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

Zemni, Inès; Sassi, Imène; Boujelbene, Nedia; Sabrine, Haddad; Doghri, Raoudha; Chargui, Riadh; Rahal, Khaled

doi:10.11604/pamj.2019.33.46.18861

Cited by 4 publications

(2 citation statements)

References 12 publications

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“…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 .…”

Section: Discussionmentioning

confidence: 99%

“…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 . It has a low potential for distant metastasis but a high grade of recurrence due to diffuse infiltration of dermis and subcutis 38 .…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

et al. 2020

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Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group

Mancari,

Cioffi,

Magazzino

et al. 2024

Cancers

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Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms “dermatofibrosarcoma protuberans”, “vulva”, and “vulvar”, alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients’ quality of life. Management by a multidisciplinary team is highly recommended.

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Reconstructive Surgery in the Elderly: A Case Report on Maintaining the Quality of Life in a Patient with Vulvar Dermatofibrosarcoma Protuberans

Jozwik,

Bednarczuk,

Osierda

et al. 2024

Diseases

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Background: Dermatofibrosarcoma protuberans (DFSP) is a rare proliferative condition representing skin sarcomas known to locally recur yet very rarely known to metastasize. A typical characteristic is trunk localization, making vulvar occurrences an exception. Complete resection is the primary treatment. Case Report: We present an unusual case of twice-recurrent vulvar DFSP in a 92-year-old woman. Wide local excision with tumor-negative margins (R0 resection) of a grapefruit-sized mass was followed by a rhomboid skin flap transplantation to address a substantial skin deficit at the post excision site. At 14-month follow-up, no signs of recurrence were present, all wounds were healed, and the esthetic results were assessed as highly adequate. Conclusions: This study is an argument for the role of reconstructive surgery in patients of advanced age undergoing oncological procedures; skin transplantations should be considered regardless of the patient’s age. We also provide an updated review on vulvar DFSP.

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

Cited by 4 publications

References 12 publications

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

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

Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group

Reconstructive Surgery in the Elderly: A Case Report on Maintaining the Quality of Life in a Patient with Vulvar Dermatofibrosarcoma Protuberans

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