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Berman G, Aung AB, Lin MY, Newman NJ, Biousse V. Subconjunctival Plasmacytoma Causing Diplopia. J Neuroophthalmol 2024; 44:e421-e422. [PMID: 37167005 DOI: 10.1097/wno.0000000000001863] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/12/2023]
Affiliation(s)
- Gabriele Berman
- Department of Ophthalmology (GB, ABA, MYL), Emory Eye Center, Emory University School of Medicine, Atlanta, Georgia; Departments of Ophthalmology, Neurology, and Neurological Surgery (NJN), Emory University School of Medicine, Atlanta, Georgia; and Departments of Ophthalmology and Neurology (VB), Emory University School of Medicine, Atlanta, Georgia
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2
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O’Donovan C, Wu AD, Lim WS. Rare presentation of orbital metastasis in multiple myeloma. Saudi J Ophthalmol 2024; 38:185-187. [PMID: 38988781 PMCID: PMC11232743 DOI: 10.4103/sjopt.sjopt_109_22] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2022] [Revised: 01/04/2024] [Accepted: 01/14/2024] [Indexed: 07/12/2024] Open
Abstract
Orbital manifestations are rarely observed in multiple myeloma (MM) and when they occur, they are often the first manifestation. We report a rare occurrence of vision loss in a 51-year-old female from orbital metastases in MM without proptosis or diplopia. The ophthalmic presentation of MM is usually progressive proptosis, pain, diplopia, and visual loss. The presence of metastasis in MM indicates poor prognosis and orbital metastases have worse survival rates. In conclusion, in cases of profound vision loss with no obvious cause, neuroimaging should be performed to evaluate the orbital extent of the disease and exclude intracranial pathology.
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Affiliation(s)
- Charles O’Donovan
- Department of Ophthalmology, Royal Eye Unit, Kingston Hospital NHS Foundation Trust, London, UK
| | - Angela D. Wu
- Department of Ophthalmology, Royal Eye Unit, Kingston Hospital NHS Foundation Trust, London, UK
| | - Wei S. Lim
- Department of Ophthalmology, Royal Eye Unit, Kingston Hospital NHS Foundation Trust, London, UK
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3
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Veisi A, Daneshvar K, Hooshmandi S, Najafi M, Mohammadi Torbati P, Hassanpour K. Superior Oblique Muscle Extramedullary Plasmacytoma in a Patient with Multiple Myeloma and a Review of Literature. Case Rep Ophthalmol 2024; 15:265-272. [PMID: 38529002 PMCID: PMC10963055 DOI: 10.1159/000538120] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2023] [Accepted: 02/27/2024] [Indexed: 03/27/2024] Open
Abstract
Introduction Multiple myeloma (MM), a plasma cell malignancy, is a systemic disease affecting various body organs. Plasmacytoma of bone and extramedullary disease (EMD) are presentations of MM. EMD is usually the sign of a more aggressive form of the disease. Herein, we report a patient with refractory MM presenting with extramedullary plasmacytoma in the superior oblique (SO) muscle. Case Presentation A 51-year-old female presented complaining of gradual protrusion of the left eye and ocular pain from 20 days prior. She received bone marrow transplantation 1 year prior and was on a chemotherapy regimen for MM for the past 1 year. Ocular examination revealed proptosis of the left eye and mild limitations of adduction and elevation. Orbital magnetic resonance imaging demonstrated remarkable enlargement of the left SO muscle with focal contrast enhancement. The patient underwent a biopsy and mass debulking. The histopathologic exam revealed fibromuscular tissue containing a neoplasm composed of sheets of plasmacytoid cells in a varying degree of differentiation with intervening scantly vascularized stromal components. The plasmacytoid cells were diffusely positive for a cluster of differentiation 138 (CD138), leading to a diagnosis of EMD involving the EOM and soft tissue of the orbit. The patient underwent palliative radiotherapy and a systemic workup. The PET-CT scan revealed involvement of the pelvic bone and left calf. Accordingly, the chemotherapy regimen was upgraded to reflect the aggressive nature of the disease. In the last follow-up, there was no sign of tumor reactivation in the orbital soft tissues. Unfortunately, the patient succumbed to her illness 7 months following her most recent presentation. Conclusion Early recognition of disease recurrence is lifesaving in MM patients; ophthalmic manifestations should be seriously considered as a sign of MM activity.
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Affiliation(s)
- Amirreza Veisi
- Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Kimia Daneshvar
- School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
| | - Sadid Hooshmandi
- Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Maryam Najafi
- Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Peyman Mohammadi Torbati
- Department of Pathology, Shahid Labbafnejad Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Kiana Hassanpour
- Ophthalmic Research Center, Research Institute for Ophthalmology and Vision Science, Shahid Beheshti University of Medical Sciences, Tehran, Iran
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Nogués-Castell J, Feu-Basilio S, Felguera García Ó, Fernández de Larrea C, Oliver-Caldés A, Balagué Ponz O, Fassi JM. Bilateral orbital plasmacytomas as first sign of extramedullary progression post CAR-T therapy: case report and literature review. Front Oncol 2023; 13:1217714. [PMID: 37637047 PMCID: PMC10450026 DOI: 10.3389/fonc.2023.1217714] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2023] [Accepted: 07/14/2023] [Indexed: 08/29/2023] Open
Abstract
Background Plasma cell leukemia (PCL) is an aggressive and rare form of plasma cell dyscrasia characterized by peripheral blood expression, poor prognosis, and high relapse rates. Extramedullary plasmacytomas are common in this entity and can affect various organs and soft tissues. Chimeric antigen receptor-T-cell (CAR-T) therapy is a novel immunotherapy for hematological malignancies with promising results. However, it is not indicated for PCL, and experience in this condition is limited. This case is a rare presentation of bilateral orbital plasmacytomas after CAR-T therapy in a patient with PCL history. Case presentation We present the case of a 51-year-old female patient with a history of previous primary PCL treated with CAR-T therapy achieving complete response and without evidence of systemic progression. Six months after the treatment, she developed subacute proptosis and ptosis on the left eye.An orbital CT scan was performed and showed an orbital tumor in both eyes. A surgical biopsy with histological examination revealed plasma cells, consistent with a plasmacytoma. PET-CT and MRI confirmed the presence of tumors in both orbits. The patient was treated with dexamethasone and chemotherapy along with palliative radiation therapy to the left orbit which had a good response. Conclusion Orbital involvement in multiple myeloma and PCL is rare, with plasmacytomas being more common in other parts of the body. In this report, we present a case of a patient with PCL history, treated with multiple therapeutic lines including CAR-T therapy, who presented bilateral orbital plasmacytomas as the first sign of extramedullary progression after the treatment. This case should be considered by specialist to be aware that the orbits are a possible location of extramedullary progression.
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Affiliation(s)
- Javier Nogués-Castell
- Institut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
| | - Silvia Feu-Basilio
- Institut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
| | - Óscar Felguera García
- Institut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
| | - Carlos Fernández de Larrea
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
- Amyloidosis and Myeloma Unit, Department of Hematology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
| | - Aina Oliver-Caldés
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
- Amyloidosis and Myeloma Unit, Department of Hematology, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
| | - Olga Balagué Ponz
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
- Centre de Diagnòstic Biomèdic, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
| | - Jessica Matas Fassi
- Institut Clínic d’Oftalmologia, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
- Institut D’ Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Fundació Clínic per a la Recerca Biomèdica (FCRB), Universitat de Barcelona, Barcelona, Spain
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Shoji MK, Chen Y, Topilow NJ, Abou Khzam R, Dubovy SR, Johnson TE. Orbital Involvement in Multiple Myeloma. Ophthalmic Plast Reconstr Surg 2023; 39:347-356. [PMID: 36661857 DOI: 10.1097/iop.0000000000002314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/21/2023]
Abstract
PURPOSE To characterize clinical and radiographic features, management, and outcomes of patients with orbital involvement of multiple myeloma (MM). METHODS A retrospective chart review identified patients with MM and orbital involvement confirmed by histopathology at a single institution between 1995 and 2021. A comprehensive literature review was performed via PubMed to identify all previously reported cases of orbital MM. RESULTS Retrospective review identified 7 patients (43% male, mean age 68.7 years). Presenting symptoms included proptosis and diplopia. Orbital lesions were primarily located laterally (42.8%) with associated extraocular muscle (57.1%) or lacrimal gland (42.9%) involvement. Five patients (71.4%) had a previous diagnosis of systemic MM. Six patients received chemoradiation (85.7%). All patients had improvement of orbital disease with 2 patients deceased due to disease at follow-up (mean 8.9 months). Literature review identified 111 cases (46.8% male, mean age 58.6 years). 48.6% presented with orbital disease as the first manifestation of systemic MM. Lesions were most commonly located superolaterally (20.2%) with extraocular muscle infiltration (25.2%), lacrimal gland involvement (7.2%), and orbital bony destruction (39.6%). Treatments included chemoradiation, chemotherapy, or radiation alone. Approximately half (51.4%) of patients experienced improvement in orbital disease following treatment, and 48.6% were deceased at follow-up (mean 20.1 months). CONCLUSIONS This study provides a new retrospective study and updated comprehensive literature review regarding orbital MM. Given its poor prognosis, characterization of orbital MM is essential for early diagnosis. Orbital MM is often unilateral, located superolaterally, and may represent the first manifestation of systemic disease. Treatment includes chemotherapy and radiation, which may improve orbital disease; however, the overall prognosis remains poor.
