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Dellino M, Magazzino F, Domenici L, Cicogna S, Miano ST, Pignata S, Mangili G, Cormio G. Aggressive Angiomyxoma of the Lower Female Genital Tract: A Review of the MITO Rare Tumors Group. Cancers (Basel) 2024; 16:1375. [PMID: 38611053 PMCID: PMC11011140 DOI: 10.3390/cancers16071375] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/12/2024] [Revised: 03/12/2024] [Accepted: 03/23/2024] [Indexed: 04/14/2024] Open
Abstract
Aggressive angiomyxoma (AAM) is a rare, locally aggressive, myxoid mesenchymal neoplasm primarily found in the pelvic and perineal regions of young adult females. It is a slow growing and locally infiltrating tumor. Preoperative diagnosis is difficult due to the rarity of these tumors and absence of characteristic signs and symptoms. The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Other non- surgical modalities have been employed, such as radiotherapy, embolization, GnRH analogues or other anti-estrogenic agents. Local relapses occur in 30-40% of the cases, and often appear many years (sometimes decades) after the first excision. Occasional distant metastasis has also been reported. A limited number of cases have been reported in the literature, mostly in the form of small case series or isolated case reports. Therefore, the aim of this paper by a team of experts from the MITO rare tumors group is to review clinical findings, pathologic characteristics and outcome of patients affected by this rare condition in order to be able to offer up-to-date guidance on the management of these cases.
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Affiliation(s)
- Miriam Dellino
- Department of Interdisciplinary Medicine (DIM), University of Bari “Aldo Moro”, Piazza Giulio Cesare 11, 70124 Bari, Italy;
| | - Francescapaola Magazzino
- Complex Operating Unit Ginecologia E Ostetricia, Ospedale Civile Di San Dona’ Di Piave (Venezia), Aulss4 Veneto Orientale, 30027 San Donà di Piave, Italy;
| | - Lavinia Domenici
- Division of Obstetrics and Gynecology, Azienda Ospedaliera Universitaria Pisana, University of Pisa, 56126 Pisa, Italy;
| | - Stefania Cicogna
- Institute for Maternal and Child Health-IRCCS “Burlo Garofolo”, 34145 Trieste, Italy;
| | - Salvatora Tindara Miano
- Complex Operating Unit of Oncology, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy;
| | - Sandro Pignata
- Department of Urology and Gynecology, Istituto Nazionale Tumori IRCCS ‘Fondazione G Pascale’, 80144 Napoli, Italy;
| | - Giorgia Mangili
- Obstetrics and Gynecology Unit, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy;
| | - Gennaro Cormio
- Department of Interdisciplinary Medicine (DIM), University of Bari “Aldo Moro”, Piazza Giulio Cesare 11, 70124 Bari, Italy;
- Gynecologic Oncology, IRCCS Istituto Tumori “Giovanni Paolo II”, 70124 Bari, Italy
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Aminimoghaddam S, Sarchami N, Mahboub SS. Second recurrence of aggressive vulvar angiomyxoma: a case report. J Int Med Res 2023; 51:3000605231189366. [PMID: 37551697 PMCID: PMC10411273 DOI: 10.1177/03000605231189366] [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] [Received: 04/20/2023] [Accepted: 07/05/2023] [Indexed: 08/09/2023] Open
Abstract
Aggressive angiomyxoma is a rare benign tumor with diverse clinical and radiologic presentations, making its differential diagnosis challenging. Here we report the second recurrence of an aggressive angiomyxoma of the vulva in a 33-year-old woman who presented with pain and swelling. Clinical examination revealed a well-circumscribed large palpable mass extending from the inferior part of the right labia major into the right side of the gluteus. A second recurrence of aggressive angiomyxoma was suspected based on the patient's history, clinical examination, and magnetic resonance imaging report, and the patient underwent surgical resection with a negative margin. Histological evaluation of the extracted lesion confirmed the diagnosis of aggressive angiomyxoma. At the 1-year follow-up, the patient was recurrence-free. The present report urges more awareness regarding the aggressiveness of angiomyxoma of the vulva. Closer attention should be given to margin-free removal of such tumors, and patients should be routinely followed up for at least 2 years postoperatively for early diagnosis of recurrence, thereby reducing the risk of morbidity.
