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Saito A, Komiyama S, Nagashima M, Kugimiya T, Mukai T. Recurrent Aggressive Angiomyxoma That Responded to the Gonadotropin-Releasing Hormone (GnRH) Antagonist Relugolix. Cureus 2025; 17:e81270. [PMID: 40291187 PMCID: PMC12033977 DOI: 10.7759/cureus.81270] [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] [Accepted: 03/27/2025] [Indexed: 04/30/2025] Open
Abstract
Aggressive angiomyxoma (AAM) is a rare benign mesenchymal tumor occurring in the vulva, vagina, and pelvis; however, it is difficult to treat because it often recurs locally. Since AAM expresses the estrogen receptor (ER) and progesterone receptor (PgR), various hormone therapies have been reportedly effective. Regardless, the efficacy of gonadotropin-releasing hormone (GnRH) antagonists has not yet been reported. We encountered a case of relapsed AAM in which the GnRH antagonist relugolix was highly effective. Although the patient initially underwent surgical resection for the AAM, recurrence occurred 11 months postoperatively. Since the immunohistochemical expression of ER and PgR was positive, relugolix (40 mg/day) was orally administered daily for six months, resulting in significant tumor regression (8.7 cm to 4.6 cm). This is the first report of successful GnRH antagonist treatment for AAM. Although AAM may be difficult to treat, GnRH antagonists are promising candidates for hormone therapy.
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Affiliation(s)
- Arisa Saito
- Department of Obstetrics and Gynecology, Toho University Omori Medical Center, Tokyo, JPN
| | - Shinichi Komiyama
- Department of Obstetrics and Gynecology, Toho University Omori Medical Center, Tokyo, JPN
| | - Masaru Nagashima
- Department of Obstetrics and Gynecology, Toho University Omori Medical Center, Tokyo, JPN
| | - Tsuyoki Kugimiya
- Department of Obstetrics and Gynecology, Toho University Omori Medical Center, Tokyo, JPN
| | - Takahumi Mukai
- Department of Obstetrics and Gynecology, Toho University Omori Medical Center, Tokyo, JPN
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Yu BR, Choi WK, Cho DH, Lee NR. Aggressive angiomyxoma of the vagina: A case report and literature review. Medicine (Baltimore) 2025; 104:e41287. [PMID: 39854765 PMCID: PMC11771726 DOI: 10.1097/md.0000000000041287] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2024] [Revised: 12/20/2024] [Accepted: 01/03/2025] [Indexed: 01/26/2025] Open
Abstract
RATIONALE Aggressive angiomyxoma (AAM) is an exceptionally rare mesenchymal tumor that predominantly manifests in the female genital organs during the reproductive age. Its rarity alone makes it a fascinating subject for study. The diagnosis of AAM necessitates differentiation from other benign or mesenchymal tumors and can be confirmed through immunohistochemistry (IHC) staining. Surgical resection is the primary treatment, and adjuvant treatment can be used as hormonal therapy with gonadotropin-releasing hormone agonists, selective estrogen receptor modulators, and aromatase inhibitors. PATIENT CONCERNS A 44-year-old premenopausal Korean woman presented with a growing perineal mass and frequent urination. DIAGNOSES Histopathological findings confirmed AAM, with IHC staining showing estrogen receptor, progesterone receptor, actin and desmin positivity, and CD34 and S100 negativity. INTERVENTIONS The mass was excised transvaginally under general anesthesia. OUTCOMES The patient showed no signs of recurrence 6 months postoperatively. LESSONS AAM in the vagina is a rare tumor that requires differential diagnosis using IHC staining. Previously, we reviewed reported cases and confirmed the feasibility and effectiveness of surgery as the main treatment. This might reassure us about the potential successful treatment of AAM. Adjuvant hormonal therapy with gonadotropin-releasing hormone agonists, selective estrogen receptor modulators, and aromatase inhibitors can further reduce the risk of recurrence.
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Affiliation(s)
- Bo Ram Yu
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Republic of Korea
| | - Won Ku Choi
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Republic of Korea
- Research Institute of Clinical Medicine, Jeonbuk National University, Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju, Republic of Korea
| | - Dong Hyu Cho
- Department of Obstetrics and Gynecology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Republic of Korea
- Research Institute of Clinical Medicine, Jeonbuk National University, Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju, Republic of Korea
| | - Na-Ri Lee
- Department of Internal Medicine, Division of Hematology and Oncology, Jeonbuk National University Hospital, Jeonbuk National University Medical School, Jeonju, Republic of Korea
- Research Institute of Clinical Medicine, Jeonbuk National University, Biomedical Research Institute of Jeonbuk National University Hospital, Jeonju, Republic of Korea
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Qu H, Liu N, Liang H, Wang Y, Zhuang H, Li H. Aggressive angiomyxoma of female pelvis and perineum: Retrospective study of 17 cases. Eur J Obstet Gynecol Reprod Biol 2024; 298:165-170. [PMID: 38762952 DOI: 10.1016/j.ejogrb.2024.05.012] [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: 03/05/2023] [Revised: 04/22/2024] [Accepted: 05/11/2024] [Indexed: 05/21/2024]
Abstract
OBJECTIVE Aggressive angiomyxoma is an uncommon mesenchymal neoplasm characterized by a high recurrence rate, usually observed in the lower genital tract of women during their reproductive age. STUDY DESIGN Seventeen cases of aggressive angiomyxoma confirmed by pathology from January 2007 to December 2021 in Beijing Chao-yang Hospital were included. We collected clinical data and summarized the clinical and immunohistochemical features. RESULTS All seventeen included patients were females, aged between 23 and 57 years (mean, 37.7 years; median, 42 years). Fourteen patients were newly diagnosed and three were recurrent. The tumors were located in vulva (58.8 %), vagina (23.5 %), buttock (11.8 %), and cervix (5.9 %). The tumors size were 2 to 15 cm in greatest dimension (mean 8 ± 4.4 cm, median 6 cm). Follow-up data was available for nine patients, which ranged from 25 to 124 months (mean, 82 months; median, 80 months). At the end of follow-up, no other recurrence or metastasis was reported. Immunohistochemical analysis showed immunoreactive for estrogen (10/11) and progesterone (8/11) receptor, desmin (6/8), smooth muscle actin (4/10), and vimentin (4/4), S-100 (1/8) and CD34 (1/7). The Ki67 level was less than 5 % in five cases. CONCLUSIONS AAM is a hormone-sensitive, distinct rare mesenchymal neoplasm with high incidence of local recurrence. Surgery is the preferred treatment, with complete resection being an essential prerequisite for minimizing the risk of recurrence.
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Affiliation(s)
- Hong Qu
- Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Na Liu
- Department of Obstetrics and Gynecology, Beijing Sixth Hospital, Beijing, China
| | - Huamao Liang
- Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, China
| | - Ying Wang
- Department of Pathology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Huiyu Zhuang
- Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China
| | - Hua Li
- Department of Obstetrics and Gynecology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, China.