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Affiliation(s)
- Marissa K Shoji
- Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Miami, Florida, U.S.A
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Hu WL, Song JY, Li X, Pei XJ, Zhang JJ, Shen M, Tang R, Pan ZY, Huang ZX. Clinical features and prognosis of multiple myeloma and orbital extramedullary disease: Seven cases report and review of literature. World J Clin Cases 2022; 10:12365-12374. [PMID: 36483807 PMCID: PMC9724521 DOI: 10.12998/wjcc.v10.i33.12365] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/10/2022] [Revised: 09/26/2022] [Accepted: 10/24/2022] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND Multiple myeloma (MM) complicated with extramedullary disease (EMD) has a poor prognosis and is a limiting factor in the treatment of MM, and no standard treatment is recommended in international guidelines. Few studies have reported MM with periorbital EMD.
CASE SUMMARY In this paper, the clinical characteristics and survival of seven patients with multiple myeloma and orbital are described and analyzed. The common ocular symptoms were blurred vision, proptosis and/or eye movement disorders, IgG type MM may be a risk factor for orbital involvement. Of them, six patients were treated with bortezomib-based regimens. The median overall survival (OS) and progression free survival for the entire cohort were 48 and 33 mo, respectively, which was much worse than the OS reported for MM patients without orbital EMD.
CONCLUSION Orbital MM may have significantly shortened survival for the entire cohort, so multidisciplinary collaboration is emphasized and recommended in the diagnosis and treatment of these difficult cases.
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Affiliation(s)
- Wan-Li Hu
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Jia-Yin Song
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Xin Li
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Xiao-Jiao Pei
- Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Jia-Jia Zhang
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Man Shen
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Ran Tang
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Zhen-Yu Pan
- Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
| | - Zhong-Xia Huang
- Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
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7
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Rawicka A, Crasta M, Guandalini A, Peruccio C, Multari D, Rondena M, D’Anna N, Giudice C. Conjunctival Extramedullary Plasmacytoma in Dogs and a Cat: Clinical Characteristics and Histopathological Findings. Animals (Basel) 2022; 12:ani12213015. [PMID: 36359139 PMCID: PMC9653729 DOI: 10.3390/ani12213015] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2022] [Revised: 10/30/2022] [Accepted: 10/31/2022] [Indexed: 11/06/2022] Open
Abstract
Simple Summary Extramedullary plasmacytoma has been occasionally reported to affect the ocular and periocular (orbit, conjunctiva) region. To better describe the clinical and histopathological features of extramedullary plasmacytoma, we reviewed the records of seven cases (six dogs, one cat). In these cases, extramedullary plasmacytoma was found mostly in the conjunctiva of the third eyelid in the form of salmon-colored nodules with clear borders. All tumors were surgically removed with no additional therapy. No signs of recurrence or systemic involvement (multiple myeloma) were observed in the follow up period. We recommend that extramedullary plasmacytoma should be considered in cases of canine and feline conjunctival masses, followed by investigations to rule out multiple myeloma. Our results suggest that conjunctival extramedullary plasmacytoma is unlikely to recur or spread, and that local surgical excision alone or combined with cryotherapy should be curative. Abstract Background: Extramedullary plasmacytoma (EMP) is a plasma cell tumor that is very rarely reported in ocular and adnexal tissue and is usually solitary and benign. Methods: This study evaluated seven cases from different ophthalmology referrals submitted for histopathological analysis between 2013 and 2022. Results: EMPs were diagnosed in a 9-year-old domestic short-haired cat and in six dogs (median age, 10 years): three English Cocker Spaniels, a Golden Retriever, a Maltese, and a Pinscher. EMPs affected the third eyelid conjunctiva in five cases (5/7), followed by the superior bulbar (1/7) and palpebral conjunctivae (1/7), respectively, and were presented mostly as well-defined, salmon-colored nodules. Histopathologically, the masses were composed of sheets and packets of round cells morphologically consistent with mature plasma cells with mild anisocytosis and anisokaryosis; mitosis and binucleated giant cells were rarely observed. Follow up for 6/7 cases ranged from 6 months to 2 years. No signs of recurrence or systemic involvement (multiple myeloma) were observed. Conclusions: EMP should be included in the differential diagnoses of canine and feline conjunctival masses. Staging recommendations should include multiple myeloma diagnostic evaluation. Our results suggest that primary conjunctival EMP does not appear to be locally aggressive and has low systemic involvement behavior. The local surgical excision alone or combined with cryotherapy should be curative.
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Affiliation(s)
- Aleksandra Rawicka
- Clinica Veterinaria Roma Sud, Via Pilade Mazza 24, 00173 Rome, Italy
- Correspondence: ; Tel.: +39-347-454-15-04
| | - Manuela Crasta
- Anicura VisionVet Eye Clinic, Via Antonio Marzocchi 6, San Giovanni in Persiceto, 40017 Bologna, Italy
| | - Adolfo Guandalini
- Department of Ophthalmology, Centro Veterinario Specialistico, Via Sandro Giovannini 53, 00137 Rome, Italy
| | - Claudio Peruccio
- Veterinary Ophthalmology Referrals Centro Veterinario Torinese, Lungo Dora Colletta 147, 10153 Torino, Italy
| | - Domenico Multari
- Centro Veterinario Oculistico Fontane, Via Donatori del Sangue 1, 31020 Villorba, Italy
| | - Marco Rondena
- Clinica Veterinaria Privata San Marco Srl, Via dell’Industria, 3, 35030 Veggiano, Italy
| | - Nunzio D’Anna
- Clinica Veterinaria Roma Sud, Via Pilade Mazza 24, 00173 Rome, Italy
| | - Chiara Giudice
- Department of Veterinary Medicine and Animal Sciences, Università degli Studi di Milano, 26009 Lodi, Italy
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Sokolenko E, Johansson P, Ting S, Bechrakis NE, Fiorentzis M. Choroidal Manifestation of Smoldering Myeloma: Case Report. Klin Monbl Augenheilkd 2022. [PMID: 35901808 DOI: 10.1055/a-1838-5027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/16/2022]
Abstract
This case illustrates clinical, histopathological, immunohistochemical, and molecular pathological diagnostic testing of smoldering myeloma with atypical ophthalmic manifestations. In our case, the choroidal lesion presented as a solitary manifestation of a systemic disease. Choroidal lesions of monoclonal plasma cells are extremely rare and should be included in the differential diagnosis of amelanotic choroidal lesions, even if the histopathological examination of the primary lesion is not informative. Clinical course, immunohistochemistry, and molecular pathology are essential components of the diagnostic pathway.
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Affiliation(s)
- Ekaterina Sokolenko
- Department of Ophthalmology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Patricia Johansson
- Institute of Cell Biology (Cancer Research), Faculty of Medicine, University of Duisburg-Essen, Essen, Germany
| | - Saskia Ting
- Institute of Pathology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Nikolaos E Bechrakis
- Department of Ophthalmology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Miltiadis Fiorentzis
- Department of Ophthalmology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
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McGrath R, Whitlow S, McCabe GA, McElnea EM. An nusual ocular manifestation of multiple myeloma. BMJ Case Rep 2022; 15:e247863. [PMID: 35868805 PMCID: PMC9315917 DOI: 10.1136/bcr-2021-247863] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/30/2022] [Indexed: 11/04/2022] Open
Abstract
A man in his 70s with a history of multiple myeloma presented with a 4-day history of right ptosis and a rapidly enlarging upper eyelid mass. On examination, a large, firm, smooth, pink lesion was found to descend from the conjunctiva of the superior fornix. The patient underwent excision biopsy. Histopathological analysis demonstrated monoclonal plasma cells expressing light-chain kappa, consistent with extramedullary plasmacytoma. Clinical ophthalmic manifestations of multiple myeloma are rare but also diverse. Ocular surface manifestations of multiple myeloma are extremely uncommon. Variable examination findings mean those involving the conjunctiva and may be particularly challenging to diagnose. Secondary ocular extramedullary plasmacytoma, despite its rarity, should be considered in patients with multiple myeloma.
