Dermatofibrosarcoma protuberans of the vulva: a case report

Öge, Tufan; Benedicic, Christoph; Tamussino, Karl; Regauer, Sigrid

doi:10.1136/bcr.07.2008.0377

Cited by 5 publications

(3 citation statements)

References 5 publications

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“…DFSP is a well-differentiated fibro-cutaneous neoplasm of low to intermediate grade malignancy commonly involving the head & neck and upper and lower extremities. Involvement of the vulva is uncommon but when present constitutes 1.5-5% of all vulvar malignancies ( 5 ). The tumor was first described in 1890 by Taylor ( 3 ).…”

Section: Discussionmentioning

confidence: 99%

Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

Goyal

Garg

Kaur

et al. 2021

JFRH

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Objective: Dermatofibrosarcoma protuberans (DFSP) of the vulva is an uncommon soft tissue tumor with fewer than 60 cases reported previously. Distant metastasis is rare with a high propensity for local invasion. Surgical management is the gold standard with adequate margin excision to prevent future recurrences. We report a case of vulvar DFSP requiring three resections to achieve primary clearance. To the best of our knowledge, this is the first case report from India. Case report: A 35-years-old female, presented with nodular vulvar mass for the third time arising from the upper part of right labia majora for the last year. She had a history of two similar episodes in the past for which excision was done and histopathology confirmed DFSP. The third wide local resection was performed with 3 cm margins and the margins were sent for intra-operative frozen section analysis, which was confirmed clear by the pathologist. The patient has been free of recurrence for two years. Conclusion: DFSP is a challenging condition presenting as a non-tender nodular mass characterized by local invasion and recurrence. Early diagnosis and appropriate management using wide local excision with accurate margin assessment can achieve optimal results and prevent future recurrences.

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Section: Discussionmentioning

confidence: 99%

Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

Goyal

Garg

Kaur

et al. 2021

JFRH

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“…It must be extensive as to remove the tumor completely, given its proclivity for irregular and often deep subclinical extensions. Re-resection is recommended if the initial surgery did not achieve clear margins [10].…”

Section: Discussionmentioning

confidence: 99%

Vulvar Darier-Ferrand dermatofibrosarcoma: unusual localization of a rare tumor

Zemni¹,

Sassi²,

Boujelbene³

et al. 2019

Pan Afr Med J

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Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade sarcoma of dermal origin that rarely presents in the vulva, typically occurring on the trunk of young to middle-aged adults. Even though it has a low potential for distant metastases, it often recurs locally. Therefore, surgical excision is the treatment of choice. We report a case of DFSP with fibrosarcoma transformation involving the vulva treated by large excision with tumor free margins followed by plastic reconstruction. Our aim is to highlight this rare disease and through literature evaluate the safety and efficacy of surgical treatment. Early recognition of this rare entity whose localization and the aggressive nature of the fibrosarcomatous component will be an issue in the surgical management.

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“…35 Relapses are common after excision, but metastatic potential is low. 35,36 Wide and deep excision with tumor-free margins is the optimal therapeutic approach, in order to avoid the risk of relapse. 37 Mohs microsurgery has been reported as an appropriate therapeutic option.…”

Section: Ewing Sarcoma Of the Vulvamentioning

confidence: 99%

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

Chokoeva¹,

Tchernev

Cardoso

et al. 2015

Int J Immunopathol Pharmacol

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Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.

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Dermatofibrosarcoma protuberans of the vulva: a case report

Cited by 5 publications

References 5 publications

Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

Vulvar Darier-Ferrand dermatofibrosarcoma: unusual localization of a rare tumor

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

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