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Zhang L, Liu R, Peng J. Misleading clinical and imaging features in atypical aggressive angiomyxoma of the female vulvovaginal or perianal region: report of three cases and review of the literature. Front Oncol 2024; 14:1373607. [PMID: 38590660 PMCID: PMC10999629 DOI: 10.3389/fonc.2024.1373607] [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: 01/20/2024] [Accepted: 03/11/2024] [Indexed: 04/10/2024] Open
Abstract
Objectives Aggressive (deep) angiomyxoma (AAM) is a rare mesenchymal tumor that typically originates from the vulvovaginal region, perineum, and pelvis in adult women. The objective of this case report and literature review is to comprehensively analyze the clinical, imaging, and pathological characteristics of atypical AAM in the female lower genital tract and pelvic floor in order to minimize preoperative misdiagnosis or missed diagnosis and ultimately optimize the clinical management strategy. Methods The data of three cases with atypical AAM, which demonstrate similarities with other lesions observed in the female lower genital tract over the past 1.5 years, were retrospectively described. This description included clinical management, images and reports of ultrasonography (US) and magnetic resonance imaging (MRI), clinicopathological features, follow-up, and outcomes. In the Discussion section, a review of the literature on MEDLINE (PubMed) and Web of Science from the past 50 years was conducted. Results The three cases all underwent preoperative ultrasonography, and two of them also underwent preoperative MRI examination. Complete resection of the lesions was performed in all three cases, followed by postoperative pathological examination. The histopathology of these three cases revealed invasive angiomyxoma, as confirmed by immunohistochemical staining, which demonstrated positive expression of desmin, vimentin, estrogen, and progesterone receptors. The patients experienced a smooth postoperative recovery. Ultrasound had a diagnostic accuracy rate of 100% (3/3) for locating and determining the extent of the lesions; however, its specific diagnostic accuracy rate for identifying the pathological type was only 33% (1/3). In contrast, MRI had a diagnostic accuracy rate of 100% (2/2) for locating and determining the extent of lesions but did not show any specific diagnostic accuracy for identifying the pathological types. Conclusions Our findings indicate that even if a vulvovaginal lesion presents with a superficial location, small size, limited scope, and regular shape, suspicion of atypical AAM should arise when palpation reveals toughness, tensility, and deformability under pressure. US reveals a well-defined hypoechoic to anechoic mass with uniformly distributed coarse dot echoes, with or without detectable intratumoral blood flow signal. MRI shows prolonged T1 and T2 signals with inhomogeneous enhancement and evident diffusion restriction on diffusion-weighted imaging (DWI).
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Affiliation(s)
- Ling Zhang
- Department of Obstetrics and Gynecology, National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Rong Liu
- Department of Obstetrics and Gynecology, National Clinical Research Center for Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
- Key Laboratory of Cancer Invasion and Metastasis (Ministry of Education), Hubei Key Laboratory of Tumor Invasion and Metastasis, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jian Peng
- Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Mayer JM, Spies SI, Mayer CK, Zubler C, Loucas R, Holzbach T. How to Treat a Cyclist's Nodule?-Introduction of a Novel, ICG-Assisted Approach. J Clin Med 2024; 13:1124. [PMID: 38398438 PMCID: PMC10889187 DOI: 10.3390/jcm13041124] [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: 12/27/2023] [Revised: 01/31/2024] [Accepted: 02/14/2024] [Indexed: 02/25/2024] Open
Abstract
BACKGROUND Perineal nodular induration (PNI) is a benign proliferation of the soft tissue in the perineal region that is associated with saddle sports, especially road cycling. The etiology has not been conclusively clarified; however, repeated microtrauma to the collagen and subcutaneous fat tissue by pressure, vibration and shear forces is considered a mechanical pathomechanism. In this context, chronic lymphedema resulting in the development of fibrous tissue has been suggested as an etiological pathway of PNI. The primary aim of this study was to introduce and elucidate a novel operative technique regarding PNI that is assisted by indocyanine green (ICG). In order to provide some context for this approach, we conducted a comprehensive review of the existing literature. This dual objective aimed to contribute to the existing body of knowledge while introducing an innovative surgical approach for managing PNI. METHODS We reviewed publications relating to PNI published between 1990 and 2023. In addition to the thorough review of the literature, we presented our novel surgical approach. We described how this elaborate approach for extensive cases of PNI involves surgical excision combined with tissue doubling and intraoperative ICG visualization for exact lymphatic vessel obliteration to minimize the risk of recurrence based on the presumed context of lymphatic congestion. RESULTS The literature research yielded 16 PubMed articles encompassing 23 cases of perineal nodular induration (PNI) or cyclist's nodule. Of these, 9 cases involved females, and 14 involved males. Conservative treatment was documented in 7 cases (30%), while surgical approaches were reported in 16 cases (70%). Notably, a limited number of articles focused on histopathological or radiological characteristics, with a shortage of structured reviews on surgical treatment options. Only two articles provided detailed insights into surgical techniques. Similarly to the two cases of surgical intervention identified in the literature research, the post-operative recovery in our ICG assisted surgical approach was prompt, meaning a return to cycling was possible six weeks after surgery. At the end of the observation period (twelve months after surgery), regular scar formation and no signs of recurrence were seen. CONCLUSION We hope that this article draws attention to the condition of PNI in times of increasing popularity of cycling as a sport. We aimed to contribute to the existing body of knowledge through our thorough review of the existing literature while introducing an innovative surgical approach for managing PNI. Due to the successful outcome, the combination of tissue doubling, intraoperative ICG visualization and postoperative negative wound therapy should be considered as a therapeutic strategy in cases of large PNI.
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Affiliation(s)
- Julius M. Mayer
- Department of Plastic and Hand Surgery, Inselspital, University Hospital Bern, 3010 Bern, Switzerland;
| | - Sophie I. Spies
- Department of Dermatology and Allergy, Technical University Munich, 80802 Munich, Germany
| | - Carla K. Mayer
- Department of Urology, Spital Thurgau, 8500 Frauenfeld, Switzerland
| | - Cédric Zubler
- Department of Plastic and Hand Surgery, Inselspital, University Hospital Bern, 3010 Bern, Switzerland;
| | - Rafael Loucas
- Department of Hand and Plastic Surgery, Spital Thurgau, 8500 Frauenfeld, Switzerland; (R.L.); (T.H.)
| | - Thomas Holzbach
- Department of Hand and Plastic Surgery, Spital Thurgau, 8500 Frauenfeld, Switzerland; (R.L.); (T.H.)
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Franza A, Gusmaroli E, Fabbroni C, Vigorito R, Pasquali S, Casali PG, Sanfilippo RG. Long-term disease stability with bicalutamide in a man with aggressive angiomyxoma: case report and state of art. Front Oncol 2024; 13:1260668. [PMID: 38298446 PMCID: PMC10829570 DOI: 10.3389/fonc.2023.1260668] [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: 07/18/2023] [Accepted: 12/26/2023] [Indexed: 02/02/2024] Open
Abstract
Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm, which is commonly diagnosed in females and located in the perineal and pelvic region. Tissue specimens of AA patients often show positivity for estrogen (ER) and progesterone receptors (PgR), while some cases of androgen receptor (AR) positivity have been reported in males. When feasible, surgical excision represent the most effective treatment of AA; however, when experiencing advanced or recurrent disease, local disease control could be achieved with systemic hormonal treatment. To date, evidence regarding AA management in male patients is scarce, and only a few cases have been reported in literature. Hereby, we describe the case of a 59-year-old-man suffering from perineal AA with positivity for androgen receptors (AR) showing a long-lasting disease stability during the treatment with an AR-blocking drug (bicalutamide). A literature review regarding the state of art of AA management with a particular look to male patients is also provided.