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Affiliation(s)
- Robert McGrath
- Ophthalmology Department, University Hospital Galway, Galway, Ireland
| | - Shane Whitlow
- Ophthalmology Department, University Hospital Galway, Galway, Ireland
| | - Grace Anne McCabe
- Ophthalmology, Royal Melbourne Hospital, Melbourne, Victoria, Australia
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10
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Oyemade KA, Stafford SL, Gertz MA, Patel SV, Baratz KH, Dalvin LA. Intraocular plasmacytoma: A case of iris involvement and a review of the literature. Am J Ophthalmol Case Rep 2022; 26:101533. [PMID: 35479520 PMCID: PMC9035397 DOI: 10.1016/j.ajoc.2022.101533] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2021] [Revised: 03/09/2022] [Accepted: 04/03/2022] [Indexed: 11/21/2022] Open
Abstract
Purpose Describe a case of intraocular plasmacytoma in a patient with multiple myeloma successfully treated with photon irradiation. Observations A 61-year-old man with a history of relapsing/refractory multiple myeloma and left frontal bone plasmacytoma treated with monthly belantamab mafodotin salvage chemotherapy developed bilateral treatment-related corneal keratopathy. An iris mass was incidentally noted in the right eye during a follow-up examination. The mass was amelanotic with diffuse intrinsic vasculature involving the pupillary margin from 1:30 to 10:30. Fundus examination showed an irregularly shaped amelanotic superotemporal scleral lesion in the right eye and two smaller amelanotic scleral lesions in the left eye. Given known systemic multiple myeloma and history of left frontal bone plasmacytoma, a presumed diagnosis of iris and scleral plasmacytoma was made. Due to rapid progression of the iris plasmacytoma despite systemic chemotherapy, the patient was treated with 20 Gy photon irradiation to the anterior and posterior segments of both eyes. One month after photon irradiation, there was complete regression of the iris plasmacytoma, and the scleral lesions in both eyes also appeared to be regressing despite systemic progression of multiple myeloma. Conclusions and importance Intraocular plasmacytoma is rare and can occur in isolation but typically occurs as a manifestation of systemic multiple myeloma. Intraocular plasmacytoma can be successfully treated with photon irradiation in patients with multiple myeloma who progress on systemic chemotherapy.
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Affiliation(s)
- Kafayat A. Oyemade
- Alix School of Medicine, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Scott L. Stafford
- Department of Radiation Oncology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Morie A. Gertz
- Department of Hematology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Sanjay V. Patel
- Department of Ophthalmology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Keith H. Baratz
- Department of Ophthalmology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
| | - Lauren A. Dalvin
- Department of Ophthalmology, Mayo Clinic, 200 1st Street SW, Rochester, MN, 55905, USA
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11
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Silverman RF, Hanson L, Salahi N, Li Z, Boruk M, Hodgson NM. Case Report: Cavernous Sinus Syndrome as the Initial Presentation of Multiple Myeloma. FRONTIERS IN OPHTHALMOLOGY 2022; 2:849343. [PMID: 38983538 PMCID: PMC11182293 DOI: 10.3389/fopht.2022.849343] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 01/06/2022] [Accepted: 02/17/2022] [Indexed: 07/11/2024]
Abstract
Multiple myeloma (MM) is the second most common hematologic malignancy and most common primary bone malignancy. Ocular manifestations of MM are extremely rare and may be the first presentation leading to diagnosis. Ophthalmologists routinely encounter cavernous sinus syndrome, and there is a wide range of possible etiologies. Here, we present a case of a patient presenting with diplopia, ptosis, and ophthalmoplegia found to have a cavernous sinus plasmacytoma with systemic workup consistent with MM. MM is a rare cause of cavernous sinus syndrome and should be considered in the setting of a skull base mass.
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Affiliation(s)
| | - Lawrence Hanson
- Department of Ophthalmology, SUNY Downstate, Brooklyn, NY, United States
| | - Navid Salahi
- Department of Pathology, SUNY Downstate, Brooklyn, NY, United States
| | - Zhonghua Li
- Department of Pathology, SUNY Downstate, Brooklyn, NY, United States
| | - Marina Boruk
- Department of Otolaryngology, SUNY Downstate, Brooklyn, NY, United States
| | - Nickisa M Hodgson
- Department of Ophthalmology, SUNY Downstate, Brooklyn, NY, United States
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12
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Denier M, Tick S, Dubois R, Dulery R, Eller AW, Suarez F, Burroni B, Maurage CA, Bories C, Konopacki J, Puech M, Bouscary D, Cantalloube A, Héron E, Marçais A, Habas C, Theillac V, Keilani C, Bonhomme GR, Gallagher DS, Boumendil J, Abarah W, Sedira N, Bertin S, Choquet S, Sahel JA, Merabet L, Brignole-Baudouin F, Putterman M, Errera MH. Hidden in the Eyes—Recurrence of Systemic Hemopathies Reportedly “In Remission”: Six Cases and Review of Literature. Medicina (B Aires) 2022; 58:medicina58030456. [PMID: 35334633 PMCID: PMC8950814 DOI: 10.3390/medicina58030456] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2022] [Revised: 03/14/2022] [Accepted: 03/15/2022] [Indexed: 11/16/2022] Open
Abstract
Background and Objectives: Secondary ocular localizations of hematological malignancies are blinding conditions with a poor prognosis, and often result in a delay in the diagnosis. Materials and Methods: We describe a series of rare cases of ocular involvement in six patients with hematological malignancies, reportedly in remission, who presented secondary ocular localizations, challenging to diagnose. Two patients had an acute lymphoblastic leukemia (ALL) and developed either a posterior scleritis or a pseudo-panuveitis with ciliary process infiltration. One patient had iris plasmacytoma and developed an anterior uveitis as a secondary presentation. Two patients had a current systemic diffuse large B-cell lymphoma (DLBCL) and were referred either for intermediate uveitis or for papilledema and vitritis with secondary retinitis. Finally, one patient with an acute myeloid leukemia (AML) presented a conjunctival localization of a myeloid sarcoma. We herein summarize the current knowledge of ophthalmologic manifestations of extramedullary hematopathies. Results: Inflammatory signs were associated with symptomatic infiltrative lesions well displayed in either the iris, the retina, the choroid, or the cavernous sinus, from the admission of the patients in the ophthalmological department. These findings suggest that patients with ALL, AML, systemic DLBCL, and myeloma can present with ophthalmic involvement, even after having been reported as in remission following an effective systemic treatment and/or allograft. Conclusions: Early detection of hidden recurrence in the eyes may permit effective treatment. Furthermore, oncologists and ophthalmologists should be aware of those rare ocular malignant locations when monitoring patient’s progression after initial treatment, and close ophthalmologic examinations should be recommended when detecting patient’s ocular symptoms after treatment.
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Affiliation(s)
- Margot Denier
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Sarah Tick
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Romain Dubois
- Institut de Pathologie, CHRU de Lille, 59000 Lille, France;
| | - Remy Dulery
- Service d’Hématologie Clinique et de Thérapie Cellulaire, Hôpital Saint Antoine, AP-HP, Sorbonne Université, INSERM UMRs938, 75012 Paris, France;
| | - Andrew W. Eller
- Ophthalmology Service, University of Pittsburgh School of Medicine, Pittsburgh, PA 75012, USA; (A.W.E.); (G.R.B.); (D.S.G.)
| | - Felipe Suarez
- Department of Hematology, Hôpital Necker-Enfants Malades, INSERM UMR 1163 et CNRS ERL 8254, Institut Imagine, Sorbonne Paris Cité, Université Paris Descartes, 149 rue de Sèvres, CEDEX 15, 75743 Paris, France; (F.S.); (A.M.)
| | - Barbara Burroni
- Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université de Paris, 75006 Paris, France;
| | - Claude-Alain Maurage
- Centre de Recherche Jean-Pierre Aubert INSERM: U837, Université du Droit et de la Santé—Lille II, Faculté de Médecine 1, Place de Verdun, CEDEX, 59045 Lille, France;
- Institut de Pathologie—CHRU de Lille, 59000 Lille, France
| | - Claire Bories
- France Department of Hematology, CHRU, 59000 Lille, France;
| | - Johanna Konopacki
- Department of Hematology, Hôpital D’instruction des Armées Percy, 92140 Clamart, France;
| | | | - Didier Bouscary
- Department of Hematology, Faculté de Médecine Sorbonne Paris Cité, Université Paris Descartes, Hôpital Cochin, AP-HP, 75014 Paris, France;
| | - Alberte Cantalloube
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Emmanuel Héron
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Ambroise Marçais
- Department of Hematology, Hôpital Necker-Enfants Malades, INSERM UMR 1163 et CNRS ERL 8254, Institut Imagine, Sorbonne Paris Cité, Université Paris Descartes, 149 rue de Sèvres, CEDEX 15, 75743 Paris, France; (F.S.); (A.M.)
| | - Christophe Habas
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Vincent Theillac
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Chafik Keilani
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Gabrielle R. Bonhomme
- Ophthalmology Service, University of Pittsburgh School of Medicine, Pittsburgh, PA 75012, USA; (A.W.E.); (G.R.B.); (D.S.G.)
| | - Denise S. Gallagher
- Ophthalmology Service, University of Pittsburgh School of Medicine, Pittsburgh, PA 75012, USA; (A.W.E.); (G.R.B.); (D.S.G.)
| | - Julien Boumendil
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Wajed Abarah
- Department of Hematology, Hôpital de Meaux, 77100 Meaux, France;
| | - Neila Sedira
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Stéphane Bertin
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Sylvain Choquet
- Department of Hematology, Hôpital Pitié-Salpêtrière, 75013 Paris, France;
| | - José-Alain Sahel
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
- Ophthalmology Service, University of Pittsburgh School of Medicine, Pittsburgh, PA 75012, USA; (A.W.E.); (G.R.B.); (D.S.G.)
| | - Lilia Merabet
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Françoise Brignole-Baudouin
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
- Sorbonne Universités, 75006 Paris, France
| | - Marc Putterman
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
| | - Marie-Hélène Errera
- Centre Hospitalier National des Quinze-Vingts, CIC 1423, DHU Sight Restore, 28 rue de Charenton, Sorbonne-Universités, Université Pierre et Marie Curie (UPMC), 75012 Paris, France; (M.D.); (S.T.); (A.C.); (E.H.); (C.H.); (V.T.); (C.K.); (J.B.); (N.S.); (S.B.); (J.-A.S.); (L.M.); (F.B.-B.); (M.P.)