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Affiliation(s)
- Soheila Aminimoghaddam
- Department of Obstetrics and Gynecology, Firoozgar Hospital, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
| | - Niloufar Sarchami
- Department of Obstetrics and Gynecology, Firoozgar Hospital, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
| | - Sarah Sadat Mahboub
- Department of Obstetrics and Gynecology, Firoozgar Hospital, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
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Aggressive Angiomyxoma of the Vulva: Which Is the Best Management Strategy? Description of a Case Report and Review of Literature of the Last Ten Years. J Clin Med 2023; 12:jcm12051726. [PMID: 36902513 PMCID: PMC10003322 DOI: 10.3390/jcm12051726] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2022] [Revised: 01/23/2023] [Accepted: 02/17/2023] [Indexed: 02/24/2023] Open
Abstract
BACKGROUND Aggressive angiomyxoma is a rare mesenchymal tumour of the genital tract with a high incidence in women of reproductive age. The aim of our work is to understand which is the best management strategy for this condition, starting from the description of a rare case report up to the performance of a narrative review of the literature. METHODS We report a case of a 46-year-old woman who came to our attention because of the growth of a 10-centimetre pedunculated, non-soft, non-tender mass of the left labium majus. She underwent surgical excision, and the histologic diagnosis was aggressive angiomyxoma. Due to a lack of tumour-free margins, radicalization surgery was carried out after three months. A review of the literature of the last ten years was performed following the PRISMA statement on MEDLINE (PubMed). We obtained data from twenty-five studies describing thirty-three cases. RESULTS Aggressive angiomyxoma is characterized by a high recurrence rate of between 36 and 72% after surgery. There is no universal consensus about hormonal therapy, and most studies (85%) describe surgical excision followed by only clinical and radiological follow-up. CONCLUSION Wide surgical excision is the gold-standard treatment for aggressive angiomyxoma, succeeded by either clinical or radiological (ultrasound or MRI) follow-up.
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Cingoz M, Kilic A, Acar A, Tosun İ, Aksoy DO, Cingoz E. Radiologic imaging findings of pelvic aggressive angiomyxoma correlated with surgical and pathological features. THE EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE 2021. [DOI: 10.1186/s43055-021-00642-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Aggressive angiomyxoma is an infrequent benign tumor that usually occurs in the pelvic region. Pelvic masses have variety of differential diagnosis but some featured findings should prompt the diagnosis of aggressive angiomyxoma by the radiologist.
Case presentation
A 40-year-old female patient presented with a two-year history of perineal swelling. Radiological examination including gray scale and color Doppler ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) was consistent with the diagnosis of aggressive angiomyxoma. The patient underwent surgical operation that ensures total resection of the tumor.
Conclusion
In the case of extensive pelvic soft tissue mass with characteristic imaging findings, the radiologists should take the diagnosis of aggressive angiomyxoma into consideration.
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Malukani K, Varma AV, Choudhary D, Dosi S. Aggressive angiomyxoma in pregnancy: A rare and commonly misdiagnosed entity. J Lab Physicians 2020; 10:245-247. [PMID: 29692596 PMCID: PMC5896197 DOI: 10.4103/jlp.jlp_179_17] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/03/2022] Open
Abstract
Aggressive angiomyxoma (AAM) is an uncommon mesenchymal tumor that predominantly involves the pelvis and perineum of young females. It is often clinically mistaken for more common superficial lesions such as vaginal cysts, labial cysts, and lipomas. A review of the medical literature reveals very few cases of AAM reported in pregnancy. We describe a rare case of AAM in pregnancy, clinically misdiagnosed as prolapsed cervical fibroid.