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Affiliation(s)
- Andrea Franza
- Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Eleonora Gusmaroli
- Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Chiara Fabbroni
- Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Raffaella Vigorito
- Department of Radiology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Sandro Pasquali
- Molecular Pharmacology Unit, Department of Applied Research and Technological Development, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
- Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - Paolo Giovanni Casali
- Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
- Department of Oncology and Hematology-Oncology, University of Milan, Milan, Italy
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Liu XJ, Su JH, Fu QZ, Liu Y. Aggressive angiomyxoma of the epididymis: A case report. World J Clin Cases 2023; 11:7214-7220. [PMID: 37946787 PMCID: PMC10631419 DOI: 10.12998/wjcc.v11.i29.7214] [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: 08/01/2023] [Revised: 09/05/2023] [Accepted: 09/18/2023] [Indexed: 10/13/2023] Open
Abstract
BACKGROUND Aggressive angiomyolipoma is an extremely rare benign mesenchymal tumor that was originally described as a locally recurrent mucinous spindle cell tumour. Aggressive angiomyolipoma originates from myofibroblasts, vascular smooth muscle cells, or fibroblasts, and displays various phenotypes of myofibroblasts and abnormal muscle arteries. Aggressive angiomyolipoma was first identified in 1983 and fewer than 50 male patients have been reported to date. It is an extremely rare mesenchymal tumour and often confused with other diseases. Patients with epididymal aggressive angiomyolipoma lack typical symptoms, most of which occur incidentally, although some patients may experience mild pain, discomfort, and swelling. Pain may be exacerbated by pressure from the mass. CASE SUMMARY A 66-year-old male was admitted to the hospital on January 14, 2022 with chief complaint of swelling in the left scrotum for one year. There was no apparent cause for the swelling. The patient did not consult with any doctor or receive any treatment for the swelling. The enlarged scrotum increased in size gradually until it reached approximately the size of a goose egg, and was accompanied by discomfort and swelling of the left cavity of the scrotum. The patient had no history of any testicular trauma, infection, or urinary tract infection. The patient urinated freely, 1-2 times at night, without urgency, dysuria (painful urination), or haematuria. There was no significant family history of malignancy. The patient underwent excision of the enlarged tumour and the left epididymis under general anaesthesia on January 18, 2022. Twelve months of follow-up revealed no recurrence. The patient was satisfied with the treatment. CONCLUSION Aggressive angiomyolipoma is extremely rare clinically and often confused with other diseases. The pathogenesis of aggressive angiomyolipoma is unclear and the clinical presentation is mostly a painless enlarged mass. The diagnosis of aggressive angiomyolipoma requires a combination of medical history, preoperative imaging such as computed tomography and magnetic resonance imaging, cytological examination and preoperative and postoperative pathological biopsy. The preferred treatment is surgery, with the possibility of a new alternative treatment option after hormonal therapy. Aggressive angiomyolipoma should be considered in the differential diagnosis of parametrial tumors of the male genital area that present as clinically significant masses. The high recurrence rate of aggressive angiomyolipoma may be related to incomplete tumor resection, and patients with aggressive angiomyolipoma are advised to undergo annual postoperative follow-up and imaging for recurrence.
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Affiliation(s)
- Xu-Jie Liu
- Department of Urology Surgery, Affiliated Zhongshan Hospital of Dalian University, Dalian 116001, Liaoning Province, China
| | - Jia-Hao Su
- Department of Urology Surgery, Affiliated Zhongshan Hospital of Dalian University, Dalian 116001, Liaoning Province, China
| | - Qi-Zhong Fu
- Department of Urology Surgery, Affiliated Zhongshan Hospital of Dalian University, Dalian 116001, Liaoning Province, China
| | - Ying Liu
- Department of Urology Surgery, Affiliated Zhongshan Hospital of Dalian University, Dalian 116001, Liaoning Province, China
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Sidhu MS, Jain K, Sood S, Kaur H. Localized aggressive angiomyxoma of axilla: A rare case report. J Cancer Res Ther 2023; 19:2101-2103. [PMID: 38376331 DOI: 10.4103/jcrt.jcrt_2286_21] [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: 12/17/2021] [Accepted: 12/29/2021] [Indexed: 02/21/2024]
Abstract
ABSTRACT Aggressive angiomyxoma (AAM) is a rare mesenchymal locally aggressive tumor arising in the pelvis and perineal region in females. On the other hand, extragenital AAMs are extremely rare, specifically in males. We are reporting male AAM which involves only axillary region along with its pathological features. To confirm it, immunohistochemistry (IHC) was done. In sum, pathological examination and IHC aid in distinguishing this lesion and help in differentiating it from other tumors of similar histology. Furthermore, it also aids in planning treatment.
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Affiliation(s)
| | - Kunal Jain
- Department of Medical Oncology, DMCH Cancer Center, Ludhiana, Punjab, India
| | - Sandhya Sood
- Department of Radiation Oncology, Ludhiana, Punjab, India
| | - Harpreet Kaur
- Department of Pathology, DMCH, Ludhiana, Punjab, India
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Cicogna S, Dellino M, Miano ST, Magazzino F, Domenici L, Pignata S, Mangili G, Cormio G. Aggressive Angiomyxoma of the Lower Female Genital Tract in Pregnancy: A Review of the MITO Rare Tumors Group. Cancers (Basel) 2023; 15:3403. [PMID: 37444513 DOI: 10.3390/cancers15133403] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/07/2023] [Revised: 06/23/2023] [Accepted: 06/24/2023] [Indexed: 07/15/2023] Open
Abstract
Deep (aggressive) angiomyxoma of the lower genital tract is a rare malignancy affecting women of reproductive age. Being a hormone-sensitive tumor, its growth is particularly benefitted during pregnancy. Surgical excision with complete resection is indicated, even if a wait-and-see approach can be considered until delivery, to avoid destructive surgeries. The mode of delivery is to be evaluated based on the location and size of the neoplasm; vaginal delivery is not contraindicated, as long as the tumor does not obstruct the birth canal. Positive surgical margins are the most important prognostic factor for recurrence. Adjuvant therapy with gonadotropin-releasing hormone analogues may be proposed after pregnancy, in the case of non-radical surgery. Despite the high local relapse rate, the outcomes for mother and child are favorable. Since recurrences can occur after many years, the patient should be included in long-term follow-up.