- Ophthalmology Service, University of Pittsburgh School of Medicine, Pittsburgh, PA 75012, USA; (A.W.E.); (G.R.B.); (D.S.G.)
- Correspondence:
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Zloto O, Vahdani K, Stack R, Verity DH, Rose GE. Periocular Presentation of Solitary Plasmacytomas and Multiple Myeloma. Ophthalmic Plast Reconstr Surg 2022; 38:180-184. [PMID: 34293789 DOI: 10.1097/iop.0000000000002023] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
AIMS To describe patients with periocular solitary extramedullary plasmacytoma (SEMP) and multiple myeloma (MM), together with an estimate of the risk of progression from SEMP to MM. PATIENTS AND METHODS A retrospective case-note review for patients seen between 1978 and 2020, examining demographics, presentation, imaging, pathology, management, and outcome. RESULTS Twenty patients (10 male; 50%) presented at a mean age of 60.9 years, with an average symptom duration of 4.5 months. Ten (50%) patients had known systemic myeloma at ophthalmic presentation (the MM group) and, on average, they presented one decade earlier than those with occult MM discovered after orbital biopsy (p = 0.06); the majority (9/15; 60%) of patients with MM were female, whereas there was a male bias (4/5; 80%) with SEMP (p = 0.30). Most tumors (15/20; 75%) were within the anterior part of the orbit, especially superolaterally (16 patients; 80%), and the soft-tissue mass often appeared to "explode" from the frontal bone or greater wing of the sphenoid (16/20; 80%). Full treatment details were known for 19 patients: 6 (32%) had solely orbital radiotherapy, 4 (21%) chemotherapy, 6 (32%) combined chemoradiation, and 3 (16%) had combined chemoradiation with stem-cell transplant (Table 3). After an average follow up of 58 months, 1/5 (20%) patients with SEMP and 11/15 (73%) with MM had tumor-related death. The overall survival probability for all 20 patients with periocular plasmacytoma was 34% at 5 and 10 years, with MM patients having a worse outlook (27% 5-year, and 18% 10-year survival) compared with SEMP (53% survival at 5 and 10 years) (p = 0.18). None of the 5 patients with SEMP progressed to systemic MM over an average follow up of 9.1 years. CONCLUSIONS Although 50% patients with periocular plasmacytoma appear to have a SEMP at ophthalmic presentation, a half of these patients were found to have occult MM within 6 months of biopsy. Of those without systemic disease around the time of biopsy, none developed MM over an average follow up of more than 9 years.
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Affiliation(s)
- Ofira Zloto
- Orbital Service, Moorfields Eye Hospital, London, United Kingdon
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McNab AA, Jones RS, Hardy TG. Spheno-orbital Lesions-A Major Review of Nonmeningioma Causes. Ophthalmic Plast Reconstr Surg 2021; 37:522-533. [PMID: 33782321 DOI: 10.1097/iop.0000000000001924] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
PURPOSE To review the imaging features of the broad range of nonmeningioma lesions of the greater wing of the sphenoid (GWS) bone and adjacent orbit to assist clinicians in differentiating these lesions from each other and from the most common lesion of the GWS and adjacent orbit, meningioma. METHODS We reviewed 32 cases of spheno-orbital lesions involving the GWS from our own practice, as well as 109 published cases (total 141), with emphasis on available imaging features on computerized tomography (CT) and MRI. Features that might assist in differentiating meningioma from its mimics were analyzed for each lesion, including the presence of an osteoblastic or hyperostotic response, bone erosion or osteolysis, homogeneous hypo- or hyperintensity on T2-weighted MRI, leptomeningeal involvement, and the absence of a "dural tail" on contrast-enhanced MRI. The clinical and imaging features were also briefly summarized for each diagnostic group. RESULTS The largest diagnostic group was metastasis (67 cases, 47.5%). The most useful imaging features that helped differentiate meningioma from its mimics were the presence of bone erosion and the absence of a "dural tail." Other features were helpful in a small minority of cases only. Metastatic prostate cancer was the largest single group (21 cases), and 18 (85.7%) of these were osteoblastic and most closely mimicked meningioma. Prostate cancer patients were generally older than males with GWS meningioma. Almost all other (44/46, or 95.7%) metastatic lesions showed evidence of bone erosion. Almost half (30 of 61, 49.2%) of patients with metastasis presented without a known diagnosis of malignancy. Among children 16 years of age and less, Langerhans cell histiocytosis (10 cases), dermoid cyst (5), and Ewing's sarcoma (5) were the most common diagnoses. CONCLUSIONS A combination of a careful history and both CT and MRI gives information, which can best guide the management of patients with spheno-orbital lesions. Metastatic prostate cancer to the GWS most closely mimics GWS meningioma but can in most cases be differentiated on clinical and imaging features. Older males with hyperostotic lesions of the GWS should be investigated for prostate cancer. Other metastatic lesions and primary tumors of the GWS, as well as benign and structural lesions can readily be differentiated from meningioma on clinical and imaging features.
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Affiliation(s)
- Alan A McNab
- Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital
- Centre for Eye Research Australia, University of Melbourne
| | - Randall S Jones
- Department of Radiology, St Vincent's Hospital, Melbourne, Victoria, Australia
| | - Thomas G Hardy
- Orbital Plastic and Lacrimal Clinic, Royal Victorian Eye and Ear Hospital
- Department of Surgery, Royal Melbourne Hospital, University of Melbourne, Parkville, Victoria, Australia
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15
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Choi JH, Kim JH, Jeong JG. Orbital and Choroidal Plasmacytoma in Recurrent Multiple Myeloma: A Case Report. KOREAN JOURNAL OF OPHTHALMOLOGY 2021; 35:335-336. [PMID: 34379971 PMCID: PMC8357614 DOI: 10.3341/kjo.2021.0074] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2021] [Accepted: 06/24/2021] [Indexed: 11/23/2022] Open
Affiliation(s)
- Ji Ho Choi
- Department of Ophthalmology, Jeonbuk National University Medical School, Jeonju, Korea
| | - Joon Hyung Kim
- Department of Ophthalmology, Seoul National University Hospital, Seoul, Korea
| | - Jin Gu Jeong
- Department of Ophthalmology, Jeonbuk National University Medical School, Jeonju, Korea
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16
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Huge Lateral Rectus Solitary Plasmacytoma Causing Shunt Extrusion. Case Rep Ophthalmol Med 2021; 2021:5563514. [PMID: 34194858 PMCID: PMC8181099 DOI: 10.1155/2021/5563514] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/18/2021] [Revised: 05/03/2021] [Accepted: 05/08/2021] [Indexed: 11/17/2022] Open
Abstract
A 54-year-old man with a history of radiotherapy for right maxillary sinus plasmacytoma 3 years previously was referred to an orbital clinic with progressive proptosis in his right eye. His vision had deteriorated after an initial improvement after phacoemulsification 2 years before. He had undergone shunt implantation and later shunt removal following plate extrusion with the diagnosis of neovascular glaucoma following CRVO. His vision remained at no light perception afterwards, despite a controlled IOP with topical medications. In his CT scan, a large orbital mass was seen with lateral rectus involvement. He underwent deep orbitotomy for tumor resection following worsening of symptoms, and his symptoms were improved afterwards. Pathology report was consistent with plasmacytoma with anaplastic features. After tumor resection, he underwent another course of radiotherapy with complete remission of symptoms afterwards.
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17
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Sandor KP, Micieli JA, Peragallo JH. Optic nerve head plasmacytoma as a manifestation of multiple myeloma. Taiwan J Ophthalmol 2021; 11:97-100. [PMID: 33767963 PMCID: PMC7971446 DOI: 10.4103/tjo.tjo_81_20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2020] [Accepted: 11/19/2020] [Indexed: 12/18/2022] Open
Abstract
Extramedullary disease in multiple myeloma is uncommon and associated with a poorer prognosis. Extramedullary disease involving the orbit is even more unusual, with optic nerve involvement being rare. We describe an optic nerve head plasmacytoma in a 45-year-old female in the setting of systemic relapsed, refractory IgA kappa multiple myeloma. The case highlights the importance of keeping extramedullary disease spread in the differential for vision loss in a patient with a history of multiple myeloma. In addition, it describes an unusual location for presentation of extramedullary disease, the optic nerve head, which has rarely been described.
