Breast and pleuropulmonary metastasis of multirecurrent scalp dermatofibrosarcoma protuberans: a case report

Nde, Nina Astrid; Ouédraogo, Nazinigouba; Bambara, Hierrhum Aboubacar; Tiemtore-Kambou, Bénilde Marie Ange; Traoré, Fagnima; Zongo, Nayi; Ouédraogo, M.; Tapsoba, Gilbert Patrice; Chtioui, Achach Thouraya; Lamien, Aline; Sanou,

doi:10.1186/s13256-017-1257-8

Cited by 3 publications

(1 citation statement)

References 12 publications

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“…Given the rarity of metastatic DFSP, an English language literature search of patients with metastatic DFSP was performed to review the most common sites of metastases, presence of fibrosarcomatous transformation, and time from initial presentation to first metastasis (Table ). The average age was 44 years old.…”

Section: Discussionmentioning

confidence: 99%

Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review

Banach

Antic

Bridge

et al. 2019

Diagnostic Cytopathology

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Dermatofibrosarcoma protuberans (DFSP) is a low-grade spindle cell tumor of the skin commonly arising on the trunk and extremities which tends to be slow growing yet locally aggressive. DFSPs are associated with a good prognosis when surgical excision with negative margins is achieved.Although local recurrences occur up to 50% of incompletely resected cases, distant metastases are very rare. Here, we report a case of DFSP metastasizing to the right hemithorax diagnosed by an endobronchial ultrasound-guided fine-needle aspiration (FNA) 9 years after initial presentation. The aspirate showed a bland spindle cell proliferation that was morphologically similar to the original skin excision; the storiform pattern was particularly prominent in tumor-tissue fragments in the cellblock.Immunostaining showed strong, diffuse positivity for CD34. Molecular studies demonstrated a characteristic COL1A1/PDGFB fusion in both original and metastatic specimens. A review of the literature revealed that metastatic DFSP most often involves the lungs, occurs usually in cases with fibrosarcomatous transformation and after a local recurrence, and presents on average 4.5 years after the original diagnosis. This case did not show fibrosarcomatous transformation or local recurrence prior to metastasis 9 years later. In summary, it is important to consider the potential for metastases years after a nonrecurring primary DFSP, despite its rarity. Cytologic features when complemented by ancillary studies and awareness of the patient's prior clinical history permit a confident diagnosis of metastatic DFSP by FNA. In addition, by confirming the characteristic translocation, tyrosinekinase inhibitor imatinib can provide additional treatment options for unresectable metastatic DFSP. K E Y W O R D S COL1A1 PDGFB fusion, dermatofibrosarcoma protuberans, metastatic dermatofibrosarcoma protuberans, pulmonary metastasis 1 | INTRODUCTION Dermatofibrosarcoma protuberans (DFSP) is a low-grade spindle cell tumor of the skin which tends to be slowly growing, locally aggressive, and associated with good prognosis when surgical excision with negative margins is achieved. Local recurrences occur up to 50% of the time, especially if incompletely excised. Distant metastases are rare, occurring in <5% of patients usually after repeated local recurrences. 1-3 Histologically, DFSP consists of a proliferation of uniform spindle cells arranged in a storiform pattern. Importantly, the spindle cells are not pleomorphic, mitoses are infrequent, and necrosis is usually absent despite the infiltrative nature of the tumor which can entrap adnexal structures and fat in a "honeycomb" pattern. 4 Rarely DFSP can undergo fibrosarcomatous transformation, which is associated with a more aggressive course and demonstrates increased mitoses, necrosis, and fascicular growth pattern. 5 A helpful advance in diagnosis of DFSP has been identification of a characteristic translocation of t (17;22)(q22;q13) which consists of the fusion of COL1A1(17q22)and PDGFB(22q13) genes. 6 This assist...

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Section: Discussionmentioning

confidence: 99%

Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review

Banach

Antic

Bridge

et al. 2019

Diagnostic Cytopathology

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Novel association of SMARCA4 deletion with Dermatofibrosarcoma protuberans: Novel insights and expansion of the molecular change of this tumour

Deng

Ying

Jia

et al. 2023

Preprint

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Dermatofibrosarcoma protuberans (DFSP) is a rare indolent soft tissue sarcoma of the skin that accounts for less than 1 % of all soft tissue sarcomas. Clinically, it is a slow-growing, localised, low-grade, malignant fibrosarcoma. Here, we report the case of a 40-year-old woman diagnosed with DFSP harbouring a deletion in the SMARCA4 gene. The tumour was completely removed after surgery and the 1-year follow-up, no recurrence or additional malignancy was observed. Our review of the existing literature suggests that DFSP with a SMARCA4 gene deletion have not been reported to date. This case furthers our understanding on the molecular biology of DFSP for soft tissue sarcomas, which will therefore help clinicians to more accurately assess the prognoses of patients.

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A case of dermatofibrosarcoma protuberans on the head with bone destruction and brain metastasis

et al. 2020

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Breast and pleuropulmonary metastasis of multirecurrent scalp dermatofibrosarcoma protuberans: a case report

Cited by 3 publications

References 12 publications

Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review

Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review

Novel association of SMARCA4 deletion with Dermatofibrosarcoma protuberans: Novel insights and expansion of the molecular change of this tumour

A case of dermatofibrosarcoma protuberans on the head with bone destruction and brain metastasis

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