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Affiliation(s)
- Stefania Cicogna
- Institute for Maternal and Child Health-IRCCS "Burlo Garofolo", 34145 Trieste, Italy
| | - Miriam Dellino
- Department of Interdisciplinary Medicine (DIM), University of Bari "Aldo Moro", Piazza Giulio Cesare 11, 70124 Bari, Italy
| | - Salvatora Tindara Miano
- Complex Operating Unit of Oncology, Azienda Ospedaliera Universitaria Senese, 53100 Siena, 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
| | - 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", 7012 Bari, Italy
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HMGA2 Is a Useful Marker of Vulvovaginal Aggressive Angiomyxoma But May Be Positive in Other Mesenchymal Lesions at This Site. Int J Gynecol Pathol 2021; 40:185-189. [PMID: 32897956 DOI: 10.1097/pgp.0000000000000689] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
Abstract
Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm occurring almost exclusively in the vulvovaginal region and which has a wide differential diagnosis. It has previously been suggested that the nuclear transcription factor HMGA2 is a useful marker of AA, although the number of studies is limited. We investigated HMGA2 immunoreactivity in a large series (n=284) of vulvovaginal mesenchymal lesions. HMGA2 nuclear staining was classified as diffuse (≥50%), focal (<50%), or negative. Of 38 cases of AA, 26 (68%) were positive; 77% (n=20) of these exhibited diffuse staining. Of the 41 smooth muscle tumors, 18 (44%) were positive with 16 (89%) exhibiting diffuse staining. 80 fibroepithelial stromal polyps were included and 15 (19%) were positive (8 diffuse; 7 focal). Most of the fibroepithelial stromal polyps that exhibited diffuse HMGA2 immunoreactivity were large and edematous. Occasional cases of a variety of other lesions were positive, including 1 of 30 superficial myofibroblastomas and 1 of 16 angiomyofibroblastomas. Cellular angiofibromas (n=12) and superficial angiomyxomas (n=6) were always negative. Our results confirm that HMGA2 is a useful marker of AA but a significant minority of cases are negative. The marker also lacks specificity, since a high percentage of smooth muscle tumors are positive, although these typically do not bear a close morphologic resemblance to AA. A novel observation in our study is positive staining of some fibroepithelial stromal polyps, particularly when large and edematous; these are particularly likely to be confused morphologically with AA and positive staining with HMGA2 represents a significant diagnostic pitfall.
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11
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Kooy J, Carlson V, Šačiragić L, Sawhney S, Nelson G. A case series of aggressive angiomyxoma: Using morphologic type and hormonal modification to tailor treatment. Gynecol Oncol Rep 2021; 36:100765. [PMID: 33912645 PMCID: PMC8066423 DOI: 10.1016/j.gore.2021.100765] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/21/2021] [Revised: 03/24/2021] [Accepted: 03/27/2021] [Indexed: 12/04/2022] Open
Abstract
Aggressive angiomyxoma tends toward two forms: deeply invasive and pedunculated. Anti-hormonal therapy can be an adjunct to aggressive angiomyxoma treatment. Pedunculated aggressive angiomyxoma can be considered for shorter follow-up. Aggressive angiomyxoma is a rare tumour type with a predilection for the female pelvis, high rates of estrogen and progesterone receptor positivity and local recurrence. A retrospective chart review of patients with aggressive angiomyxoma treated at 2 cancer centres is presented. Nine patients were identified with a mean age of 41. Five patients had deeply invasive tumours that were difficult to surgically resect. Four patients had pedunculated tumours with less complex resections. In only two cases was aggressive angiomyxoma considered before resection: one due to classic magnetic resonance imaging findings and one with a preoperative biopsy. Four patients had positive margins after resection, with only one having persistent disease. Two patients were treated with gonadotropin-releasing hormone (GnRH) agonists resulting in tumour regression in one and no recurrence in the other. In this case series, aggressive angiomyxoma presented in deeply invasive and pedunculated forms. Previously reported high rates of recurrence were not observed in this group, perhaps secondary to easier resection in the pedunculated forms. GnRH agonists were successfully used as adjuncts to surgery. Evidence in this case series could be used to provide tailored treatment to patients with aggressive angiomyxoma.
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Affiliation(s)
- Joni Kooy
- Tom Baker Cancer Center, Calgary, Alberta, Canada
| | | | - Lana Šačiragić
- Nova Scotia Health Authority, Halifax, Nova Scotia, Canada
| | | | - Gregg Nelson
- Tom Baker Cancer Center, Calgary, Alberta, Canada
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12
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Alosaimi AM, Al-Jifree HM, Alharbi SY, Algethami AS. Aggressive Angiomyxoma of the Posterior Wall of the Uterus. Cureus 2020; 12:e12023. [PMID: 33329983 PMCID: PMC7734890 DOI: 10.7759/cureus.12023] [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] [Indexed: 11/05/2022] Open
Abstract
Aggressive angiomyxoma (AA) is a rare benign mesenchymal tumor that usually arises in the vulvovaginal and perineal regions of premenopausal females. The treatment of choice is surgical excision. Hormonal therapy or radiotherapy have emerged as alternative forms of treatment but are indefinite. In this article, we report a case of aggressive angiomyxoma in the posterior wall of the uterus of a 35-year-old Saudi female patient. The clinical data, imaging, histopathology, treatment, and prognosis were analyzed, and related literatures were reviewed. The frequency of recurrence in these tumors emphasizes the importance of long-term follow-ups.
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Affiliation(s)
- Amal M Alosaimi
- Department of Oncology, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, SAU.,Department of Oncology, King Abdullah International Medical Research Center, Jeddah, SAU
| | - Hatim M Al-Jifree
- Department of Gynecologic Oncology, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, SAU.,Department of Oncology, King Abdullah International Medical Research Center, Jeddah, SAU.,Department of Oncology, Ministry of National Guard Health Affairs, Jeddah, SAU
| | - Suzan Y Alharbi
- Department of Oncology, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, SAU.,Department of Oncology, King Abdullah International Medical Research Center, Jeddah, SAU
| | - Amirah S Algethami
- Department of Oncology, College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, SAU.,Department of Oncology, King Abdullah International Medical Research Center, Jeddah, SAU
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13
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Chapel DB, Cipriani NA, Bennett JA. Mesenchymal lesions of the vulva. Semin Diagn Pathol 2020; 38:85-98. [PMID: 32958293 DOI: 10.1053/j.semdp.2020.09.003] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2020] [Accepted: 09/03/2020] [Indexed: 02/01/2023]
Abstract
Mesenchymal lesions of the vulva include site-specific entities limited to the lower genital tract, as well as a range of non-site-specific tumors that are more common at extragenital sites. Site-specific lesions include fibroepithelial stromal polyp, cellular angiofibroma, angiomyofibroblastoma, and aggressive angiomyxoma. Non-site-specific tumors that may occur in the vulva include those of smooth muscle, skeletal muscle, vascular, neural, adipocytic, and uncertain differentiation. This review discusses both site-specific and non-site-specific vulvar mesenchymal lesions including non-neoplastic proliferations, benign neoplasms, locally aggressive neoplasms with a predilection for local recurrence, neoplasms of indeterminate biologic potential, and frankly malignant neoplasms with a high risk of distant metastasis and death. Accurate diagnosis is essential for proper management, and is facilitated by correlation with clinical findings and targeted application of immunohistochemical and molecular studies.
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Affiliation(s)
- David B Chapel
- Division of Women's and Perinatal Pathology, Department of Pathology, Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA
| | - Nicole A Cipriani
- Department of Pathology, The University of Chicago, 5841 S. Maryland Ave, Chicago, IL 60637, USA
| | - Jennifer A Bennett
- Department of Pathology, The University of Chicago, 5841 S. Maryland Ave, Chicago, IL 60637, USA.
