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Oh YJ, Lee JE, You SK, Ohm JY, Han HY, Kim JM, Shin KS. Multimodality Imaging Features of Various Splenic Lesions: Clinical and Histopathologic Correlation. JOURNAL OF THE KOREAN SOCIETY OF RADIOLOGY 2024; 85:1099-1125. [PMID: 39660321 PMCID: PMC11625836 DOI: 10.3348/jksr.2024.0004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 01/03/2024] [Revised: 04/02/2024] [Accepted: 04/24/2024] [Indexed: 12/12/2024]
Abstract
The spleen is occasionally referred to as the 'forgotten organ' because splenic lesions are less common and encountered rarely compared to pathologies of other abdominal solid organs. Therefore, although well-demonstrated using various abdominal imaging modalities, radiologists tend to be less familiar with splenic diseases, making interpretation challenging. This study aimed to review common and uncommon splenic diseases and illustrate the multimodal imaging (including ultrasonography, CT, MRI, and PET/CT) features of these lesions in correlation with their histopathology. Recognizing the radiological findings of various splenic lesions helps narrow down the differential diagnosis and guide appropriate clinical decision-making for radiologists.
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Milickovic M, Rasic P, Cvejic S, Bozic D, Savic D, Mijovic T, Cvetinovic S, Djuricic SM. Splenic hamartomas in children. World J Clin Cases 2024; 12:1909-1917. [PMID: 38660549 PMCID: PMC11036520 DOI: 10.12998/wjcc.v12.i11.1909] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/27/2024] [Revised: 03/06/2024] [Accepted: 03/22/2024] [Indexed: 04/11/2024] Open
Abstract
Splenic hamartomas (SHs) are uncommon, benign vascular lesions of unclear etiology and are mostly found incidentally on abdominal images, at surgery, or at autopsy. Since the first case description, in 1861, less than 50 pediatric SH cases have been reported in the literature. In this article, we have performed an analysis of all SH cases in children published in the literature to date and presented our case of an 8-year-old male with SH. These lesions in children were shown to cause symptoms more often than in the adult population. The observed SH sizes in children ranged from a few millimeters to 18 cm, and the symptomatic lesions were mostly larger or multiple. The most common clinical finding was splenomegaly. Signs of hypersplenism were present in children with a single SH larger than 4.5 cm (diameter range: 4.5-18.0 cm) and in those with multiple hamartomas, ranging from a few millimeters to 5 cm. Eighty percent of patients with available laboratory findings had hematological abnormalities such as anemia, thrombocytopenia, or pancytopenia. Other symptoms and signs included abdominal pain, recurrent infections, fever, night sweats, lethargy, growth retardation, and weight loss. The use of multiple imaging modalities may suggest the preoperative diagnosis of a splenic mass in children and determine the therapeutic approach. However, the final diagnosis of SH relies on histopathological evaluation. Surgery, including total or partial splenectomy (PS), is the mainstay of SH management. Although total splenectomy carries a greater risk of overwhelming post-splenectomy infection than PS it has remained the most performed surgical procedure in children with SH. In the majority of pediatric patients with symptomatic SH, resolution of symptoms and resolution or improvement of cytopenias occurred after surgical treatment.
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Affiliation(s)
- Maja Milickovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Petar Rasic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Sofija Cvejic
- Department of Radiology, The Children’s University Hospital, Belgrade 11000, Serbia
| | - Dejana Bozic
- Department of Clinical Pathology, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Djordje Savic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
- Faculty of Medicine, University of Belgrade, Belgrade 11000, Serbia
| | - Tanja Mijovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Sava Cvetinovic
- Department of Abdominal Surgery, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
| | - Slavisa M Djuricic
- Faculty of Medicine, University of Banja Luka, Banja Luka 78000, Bosnia and Herzegovina
- Department of Clinical Pathology, Mother and Child Health Care Institute of Serbia "Dr. Vukan Cupic", Belgrade 11000, Serbia
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Kim N, Auerbach A, Manning MA. Algorithmic Approach to the Splenic Lesion Based on Radiologic-Pathologic Correlation. Radiographics 2022; 42:683-701. [PMID: 35302864 DOI: 10.1148/rg.210071] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Splenic lesions are commonly discovered incidentally at imaging, without clinical signs or symptoms that may aid in diagnosis. As such, the differential diagnosis and subsequent management are based primarily on imaging characteristics. Much has been written about the myriad pathologic conditions that can occur in the spleen; however, there is little guidance on the approach to an incidental splenic mass. Applying an approach frequently used in imaging to the splenic mass-based on the number and consistency of lesions and refined by supplementary imaging features-allows formulation of a useful differential diagnosis. Solitary cystic masses include true cysts, pseudocysts, and parasitic cysts. When multiple cystic lesions are present, the differential diagnosis expands to include infectious lesions (abscess or microabscesses) and lymphangioma (a benign cystic neoplasm). Hemangioma is the most common solitary solid mass, although other vascular lesions (hamartoma, sclerosing angiomatoid nodular transformation) and nonvascular lesions (inflammatory pseudotumor, lymphoma) manifest as solitary and solid. When multiple solid masses are present, diffuse inflammatory disease (sarcoidosis), littoral cell angioma, and lymphoma should be considered. Malignancies, such as angiosarcoma or metastasis, can manifest as solitary or multiple and solid or cystic masses but are typically associated with symptoms or widespread primary malignancy. Careful assessment of the multimodality imaging characteristics of splenic lesions based on this approach aids the radiologist faced with the incidental splenic lesion. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.
