Giant Darier–Ferrand dermatofibrosarcoma protuberans of the abdomen and pelvis: a case report

Triki, A; Aloui, M.; Ghalleb, Montassar; Jbir, Ichraf; Naceur, Ben; Hassouna, Jamel Ben; Chargui, R; Rahal, Khaled

doi:10.1186/s13256-021-02687-8

Cited by 3 publications

(2 citation statements)

References 19 publications

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“…First described by Darier and Ferrand in 1924, dermatofibrosarcomas are rare tumors 1–3 . Its incidence varies between 0.1% and 4.1% worldwide 4,5 .…”

Section: Introductionmentioning

confidence: 99%

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Dermatofibrosarcoma protuberans surgery: Experiences of four African surgical oncology units and literature review

Zongo

Guigemdé

Yameogo

et al. 2022

Journal of Surgical Oncology

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Background and Objectives Dermatofibrosarcoma is a locally malignant tumor. This gives surgery a place of choice. The advent of imatinib has improved outcomes. Our aim is to describe the indications, techniques and results of surgery. Methods A retrospective, multicenter, descriptive study conducted in four West African surgical oncology units. It covers dermatofibrosarcoma surgery performed between January 1, 1988 and December 31, 2020. We took into account the surgical procedure, the mode of skin coverage, adjuvant treatments and survival. Comparisons were possible using Student's t‐test and Pearson's χ2. Results We recorded 81 cases of dermatofibrosarcoma. Surgery was effective in 90.1% of cases. Wide resection was the principle with a mean resection margin of 3.8 ± 1.9 cm. healing by primary intention, flaps and healing by secondary intention were the methods of skin coverage in 30.1%, 24.7% and 41.1% respectively. The type of skin coverage was related to the topography and size of the tumor (p < 0.0001). The healing time is associated with the type of skin cover. The recurrence rate was not related to the type of skin coverage (p = 0.8). Conclusions Wide and deep resection in the absence of Mohs micrographic surgery ensures healthy margins. Oncoplasty reduces the healing time without increasing the risk of recurrence.

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“…First described by Darier and Ferrand in 1924, dermatofibrosarcomas are rare tumors 1–3 . Its incidence varies between 0.1% and 4.1% worldwide 4,5 .…”

Section: Introductionmentioning

confidence: 99%

“…First described by Darier and Ferrand in 1924, dermatofibrosarcomas are rare tumors. [1][2][3] Its incidence varies between 0.1% and 4.1% worldwide. 4,5 In Africa, they are reported in case series that do not allow reliable conclusions on their therapeutic and evolutionary aspects.…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans surgery: Experiences of four African surgical oncology units and literature review

Zongo

Guigemdé

Yameogo

et al. 2022

Journal of Surgical Oncology

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Dermatofibrosarcoma- An uncommon entity, commonly mismanaged: a case report

Tanwar

Singh

Pratap

et al. 2021

International Journal of Surgery Case Reports

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Introduction & importance Dermatofibrosarcoma protuberans (DFS) is a slow-growing, recurrent, cutaneous soft tissue sarcoma with low metastatic potential. It is not uncommon for DFS to get misdiagnosed and treated like commoner parietal wall swellings, viz., epidermal cysts, lipomas and fibromas. Suboptimal management often leads to recurrence, which may be difficult to manage. Case report A 33-year male was referred to us with a lump in his lower abdomen for 15 years, without any symptoms. The patient underwent medical management for 6 years, followed by excision. The swelling recurred four years later and progressed in size till the next 5 years. At our centre, the patient underwent imaging and aspiration cytology, which established diagnosis of DFS, followed by wide local excision. Biopsy was conclusive and margins negative. Discussion DFS presents commonly as an asymptomatic indurated plaque that slowly enlarges over months to years. Untreated, DFS can attain massive dimensions, producing the large “protuberant” nodules, and hence the name. In the current report, 15 years elapsed before the correct diagnosis was established. In this case, recurrence first appeared after four years of excision. The possibility of primary wound closure after wide excision decreases with every subsequent excision, and reconstructive options may not be readily available. Conclusion The current report highlights a diagnostic delay of 15 years in a case of DFS. Asymptomatic, indolent nature combined with low awareness among community doctors contribute to delay in timely diagnosis. Community doctors should consider DFS as a differential in any long-standing, indolent, asymptomatic parietal wall swelling, especially with a history of recurrence.

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Localization on the palmar face of the hand of a Darier-Ferrand dermatofiborosarcoma. About a case

Ai¹,

Ramampisendrahova

Has³

et al. 2021

JAMRMHSS

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Darier-Ferrand dermatofibrosarcoma is a rare but not exceptional malignant mesenchymal skin tumor, representing 0.1 % of malignant skin tumors. It is characterized by high recurrence, slow growth and low metastatic potential. Although several clinical cases of an unusual variant of Darier-Ferrand dermatofibrosarcoma have been reported in the literature, localization on the palmar face of the hand is not common. We report a case of Darier and Ferrand dermatofibrosarcoma at the level of the fourth commissure of the palm of the left hand in a 43- year-old young Malagasy adult treated by a large surgical excision with a healthy margin of two centimeters associated with adjuvant chemotherapy with Imatinib.

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Giant Darier–Ferrand dermatofibrosarcoma protuberans of the abdomen and pelvis: a case report

Cited by 3 publications

References 19 publications

Dermatofibrosarcoma protuberans surgery: Experiences of four African surgical oncology units and literature review

Dermatofibrosarcoma protuberans surgery: Experiences of four African surgical oncology units and literature review

Dermatofibrosarcoma- An uncommon entity, commonly mismanaged: a case report

Localization on the palmar face of the hand of a Darier-Ferrand dermatofiborosarcoma. About a case

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