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14
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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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15
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Pannier D, Cordoba A, Ryckewaert T, Robin YM, Penel N. Hormonal therapies in uterine sarcomas, aggressive angiomyxoma, and desmoid-type fibromatosis. Crit Rev Oncol Hematol 2019; 143:62-66. [DOI: 10.1016/j.critrevonc.2019.08.007] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/18/2019] [Revised: 08/23/2019] [Accepted: 08/28/2019] [Indexed: 10/26/2022] Open
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16
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Serao A, Tiranti D, Ferraro M, Malinaric R, Re P, Calamaro P. Incidental finding of paratesticular aggressive angiomyxoma in a 72-year-old monorchid male. Urologia 2019; 87:194-198. [PMID: 31618126 DOI: 10.1177/0391560319881082] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022]
Abstract
Aggressive angiomyxoma is an uncommon mesenchymal neoplasm. It arises from soft tissue of pelvis and perineum in fertile females, occurring very rarely in males. Its name emphasizes the common local recurrence and infiltrative tendency, but usually it does not metastasize. Currently, the first line of therapy is surgical excision, achieving R0 surgical margins, and radiological follow-up. This case report describes an uncommon, incidental finding of paratesticular aggressive angiomyxoma in an asymptomatic, cryptorchid 72-year-old male patient.
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Affiliation(s)
- Armando Serao
- Department of Urology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio Arrigo, Alessandria, Italy
| | - Donatella Tiranti
- Department of Urology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio Arrigo, Alessandria, Italy
| | - Muriel Ferraro
- Department of Urology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio Arrigo, Alessandria, Italy
| | - Rafaela Malinaric
- Department of Urology, Ospedale Policlinico San Martino, University of Genoa, Genoa, Italy
| | - Paola Re
- Department of Pathology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio Arrigo, Alessandria, Italy
| | - Paola Calamaro
- Department of Pathology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio Arrigo, Alessandria, Italy
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17
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Secondaries of Synovial Sarcoma in Vagina: A Diagnostic Dilemma. INDIAN JOURNAL OF GYNECOLOGIC ONCOLOGY 2019. [DOI: 10.1007/s40944-019-0260-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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18
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PDGFB Rearrangements in Dermatofibrosarcoma Protuberans of the Vulva: A Study of 11 Cases Including Myxoid and Fibrosarcomatous Variants. Int J Gynecol Pathol 2019; 37:537-546. [PMID: 29140881 DOI: 10.1097/pgp.0000000000000472] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Dermatofibrosarcoma protuberans (DFSP) is a low-grade fibroblastic sarcoma that tends to arise in young to middle age adults and involve the trunk and proximal extremities. Rare examples of vulvar DFSP have been reported, including myxoid, myoid, and fibrosarcomatous variants, but detection of the characteristic t(17;22)(q22;q13) that produces COL1A1-PDGFB gene fusion has not been evaluated in a large series of primary vulvar tumors. The clinical, morphologic, immunohistochemical, and molecular cytogenetic features of 11 cases were examined. Patient age ranged from 29 to 75 yr (mean, 46 yr; median, 43 yr). Seven tumors were purely classic DFSP, 1 was purely myxoid DFSP and the remaining 3 had varying quantities of fibrosarcomatous DFSP. All cases of classic DFSP had diffuse expression of CD34 and low-level p53 immunoreactivity. Myxoid variants had strong, but reduced expression of CD34. Fibrosarcomatous DFSP showed focal CD34 expression and increased p53 reactivity. Nine of 11 tumors (82%) had rearrangement of PDGFB by fluorescence in situ hybridization. The 2 nonrearranged tumors were a classic DFSP and a myxoid DFSP with fibrosarcomatous transformation. Follow-up was available for 9 patients (82%) and ranged from 1 to 108 mo (mean, 30 mo; median, 21 mo). Eight patients had tumors with positive margins, one of which developed local recurrence after no further therapy. No patient developed metastasis. The high frequency of PDGFB rearrangement in vulvar DFSP provides a useful exploit in diagnostically challenging cases and genetic evidence of probable clinical response to targeted therapeutics in cases of locally advanced or metastatic tumors.
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19
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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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20
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Neyaz A, Husain N, Anand N, Srivastava P. Rare paratesticular aggressive angiomyxoma with negative oestrogen and progesterone receptors in a male patient. BMJ Case Rep 2018; 2018:bcr-2017-222164. [PMID: 29866663 DOI: 10.1136/bcr-2017-222164] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
Aggressive angiomyxoma (AAM) is a rare mesenchymal myxoid tumour localised to the pelvis and/or perineum in adult females in reproductive age group. AAM is very rare in males, with <50 cases described in literature, and involves scrotum, spermatic cord and perineum. It is slow growing, with a marked tendency for local recurrence after excision, but without metastatic potential. We present a rare case of a paratesticular AAM in a man aged 53 years. Tumour cells were immunoreactive for desmin, smooth muscle actin (SMA), vimentin, CD34 and were negative for S100. Unlike AAMs in females which express oestrogen receptor (ER) and/or progesterone receptor (PR) in >90% cases, the tumour cells in our case were negative for ER and PR, suggesting that the hypothesis that these markers play a role in tumour development and pathogenesis, does not apply in males. Androgen receptor positivity was noted in 2%-5% tumour cells.
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Affiliation(s)
- Azfar Neyaz
- Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Nuzhat Husain
- Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Nidhi Anand
- Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
| | - Pallavi Srivastava
- Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
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21
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Dehuri P, Gochhait D, Srinivas BH, Sistla SC. Aggressive Angiomyxoma in Males. J Clin Diagn Res 2017; 11:ED01-ED03. [PMID: 28764175 DOI: 10.7860/jcdr/2017/26738.9960] [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] [Received: 01/12/2017] [Accepted: 03/06/2017] [Indexed: 11/24/2022]
Abstract
Paratesticular aggressive angiomyxoma is a very rare tumour in males. Most of documented cases of aggressive angiomyxomas have been seen in genital, perineal and pelvic regions in women of child bearing age. We report two cases of aggressive angiomyxomas in males who presented with inguinal swellings. A globular mass with greyish white, glistening cut surface was received after excision of the mass. Microscopic examination revealed a paucicellular tumour comprising of spindle shaped cells along with vessels of varying calibre. The accompanying stroma was myxocollagenous. In addition there was evidence of fat infiltration in one of the cases. Immunohistochemical staining showed CD34, desmin, vimetin positivity and negative staining for S100, actin, Estrogen Receptors (ER) and Progesterone Receptors (PR). The microscopic and immunohistochemical features favoured the diagnosis of aggressive angiomyxoma. This report of angiomyxoma in two cases of males assumes great significance in view of the extreme rarity of the tumour in males and its locally infiltrative nature.