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Affiliation(s)
- Nancy Kim
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Aaron Auerbach
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Maria A Manning
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
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Franke D, Anupindi SA, Barnewolt CE, Green TG, Greer MLC, Harkanyi Z, Lorenz N, McCarville MB, Mentzel HJ, Ntoulia A, Squires JH. Contrast-enhanced ultrasound of the spleen, pancreas and gallbladder in children. Pediatr Radiol 2021; 51:2229-2252. [PMID: 34431006 DOI: 10.1007/s00247-021-05131-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2021] [Revised: 04/30/2021] [Accepted: 06/10/2021] [Indexed: 12/14/2022]
Abstract
Gray-scale and color/power Doppler ultrasound (US) are the first-line imaging modalities to evaluate the spleen, gallbladder and pancreas in children. The increasing use of contrast-enhanced ultrasound (CEUS) as a reliable and safe method to evaluate liver lesions in the pediatric population promises potential for imaging other internal organs. Although CEUS applications of the spleen, gallbladder and pancreas have been well described in adults, they have not been fully explored in children. In this manuscript, we present an overview of the applications of CEUS for normal variants and diseases affecting the spleen, gallbladder and pancreas. We highlight a variety of cases as examples of how CEUS can serve in the diagnosis and follow-up for such diseases in children. Our discussion includes specific examination techniques; presentation of the main imaging findings in various benign and malignant lesions of the spleen, gallbladder and pancreas in children; and acknowledgment of the limitations of CEUS for these organs.
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Affiliation(s)
- Doris Franke
- Department of Pediatric Kidney, Liver and Metabolic Diseases, MHH, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
| | - Sudha A Anupindi
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.,Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA
| | - Carol E Barnewolt
- Department of Radiology, Boston Children's Hospital, Harvard University, Boston, MA, USA
| | - Thomas G Green
- Department of Radiology, Crouse Hospital, Syracuse, NY, USA
| | - Mary-Louise C Greer
- Department of Diagnostic Imaging, The Hospital for Sick Children, Department of Medical Imaging, University of Toronto, Toronto, ON, Canada
| | - Zoltan Harkanyi
- Department of Radiology, Heim Pal National Pediatric Institute, Budapest, Hungary
| | - Norbert Lorenz
- Children's Hospital, Dresden Municipal Hospital, Teaching-Hospital of Technical University Dresden, Dresden, Germany
| | - M Beth McCarville
- Department of Diagnostic Imaging, St. Jude Children's Research Hospital, Memphis, TN, USA
| | - Hans-Joachim Mentzel
- Section of Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, University Hospital, Jena, Germany
| | - Aikaterini Ntoulia
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
| | - Judy H Squires
- Department of Radiology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA
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Laparoscopic partial splenectomy for a splenic hamartoma. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2021. [DOI: 10.1016/j.epsc.2020.101737] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
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Splenic hamartoma associated with visceral leishmaniasis mimicking aggressive lymphoma. Hematol Transfus Cell Ther 2019; 42:390-393. [PMID: 31836460 PMCID: PMC7599261 DOI: 10.1016/j.htct.2019.08.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/22/2019] [Revised: 08/15/2019] [Accepted: 08/22/2019] [Indexed: 11/22/2022] Open
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7
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Torsion of wandering spleen in an infant associated with hamartomatous vascular malformation. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2019. [DOI: 10.1016/j.epsc.2018.10.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
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A giant splenic hamartoma associated with hematologic disorders: A case report. Ann Med Surg (Lond) 2018; 36:199-202. [PMID: 30505440 PMCID: PMC6249354 DOI: 10.1016/j.amsu.2018.11.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/03/2018] [Accepted: 11/06/2018] [Indexed: 12/17/2022] Open
Abstract
Introduction Splenic hamartoma is a primary benign tumor of the spleen, with approximately 150 cases documented in the literature to date, with only a few cases associated with symptoms and hematologic disorders. Presentation of case A 49-year-old female with no past medical history, presented to the emergency department complaining of a three-month history of intermittent abdominal pain and 12 kg of weight loss. Physical examination revealed abdominal distension and a big palpable and painless mass on the left side of her abdomen measuring 14 cm. Laboratory tests were significant for anemia and thrombocytopenia, with levels of 9.7 g/dL and 47 × 109/L respectively. Ultrasonography showed splenomegaly with a hypoechoic splenic mass and the computed tomography showed a 14 cm splenic mass with heterogeneous enhancement during the arterial phase. A laparotomy with splenectomy was unremarkably accomplished. Histological examination revealed abnormal red pulp proliferation and showed unorganized sinusoid-like vascular channels, compatible with splenic hamartoma. The patient was discharged on postoperative day 3 without complications. She was seen at the ambulatory clinic 6-months after the surgical procedure with a normal blood count. Discussion Although splenic hamartoma is very rare, it must be included in the differential diagnosis of splenic mass-forming lesions. This type of tumor has some specific radiological features. However, the diagnosis of this disease must be based on clinical features and confirmed by pathology. Conclusion In patients with splenic tumors, splenectomy is indicated in cases where malignancy cannot be excluded, when symptoms occur, or in the rare cases of consequent hematologic disorders.