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Affiliation(s)
- Kamal Malukani
- Department of Pathology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India
| | - Amit V Varma
- Department of Pathology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India
| | - Devashish Choudhary
- Department of Pathology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India
| | - Shilpi Dosi
- Department of Pathology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India
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Abduljabbar A, Wazzan M. Recurrent aggressive angiomyxoma presented with perianal mass and typical imaging swirl sign. Int J Surg Case Rep 2020; 72:486-489. [PMID: 32698271 PMCID: PMC7322231 DOI: 10.1016/j.ijscr.2020.06.050] [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: 04/01/2019] [Revised: 05/23/2020] [Accepted: 06/03/2020] [Indexed: 11/18/2022] Open
Abstract
Silent growing pelvic mass in middle age women with tendency to insinuate within the pelvis is commonly seen in aggressive angiomyxoma. The presence of swirl sign with the mass and striated appearance in T1 weighted and T2 weighted images are commonly seen with this tumour. Surgical intervention, radiotherapy, hormonal therapy and chemotherapy are valid treatment options. Introduction Aggressive angiomyxoma is a rare pelvic neoplasm that arises commonly in middle aged women and usually has delayed presentation due to its typically silent and slow growing behavior. Clinical presentation A 47-year-old woman presented at our hospital with recurrent attacks of pelvic pain and a perianal bulge. Her past medical history was relevant due to previous en bloc resection of a right ovarian angiomyxoma. Current cross sectional imaging, including computed tomography and magnetic resonance imaging, was performed and showed evidence of local recurrence. Various available treatment options were offered to the patient. Discussion The imaging features of the aggressive angiomyxoma were peculiar and showed a swirl and lamellate appearance in T1 and T2 weighted images. Conclusion A locally aggressive tumor with benign histopathology and swirl sign in imaging is highly suggestive of aggressive angiomyxoma.
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Affiliation(s)
- Ahmed Abduljabbar
- King Abdul Aziz University, Radiology Department, Faculty of Medicine, P.O. Box 80215. Jeddah, 21589, Jeddah, Saudi Arabia.
| | - Mohammad Wazzan
- King Abdul Aziz University, Radiology Department, Faculty of Medicine, P.O. Box 80215. Jeddah, 21589, Jeddah, Saudi Arabia
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Canda MT, Erkan MM, Caglayan LD, Demir N. Angiomyofibroblastoma of the left broad ligament mimicking ovarian tumour. J OBSTET GYNAECOL 2019; 41:316-318. [PMID: 31847648 DOI: 10.1080/01443615.2019.1692803] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/25/2022]
Affiliation(s)
| | | | | | - Namık Demir
- Obstetrics and Gynecology Unit, Kent Hospital, Izmir, Turkey
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8
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Zhao CY, Su N, Jiang YX, Yang M. Application of ultrasound in aggressive angiomyxoma: Eight case reports and review of literature. World J Clin Cases 2018; 6:811-819. [PMID: 30510948 PMCID: PMC6264985 DOI: 10.12998/wjcc.v6.i14.811] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/22/2018] [Revised: 10/16/2018] [Accepted: 10/22/2018] [Indexed: 02/05/2023] Open
Abstract
Aggressive angiomyxoma (AAM) is a rare tumour that often occurs in soft tissues of the female genital tract. Eight cases of AAM are reported in this article, and the clinical features and ultrasound and magnetic resonance imaging (MRI) results of the eight cases are reviewed and summarized. The main complaints of all the patients were palpable and painless masses in the vulva or scrotum. The lesions were mainly located in the vulva, pelvis, and perineal region, with a large scope of involvement. The sonographic features of AAM were characteristic. On sonography, all of the masses were of irregular shape and showed hypoechogenicity, with a heterogeneous inner echotexture. Intratumoural and peritumoural blood flows were detected by colour Doppler imaging. On real-time ultrasonic imaging, prominent deformation of the lesions was observed by compressing the masses with the probe. Some special imaging features were also revealed, including a laminated or swirled appearance of inner echogenicity, and a finger-like or tongue-like growth pattern. On MRI imaging, the lesions showed intermediate-intensity signals and intermediate to high-intensity signals on TI-weighted and T2-weighted sequences. A rapid and uneven enhancement pattern was demonstrated. After the comparison of sonographic features with MRI and pathological findings, we found the relevance of the ultrasonographic characteristics with MRI and histological features of AAM. Ultrasound can be a valuable imaging method for the preoperative diagnosis, evaluation of scope, and follow-up of AAM.