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Affiliation(s)
- Priyadarshini Dehuri
- Senior Resident, Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, Pondicherry, India
| | - Debasis Gochhait
- Assistant Professor, Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, Pondicherry, India
| | - B H Srinivas
- Associate Professor, Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, Pondicherry, India
| | - Sarath Chandra Sistla
- Professor, Department of Surgery, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, Pondicherry, India
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22
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Sun Y, Zhu L, Chang X, Chen J, Lang J. Clinicopathological Features and Treatment Analysis of Rare Aggressive Angiomyxoma of the Female Pelvis and Perineum - a Retrospective Study. Pathol Oncol Res 2016; 23:131-137. [PMID: 27571990 DOI: 10.1007/s12253-016-0109-y] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/16/2015] [Accepted: 08/24/2016] [Indexed: 02/07/2023]
Abstract
The study was to evaluate the clinicopathological features of aggressive angiomyxoma (AAM) of the female pelvis and perineum and its treatments. This was a retrospective study of female patients with AAM admitted to our hospital. Clinical and pathological data were analyzed, as well as the postsurgical follow-up. Median age at initial presentation was 41 years. Thirteen patients had lesions involving adjacent organs. Eighteen patients underwent complete tumor resection, while one patient underwent partial tumor resection. The tumors were soft in texture, pink in color, and had mucus on the surface. A microscopic examination revealed that the tumors were non-encapsulated, with spindle cells and stellate cells of almost identical size loosely distributed in the myxoid stroma, and vessels of different sizes and wall thicknesses. Immunohistochemistry indicated that AAMs were strongly positive for CD34 and smooth muscle actin, moderately positive for desmin, estrogen receptors and progesterone receptor, and mostly negative for S-100. After a median follow-up of 24 months, the recurrence rate was 33.3 %. Four recurrences were in patients with positive initial margins. AAM is a slow growing, locally invasive, benign tumor. Complete resection could lead to lower recurrence rate compared with incomplete resection. Follow-up is necessary for recurrent cases with repeated surgeries. The overall prognosis could be favorable.
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Affiliation(s)
- Yin Sun
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Beijing, 100000, China
| | - Lan Zhu
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Beijing, 100000, China.
| | - Xiaoyan Chang
- Department of Pathology, Peking Union Medical College Hospital, Beijing, 100000, China
| | - Jie Chen
- Department of Pathology, Peking Union Medical College Hospital, Beijing, 100000, China
| | - Jinghe Lang
- Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Beijing, 100000, China
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23
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Zangmo R, Kumar S, Singh N, Meena J. Aggressive Angiomyxoma of Vulva in Pregnancy: A Case Report. J Obstet Gynaecol India 2016; 66:610-612. [PMID: 27803522 DOI: 10.1007/s13224-016-0870-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/08/2016] [Accepted: 03/19/2016] [Indexed: 10/22/2022] Open
Affiliation(s)
- Rinchen Zangmo
- Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, India
| | - Sunesh Kumar
- Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, India
| | - Neeta Singh
- Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, India
| | - Jyoti Meena
- Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, New Delhi, India
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24
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Argiró R, Sacconi B, Iannarelli A, Collini P, Bezzi M. Perineal and pelvic aggressive angiomyxoma: spontaneous regression after hormonal replacement therapy withdrawal assessed by 3T magnetic resonance. BJR Case Rep 2015; 1:20150110. [PMID: 30363609 PMCID: PMC6180818 DOI: 10.1259/bjrcr.20150110] [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: 03/14/2015] [Revised: 06/10/2015] [Accepted: 06/16/2015] [Indexed: 11/15/2022] Open
Abstract
Aggressive angiomyxoma (AA) is a rare mesenchymal tumour which mainly arises in the soft tissue of the pelvis and perineum in women of reproductive age. AA usually shows an aggressive behaviour, with a high rate of incomplete surgical excision and post-surgical recurrence. Most cases of AA exhibit oestrogen and/or progesterone receptors; in these cases, maintenance of a hypo-oestrogenic state can be helpful in the medical management of this tumour. We describe a case of spontaneous reduction in size of an AA during a 6-month period of hormonal replacement therapy withdrawal, assessed by MRI using a 3T magnet.
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Affiliation(s)
- R Argiró
- Department of Radiological, Oncological and Anatomopathological Sciences-Radiology-Sapienza, University of Rome, Italy
| | - B Sacconi
- Department of Radiological, Oncological and Anatomopathological Sciences-Radiology-Sapienza, University of Rome, Italy
| | - A Iannarelli
- Department of Radiological, Oncological and Anatomopathological Sciences-Radiology-Sapienza, University of Rome, Italy
| | - P Collini
- Department of Pathology, Istituto Nazionale dei Tumori, Milano Italy
| | - M Bezzi
- Department of Radiological, Oncological and Anatomopathological Sciences-Radiology-Sapienza, University of Rome, Italy
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25
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Myxoid soft-tissue neoplasms: comprehensive update of the taxonomy and MRI features. AJR Am J Roentgenol 2015; 204:374-85. [PMID: 25615761 DOI: 10.2214/ajr.14.12888] [Citation(s) in RCA: 57] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
OBJECTIVE. The purpose of this article is to review the classification, clinical presentation, and histopathologic and MRI features of myxoid soft-tissue neoplasms. CONCLUSION. MRI is the modality of choice for characterization of myxoid soft-tissue tumors. A combination of imaging features (including certain characteristic signs), clinical features, and patient demographics can help the radiologist in coming to a specific diagnosis or in narrowing down the differential diagnoses.
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26
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Aggressive angiomyxoma of scrotum presenting as scrotal lymphedema in a case of postoperative carcinoma penis. Indian J Surg 2015; 77:170-1. [PMID: 25972685 DOI: 10.1007/s12262-015-1233-8] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2014] [Accepted: 01/12/2015] [Indexed: 10/24/2022] Open
Abstract
Aggressive angiomyxoma tumor is a rare tumor in males. We report a case of incidentally diagnosed aggressive angiomyxoma of scrotum presenting as scrotal lymphedema in a patient who underwent partial penectomy and radiotherapy for carcinoma penis.
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27
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Coxalgia? Call the gynecologist. J Minim Invasive Gynecol 2015; 22:663-7. [PMID: 25614345 DOI: 10.1016/j.jmig.2015.01.008] [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: 12/15/2014] [Revised: 01/06/2015] [Accepted: 01/08/2015] [Indexed: 11/23/2022]
Abstract
Cellular angiofibroma is a rare mesenchymal tumor that usually affects the vulvar region. It is commonly asymptomatic and treated with radical excision. We describe the first case of cellular angiofibroma located in the retroperitoneum presenting with coxalgia and laparoscopically treated. At the 6-month follow-up, the patient was coxalgia and disease free. Coxalgia is an unusual symptom for gynecologists; nevertheless, it is possible that several neoplasms may originate in the female pelvis, so any symptom requires careful evaluation. The laparoscopic approach may be effective in removing cellular angiofibroma, avoiding injuries to surrounding structures and unesthetic scarring and leading to a faster recovery.
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28
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Nayal B, Rao L, Rao ACK, Sharma S, Shenoy R. Extragenital aggressive angiomyxoma of the axilla and the chest wall. J Clin Diagn Res 2013; 7:718-20. [PMID: 23730656 DOI: 10.7860/jcdr/2013/5458.2891] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/14/2012] [Accepted: 01/22/2013] [Indexed: 11/24/2022]
Abstract
Aggressive angiomyxomas are uncommon mesenchymal tumours which most often arise in the perineal and the pelvic regions in women. Extragenital aggressive angiomyxonas are extremely rare. We are reporting a young male with an aggressive angiomyxoma which involved the axillary region and extended into the anterior chest wall, which demonstrated its characteristic histomorphological features. The diagnosis was confirmed by immunohistochemistry. A careful histological examination, along with immunohistochemistry, aids in diagnosing this lesion and differentiating it from tumours which have similar histologies.