Case report of a 49-year-old female patient presented with abdominal pain and hematologic disorders due to a spleen hamartoma. Little over 150 cases have been reported in medical literature. Splenic hamartoma must be included in the differential diagnosis of splenic mass-forming lesions. Splenectomy is the treatment of choice for these lesions.
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Serra F, Sorrentino L, Cabry F, Biondini D, Ceccarelli PL, Campanelli M, Gelmini R. First case of laparoscopic partial splenectomy in a child with hamartoma: Case report and review of the literature. Int J Surg Case Rep 2018; 53:140-143. [PMID: 30396124 PMCID: PMC6216043 DOI: 10.1016/j.ijscr.2018.09.018] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/18/2018] [Revised: 09/12/2018] [Accepted: 09/13/2018] [Indexed: 12/11/2022] Open
Abstract
INTRODUCTION To date, laparoscopic surgery has played a key role in the treatment of not only splenic hematologic pathologies but also solid ones. Hamartoma is a rare disease; only twenty percent of them are of pediatric relevance; it is a benign tumor, but radiological features never allow proper differentiation from malignant neoplasms. In children, hamartoma may be associated with other morbid conditions, such as sickle cell disease or other hematological alterations. PRESENTATION OF THE CASE We report a case of hamartoma in a 7-year-old child treated with partial laparoscopic splenectomy. After a multidisciplinary evaluation, the indication of laparoscopic splenectomy was decided; upon evaluating the age of the patient and the affected spleen portion, a partial splenectomy was proposed. The histological examination during surgery was performed to exclude any form of malignancy. The intraoperative frozen section of the specimen was negative for malignancies, and a partial splenectomy was performed. DISCUSSION Surgery remains the first choice in the definitive treatment of solid lesions of the spleen; minimally invasive technique, namely, laparoscopy, has set itself as the technique of choice for surgical treatment. In this case, the possibility of obtaining an intraoperative pathological diagnosis by frozen section of the specimen, confirming the benign nature of the lesion, allowed the surgeon to decide in favor of a laparoscopic partial splenectomy. CONCLUSION Partial laparoscopic splenectomy can be considered a safe, effective and reproducible alternative in patients suffering from benign solid diseases, safeguarding the hematological functions of the organ itself in pediatric age.
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Affiliation(s)
- Francesco Serra
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Lorena Sorrentino
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Francesca Cabry
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Diego Biondini
- Department of Pediatric Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Pier Luca Ceccarelli
- Department of Pediatric Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Michela Campanelli
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
| | - Roberta Gelmini
- Department of Surgery, University of Modena and Reggio Emilia, Policlinico of Modena, Via del Pozzo, 71 41100 Modena, Italy.
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Illuminati G, Prezioso G, Pizzardi G, Pasqua R, Perotti B, Amatucci C, De Vincentiis L. Splenic hamartoma associated with abdominal discomfort and pain: Case report. INTERNATIONAL JOURNAL OF SURGERY OPEN 2018. [DOI: 10.1016/j.ijso.2018.07.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
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Elenga N, Labbé S, Leduc N, Sika A, Cuadro E, Long L, Njuieyon F, Kom-Tchameni R, Basset T. Asymptomatic multinodular splenoma (splenic hamartoma) in a child with sickle cell anemia. Int Med Case Rep J 2017; 10:233-236. [PMID: 28744165 PMCID: PMC5513840 DOI: 10.2147/imcrj.s125988] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Splenoma is a rare and benign malformation usually fortuitously diagnosed during imaging, surgery or, unfortunately, at autopsy. Although splenoma was first described in 1861, its association with hematological pathology is a very unusual condition in children. We report the case of an asymptomatic splenoma in an 8-year-old boy with sickle cell anemia, whose diagnosis was confirmed after conventional splenectomy.
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Affiliation(s)
- Narcisse Elenga
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Sylvain Labbé
- Pathological Anatomy Department, Cayenne Hospital, Cayenne Cedex, French Guiana
| | - Nicolas Leduc
- Pathological Anatomy Department, Cayenne Hospital, Cayenne Cedex, French Guiana
| | - Anicet Sika
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Emma Cuadro
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Laurence Long
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Falucar Njuieyon
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Rémi Kom-Tchameni
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
| | - Thierry Basset
- Department of Pediatrics and Pediatric Surgery, Pediatric Medicine and Surgery
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Radouani MA, Azzaoui S, Mammad C, Knouni H, Chefchaouni K, Benchekroun I, Barkat A. [Splenic hamartoma, severe thrombocytopenia, and macrocrania in a neonate: A case study]. Arch Pediatr 2015; 22:1260-2. [PMID: 26552616 DOI: 10.1016/j.arcped.2015.09.016] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2014] [Revised: 01/26/2015] [Accepted: 09/23/2015] [Indexed: 11/19/2022]
Abstract
We report a case of splenic hamartoma associated with neonatal macrocrania diagnosed in a newborn. The diagnosis was made in the first 2 days of life upon the existence of an abdominal mass in the newborn infant. Abdominal ultrasound and abdominal computed tomography supported the diagnosis, but the histological study was inconclusive. The indication for surgery is still controversial. Through a literature review, the clinical, histological, and radiological aspects are discussed. The combination of neonatal hamartoma, thrombocytopenia, and macrocrania has never been reported.