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Affiliation(s)
- Chen-Yang Zhao
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital, Beijing 100730, China
| | - Na Su
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital, Beijing 100730, China
| | - Yu-Xin Jiang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital, Beijing 100730, China
| | - Meng Yang
- Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College Hospital, Beijing 100730, China
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Robotic extralevator excision of a retrorectal giant aggressive angiomyxoma. Obstet Gynecol Sci 2018; 61:693-697. [PMID: 30474017 PMCID: PMC6236091 DOI: 10.5468/ogs.2018.61.6.693] [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: 10/11/2017] [Revised: 03/06/2018] [Accepted: 03/07/2018] [Indexed: 12/17/2022] Open
Abstract
Aggressive angiomyxoma (AA) is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. For the complete excision of retrorectal tumors, with extension through the levator muscle into the ischioanal space, open anterior and posterior approaches are typically required. Herein, we report our experience with robotic excision of a giant presacral AA with extralevator extension into the ischioanal space and extraction via Pfannenstiel incision, which we found to be technically feasible, efficacious, and safe to perform. Mayo Clinic Institutional Review Board exemption status was obtained for this study.
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Wong YP, Tan GC, Ng PF. Cervical angiomyofibroblastoma: a case report and review of literature. J OBSTET GYNAECOL 2017; 37:681-682. [PMID: 28604180 DOI: 10.1080/01443615.2017.1281236] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Affiliation(s)
- Yin Ping Wong
- a Department of Pathology , Universiti Kebangsaan Malaysia Medical Centre , Kuala Lumpur , Malaysia
| | - Geok Chin Tan
- a Department of Pathology , Universiti Kebangsaan Malaysia Medical Centre , Kuala Lumpur , Malaysia
| | - Pui Foong Ng
- a Department of Pathology , Universiti Kebangsaan Malaysia Medical Centre , Kuala Lumpur , Malaysia
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Tan GHC, Ng D, Hennedige T, Teo M. A solitary fibrous tumour mimicking an aggressive angiomyxoma/liposarcoma. BMJ Case Rep 2017; 2017:bcr-2016-218202. [PMID: 28476856 DOI: 10.1136/bcr-2016-218202] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
We present a case of a solitary fibrous tumour (SFT) resembling an aggressive angiomyxoma/liposarcoma on radiological imaging, causing significant diagnostic challenges preoperatively. A 76-year-old male was incidentally found to have a large pelvic mass on a CT scan. Further evaluation with an MRI scan confirmed a presacral mass containing fat and soft tissue components. It was inseparable from the sacrococcygeal spine, rectal serosa and the posterior wall of the urinary bladder, but no evidence of invasion was seen. A prominent vascular pedicle arising from the epidural vasculature was also noted. Differentials discussed at the multidisciplinary tumour board were an aggressive angiomyxoma versus a liposarcoma. The patient underwent wide resection of the pelvic tumour, anterior resection and end colostomy. Intraoperatively, a large 20 cm pelvic mass involving the sigmoid mesocolon and presacral fascia was found. Final histology reported an SFT with extensive adipocytic metaplasia.
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Affiliation(s)
- Grace Hwei Ching Tan
- Division of Surgical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Deanna Ng
- Division of Surgical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Tiffany Hennedige
- Division of Surgical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Melissa Teo
- Division of Surgical Oncology, National Cancer Centre Singapore, Singapore, Singapore
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Aggressive Angiomyxoma of the Vulva Mimicking Clitoromegaly in a Young Child. Urology 2017; 101:142-144. [DOI: 10.1016/j.urology.2016.10.034] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2016] [Revised: 10/19/2016] [Accepted: 10/25/2016] [Indexed: 11/18/2022]
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Al-Umairi RS, Kamona A, Al-Busaidi FM. Aggressive Angiomyxoma of the Pelvis and Perineum: A Case Report and Literature Review. Oman Med J 2016; 31:456-458. [PMID: 27974964 DOI: 10.5001/omj.2016.92] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/13/2023] Open
Abstract
Aggressive angiomyxoma (AA) is a rare mesenchymal tumor that usually occurs in the pelvis and perineum of young females. AA can simulate Bartholin's gland cyst, abscess, lipoma, simple labial cyst, or other pelvic soft tissue tumors. Cross-sectional imaging, particularly magnetic resonance imaging (MRI), plays an essential role in the diagnosis and management of AA. We report a case of AA in a 38-year-old woman with typical MRI features. We also present the findings of a literature review on the radiological features of this disease.
