Aggressive angiomyxoma of the vulva: An uncommon entity

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Abstract

Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor occurring usually in women of reproductive age, which carries a high risk for local relapse; hence the need to differentiate it from the other mesenchymal tumors occurring in this region. We describe a case of a 44-year-old female presenting with a large pedunculated swelling on the right labia majora.

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Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm.

T Steeper, J Rosai (1983)

Nine case of a distinctive soft tissue tumor of the female pelvis and perineum are described. They were characterized by their occurrence in young women (ages 21-38), large size (up to 60 X 20 cm), locally infiltrative nature, and grossly gelatinous appearance. The initial clinical impression was usually that of a Bartholin gland cyst. The microscopic appearance was that of spindle or stellate cells widely separated by a loose myxoid stroma focally rich in collagen fibrils, a prominent vascular component, including many large thick-walled vessels without an arborizing pattern, and foci of proliferating glandular elements in two cases. Mitotic activity was exceedingly low. Ultrastructural study of the spindle cells showed features consistent with myofibroblastic differentiation. Four patients developed large local recurrences; one tumor recurred twice, 14 and 15 years after initial excision. No distant metastases have been documented to date, and all patients are alive and well. The differential diagnosis of this unusual tumor includes myxoma, myxoid liposarcoma, sarcoma botryoides, myxoid variant of malignant fibrous histiocytoma, nerve sheath myxoma, and other soft tissue tumors with secondary myxoid changes. We have chosen the term "aggressive angiomyxoma" for this neoplasm to emphasize the neoplastic nature of the blood vessels and its locally infiltrative and recurrent nature.

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Aggressive angiomyxoma: a second case of metastasis with patient's death.

J. L. Faure, Isidro Machado, Juan Cruz … (2003)

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient. We document this case and report a similar one, found in the literature, of a postmenopausal woman with pulmonary and mediastinic metastases. These cases may expand the current concepts of potential behavior of aggressive angiomyxoma.

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Aggressive angiomyxoma: findings on CT and MR imaging.

B E Marchetto, Ulrich Wagner, E S Siegelman … (1999)

Aggressive angiomyxoma is a benign tumor affecting the pelvis and perineum, predominantly in women. Because of its variable presentation as a soft mass in the vulva, perianal region, buttock, or pelvis, the tumor is often clinically misdiagnosed and initial surgery is usually unsuccessful in extirpating it. This study describes the imaging features of these tumors. Aggressive angiomyxomas display unusual growth patterns of translevator extension with growth around perineal structures. Both CT and MR imaging show the transdiaphragmatic extent of these tumors. High signal intensity on T2-weighted MR images may reflect the myxomatous stroma of these tumors.

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Author and article information

Journal

Journal ID (nlm-ta): Indian Dermatol Online J

Journal ID (iso-abbrev): Indian Dermatol Online J

Journal ID (publisher-id): IDOJ

Title: Indian Dermatology Online Journal

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 2229-5178

ISSN (Electronic): 2249-5673

Publication date (Print): May-Aug 2012

Volume: 3

Issue: 2

Pages: 128-130

Affiliations

[1]Department of Dermatology, Venereology and Leprosy, Grant Medical College, Mumbai, India

Author notes

Address for correspondence: Dr. M. M. Kura, Department of Dermatology, Venereology and Leprosy, Grant Medical College and Sir JJ Hospital, Byculla, Mumbai – 400 008, India. E-mail: drkura@ 123456gmail.com

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Publisher ID: IDOJ-3-128

DOI: 10.4103/2229-5178.96712

PMC ID: 3481881

PubMed ID: 23130288

SO-VID: fbef5b50-d246-4300-b396-535626031644

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This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Aggressive angiomyxoma of the vulva: An uncommon entity

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Karger: Dermatology

Most cited references 10

Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm.

Aggressive angiomyxoma: a second case of metastasis with patient's death.

Aggressive angiomyxoma: findings on CT and MR imaging.

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