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Affiliation(s)
- M A Radouani
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - S Azzaoui
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - C Mammad
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - H Knouni
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - K Chefchaouni
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - I Benchekroun
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc
| | - A Barkat
- Service de médecine et réanimation néonatales, Centre national de néonatologie et nutrition, hôpital d'Enfants, centre hospitalier Ibn Sina, boulevard Ibn Rochd, Souissi 10100, Rabat, Maroc; Équipe de recherche en santé et nutrition du couple mère-enfant, faculté de médecine et de pharmacie de Rabat, université Mohammed V Souissi, Rabat, Maroc.
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Eker T, Kocaay AF, Sevim Y, Çakmak A. Splenic hamartoma is a rare cause of abdominal pain: Case report and literature review. Turk J Surg 2015; 33:294-295. [PMID: 29260137 DOI: 10.5152/ucd.2015.3048] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/25/2014] [Accepted: 02/21/2015] [Indexed: 12/21/2022]
Abstract
Hamartoma is a rare benign tumor of the spleen. It is often asymptomatic and diagnosed incidentally. In this study, we report the case of a 51-year-old female patient who was admitted to our department for intermittent epigastric pain since the last 6 months and left upper quadrant fullness. She was diagnosed with splenic hamartoma histopathologically after splenectomy. Although splenic hamartoma is very rare, it must be included in the differential diagnosis of splenic mass-forming lesions.
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Affiliation(s)
- Tevfik Eker
- Department of General Surgery, Ankara University School of Medicine, Ankara, Turkey
| | - Akın Fırat Kocaay
- Department of General Surgery, Ankara University School of Medicine, Ankara, Turkey
| | - Yusuf Sevim
- Clinic of General Surgery, Kayseri Training and Research Hospital, Kayseri, Turkey
| | - Atıl Çakmak
- Department of General Surgery, Ankara University School of Medicine, Ankara, Turkey
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Wang RT, Xu XS, Hou HL, Qu K, Bai JG. Symptomatic multinodular splenic hamartoma preoperatively suspected as metastatic tumor: A case report. World J Gastroenterol 2014; 20:10637-10641. [PMID: 25132786 PMCID: PMC4130877 DOI: 10.3748/wjg.v20.i30.10637] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2014] [Revised: 01/26/2014] [Accepted: 05/05/2014] [Indexed: 02/06/2023] Open
Abstract
Splenic hamartoma (SH) is a rare benign tumor usually detected accidentally, which is composed of an aberrant mixture of normal splenic elements. Here, we report a case of 54-year-old man who presented with symptomatic multinodular SH and was admitted initially for thrombocytopenia and anemia. Physical examination revealed that the patients had an anemic appearance and palpable spleen, extending 10 cm below the costal margin. Preoperative ultrasound and computed tomography (CT) indicated splenomegaly with multinodular lesions. On enhanced CT scanning, during the arterial phase, the lesions demonstrated inhomogeneous enhancement, and in the portal phase the lesions were more hyperdense than the splenic parenchyma. The images were highly suggestive of a metastatic tumor. Splenectomy was performed 1 wk later. The tumor was eventually diagnosed as SH according to the morphological features and immunohistochemical detection, by which CD34 was positive in lining cells and some spindle cells, vimentin was positive in the tumor, factor-VIII-related antigen was positive multifocally in lining cells, and smooth muscle actin was positive in some spindle cells. Thrombocytopenia and anemia were cured after splenectomy.
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Wang JH, Ma XL, Ren FY, Zuo CJ, Tian JM, Wang ZF, Zheng JM. Multi-modality imaging findings of splenic hamartoma: a report of nine cases and review of the literature. ACTA ACUST UNITED AC 2013; 38:154-62. [PMID: 22539044 DOI: 10.1007/s00261-012-9880-8] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
PURPOSE To investigate the presentation of splenic hamartomas (SHs) on ultrasonography (US), CT and MRI. METHODS Nine patients (5 males and 4 females, mean age, 52.8 years) with pathologically proven SHs were included in this study. US, CT and MRI images were analyzed retrospectively, and imaging features were correlated with pathological findings. RESULTS SHs appeared solitary lesion (n = 8) and multiple lesions (n = 1) in the present study. (1) In 8 cases of solitary lesion, the lesions appeared as solid nodules or masses with well-defined margins and varying echogenicity (hyperecho = 5, hypoecho = 2, strong echo = 1) on ultrasound. The lesions showed iso-attenuation (n = 3) or slightly hypo-attenuation (n = 4) on unenhanced CT, and calcification were revealed in 3 lesions. MRI showed isointensity (n = 3) or hypointensity (n = 2) on the T1-weighted image, and heterogeneous hypointensity (n = 2), slightly hyperintensity (n = 2) and hyperintensity (n = 1) on the T2-weighted image. The enhanced patterns of SHs showed mild diffuse heterogeneous enhancement (n = 6) and prominent enhancement (n = 1) during arterial phase and above 7 lesions were demonstrated progressive enhancement at delayed phase on enhanced CT. One lesion without any enhancement was revealed in another patient. (2) One case of multiple lesions included 1 cystic lesion with irregular calcification and 7 solid lesions with progressive enhancement on CT images. CONCLUSIONS Combination of a variety of imaging modalities could more fully reflect the pathological characteristics and contribute to the diagnosis of SH.
