Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review

Nguyen, Austin Huy; Detty, Shannon Q.; Gonzaga, Maria I.; Huerter, Christopher J.

doi:10.1097/dss.0000000000001113

Cited by 18 publications

(14 citation statements)

References 5 publications

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“…Some cases of breast DFSP with negative margin reported the use of postoperative radiotherapy, but no long-term follow-up data included. It is reported that most DFSP have the translocation t(17; 22) (q22; q13), resulting in the COL1A1-PDGF β fusion gene, which provides a biological basis for treatment with tyrosine kinase inhibitors, such as imatinib [ 52 ]. In the advanced or metastatic group, imatinib therapy is warranted if a unique translocation is confirmed [ 53 , 54 ].…”

Section: Discussionmentioning

confidence: 99%

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Wang

Chen

et al. 2020

BioMed Research International

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Dermatofibrosarcoma protuberans (DFSP) is a rare low-grade fibroblastic mesenchymal tumor derived from the dermis. The aim of this retrospective analysis was to summarize the clinicopathological data from our cases and published cases to offer more evidence for the recognition of dermatofibrosarcoma protuberans (DFSP). A total of 6 breast DFSP patients who had received treatment in our hospital were retrospectively enrolled, and detailed clinicopathological data were gathered for analysis. The median age was 29.5 years (ranging from 17 to 42 years). Most cases presented a red or brown-red, mobile, well-circumscribed, protruding, breast mass (ranging from 1 to 3 cm). For histopathology, all cases (6/6) showed a storiform pattern of spindle cells that were positive for CD34 (6/6) and Vimentin (5/6) and negative for smooth muscle actin (0/6) and S-100 protein (0/6). The majority of patients (5/6) underwent wide local excision, with 2 cases treated with radiotherapy. With a median follow-up of 36 months, all 6 patients survived without recurrence or metastasis. The PubMed database was used to search for similar cases. Eventually, 36 cases were included in this review, while cases without detailed clinical information or not reported in English were excluded from the analysis. To summarize, DFSP of the breast is an extremely rare malignancy characterized by spindle tumor cells arranged in a storiform pattern and positivity for CD34. The core needle biopsy is one of the crucial methods for its preoperative diagnosis. Management of DFSP is mainly based on surgical excision. It is prone to local recurrence, so long-term follow-up is required.

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Section: Discussionmentioning

confidence: 99%

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Wang

Chen

et al. 2020

BioMed Research International

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Section: Discussionmentioning

confidence: 99%

“…DFSP is rarely described in the vulva 36,37 . Nguyen et al published the largest series of cases of vulvar DFSP, 33 cases, arising slowly as tender asymptomatic masses on labia majora 36 . This soft tissue tumor is described overall as a superficial low‐grade sarcoma of intermediate malignancy that typically arises in the trunk and proximal extremities 37 .…”

Section: Discussionmentioning

confidence: 99%

Non‐human‐papillomavirus‐related malignancies of the vulva: A clinicopathological study

et al. 2020

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Background: Malignant tumor of the vulva is the fourth gynecological malignancy in frequency. Close to 70% of all vulvar malignancies are related to high-risk human papillomavirus (HPV) infection. Methods: A search for non-HPV-related malignant tumors of the vulva was performed in the last 20 years (2000-2020) in the pathology database of a single tertiary institution. We aim to estimate the prevalence of non-HPV-related malignancies in our population, describe clinicopathological features of these tumors and investigate the expression of some potential therapeutic targets. Results: A total of 71 patients were recovered; 26 patients (36%) had the diagnosis of extramammary Paget disease, 17 patients (24%) had basal cell carcinomas, 17 patients (24%) had primary melanomas, 10 patients (14%) had metastatic disease to the vulva and one patient (1%) had a primary dermatofibrosarcoma protuberans. Fifty-four percent of patients with extramammary Paget disease had a secondary malignancy and 12.5% had invasive disease. Programmed death-ligand 1 (PDL-1) was positive in seven out of nine primary melanomas and Her2/neu was overexpressed in six out of seven extramammary Paget disease. Conclusion: Non-HPV-related malignancies are important differential diagnoses in patient with vulvar lesions. Additional research is necessary to further understand these complex malignancies and potential new therapeutic targets.

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“…Lesions presented with erythema, hyperpigmentation, ulceration, or even of orange peel skin appearance. Patients reported slow to minimal change in size (ranging from 3 months to 10 years) [7]. As with all solid tumors, clinical suspicion is confirmed by biopsy.…”

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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Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review

Abstract: This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.

Cited by 18 publications

References 5 publications

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

A Rare Malignant Disease, Dermatofibrosarcoma Protuberans of the Breast: A Retrospective Analysis and Review of Literature

Non‐human‐papillomavirus‐related malignancies of the vulva: A clinicopathological study

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

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