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Affiliation(s)
| | - Jonathan A Micieli
- Department of Ophthalmology and Vision Sciences, University of Toronto, Toronto, ON, Canada
| | - Jason H Peragallo
- Department of Ophthalmology, Emory University, Atlanta, GA, USA.,Department of Pediatrics, Emory University, Atlanta, GA, USA
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Solitary Conjunctivocorneal Plasmacytoma Arising After Cataract Surgery. Cornea 2020; 40:1065-1066. [PMID: 33252386 DOI: 10.1097/ico.0000000000002569] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2020] [Accepted: 09/03/2020] [Indexed: 11/26/2022]
Abstract
PURPOSE To report a case of solitary plasmacytoma of the conjunctiva with corneal encroachment and to review the literature of all published cases. METHODS A 70-year-old man presented with 1-month history of pain and watering in the right eye, 7 months after undergoing cataract surgery in the same eye. RESULTS The mass was surgically excised. Pathology and immunohistochemistry were consistent with plasmacytoma. Staging studies found no evidence of systemic disease. CONCLUSIONS To our knowledge, this is the first case of plasmacytoma in the literature to involve the cornea. This case was not associated with systemic disease. Surgical excision and brachytherapy remain options for cases without corneal involvement.
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Barmas-Alamdari D, Sodhi GS, Shenouda TA. Bilateral Proptosis in a Case of Recurring Multiple Myeloma: Uncommon Orbital Presentation of Plasmacytoma. Int Med Case Rep J 2020; 13:297-301. [PMID: 32884366 PMCID: PMC7434372 DOI: 10.2147/imcrj.s260472] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2020] [Accepted: 07/08/2020] [Indexed: 11/23/2022] Open
Abstract
Multiple myeloma is a malignant plasma cell dyscrasia that may invade the orbits in extramedullary presentations. Common manifestations of orbital involvement include unilateral proptosis, injection, chemosis, diplopia, and occasionally pain. Additionally, the soft-tissue tumors associated with multiple myeloma typically cause bony destruction of adjacent structures. However, in certain patients, bilateral proptosis and a lack of bony destruction may occur. In these instances, it is important for physicians to consider patient history, presentation, and progression in order to ensure that a potentially fatal diagnosis is not missed. The reason this case is important is two-fold: 1) we describe a case of an individual with relapsing multiple myeloma presenting as bilateral proptosis and lacking bony destruction, highlighting the importance of understanding the disease process and 2) we discuss why patients with multiple myeloma may be more prone to developing tumors of the orbit in recurrent cases.
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Affiliation(s)
| | - Guneet S Sodhi
- Department of Ophthalmology, Eastern Virginia Medical School, Norfolk, VA, USA
| | - Teresa A Shenouda
- Department of Ophthalmology, Eastern Virginia Medical School, Norfolk, VA, USA
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20
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Kakkassery V, Coupland SE, Heindl LM. Iris lymphoma-a systematic guide for diagnosis and treatment. Surv Ophthalmol 2020; 66:41-53. [PMID: 32585164 DOI: 10.1016/j.survophthal.2020.06.003] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2019] [Revised: 06/12/2020] [Accepted: 06/15/2020] [Indexed: 12/16/2022]
Abstract
Iris lymphomas are rare malignant neoplasms arising either as primary tumors in the iris or as secondary tumors involving the iris. We summarize previously published data and make recommendations for work-up strategies for cases of suspected iris lymphoma. Our objective is to provide a structured overview of the typical clinical symptoms and signs, the pathologic, ophthalmic, as well as hematologic work-up for diagnosis, treatment, and follow-up of iris lymphomas and offer a flowchart on how to diagnose and treat these tumors.
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Affiliation(s)
| | - Sarah E Coupland
- Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom; Liverpool Clinical Laboratories, Liverpool University Hospitals Foundation Trust, Liverpool, UK
| | - Ludwig M Heindl
- Department of Ophthalmology, University of Cologne, Faculty of Medicine and University Hospital Cologne, Cologne, Germany; Center for Integrated Oncology (CIO), Aachen-Bonn-Cologne-Duesseldorf, Cologne, Germany.
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Mani M, Kasturi N, Sravya R, Kaliaperumal S, Gochhait D. Orbital plasmacytoma as the presenting feature in multiple myeloma. Eur J Ophthalmol 2020; 31:NP1-NP4. [PMID: 32493136 DOI: 10.1177/1120672120929959] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
A 50-year-old female patient presented with protrusion of the left eye for 1 month. Examination showed abaxial proptosis, restriction of extraocular movements, and elevated intraocular pressure. Computed tomography of the orbits showed soft tissue enhancing lesion in the superolateral aspect of the left orbit with lytic lesions in calvarium. Fine needle aspiration cytology of the lesion revealed a diagnosis of plasmacytoma with positive CD138 and CD38 immunohistochemical stains. Erythrocyte sedimentation rate, C-reactive protein and serum lactate dehydrogenase were elevated. Serum protein electrophoresis revealed hypergammaglobulinemia, and bone marrow biopsy revealed 6% plasma cells. The patient was started on chemotherapy with bortezomib, dexamethasone and lenalidomide by the medical oncologist. Significant improvement in proptosis and extraocular movements noted on follow-up. Orbital myeloma may be the first manifestation of systemic disease.
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Affiliation(s)
- Malavika Mani
- The Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Nirupama Kasturi
- The Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Rekha Sravya
- The Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Subashini Kaliaperumal
- The Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
| | - Debasis Gochhait
- The Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
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Alshomar KM, Altariqi SM, Alrikabi AC, Alkatan HM, Al-Faky YH. Primary extramedullary plasmacytoma of the eyelid conjunctiva - A case report and review of the literature. Ann Med Surg (Lond) 2020; 55:1-4. [PMID: 32435472 PMCID: PMC7229271 DOI: 10.1016/j.amsu.2020.04.028] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2020] [Revised: 04/22/2020] [Accepted: 04/28/2020] [Indexed: 11/24/2022] Open
Abstract
Extramedullary plasmacytomas (EMPs) are uncommon plasma cell tumors that develop in soft tissue as isolated tumors without osseous involvement while secondary lesions are associated with systemic multiple myeloma (MM). Primary extramedullary lesions are most commonly found in upper respiratory tract, gastrointestinal tract and lymph nodes. They can be found either in patients with history of MM or preceding the manifestation of a systemic disease. Orbital manifestation of the lesion is rare but conjunctival involvement is very unusual. The reported cases in the English-written literature are only five cases. Herein, we report the sixth case of primary EMP in a middle-aged adult who presented with a lesion confined to the conjunctiva, unremarkable present and past medical history, and confirmed tissue diagnosis. In addition, a review and summary of the previously reported cases in the literature is presented. We aim to attract the attention of ophthalmic surgeons to consider plasmacytoma within the differential diagnosis of a conjunctival lesion.
Extramedullary plasmacytomas (EMP) are isolated primary soft tissue plasma cell tumors without osseous involvement. Secondary lesions are associated with systemic multiple myeloma. The conjunctiva is a very unusual location for primary lesions. We are reporting the sixth case of conjunctival primary EMP. All cases including ours are summarized.
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Affiliation(s)
- Khalid M Alshomar
- Ophthalmology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Sulaiman M Altariqi
- Ophthalmology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Ammar C Alrikabi
- Pathology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Hind M Alkatan
- Ophthalmology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia.,Pathology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia
| | - Yasser H Al-Faky
- Ophthalmology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia
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Selvan H, Kashyap S, Sen S, Chawla B, Lomi N, Gupta V. Trabeculectomy in an unsuspected extramedullary iris-ciliary body plasmacytoma. Eur J Ophthalmol 2020; 31:NP5-NP8. [PMID: 32314602 DOI: 10.1177/1120672120920225] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Extramedullary plasmacytoma of the iris and ciliary body is extremely rare. We present a case which was misdiagnosed as granulomatous uveitis with neovascular glaucoma, and underwent a trabeculectomy with mitomycin-c along with iris biopsy. The post-operative period showed early bleb failure and catastrophic growth of the suspected mass. Histopathological examination revealed a diagnosis iris plasmacytoma. Subsequent ultrasound biomicroscopy showed involvement of the iris and ciliary body. A prompt systemic workup was done, and an associated systemic plasma cell dyscrasia was ruled out. The affected eye was enucleated, and the patient remains disease free at the end of 3-year follow-up.