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Affiliation(s)
- Bhavna Nayal
- Assistant Professor, Department of Pathology, KMC, Manipal University , Manipal, India
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29
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Abstract
Myxomas of the hand are rare tumours and the diameters are usually smaller than 2 cm. In this report we describe the biggest reported myxoma that localised on the dorsal hand and the comprehensively review of hand myxomas. This case is to our knowledge the first hormone-receptor-positive myxoma of the hand.
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Affiliation(s)
- Serdar Nasır
- Department of Plastic and Reconstructive Surgery, Süleyman Demirel University School of Medicine, Isparta, Turkey.
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30
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Affiliation(s)
- Q Liu
- Department of Minimally Invasive Gynecological Surgery, Specialist Clinic, Fourth Military Medical University, Xian, People's Republic of China.
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31
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Shaffer VO, Berho M, Rosen L. A case of intraluminal rectal aggressive angiomyxoma. Colorectal Dis 2012; 14:e78-9. [PMID: 21831173 DOI: 10.1111/j.1463-1318.2011.02757.x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Affiliation(s)
- V O Shaffer
- Department of Colorectal Surgery, Cleveland Clinic Florida, USA.
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32
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Aggressive Angiomyoma of the Female Genital Tract and Pelvis—Clinicopathologic Features With Immunohistochemical Analysis. Int J Gynecol Pathol 2011; 30:505-13. [DOI: 10.1097/pgp.0b013e318211d56c] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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33
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34
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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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HMGA2 is a Sensitive But Not Specific Immunohistochemical Marker of Vulvovaginal Aggressive Angiomyxoma. Am J Surg Pathol 2010; 34:1037-42. [DOI: 10.1097/pas.0b013e3181e32a11] [Citation(s) in RCA: 43] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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36
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Huge pedunculated angiomyofibroblastoma of the vulva. Int J Clin Oncol 2010; 15:201-5. [DOI: 10.1007/s10147-010-0026-0] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2009] [Accepted: 07/31/2009] [Indexed: 12/30/2022]
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37
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Myoepithelial neoplasms involving the vulva and vagina: report of 4 cases. Hum Pathol 2009; 40:1747-53. [DOI: 10.1016/j.humpath.2009.04.025] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/10/2009] [Revised: 04/07/2009] [Accepted: 04/09/2009] [Indexed: 11/22/2022]
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McCluggage WG. Immunohistochemical Markers of Value in the Diagnosis of Mesenchymal Lesions of the Female Genital Tract. Surg Pathol Clin 2009; 2:785-811. [PMID: 26838779 DOI: 10.1016/j.path.2009.08.017] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
Pure mesenchymal tumors may occur anywhere in the female genital tract, but they are most common in the uterine corpus, leiomyoma being by far the most prevalent. These tumors, and other uncommon mesenchymal lesions within the uterine corpus and elsewhere in the female genital tract, may result in several diagnostic problems. Morphology remains paramount and, in most cases, an unequivocal diagnosis can be made based on examination of the hematoxylin and eosin stained sections, combined with an appreciation of the clinical and gross pathologic features. In difficult cases, immunohistochemistry can significantly contribute to the final diagnosis. In this article, the immunohistochemistry of neoplastic and nonneoplastic mesenchymal lesions of the female genital tract is discussed site by site, concentrating on markers that are of value in diagnosis and in differential diagnosis. The immunophenotype of mixed epithelial and mesenchymal neoplasms and pure epithelial neoplasms with a component of spindle cells is discussed, where appropriate, because these can enter into the differential diagnosis of a pure mesenchymal lesion.
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Affiliation(s)
- W Glenn McCluggage
- Department of Pathology, Royal Group of Hospitals Trust, Grosvenor Road, Belfast BT12 6BA, Northern Ireland, UK.
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Dierickx I, Deraedt K, Poppe W, Verguts J. Aggressive angiomyxoma of the vulva: a case report and review of literature. Arch Gynecol Obstet 2008; 277:483-7. [PMID: 18214507 DOI: 10.1007/s00404-008-0561-3] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2007] [Accepted: 01/03/2008] [Indexed: 10/22/2022]
Abstract
BACKGROUND Aggressive angiomyxoma (AA) is a rare vulvovaginal mesenchymal neoplasm with a marked tendency to local recurrence but which usually does not metastasize. CASE REPORT We describe a case of AA in the left labium majus pudendi in a 47-year-old woman who underwent incomplete surgical excision. Follow-up 2years later revealed no recurrence. CONCLUSION In the past, most authors advocated wide excision even if genitourinary and digestive tract resection were necessary. These days, a less radical surgery is recommended, but the significance of hormonal treatment and/or radiation therapy is not clear yet. Further investigation is necessary.
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Affiliation(s)
- Inge Dierickx
- Department of Obstetrics and Gynaecology, Katholieke Universiteit Leuven, Leuven, Belgium
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41
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Luteinized Thecomas (Thecomatosis) of the Type Typically Associated With Sclerosing Peritonitis. Am J Surg Pathol 2008; 32:1273-90. [PMID: 18636018 DOI: 10.1097/pas.0b013e3181666a5f] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/07/2023]
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42
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Massive Vulval Edema Secondary to Obesity and Immobilization: A Potential Mimic of Aggressive Angiomyxoma. Int J Gynecol Pathol 2008; 27:447-52. [DOI: 10.1097/pgp.0b013e31816017a7] [Citation(s) in RCA: 26] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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43
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Aggressive angiomyxoma of the thigh. Skeletal Radiol 2008; 37:673-8. [PMID: 18338163 DOI: 10.1007/s00256-008-0465-0] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/13/2007] [Revised: 01/16/2008] [Accepted: 01/21/2008] [Indexed: 02/02/2023]
Abstract
Aggressive angiomyxoma is a rare tumour that typically occurs in the perineum in women of reproductive age. A small number of cases occurring in men have been reported, all of which were located in the low pelvis, perineum or scrotum. While benign, the tumour is locally infiltrative and consequently has a high rate of local recurrence following surgery; therefore, accurate pre-operative diagnosis is important. The characteristic location of these tumours in the low pelvis or perineum has led to speculation that aggressive angiomyxomas arise from a mesenchymal cell that is unique to the perineum. We describe a case of aggressive angiomyxoma arising in the thigh of a 54-year-old man, which we believe is the first reported instance of this rare neoplasm occurring remote from the pelvis or perineum in a male patient. Cross-sectional imaging demonstrated a well-defined mass that had low density on CT and high intensity on fluid-sensitive MR sequences. Biopsy was non-diagnostic and excision was performed. At histological analysis, the tumour exhibited the characteristic features of aggressive angiomyxoma, with bland spindle cells and large, hyalinised blood vessels in a hypocellular myxoid matrix. Extensive immunohistochemical staining further supported the diagnosis. While the imaging features of these tumours are non-specific and suggestive of myxoid neoplasms, the diagnosis should be considered whenever biopsy of a myxoid-appearing mass yields hypocellular, non-diagnostic material, despite adequate sampling.