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Affiliation(s)
- Jian-hua Wang
- Department of Radiology, The Affiliated Hospital of Ningbo University School of Medicine, Ningbo No. 2 Hospital, Ningbo, 315010, Zhejiang, China
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16
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Pisani Ceretti A, Bislenghi G, Virdis M, Maroni N, Gatti A, Opocher E. Laparoscopic splenectomy for splenic hamartoma: a case report. Case Rep Gastrointest Med 2012; 2012:435802. [PMID: 23125940 PMCID: PMC3485498 DOI: 10.1155/2012/435802] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/16/2012] [Accepted: 09/30/2012] [Indexed: 12/11/2022] Open
Abstract
Hamartoma is a rare splenic benign tumor usually accidentally detected as a radiologic finding. Preoperative diagnosis poses a challenge and thus surgery becomes necessary to confirm the clinical suspicion. Laparoscopic splenectomy has gained consensus as a standard surgical procedure particularly for autoimmune hematological diseases. This former experience has allowed this technique to be extended to other splenic pathologies. Here we report a case of total laparoscopic splenectomy for a bulky splenic hamartoma in a young male patient.
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Affiliation(s)
| | - Gabriele Bislenghi
- Department of General Surgery, San Paolo Hospital, University of Milan, Via Cesariano 10, 20154 Milano, Italy
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18
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Okamoto K, Maeda H, Okabayashi T, Dabanaka K, Namikawa T, Sugimoto T, Kobayashi M, Hanazaki K. Splenic hamartoma: a case report and clinicopathological analysis of Japanese cases. Clin J Gastroenterol 2011; 4:381-386. [PMID: 26189740 DOI: 10.1007/s12328-011-0255-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/13/2011] [Accepted: 08/08/2011] [Indexed: 02/05/2023]
Abstract
A 75-year-old man was referred to our department for the treatment of a gastric submucosal tumor-like protrusion which had gradually enlarged over 3 years. After examination with various types of imaging modalities, a splenic mass was diagnosed. Splenectomy was performed because it was difficult to determine preoperatively whether the splenic mass was benign or malignant. The postoperative pathological examination revealed a splenic hamartoma without malignancy. Despite the preoperative examination with advanced diagnostic imaging techniques and equipment, an accurate diagnosis of splenic hamartoma is difficult due to its rarity, lack of specific symptoms, and various inadequacies of radiological imaging techniques. Here we present a rare case of splenic hamartoma and discuss the clinical characteristics obtained from a review of Japanese cases of this rare condition.
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Affiliation(s)
- Ken Okamoto
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan.
| | - Hiromichi Maeda
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Takehiro Okabayashi
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Ken Dabanaka
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Tsutomu Namikawa
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Takeki Sugimoto
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Michiya Kobayashi
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
| | - Kazuhiro Hanazaki
- Department of Surgery, Kochi Medical School, Kohasu-Okocho, Nankoku, Kochi, 783-8505, Japan
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19
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Chiu A, Czader M, Cheng L, Hasserjian RP, Wang M, Bhagavathi S, Hyjek EM, Al-Ahmadie H, Knowles DM, Orazi A. Clonal X-chromosome inactivation suggests that splenic cord capillary hemangioma is a true neoplasm and not a subtype of splenic hamartoma. Mod Pathol 2011; 24:108-16. [PMID: 20852592 DOI: 10.1038/modpathol.2010.168] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Splenic hamartoma is a rare tumor-like lesion composed of structurally disorganized red pulp elements. It has been hypothesized that two other splenic lesions, cord capillary hemangioma and myoid angioendothelioma, may fall within the spectrum of splenic hamartoma, simply representing morphological variants. In this study, we compared the vascular and stromal composition of cord capillary hemangioma and myoid angioendothelioma with those of classical hamartoma. In addition, we assessed the clonal vs polyclonal nature of the lesions in nine female cases by performing clonality analysis for X-chromosome inactivation at the human androgen receptor locus (HUMARA) on laser-assisted microdissected samples. In 15 of 17 cases, increased reticulin and/or collagen content was observed. The classical hamartoma cases showed a vasculature predominantly composed of CD8+ CD31+ CD34- splenic sinuses, whereas cases of cord capillary hemangioma and myoid angioendothelioma contained many CD8- CD31+ CD34+ cord capillaries, but very little CD8+ vasculature. All cases lacked expression of D2-40 and Epstein Barr virus-encoded RNA. All cases showed a proliferation index of ≤5% by Ki-67. Cases of classical hamartoma lacked significant perisinusoidal expression of collagen IV and low-affinity nerve growth factor receptor. Both markers were variably expressed in the other lesions. Increased CD163-positive histiocytes were found in four cases (three cord capillary hemangiomas and one myoid angioendothelioma). HUMARA analysis was informative in all nine tested cases, of which three cases showed a non-random X-chromosome inactivation pattern, indicating clonality. All three clonal cases were cord capillary hemangiomas. Our study has shown that in spite of considerable morphologic heterogeneity and overlapping features, classical hamartoma and cord capillary hemangioma and myoid angioendothelioma are different in terms of their vascular and stromal composition. Clonality analysis supports a true neoplastic origin for the cord capillary hemangioma. A larger study using additional immunohistochemical and molecular studies is necessary to further evaluate the biological significance of the current findings.