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Affiliation(s)
- Harathy Selvan
- Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Seema Kashyap
- Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Seema Sen
- Department of Ocular Pathology, Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Bhavna Chawla
- Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Neiwete Lomi
- Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
| | - Viney Gupta
- Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
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Abstract
Orbital lymphomas constitute 50-60% of ocular adnexal lymphomas. A total of 2211 cases of orbital lymphoma with a known subtype have been reported in the last 24 years (1994-2017). The vast majority of orbital lymphomas are of B-cell origin (97%), of which extranodal marginal zone B-cell lymphoma (EMZL) (59%) is the most common subtype, followed by diffuse large B-cell lymphoma (23%), follicular lymphoma (9%), and mantle cell lymphoma (5%). Orbital lymphoma is primarily a disease of the elderly. Gender distribution varies according to lymphoma subtype. However, extranodal marginal zone B-cell lymphoma (53%) and follicular lymphoma (75%) show a female predominance, whereas diffuse large B-cell lymphoma shows an even gender distribution. Mantle cell lymphoma has a striking male predominance of 80%. The histopathological subtype and the clinical stage of the disease are the best indicators of prognosis and patient outcome. Low-grade lymphomas such as extranodal marginal zone B-cell lymphoma and FL have a good prognosis, whereas high-grade lymphomas (diffuse large B-cell lymphoma and mantle cell lymphoma) are associated with a poor prognosis. When managing solitary low-grade lymphomas, radiotherapy is the treatment of choice. Chemotherapy, with or without radiotherapy, should be chosen for disseminated and high-grade lymphomas.
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Affiliation(s)
- Tine Gadegaard Olsen
- Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark
| | - Steffen Heegaard
- Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Ophthalmology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark.
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Thuro BA, Sagiv O, Shinder R, Debnam JM, Ozgur O, Ng JD, Rootman D, Thomas SK, Esmaeli B. Clinical Presentation and Anatomical Location of Orbital Plasmacytomas. Ophthalmic Plast Reconstr Surg 2018; 34:258-261. [PMID: 28582371 DOI: 10.1097/iop.0000000000000939] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
PURPOSE To evaluate the clinical and anatomical location of orbital plasmacytomas and assess local control following therapy. METHODS The American Society of Ophthalmic Plastic and Reconstructive Surgery Oncology Database was queried to identify patients diagnosed with orbital plasmacytoma. These patients' records were reviewed for demographic characteristics, clinical and radiologic findings, treatments, and outcomes. RESULTS Thirty patients from 4 institutions (24 from MD Anderson Cancer Center, 3 from SUNY Downstate Medical Center, 2 from University of California, Los Angeles, and 1 from Oregon Health and Science University) were identified. Eighteen patients (60%) were diagnosed with multiple myeloma (MM) before and 11 (37%) were diagnosed with MM immediately after orbital plasmacytoma. Based on imaging, 4 distinct anatomical patterns were identified: 1) bony plasmacytoma affecting the superotemporal orbit, epidural space, and temporal fossa (15 patients; 50%); 2) discrete orbital plasmacytoma (7 patients; 23%); 3) infiltrative plasmacytoma either originating from a sinus (4 patients; 13%); or 4) originating from the orbital floor and infiltrating facial soft tissue (4 patients; 13%). Of the 29 patients with available treatment data, 2 had radiation only, 3 had chemotherapy only, 6 had chemoradiation, and 18 had stem cell transplant following chemoradiation (n = 17) or only chemotherapy (n = 1). Following treatment, 10 patients achieved complete and 11 achieved partial responses. CONCLUSION Orbital plasmacytomas were found exclusively in patients with MM diagnosed before or immediately after orbital plasmacytoma. Plasmacytomas can have 4 distinct anatomical patterns of origin. Following treatment, all patients had good to excellent local control of their orbital lesions.
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Affiliation(s)
- Bradley A Thuro
- Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
| | - Oded Sagiv
- Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
| | - Roman Shinder
- Department of Ophthalmology, State University of New York Downstate Medical Center, Brooklyn, New York
| | | | - Omar Ozgur
- Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
| | - John D Ng
- Department of Ophthalmology, Oregon Health and Science University, Portland, Oregon
| | - Daniel Rootman
- Department of Ophthalmology, University of California, Los Angeles, Los Angeles, California
| | - Sheeba K Thomas
- Department of Lymphoma and Myeloma, M.D. Anderson Cancer Center, Houston, Texas, U.S.A
| | - Bita Esmaeli
- Orbital Oncology and Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas M.D. Anderson Cancer Center, Houston, Texas
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Wang SSY, Lee MB, George A, Wang SB, Blackwell J, Moran S, Francis IC. Five cases of orbital extramedullary plasmacytoma: diagnosis and management of an aggressive malignancy. Orbit 2018; 38:218-225. [PMID: 29985709 DOI: 10.1080/01676830.2018.1490437] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
Purpose: Multiple myeloma is an insidious haematological malignancy characterised by monoclonal proliferation of plasma cells in the bone marrow. Extramedullary plasmacytoma is a rare manifestation of multiple myeloma and usually occurs in the upper respiratory tract. Orbital involvement is particularly uncommon, but may be associated with devastating visual impairment and poor clinical outcomes. Therefore, this article aims to highlight the need for multidisciplinary management of orbital extramedullary plasmacytoma. Methods: This is a retrospective observational case series of five patients. All presented to the authors for management of orbital extramedullary plasmacytomas from 2004 to 2015 at Prince of Wales and Mater Hospitals in Sydney, Australia. Medical records were reviewed for pertinent information including demographics, disease features, management strategy, and clinical progress. The study met Medical Ethics Board standards and is in accordance with the Helsinki Agreements. Results: This case series of five patients underscores the poor prognosis of orbital extramedullary plasmacytoma. Despite aggressive multidisciplinary management, four of these five patients succumbed to their illness during the study period. However, multidisciplinary management did manage to minimise symptoms and preserve quality of life. Conclusions: On a case-by-case basis, patients may derive palliative benefit from orbital surgery in conjunction with radiotherapy and chemotherapy. Orbital surgeons are encouraged to work within a multidisciplinary framework of medical specialists, including haematologists and radiation oncologists, when determining the optimal management plan in cases of orbital extramedullary plasmacytoma.
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Affiliation(s)
- Samuel S Y Wang
- a Faculty of Medicine , University of New South Wales , Sydney, Australia
| | - Mitchell B Lee
- b Sydney Medical School , University of Sydney , Sydney, Australia
| | - Adarsh George
- a Faculty of Medicine , University of New South Wales , Sydney, Australia
| | - Sarah B Wang
- b Sydney Medical School , University of Sydney , Sydney, Australia
| | | | - Steve Moran
- c Department of Haematology , Mater Hospital , Sydney , Australia
| | - Ian C Francis
- a Faculty of Medicine , University of New South Wales , Sydney, Australia.,d Department of Ophthalmology , Prince of Wales Hospital , Sydney , Australia
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Duletic-Nacinovic A, Stifter S, Marijic B, Miletic D, Loncarek K, Manestar D, Jonjic N. Dacryocystitis Provoked by Recurrence of Extramedullary Plasmacytoma of the Orbit: A Case Report. TUMORI JOURNAL 2018; 96:164-7. [DOI: 10.1177/030089161009600128] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Extramedullary plasmacytoma (EMP) of the orbit is an extremely rare tumor, which frequently manifests with nonspecific symptoms. In the case reported, the symptoms of relapsing dacryocystitis appeared before the diagnosis of orbital EMP in a 60-year-old man. Moreover, EMP of the right submandibular gland that had been excised and treated by radiotherapy preceded the orbital lesion by seven years. The present report emphasizes the importance of an extensive medical workup to rule out multiple myeloma or other malignant lymphoproliferative diseases, because their treatment and prognosis are very different. Additionally, it is necessary to consider all tumors that can provoke relapsing dacryocystits.
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Affiliation(s)
| | - Sanja Stifter
- University Department of Pathology, School of Medicine, Rijeka, Croatia
| | - Blazen Marijic
- University Department of Hematology, Rijeka University Hospital Center, Rijeka, Croatia
| | - Damir Miletic
- University Department of Radiology, Rijeka University Hospital Center, Rijeka, Croatia
| | - Karmen Loncarek
- University Department of Ophthalmology, Rijeka University Hospital Center, Rijeka, Croatia
| | - Darko Manestar
- University Department of ENT, Rijeka University Hospital Center, Rijeka, Croatia
| | - Nives Jonjic
- University Department of Pathology, School of Medicine, Rijeka, Croatia
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Solitary Extramedullary Plasmacytoma of the Orbital Apex and Dura Masquerading as Idiopathic Inflammation. Ophthalmic Plast Reconstr Surg 2017; 33:S92-S94. [DOI: 10.1097/iop.0000000000000533] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Abstract
An 83-year-old male with a 15-month history of multiple myeloma presented with acute onset of swelling, redness, and pain around his right eye. CT scan was consistent with an orbital abscess. The patient was taken to the operating room for drainage of the orbital abscess. Abnormal tissue was encountered intraoperatively so biopsies were taken. His cultures grew only one colony of coagulase-negative Staphylococcus aureus. The histopathology from the biopsies showed a CD-138 positive plasma cell neoplasia consistent with a plasmacytoma. Plasmacytomas have been reported to present as orbital cellulitis and as abscesses in other locations in the body, but to our knowledge, this is the first case of a plasmacytoma presenting as an orbital abscess.