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Luengo A, Fernández-Corona A, Salas J, Zornoza V. Neoplasias mesenquimatosas vulvares: descripción de un caso de angiofibroma celular vulvar. CLINICA E INVESTIGACION EN GINECOLOGIA Y OBSTETRICIA 2008. [DOI: 10.1016/s0210-573x(08)73046-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Mittal K, Soslow R, McCluggage WG. Application of immunohistochemistry to gynecologic pathology. Arch Pathol Lab Med 2008; 132:402-23. [PMID: 18318583 DOI: 10.5858/2008-132-402-aoitgp] [Citation(s) in RCA: 59] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/16/2007] [Indexed: 11/06/2022]
Abstract
CONTEXT A large variety of tumors and lesions arise in the female genital tract. Although the majority of these can be correctly recognized on routine hematoxylin-eosin-stained slides, occasional cases present a diagnostic challenge. Immunohistochemical stains are extremely useful in resolving many of these problematic cases. As the knowledge in this area is constantly expanding, it is useful to have this updated information in a review form for easy access. OBJECTIVE To present our current knowledge of immunohistochemistry of the lesions of the female genital tract in a readily accessible form. DATA SOURCES The review is based on previously published articles on this topic. CONCLUSIONS Immunohistochemical stains help in reaching a conclusive diagnosis in a variety of problematic lesions seen in gynecologic pathology. As in any other system, immunohistochemical findings need to be interpreted in light of the clinical history and morphologic findings.
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Affiliation(s)
- Khush Mittal
- Department of Pathology, NYU School of Medicine and Surgical and Ob-Gyn Pathology, Bellevue Hospital, Room 4 west 1, 462 First Ave, New York, NY 10016, USA.
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Giles DL, Liu PT, Lidner TK, Magtibay PM. Treatment of aggressive angiomyxoma with aromatase inhibitor prior to surgical resection. Int J Gynecol Cancer 2008; 18:375-9. [PMID: 18334016 DOI: 10.1111/j.1525-1438.2007.01005.x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/29/2025] Open
Abstract
Aggressive angiomyxoma (AA) is a rare, locally infiltrating soft tissue tumor typically located in the genital tract of young, reproductive age women. Surgical excision has been the mainstay of treatment. Recently, gonadotropin-releasing hormone agonist therapy has been reported to decrease the size of estrogen-receptor positive AA. We present the first case of a postmenopausal woman treated with an aromatase inhibitor to shrink the size of tumor prior to surgical resection.
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Affiliation(s)
- D L Giles
- Department of Gynecologic Surgery, Mayo Clinic, Scottsdale, Arizona, USA.
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47
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Bagga R, Keepanasseril A, Suri V, Nijhawan R. Aggressive angiomyxoma of the vulva in pregnancy: a case report and review of management options. MEDGENMED : MEDSCAPE GENERAL MEDICINE 2007; 9:16. [PMID: 17435625 PMCID: PMC1925027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Subscribe] [Scholar Register] [Indexed: 05/14/2023]
Affiliation(s)
- Rashmi Bagga
- Department of Obstetrics & Gynaecology, Postgraduate Institute of Medical Education & Research (PGIMER), Sector 12, Chandigarh, India.
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Andres LA, Mishra MJ. Angiomyxoma diagnosed in a man presenting for abdominal lipectomy. THE CANADIAN JOURNAL OF PLASTIC SURGERY = JOURNAL CANADIEN DE CHIRURGIE PLASTIQUE 2007; 15:163-4. [PMID: 19554150 PMCID: PMC2687501 DOI: 10.1177/229255030701500301] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Aggressive angiomyxoma is a rare tumour that is locally infiltrative but nonmetastasizing. It occurs nearly exclusively in adult women of childbearing age and almost always arises in the perineum and pelvic area. A case of angiomyxoma occurring in a middle-aged, morbidly obese man is reported. The clinical presentation was one of progressing scrotal edema and enlargement with subsequent development of scrotal abscesses, requiring several incisions and drainages as well as oral and intravenous antibiotics. His symptoms and body habitus left him in a significantly debilitated state, prompting him to seek treatment for his conditions. He initially presented for abdominal lipectomy. However, due to the chronic scrotal infections and enlargement, scrotectomy was recommended before any other surgical procedures. A scrotectomy was performed by the urology service. Pathological diagnosis of the excised tumour was an aggressive angiomyxoma. Given the rarity of this tumour, especially in males, the proper diagnosis and treatment of angiomyxoma is still being investigated. With this in mind, the physical signs and symptoms that characterize this condition are presented and treatment options are reviewed.
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Affiliation(s)
- Lewis A Andres
- Grand Rapids/Michigan State University Plastic & Reconstructive Surgery Residency Program; Grand Rapids Medical Education & Research Center; Spectrum Health, Grand Rapids, Michigan, USA
| | - Mithilesh J Mishra
- Grand Rapids/Michigan State University Plastic & Reconstructive Surgery Residency Program; Grand Rapids Medical Education & Research Center; Spectrum Health, Grand Rapids, Michigan, USA
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Rilo I, Subinas A, Velasco S, Laraudogoitia E. Angiomixoma agresivo de pelvis con afectación cardiaca. Rev Esp Cardiol 2006. [DOI: 10.1157/13095791] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
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Idrees MT, Hoch BL, Wang BY, Unger PD. Aggressive angiomyxoma of male genital region. Report of 4 cases with immunohistochemical evaluation including hormone receptor status. Ann Diagn Pathol 2006; 10:197-204. [PMID: 16844560 DOI: 10.1016/j.anndiagpath.2005.09.002] [Citation(s) in RCA: 44] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
Aggressive angiomyxoma (AA), first described by Steeper and Rosai (Am J SurgPathol. 1983;7:463-475), is a rare locally infiltrative tumor that usually arises in the pelvic and perineal soft tissues of young women. Approximately 150 cases have been reported in women. Aggressive angiomyxoma has a high rate of local recurrence because of its infiltrative growth and anatomical location making complete excision with wide margins difficult. To our knowledge, 39 cases of AA occurring in men have been reported in the literature. Sites frequently involved include the scrotum, spermatic cord, inguinal region, and perineum. The gross and microscopic appearances and clinical course are similar to those described in female cases. Immunohistochemistry evaluating estrogen and progesterone receptors (ER and PR, respectively), although frequently positive in the female cases, has rarely been studied in the male cases. We report the clinicopathologic features of 4 additional cases of AA in men with particular emphasis on hormone (ER/PR) receptor status. Hormone reactivity is significant in that AA may arise from specialized hormonally responsive stromal cells of the perineum and may potentially play a therapeutic role in unresectable tumors. From our small series, hormone positivity (1 case of ER+, 3 cases of PR+) does occur in the male cases of AA, and a large number of cases should be examined to determine the frequency at which these tumors express hormone receptors.
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Affiliation(s)
- Mohammed T Idrees
- Department of Pathology, The Lilian and Henry M. Stratton-Hans Popper, Mount Sinai Medical Center, New York, NY 10029, USA
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