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Affiliation(s)
| | - Atheel Kamona
- Department of Radiology, Royal Hospital, Muscat, Oman
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Sozutek A, Irkorucu O, Reyhan E, Yener K, Besen AA, Erdogan KE, Gonlusen G, Doran F. A Giant Aggressive Angiomyxoma of the Pelvis Misdiagnosed as Incarcerated Femoral Hernia: A Case Report and Review of the Literature. Case Rep Surg 2016; 2016:9256749. [PMID: 27274880 PMCID: PMC4871960 DOI: 10.1155/2016/9256749] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2016] [Accepted: 04/19/2016] [Indexed: 11/18/2022] Open
Abstract
Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions. AA is a locally infiltrative slow growing tumor with a marked tendency to local recurrence. Painless swelling located around the genitofemoral region is the common symptom; thus, it is often misdiagnosed as a gynecological malignancy or a groin hernia. A 35-year-old female patient who previously underwent surgery for left femoral hernia operation resulting in surgical failure was reoperated for a giant AA located in the pelvis. The tumor was completely excised with free margins. Histopathologic examination revealed an AA. The tumor size was measured as 24 × 12 × 6 cm with a weight of 4.2 kg. Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor. S100, MUC4, CD34, and SMA were negative in the tumor cells. AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The principle treatment should be complete surgical excision with tumor-free margins. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence.
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Affiliation(s)
- Alper Sozutek
- Adana Numune Training and Research Hospital, Department of General Surgery, Division of Gastroenterological Surgery, Adana, Turkey
| | - Oktay Irkorucu
- Adana Numune Training and Research Hospital, Department of General Surgery, Division of Gastroenterological Surgery, Adana, Turkey
| | - Enver Reyhan
- Adana Numune Training and Research Hospital, Department of General Surgery, Division of Gastroenterological Surgery, Adana, Turkey
| | - Kemal Yener
- Adana Numune Training and Research Hospital, Department of General Surgery, Division of Gastroenterological Surgery, Adana, Turkey
| | - Ali Ayberk Besen
- Adana Numune Training and Research Hospital, Department of Medical Oncology, Adana, Turkey
| | | | - Gulfiliz Gonlusen
- Department of Pathology, Cukurova University Medical Faculty, Adana, Turkey
| | - Figen Doran
- Department of Pathology, Cukurova University Medical Faculty, Adana, Turkey
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Aggressive angiomyxomas: a comprehensive imaging review with clinical and histopathologic correlation. AJR Am J Roentgenol 2014; 202:1171-8. [PMID: 24848813 DOI: 10.2214/ajr.13.11668] [Citation(s) in RCA: 42] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
OBJECTIVE Aggressive angiomyxomas are rare infiltrative mesenchymal neoplasms that commonly recur locally. The purpose of this study was to conduct a retrospective review of imaging findings of aggressive angiomyxomas with clinicopathologic correlation in 16 patients. MATERIALS AND METHODS CT and MRI studies and clinical data of 16 patients with histopathologic evidence of aggressive angiomyxoma who had been referred to our institutions from January 2002 through January 2012 were retrospectively reviewed. The tumors were evaluated with respect to location, morphology, attenuation or signal intensity, and enhancement characteristics. RESULTS The most common location was the pelvis and perineum with the mass on either side of the pelvic diaphragm (12/16, 75%). The characteristic "laminated" appearance was seen in 10 of 12 patients on MRI. Aggressive angiomyxomas showed only mild diffusion restriction and mild (18)F-FDG avidity in both of the two patients who underwent DWI and PET/CT, in keeping with histologic low-mitotic activity. Imaging features, such as collateral vessels and fingerlike growth pattern, were seen in seven of 16 (44%) aggressive angiomyxomas. Internal cystic degeneration was seen in three of 16 (19%) aggressive angiomyxomas. CONCLUSION The finding of a large multicompartmental tumor with a characteristic internal laminated morphology or extension on either side of the pelvic diaphragm should alert the radiologist to the possible diagnosis of aggressive angiomyxoma. Imaging features, such as large peripheral vessels and cystic degeneration are less common, but presence of these features in the background of laminated morphology should not deter the radiologist from suggesting a diagnosis of aggressive angiomyxoma.