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Affiliation(s)
- A Chiu
- Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY 10065, USA
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20
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Koh YW, Lee H, Choi G, Kwon GY, Kim EJ, Huh J. Primary Splenic Vascular Lesions: A Clinicopathologic, Immunophenotypic and Radiopathologic Correlation Study of 40 Cases. KOREAN JOURNAL OF PATHOLOGY 2010. [DOI: 10.4132/koreanjpathol.2010.44.5.502] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Affiliation(s)
- Young Wha Koh
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Heejin Lee
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gawon Choi
- Department of Pathology, Mizmedi Hospital, Seoul, Korea
| | - Gui Young Kwon
- Department of Pathology, Chung-Ang University Hospital, Chung-Ang School of Medicine, Seoul, Korea
| | - Eun-Ju Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jooryung Huh
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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21
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Abstract
Vascular proliferations of the spleen reflect the variability of vascular structures occurring in the normal spleen. Besides haemangiomas, there is a spleen-specific vascular neoplasm, littoral cell angioma, that often occurs as a paraneoplastic lesion and thus may require the differential diagnostic delineation of metastases to the spleen in patients with known neoplasms. The most common malignant vascular tumours of the spleen are angiosarcomas. A recently described vascular lesion of unknown pathogenesis, sclerosing angiomatoid nodular transformation (SANT) of the spleen, usually is an incidental finding detected in the course of imaging studies.
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22
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Abstract
Splenic hamartoma is a rare, benign vascular proliferation that is often found incidentally while working up other complaints or at autopsy. Women more commonly present with symptoms related to mass effect than men. Histologic findings consist of unorganized vascular channels of varying width, with intervening red pulp-like disorganized stroma with or without lymphoid follicles. The endothelial cells are similar to those of normal splenic sinuses. Although rendering a diagnosis can be difficult, endothelial cells that are positive for CD8 are a key feature that differentiate hamartoma from other vascular lesions of the spleen. Clinical, radiologic, and histologic correlation is essential to ensure this benign lesion is not mistaken for malignancy.
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Affiliation(s)
- Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI 48202, USA.
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23
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Abstract
We presented a case of splenic hamartoma. A 12-year-old girl has had an abdominal pain since 9 years of age. Contrast-enhanced computed tomography scan at a local hospital revealed heterogeneous enhancement of a mass which is 5 cm in diameter, located near the surface of the spleen, diagnosed as hemangioma. She had an investigation with Levovist-enhanced ultrasonography and superparamagnetic iron oxide-enhanced magnetic resonance imaging on T2-weighted gradient-echo imaging, which showed a hypervascular echoic mass and a decrease of signal intensity. The imaging diagnosis was splenic hamartoma. She underwent laparoscopic splenectomy to prevent the spontaneous rupture. The splenic artery at the pancreas body was exposed, and doubly ligated by vessel clip and furthermore ligated by 3-0 silk. At the splenic hilum, the splenic artery and vein were dissected with the Endo GIA vascular linear cutting stapler. The spleen was captured into the Endocatch II retrieval bag and removed from a transverse lower abdominal (suprapubic) incision extended up 7 cm in length through a port site. In pathological findings, the tumor was compatible with hamartoma. Splenic hamartomas in pediatric patients have been described extremely rare. In clinical symptoms, spontaneously ruptured splenic hamartoma has been rarely reported.
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Affiliation(s)
- Yukihiro Tatekawa
- Department of Surgery, Nara Medical University, 840, Shijyo-cho, Kashiharashi, Nara 634-8522, Japan.
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24
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Ramdall RB, Alasio TM, Cai G, Yang GCH. Primary vascular neoplasms unique to the spleen: littoral cell angioma and splenic hamartoma diagnosis by fine-needle aspiration biopsy. Diagn Cytopathol 2007; 35:137-42. [PMID: 17304535 DOI: 10.1002/dc.20568] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
We report the fine-needle aspiration (FNA) biopsy diagnosis of two rare cases of primary vascular neoplasms unique to the spleen: a littoral cell angioma from a 31-yr-old Caucasian woman and a splenic hamartoma from a 46-yr-old black man. The cytologic features of splenic hamartoma and of littoral cell angioma of the spleen were described three times in cytologic literature: two were bench-top aspirates and one was FNA biopsy thought to be metastatic carcinoma. To the best of our knowledge, the current two cases were the first diagnosed by FNA biopsy. Our approach to the FNA biopsy diagnosis of these rare vascular neoplasms via compact cell block and immunohistochemistry is described. The differential diagnosis with other primary vascular splenic neoplasms is also discussed.