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Tenzel PA, Mishra K, Andron A, Della Rocca RC, Reddy HS. Extramedullary plasmacytoma of the lateral rectus muscle. Orbit 2017; 36:78-80. [PMID: 28267386 DOI: 10.1080/01676830.2017.1279656] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Plasmacytoma is an uncommon presentation of plasma cell malignancy, especially in the absence of multiple myeloma. Orbital plasmacytomas generally originate from bone, although few cases in the literature report orbital extramedullary plasmacytomas. We present the case of a 68-year-old man found to have a solitary extramedullary plasmacytoma in the lateral rectus muscle without further evidence of multiple myeloma. This case demonstrates a rare presentation for such a malignancy, and a review of the literature highlights the importance of proper workup and close monitoring to rule out multiple myeloma to guide management.
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Affiliation(s)
- Phillip A Tenzel
- a Department of Ophthalmology , New York Eye and Ear Infirmary of Mount Sinai , New York , New York , USA
| | - Kapil Mishra
- a Department of Ophthalmology , New York Eye and Ear Infirmary of Mount Sinai , New York , New York , USA
| | - Aleza Andron
- a Department of Ophthalmology , New York Eye and Ear Infirmary of Mount Sinai , New York , New York , USA
| | - Robert C Della Rocca
- a Department of Ophthalmology , New York Eye and Ear Infirmary of Mount Sinai , New York , New York , USA
| | - Harsha S Reddy
- a Department of Ophthalmology , New York Eye and Ear Infirmary of Mount Sinai , New York , New York , USA
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Vatansever M, Bozkurt FM, Dinç E, Yılmaz EB, Nayir E, Sarı AA, Yıldırım Ö, Kara T. Orbital Metastasis of Multiple Myeloma: Case Report. Turk J Ophthalmol 2016; 46:148-150. [PMID: 27800278 PMCID: PMC5076298 DOI: 10.4274/tjo.73645] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/16/2014] [Accepted: 09/19/2014] [Indexed: 12/01/2022] Open
Abstract
A 68-year-old woman with a history of multiple myeloma presented to the clinic with pain and vision loss in her right eye. Proptosis was observed in her right eye and eye movements were restricted in all directions. Best corrected visual acuity was 3/10 in her right eye. On biomicroscopic examination, hyperemia and subconjunctival hemorrhage were present. Fundus examination of the right eye revealed optic disc edema and choroidal folds. In magnetic resonance imaging two orbital masses were detected. Based on the patient's history and ocular examination, we evaluated the masses as orbital metastasis of multiple myeloma. Palliative radiotherapy was recommended.
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Affiliation(s)
- Mustafa Vatansever
- Mersin University Faculty of Medicine, Department of Ophthalmology, Mersin, Turkey
| | - Fatma Merve Bozkurt
- Mersin University Faculty of Medicine, Department of Ophthalmology, Mersin, Turkey
| | - Erdem Dinç
- Mersin University Faculty of Medicine, Department of Ophthalmology, Mersin, Turkey
| | - Eda Bengi Yılmaz
- Mersin University Faculty of Medicine, Department of Radiation Oncology, Mersin, Turkey
| | - Erdinç Nayir
- Mersin University Faculty of Medicine, Department of Oncology, Mersin, Turkey
| | - Ayşe Ayça Sarı
- Mersin University Faculty of Medicine, Department of Ophthalmology, Mersin, Turkey
| | - Özlem Yıldırım
- Mersin University Faculty of Medicine, Department of Ophthalmology, Mersin, Turkey
| | - Tuba Kara
- Mersin University Faculty of Medicine, Department of Pathology, Mersin, Turkey
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Isolated Extraocular Muscle Infiltration With Plasmacytoma Treated With Localized Injection of Dexamethasone. J Neuroophthalmol 2015; 35:168-70. [DOI: 10.1097/wno.0000000000000221] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
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Lee SJ, Choi CW, Kim SD. A Case of Extramedullary Plasmacytoma in the Lacrimal Excretory System. JOURNAL OF THE KOREAN OPHTHALMOLOGICAL SOCIETY 2015. [DOI: 10.3341/jkos.2015.56.3.427] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Affiliation(s)
- Seung Joon Lee
- Institute of Wonkwang Medical Science, Department of Ophthalmology, Wonkwang University School of Medicine, Iksan, Korea
| | - Chang Wook Choi
- Institute of Wonkwang Medical Science, Department of Ophthalmology, Wonkwang University School of Medicine, Iksan, Korea
| | - Sang Duck Kim
- Institute of Wonkwang Medical Science, Department of Ophthalmology, Wonkwang University School of Medicine, Iksan, Korea
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Optic nerve and dura mater involvement as the first sign of multiple myeloma. Eur J Ophthalmol 2014; 25:77-9. [PMID: 24832040 DOI: 10.5301/ejo.5000489] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/28/2014] [Indexed: 11/20/2022]
Abstract
PURPOSE To report a case of optic nerve and dura mater involvement as the first sign of multiple myeloma. METHODS Case report. RESULTS A 43-year-old woman presented with a headache and decreased vision in both eyes. Ophthalmic examination revealed anterior uveitis and subretinal mass around the optic nerves with accompanying disc edema bilaterally. Magnetic resonance imaging showed dural and optic nerve infiltration with tram-track enhancement in the optic nerve sheath. The diagnosis of multiple myeloma was made as a result of systemic investigations. The patient underwent systemic chemotherapy and cranial radiotherapy. After treatment, the patient's headache disappeared, the papilledema regressed, and the ocular findings improved but complete recovery could not be achieved because of fibrous subretinal tissue and degenerative changes of the optic nerve. CONCLUSIONS Neurologic and ophthalmic involvement in multiple myeloma may appear as the first manifestation of disease. The correct diagnosis is important because it can be life-saving.
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Galea M, McMillan N, Weir C. Diplopia and variable ptosis as the sole initial findings in a case of orbital plasmacytoma and multiple myeloma. Semin Ophthalmol 2013; 30:235-7. [PMID: 24164055 DOI: 10.3109/13506129.2013.839807] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Plasmacytomas are monoclonal plasma cell tumors. They represent 3% of all orbital tumors. We present an unusual case of orbital plasmacytoma and advanced multiple myeloma where the sole presenting symptoms were those of diplopia and ptosis. Clinical examination revealed right hypertropia and variable left upper lid ptosis but no proptosis. The visual acuity and the rest of the ocular examination were normal in both eyes. An MRI scan of the brain and orbits revealed a frontal bone lesion consistent with a plasmacytoma. A bone marrow biopsy showed a light chain multiple myeloma with free lambda light chains. Although the literature reports diplopia and ptosis as being clinical features of orbital plasmacytomas, to our knowledge this is the first reported case where these symptoms were the only presenting features, despite widespread disease.
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Affiliation(s)
- Martin Galea
- Department of Ophthalmology, Tennent Institute of Ophthalmology, Gartnavel General Hospital , Glasgow , UK and
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Felici S, Villivà N, Balsamo G, Andriani A. Efficacy of lenalidomide in association with cyclophosphamide and dexamethasone in multiple myeloma patient with bilateral retro-orbital localisation. Ecancermedicalscience 2013; 7:331. [PMID: 24723969 PMCID: PMC3965188 DOI: 10.3332/ecancer.2013.331] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2013] [Indexed: 12/19/2022] Open
Abstract
Extramedullary localisation is an uncommon manifestation in multiple myeloma (MM). Ocular involvement is rare. Here, we describe a relapse of MM with bilateral retro-orbital localisation without any bone involvement with good and rapid response to therapy with lenalidomide, dexamethasone, and cyclophosphamide.