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Abstract
OBJECTIVE Aggressive angiomyxoma (AA) is found mainly in the pelvis and perineum, with a female-to-male ratio of approximately 6:1. Although it is a slow growing tumor, AA has a marked tendency to local recurrence with a low metastasis capacity. The study aimed to describe a case of vulvar angiomyxoma recurred almost 20 years after its initial surgery. MATERIALS AND METHODS We report the case of a 57-year-old gravida 5 para 4 woman with vulvar AA arising from the left labium majus, which recurred 20 years after initial surgery. There was a nontender, solid, mobile mass on the left vulva, which was 25 x 30 cm on physical examination. A pelvic computed tomographic scan showed a mass measuring 26 x 10 x 14 cm originating from left vulvar region, which has a fatty tissue density. RESULT Under general anesthesia, total excision of the tumor was performed. Macroscopically, the tumor weighed 723 g and measured 33 x 20 x 10 cm. The histopathological examination of the specimen revealed a myxoid tumor with sparse infiltrates of spindle-shaped to stellate cells and vessels of varying sizes. The final histopathological diagnosis was AA. CONCLUSIONS Aggressive angiomyxoma may form extremely large tumors, and recurrence is not rare even many years after primary surgery.
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Angiomyxome agressif du pelvis et du périnée : à propos d’un cas. IMAGERIE DE LA FEMME 2012. [DOI: 10.1016/j.femme.2012.08.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
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Foust-Wright C, Allen A, Shobeiri SA. Periurethral aggressive angiomyxoma: a case report. Int Urogynecol J 2012; 24:877-80. [PMID: 22790490 DOI: 10.1007/s00192-012-1867-z] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/11/2012] [Accepted: 06/16/2012] [Indexed: 12/16/2022]
Abstract
Aggressive angiomyxoma is a rare soft tissue tumor of the pelvis. Notorious for its locally infiltrative behavior, this tumor is identified by pathological appearance. Grossly gelatinous, spindle cells widely separated by collagen fibrils with vascular components can be microscopically visualized. Wide local excision is the treatment of choice. A 19-year-old woman presented with a periurethral mass that extended beyond the hymen with Valsalva. With imaging, the differential was narrowed to a soft tissue mass. Surgical excision was performed and histopathological findings were consistent with the diagnosis of aggressive angiomyxoma. Twenty-four months later the patient remains recurrence free. The typical findings of aggressive angiomyxoma are highlighted as well as the novel presentation as a periurethral mass. Histological and radiological findings are reviewed as well as current treatment options.
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Affiliation(s)
- Caroline Foust-Wright
- Department of Obstetrics and Gynecology, Maine Medical Center, 22 Bramhall Street, Portland, ME, USA.
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Aggressive angiomyxoma of vulva and vagina: a series of three cases and review of literature. Arch Gynecol Obstet 2010; 283:1145-8. [PMID: 20596716 DOI: 10.1007/s00404-010-1575-1] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2010] [Accepted: 06/17/2010] [Indexed: 10/19/2022]
Abstract
BACKGROUND Aggressive angiomyxoma is a rare locally aggressive mesenchymal tumor of unknown etiology usually affecting the vulva, perianal region, buttocks or pelvis of reproductive age women. MATERIAL A series of three cases, one each of vaginal, vulval and labial angiomyxoma is being presented. The etiology, presentation, diagnosis and management of this rare genital tumor are outlined. CONCLUSION Angiomyxoma of vulva and vagina refers to a rare disease; diagnosis is not at all clinical, thus, cases presenting as bartholin cyst, benign vulval lesions and vaginal wall cysts should have complete radiological work up before excision, as pre-diagnosis can change the treatment modality and prognosis of patient.
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