Dermatofibrosarcoma Protuberans of the Vulva: A Case Report and Review of the Literature

doi:10.1006/gyno.2000.5821

Gynecologic Oncology

Volume 78, Issue 1, July 2000, Pages 74-75

https://doi.org/10.1006/gyno.2000.5821 Get rights and content

Abstract

Objective. The purpose of this study is to describe the management of a patient with dermatofibrosarcoma protuberans (DFSP) of the vulva and to review the literature.

Methods. A 39-year-old was referred by a district hospital for incomplete excision of a vulval mass. The lesion involved the left labium major and measured 8 × 12 cm. The lesion was reexcised with a 3-cm margin of normal skin.

Results. The patient made an uneventful postoperative recovery. Histology confirmed a diagnosis of DFSP with clear margins. Immunohistochemistry was positive for CD34 glycoprotein.

Conclusions. DFSP of the vulva is a rare fibrous tumor of intermediate grade malignancy, with a tendency for local recurrence. However, it rarely metastasizes. Management should be multidisciplinary and Mohs' micrographic surgery is generally advocated to ensure precise margin control. Survival rates range from 91 to 100%, and local recurrence rates of 20 to 49% have been reported. Therefore close follow-up is recommended.

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Dermatofibrosarcoma protuberans (DFSP) is a low-grade dermal sarcoma, which predominantly affects the trunk and proximal limbs of young to middle-aged adults. However, DFSP can arise at virtually any cutaneous site, including the vulva.96–104 Vulvar DFSP occurs across a wide age range, but most commonly affects women in the fourth and fifth decades.98,101,104
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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Vulvovaginal mesenchymal neoplasms and non-neoplastic proliferations are rare. Nonetheless, the spectrum of lesions is wide. A major subset includes tumors preferentially seen in this anatomic location (vulvovaginal stromal tumors). Among them, the only one with propensity for aggressive behavior and recurrence is deep (aggressive) angiomyxoma; others are, by comparison, more indolent. With the exception of superficial angiomyxoma, vulvovaginal stromal tumors express hormone receptors, which has been implicated in their pathogenesis. Given the morphologic overlap among most primary vulvovaginal stromal lesions, an immunohistochemical panel including estrogen receptor, desmin, smooth muscle actin and CD34 is commonly performed. However, overlap in marker expression also occurs, and the diagnosis needs to rely in a combination of clinical, pathologic and immunophenotypic features. In ambiguous cases, an effort should be made to at least exclude deep angiomyxoma. Most fibrohystiocytic, lipomatous, muscular, vascular and neural lesions involving the distal female genital tract are morphologically identical to their extragenital counterparts. Important exceptions exist: for instance, criteria for classification of smooth muscle neoplasms as benign, malignant or of low malignant potential is specific for vulvovaginal lesions, and differs from uterus or other soft tissue locations. Many tumors harbor distinctive translocations or other genomic alterations, for example: FOXO1 monoallelic loss (cellular angiofibroma, mammary-type myofibroblastoma), COL1A1-PDGFB chimeric fusion (dermatofibrosarcoma protruberans), FUS-BBF2H7 fusion (low grade fibromyxoid sarcoma) and EWS alterations (angiomatoid fibrous histiocytoma, Ewing sarcoma). When available, molecular testing should be considered if tumors with specific genomic abnormalities are in the differential.
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A wide variety of other mesenchymal lesions may occasionally involve the vulvovaginal region and it is stressed that when dealing with a mesenchymal lesion at this site, the pathologist should think widely and consider other lesions rather than just the site specific ones discussed. Mesenchymal lesions that have been reported in the vulvovaginal region include nodular fasciitis, fibromatosis, spindle cell lipoma, liposarcoma, neural tumours (benign and malignant, including myxoid variants), haemangiopericytoma, solitary fibrous tumour, mammary-type myofibroblastoma, dermatofibrosarcoma protuberans (including myxoid variants), myoepithelial neoplasms (benign and malignant), epithelioid sarcoma, synovial sarcoma, angiosarcoma, alveolar soft part sarcoma, perivascular epithelioid cell tumour (PEComa) and neoplasms in the Ewing family of tumours.93–113 It should also be remembered that occasional, epithelial neoplasms may contain a population of spindle cells and, especially in a small biopsy, be mistaken for a mesenchymal lesion.
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MMS (especially with multiple preoperative scouting biopsies) is becoming the surgery of choice for extramammary Paget disease, with overall lower recurrence rates (16%–23% vs 31%–61% for standard excision) (see Table 2).37,38 A multidisciplinary approach should be taken for genital cancers and may involve urologists, gynecologists, oncologists, radiation oncologists, plastic surgeons, dermatologic surgeons, dermatologists, and dermatopathologists.33 Repair options for genital surgery include secondary intention healing, primary closures, split or full-thickness skin graft, and fasciocutaneous or myocutaneous flaps.30,33
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¹: To whom correspondence should be addressed at Department of Obstetrics and Gynaecology, University of Natal Medical School, Private Bag 7, Congella, 4013, South Africa. Fax: +27-031-2604427. E-mail: [email protected].

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Case ReportDermatofibrosarcoma Protuberans of the Vulva: A Case Report and Review of the Literature

Abstract

J Am Acad Dermatol

J Am Acad Dermatol

Cancer Lett

Int J Radiat Oncol Biol Phys

Gynecol Oncol

Malignant fibrous tumours

Semin Cutan Med Surg

Case Report
Dermatofibrosarcoma Protuberans of the Vulva: A Case Report and Review of the Literature