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Affiliation(s)
- S Felici
- Haematology Unit, Nuovo Regina Margherita, Hospital, 00153 Rome, Italy
| | - N Villivà
- Haematology Unit, Nuovo Regina Margherita, Hospital, 00153 Rome, Italy
| | - G Balsamo
- Histopathology Complex Unit, Santo Spirito Hospital, 00193 Rome, Italy
| | - A Andriani
- Haematology Unit, Nuovo Regina Margherita, Hospital, 00153 Rome, Italy
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Intraocular collision tumour: case report and literature review. Graefes Arch Clin Exp Ophthalmol 2012; 251:1383-8. [PMID: 23232651 DOI: 10.1007/s00417-012-2216-0] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2012] [Revised: 11/13/2012] [Accepted: 11/19/2012] [Indexed: 10/27/2022] Open
Abstract
BACKGROUND "Collision" tumours consist of different neoplasms coexisting within a single lesion. Whilst quite common in the skin, the gastrointestional tract, and the ovaries, intraocular collision tumours are exceedingly rare. We describe an exceptional case of a combined uveal melanoma and intraocular plasmacytoma. METHODS Observational case report. A 61-year-old woman underwent enucleation for rubeotic glaucoma and cells in the anterior chamber after proton-beam radiotherapy of a cilio-choroidal melanoma of the right eye. Examination of the enucleated eye was performed with immunohistochemistry, multiplex ligation dependent probe amplification (MLPA), and polymerase chain reaction (PCR) for immunoglobulin heavy- and light-chain gene rearrangements. A review of the literature on ocular collision tumours and uveal involvement by plasma cell neoplasms was also performed. RESULTS Morphological, immunophenotypical, and genotypical examination of the tumour revealed the co-existence of both a melanoma and a plasmacytoma within the choroid and ciliary body. The glaucoma was caused by extensive infiltration of the iris and trabecular meshwork by the plasmacytoma cells. Review of the literature revealed only four collision tumours involving the eyelid and three involving the choroid. All three intraocular collision tumours consisted of uveal melanoma and choroidal non-Hodgkin lymphoma. Uveal involvement by plasma cell neoplasms is also extremely rare, with only six reported cases. CONCLUSIONS This is the first documented intraocular collision tumour consisting of a uveal melanoma and isolated plasmacytoma. If a patient presents with 'uveitis' after proton-beam radiotherapy of a cilio-choroidal melanoma, there may be scope for performing biopsies to determine whether the lymphoid infiltrate is reactive or neoplastic.
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Kershaw O, Linek J, Linke RP, Gruber AD. Intraocular ALλ amyloidoma with plasma cell neoplasia in a cat. Vet Ophthalmol 2011; 14 Suppl 1:88-92. [DOI: 10.1111/j.1463-5224.2011.00900.x] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Abstract
Plasmacytomas are plasma cell neoplasms that rarely involve ocular adnexal tissues as a primary lesion or secondary manifestation of plasma cell myeloma. Only one case of plasmacytoma involving the lacrimal drainage system, to our knowledge, is described in the literature. The clinical presentation of ocular adnexal primary plasmacytoma typically relates to its mass effect. In this clinicopathologic report, we describe an unusual presentation of primary plasmacytoma of the lacrimal canaliculus as infectious canaliculitis.
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Lymphomas and metastases of the extra-ocular musculature. Neuroradiology 2011; 53:909-16. [DOI: 10.1007/s00234-011-0873-z] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2011] [Accepted: 04/11/2011] [Indexed: 12/20/2022]
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Chin KJ, Kempin S, Milman T, Finger PT. Ocular manifestations of multiple myeloma: Three cases and a review of the literature. ACTA ACUST UNITED AC 2011; 82:224-30. [DOI: 10.1016/j.optm.2010.10.009] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/09/2009] [Revised: 07/09/2010] [Accepted: 10/06/2010] [Indexed: 12/21/2022]
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Pan SW, Wan Hitam WH, Mohd Noor RA, Bhavaraju VMK. Recurrence of multiple myeloma with soft tissue plasmacytoma presenting as unilateral proptosis. Orbit 2011; 30:105-107. [PMID: 21322793 DOI: 10.3109/01676830.2010.546553] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/30/2023]
Abstract
PURPOSE To describe a rare case of soft tissue plasmacytoma of the orbit presenting with proptosis. RESULTS A 57 year-old Malay lady presented with right eye proptosis of 3 weeks' duration. It was associated with pain, redness and progressive blurring of vision. The patient was diagnosed to have multiple myeloma a year previously when she was investigated for backache, and had been treated with eight cycles of Pamidronate chemotherapy. On examination visual acuity was 6/60 in the right eye with impaired optic nerve function. Severe proptosis with chemosis and complete ophthalmoplegia were found. The corneal and anterior chamber examinations were normal. Fundoscopy revealed a slightly hyperaemic disc with presence of choroidal folds infero-temporally. The left vision and eye examination findings were normal. Fine needle biopsy and MRI showed recurrence of multiple myeloma with soft tissue plasmacytoma of the extraconal region compressing the right optic nerve. She was managed with orbital radiotherapy, resulting in resolution of proptosis, but without improvement in vision. CONCLUSION Proptosis may be an indicator of recurrence of multiple myeloma in patients thought to be in remission.
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Affiliation(s)
- Shin Wei Pan
- Department of Ophthalmology, Universiti Sains Malaysia, Health Campus, Kelantan, Malaysia.
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Multi-institutional analysis of solitary extramedullary plasmacytoma of the head and neck treated with curative radiotherapy. Int J Radiat Oncol Biol Phys 2011; 82:626-34. [PMID: 21277117 DOI: 10.1016/j.ijrobp.2010.11.037] [Citation(s) in RCA: 68] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/19/2010] [Revised: 11/06/2010] [Accepted: 11/09/2010] [Indexed: 01/16/2023]
Abstract
PURPOSE The purpose of this study was to elucidate the efficacy and optimal method of radiotherapy in the management of solitary extramedullary plasmacytoma occurring in the head and neck regions (EMPHN). METHODS AND MATERIALS Sixty-seven patients (43 male and 24 female) diagnosed with EMPHN between 1983 and 2008 at 23 Japanese institutions were reviewed. The median patient age was 64 years (range, 12-83). The median dose administered was 50 Gy (range, 30-64 Gy). Survival data were calculated by the Kaplan-Meier method. RESULTS The median follow-up duration was 63 months. Major tumor sites were nasal or paranasal cavities in 36 (54%) patients, oropharynx or nasopharynx in 16 (23%) patients, orbita in 6 (9%) patients, and larynx in 3 (5%) patients. The 5- and 10-year local control rates were 95% and 87%, whereas the 5- and 10-year disease-free survival rates were 56% and 54%, respectively. There were 5 (7.5%), 12 (18%), and 8 (12%) patients who experienced local failure, distant metastasis, and progression to multiple myeloma, respectively. In total, 18 patients died, including 10 (15%) patients who died due to complications from EMPHN. The 5- and 10-year overall survival (OS) rates were 73% and 56%, respectively. Radiotherapy combined with surgery was identified as the lone significant prognostic factor for OS (p = 0.04), whereas age, gender, radiation dose, tumor size, and chemotherapy were not predictive. No patient experienced any severe acute morbidity. CONCLUSIONS Radiotherapy was quite effective and safe for patients with EMPHN. Radiotherapy combined with surgery produced a better outcome according to survival rates. These findings require confirmation by further studies with larger numbers of patients with EMPHN.
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Yumori JW, Ilsen P, Bright DC. Conjunctival plasmacytoma. ACTA ACUST UNITED AC 2010; 81:234-9. [DOI: 10.1016/j.optm.2009.11.006] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/13/2009] [Accepted: 11/22/2009] [Indexed: 11/29/2022]
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Guerriero S, Piscitelli D, Ciracì L, Carluccio P, Furino C, Specchia G. Hypertensive Uveitis as a Feature of Multiple Myeloma. Ocul Immunol Inflamm 2010; 18:104-6. [DOI: 10.3109/09273940903402645] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
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Burkat CN, Van Buren JJ, Lucarelli MJ. Characteristics of orbital multiple myeloma: a case report and literature review. Surv Ophthalmol 2009; 54:697-704. [PMID: 19709708 DOI: 10.1016/j.survophthal.2009.04.012] [Citation(s) in RCA: 56] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2009] [Revised: 04/06/2009] [Accepted: 04/07/2009] [Indexed: 12/16/2022]
Abstract
Multiple myeloma is a plasma cell malignancy that destroys skeletal, renal, and neurological function. Orbital involvement is rare, but has been considered an initial presentation for the malignancy. Furthermore, an association between the subtype of multiple myeloma and the likelihood of orbital infiltration has been suggested. We present a case of an orbital mass that was a recurrence of multiple myeloma. A literature search was performed to evaluate the presentation characteristics of orbital multiple myeloma, plasmacytoma and primary (or solitary) extramedullary plasmacytoma. Past reports were analyzed for age, sex, symptoms at presentation, time from symptom onset to presentation, prior diagnosis before presentation for orbital symptoms, radiological characteristics, immunoglobulin subtype, and survival times. Less than half of published cases had orbital multiple myeloma as the primary presentation. Proptosis is the major presenting sign of orbital multiple myeloma, and radiological evaluation shows that the majority of masses originate in the superotemporal quadrant. The dominant immunoglobulin subtype was IgG.
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Affiliation(s)
- Cat N Burkat
- Oculoplastics Service, Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, Wisconsin 53792-3284, USA.
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Choroidal plasmacytoma in a patient with multiple myeloma. Diagnosis by fine-needle aspiration biopsy. Graefes Arch Clin Exp Ophthalmol 2008; 246:1195-7. [DOI: 10.1007/s00417-008-0804-9] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2008] [Revised: 02/14/2008] [Accepted: 02/25/2008] [Indexed: 10/22/2022] Open
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Lazaridou MN, Micallef-Eynaud P, Hanna IT. Soft tissue plasmacytoma of the orbit as part of the spectrum of multiple myeloma. Orbit 2007; 26:315-318. [PMID: 18097976 DOI: 10.1080/01676830601169122] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/25/2023]
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