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      Aggressive angiomyxoma of the vulva: A case report

      case-report

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          Abstract

          Aggressive angiomyxoma (AA) is an unusual mesenchymal tumor. AA occurs most commonly in women of reproductive age and is located in the perineal or pelvic region. This is a distinct soft tissue tumor that has a prominent myxoid matrix and numerous thin-walled blood vessels and may have an aggressive local recurrence. The tumors have the characteristics of large size (usually greater than 10 cm) and slow growth, and are not painful. The standard treatment for AA is total excision and close follow-up. We announce a case of a 35 year-old female presenting with a pedunculated AA on the right labium majora that has not relapsed for seven years.

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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: 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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              Myxoid tumours of soft tissue.

              J Ende, Christopher Fletcher, P Hogendoorn (1999)
              Myxoid tumours of soft tissue encompass a heterogeneous group of lesions characterized by a marked abundance of extracellular mucoid (myxoid) matrix. This group of tumours demonstrate significant variability in their biological behaviour thus including tumours which are entirely harmless, tumours with a tendency to recur locally but not metastasize, and malignant tumours. There appears to be a considerable degree of overlap clinically and morphologically between the various tumour types in this group, generating potential diagnostic problems for the clinician and pathologist alike. While diligent microscopy remains the basis of diagnostic pathology, the continuous developments and refinements within the fields of immunohistochemistry and molecular cytogenetics are providing substantial new information, allowing the development of new diagnostic criteria and hence facilitating an accurate diagnosis. It is the aim of this short review to highlight the most prevalent soft tissue tumours with predominantly myxoid morphology, to describe the features by which the majority of these myxoid lesions may be identified, and to discuss the differential diagnosis where appropriate.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Obstet Gynecol Sci
                Journal ID (iso-abbrev): Obstet Gynecol Sci
                Journal ID (publisher-id): OGS
                Title: Obstetrics & Gynecology Science
                Publisher: Korean Society of Obstetrics and Gynecology; Korean Society of Contraception and Reproductive Health; Korean Society of Gynecologic Endocrinology; Korean Society of Gynecologic Endoscopy and Minimal Invasive Surgery; Korean Society of Maternal Fetal Medicine; Korean Society of Ultrasound in Obstetrics and Gynecology; Korean Urogynecologic Society
                ISSN (Print): 2287-8572
                ISSN (Electronic): 2287-8580
                Publication date (Print): March 2014
                Publication date (Electronic): 15 March 2014
                Volume: 57
                Issue: 2
                Pages: 164-167
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
                [2 ]Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
                Author notes
                Corresponding author: Jeong-Won Lee. Department of Obstetrics and Gynecology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 135-710, Korea. Tel: +82-2-3410-1382, Fax: +82-2-3410-0630, garden.lee@ 123456samsung.com
                Article
                DOI: 10.5468/ogs.2014.57.2.164
                PMC ID: 3965702
                PubMed ID: 24678492
                SO-VID: 2301a3f2-7a90-4e36-b108-f936e9b9a9ee
                Copyright © Copyright © 2014 Korean Society of Obstetrics and Gynecology
                License:

                Articles published in Obstet Gynecol Sci are open-access, distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 14 May 2013
                Date revision received : 14 August 2013
                Date accepted : 29 September 2013
                Categories
                Subject: Case Report
                Subject: General Gynecology

                Keywords: myxoma,mesenchymal tumor,vulva
                Data availability:
                Keywords: myxoma, mesenchymal tumor, vulva

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