Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature
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...
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| Format: | Article |
| Language: | English |
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Tehran University of Medical Sciences
2021-06-01
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| Series: | Journal of Family and Reproductive Health |
| Subjects: | |
| Online Access: | https://jfrh.tums.ac.ir/index.php/jfrh/article/view/1674 |
| _version_ | 1818394908445114368 |
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| author | Lajya Devi Goyal Priyanka Garg Manmeet Kaur Diksha Sharma |
| author_facet | Lajya Devi Goyal Priyanka Garg Manmeet Kaur Diksha Sharma |
| author_sort | Lajya Devi Goyal |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-14T06:08:41Z |
| format | Article |
| id | doaj.art-139180dca74e409395717351c1b44a50 |
| institution | Directory Open Access Journal |
| issn | 1735-8949 1735-9392 |
| language | English |
| last_indexed | 2024-12-14T06:08:41Z |
| publishDate | 2021-06-01 |
| publisher | Tehran University of Medical Sciences |
| record_format | Article |
| series | Journal of Family and Reproductive Health |
| spelling | doaj.art-139180dca74e409395717351c1b44a502022-12-21T23:14:13ZengTehran University of Medical SciencesJournal of Family and Reproductive Health1735-89491735-93922021-06-0115210.18502/jfrh.v15i2.6456Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of LiteratureLajya Devi Goyal0Priyanka Garg1Manmeet Kaur2Diksha Sharma3Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Bathinda, IndiaDepartment of Obstetrics and Gynecology, All India Institute of Medical Sciences, Bathinda, IndiaDepartment of Pathology, All India Institute of Medical Sciences, Bathinda, IndiaGuru Gobind Singh Medical College, Faridkot, Punjab, IndiaObjective: 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.https://jfrh.tums.ac.ir/index.php/jfrh/article/view/1674DermatofibrosarcomaVulvaMargins of ExcisionLocal Neoplasm Recurrence |
| spellingShingle | Lajya Devi Goyal Priyanka Garg Manmeet Kaur Diksha Sharma Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature Journal of Family and Reproductive Health Dermatofibrosarcoma Vulva Margins of Excision Local Neoplasm Recurrence |
| title | Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature |
| title_full | Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature |
| title_fullStr | Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature |
| title_full_unstemmed | Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature |
| title_short | Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature |
| title_sort | recurrent dermatofibrosarcoma protuberans of the vulva a rare occurrence and review of literature |
| topic | Dermatofibrosarcoma Vulva Margins of Excision Local Neoplasm Recurrence |
| url | https://jfrh.tums.ac.ir/index.php/jfrh/article/view/1674 |
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