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Affiliation(s)
- Risha B Ramdall
- Department of Pathology, New York University School of Medicine, New York, NY 10016, USA
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25
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Benkirane A, Berrebi D, Olaya N, Ferkdadji L, Chomette PP, Benkerrou M, Peuchmaur M. Hamartome de la rate (splénome) chez un enfant drépanocytaire. Ann Pathol 2007; 27:27-30. [PMID: 17568356 DOI: 10.1016/s0242-6498(07)88681-0] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/22/2022]
Abstract
Hamartomas of the spleen or splenomas are uncommon benign lesions that predominantly occur in adults. We report a case of an 11-year-old girl with sickle cell anemia who had a single splenic 1.8 cm nodule incidentally found during splenectomy and histologically characterized by disorganized red pulp tissue without interspersed white pulp leading to the diagnosis of hamartoma. The association of hamartoma and hematological conditions is a very unusual condition in children.
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Affiliation(s)
- Amal Benkirane
- Anatomie et Cytologie pathologiques, Hôpital Robert Debré, 48, boulevard Sérurier 75019 Paris
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26
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Ali TZ, Beyer G, Taylor M, Volpe C, Papadimitriou JC. Splenic hamartoma: immunohistochemical and ultrastructural profile of two cases. Int J Surg Pathol 2005; 13:103-11. [PMID: 15735864 DOI: 10.1177/106689690501300116] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Splenic hamartoma (SH) is a rare, benign lesion. We present 2 cases, both in females (2 and 30 years, respectively) with multiple urinary tract infections, and left upper quadrant abdominal pain. Immunohistochemical staining with factor VIII displayed intense diffuse staining in the SH with corresponding weak staining in the adjacent spleen. CD31 showed a reverse pattern from that of factor VIII. CD34 staining pattern was identical in both the spleen and the SH. Ultrastructurally, the SH showed endothelial cells with relatively empty cytoplasm, scattered Weibel Palade bodies, and lining by basement membrane surrounded by fibrous long-spacing collagen. Our study highlights the unique immunohistochemical profile of SH. The ultrastructural features are interesting, although their diagnostic significance remains to be confirmed in future studies.
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Affiliation(s)
- Tehmina Z Ali
- Department of Pathology, University of Maryland Medical Center, Baltimore, Maryland 21201, USA
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27
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Abstract
Splenic hamartoma is a rare entity characterized by disorganized red pulp tissue in the absence of interspersed white pulp. We report 3 cases with scattered bizarre stromal cells, a feature not previously reported in the literature. The patients were adults who presented with abdominal pain (2 cases) or were incidentally found to have a splenic mass (1 case). The lesion was solitary, circumscribed, and unencapsulated, comprising disorganized slit-like, tubular, ectatic or cavernous vascular channels. There was a loose stroma that contained lymphocytes, plasma cells, siderophages, and fibrin exudate. Scattered haphazardly were isolated bizarre large cells with oval, convoluted, or multilobated nuclei; pale or smudged chromatin; and scanty cytoplasm. Mitotic figures were not found. The bizarre cells were negative for lymphoid, dendritic cell, histiocytic, myeloid, endothelial, epithelial, and melanocytic markers. Only rare bizarre cells stained for desmin in 1 case. It is important to recognize this morphologic variant of splenic hamartoma in order not to misinterpret the bizarre cells as being indicative of a malignancy.
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Affiliation(s)
- Wah Cheuk
- Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
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28
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Abstract
AIM: To assess CT and MR manifestations and their diagnostic value in splenic hamartoma with review of literatures.
METHODS: We described a woman who was accidentally found to have a splenic tumor by ultrasound of the abdomen. CT and MR findings of this splenic hamartoma were proved by pathology retrospectively.
RESULTS: The CT and MR findings in this case included a ball-like mass with homogeneous mild-hypodensity lesions on non-enhanced CT scans or isointensity on T1-weighted images and mild hypointensity on T2-weighted images, progressive homogeneous enhancement on multiple-phase spiral CT and MR enhanced scans, and isodense enhancement on delayed post-contrast CT scans and obvious hyperintensity relative to the spleen on delayed MR images.
CONCLUSION: Splenic hamartoma has some specific radiological features. However, the diagnosis of this disease must be based on clinical features and confirmed by pathology.
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Affiliation(s)
- Ri-Sheng Yu
- Department of Radiology, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, Zhejiang Province, China.
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29
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Abramowsky C, Alvarado C, Wyly JB, Ricketts R. "Hamartoma" of the spleen (splenoma) in children. Pediatr Dev Pathol 2004; 7:231-6. [PMID: 15037946 DOI: 10.1007/s10024-003-9097-5] [Citation(s) in RCA: 27] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/17/2003] [Accepted: 01/05/2004] [Indexed: 02/06/2023]
Abstract
Hamartomas of the spleen or splenomas, are uncommon benign tumorous growths in this organ which have not been well characterized in children. We report four patients, 4 to 11 years old, who had splenomegaly and splenic "hamartomas" associated with different hematologic conditions (refractory microcytic anemia, sickle cell anemia, hereditary spherocytosis, and dyserythropoietic hemolytic anemia). All patients had total splenectomy as a primary therapeutic approach or to lessen their transfusion requirements. In only one patient was a focal splenic mass identified preoperatively with contrasted computed tomography (CT) scans and magnetic resonance imaging (MRI). None of the patients showed a mass by ultrasonography. Gross examination showed enlarged spleens (315-724 g) which on cut surface revealed a single nodule in one and multiple bulging nodules in three specimens. The nodules varied from 1.3 to 7 cm and were indistinct from the surrounding nonlymphoid splenic (i.e., red pulp) parenchyma. Histology of the nodules showed red splenic pulp with variable histiocytic proliferation, focal extramedullary hematopoiesis, lympho-plasmacytosis, fibrosis, and siderotic-calcific deposits. Intranodular small T- and B-cell lymphoid aggregates but no organized secondary follicles or periarteriolar sheaths were seen. Proliferation antigen Ki-67 (Mib-1) immunostains showed a low (< 5%) proliferation index in the nodules and surrounding tissue. Reticulin stains did not show a capsule or border between the normal spleen and the nodules. The critical histologic differential diagnosis for these lesions is with benign vascular tumors. These can be identified by their more disorderly pattern, by immunohistochemistry and by their higher proliferation index. It is our contention that these splenic nodules are not true hamartomas, as they seem to result from remote ischemic or infectious/inflammatory insults, leading to the fibro-inflammatory reaction and deposition of calcium and hemosiderin that is better designated with the descriptive term of splenoma. Review of the literature and our own experience indicates that most children with splenic hamartomas or splenomas as we prefer to call them, have an underlying hematologic disorder likely made worse by a state of hypersplenism that explains the consistent improvement in the blood values after splenectomy.
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Affiliation(s)
- Carlos Abramowsky
- Department of Pathology, Emory University School of Medicine, Egleston Children's Hospital, 1405 Clifton Road, Atlanta, GA 30322, USA.
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30
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Abstract
This short review is dedicated to a precise pathologic characterization of 2 uncommon and poorly defined lesions of the spleen and to their distinction from histologically similar processes. Splenic hamartoma represents an abnormally formed red pulp and is characterized by the presence of sinus-like structures lined by CD8(+) endothelia. The great variety of its morphologic appearances may result from the preponderant growth of one or another of the several components of the red pulp, ie, CD34(+) capillaries, myoid cells and macrophages. Therefore, it is proposed that "cord capillary hemangioma," myoid angio-endothelioma, and histiocyte-rich tumors are part of the spectrum of splenic hamartoma. Inflammatory pseudotumor (IPT) of the spleen is a reactive lesion, probably of multiple etiologies, characterized by a mixture of inflammatory cells and a minor, disorganized component of spindle cells. The latter include fibroblasts, SMA(+) myofibroblasts, and CD68(+) spindled histiocytes, establishing a close similarity with the IPT of the lymph node. This benign process needs to be distinguished from 2 others that have a predominant spindle cell component arranged in parallel bundles: the IPT-like follicular dendritic cell tumor, which is consistently associated with Epstein-Barr Virus; and the inflammatory myofibroblastic tumor, also often Epstein-Barr Virus-related and similar to those of the soft tissues, lung and other organs. These 2 lesions are neoplastic and therefore have a potentially worse prognosis than IPT.
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Affiliation(s)
- Jayashree Krishnan
- Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY, USA
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31
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Cualing H, Wang G, Noffsinger A, Fenoglio-Preiser C. Heterotopic ovarian splenoma: report of a first case. Arch Pathol Lab Med 2001; 125:1483-5. [PMID: 11698008 DOI: 10.5858/2001-125-1483-hos] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
We present a case of ovarian splenoma, a form of heterotopic splenic hamartoma consisting of red pulp tissue. The hamartoma was located in ovarian stroma in an otherwise normal ovary. The histology showed interanastomosing vascular channels of splenic sinusoidal red pulp lined by cells that were immunoreactive for antibodies to von Willebrand antigen and CD8, findings consistent with splenic lining cells. The sinuses were lined by cuboidal to flattened cells with ovoid and grooved bland-looking nuclei. Ultrastructurally, the tumor cells showed Weibel-Palade bodies and lysosomes. To our knowledge, this is the first case of splenic tissue arising in an ovary, and it underlines the trend noted in the literature that splenic hamartoma,although a rare entity, can arise in many retroperitoneal organs, including the ovary.
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Affiliation(s)
- H Cualing
- Department of Pathology and Laboratory Medicine, University of Cincinnati Medical Center, Cincinnati, OH 45267-0529, USA.
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32
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Suárez Alvarez CG, Górgolas Hernández-Mora M, Lago M, Franco López MA, Sarasa Corral JL, Fernández Guerrero ML. [Pancytopenia and splenic tumor in a young woman]. Rev Clin Esp 2000; 200:231-2. [PMID: 10857412 DOI: 10.1016/s0014-2565(00)70614-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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