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Fine‐needle aspiration of dermatofibrosarcoma protuberans metastasizing to hemithorax with superior vena cava compression: Case report and literature review
Author(s) -
Banach Bridget S.,
Antic Tatjana,
Bridge Julia A.,
Cipriani Nicole A.,
Frye Laura,
Krausz Thomas N.,
Biernacka Anna
Publication year - 2019
Publication title -
diagnostic cytopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.417
H-Index - 65
eISSN - 1097-0339
pISSN - 8755-1039
DOI - 10.1002/dc.24179
Subject(s) - dermatofibrosarcoma protuberans , medicine , pdgfb , fine needle aspiration , wide local excision , cd34 , radiology , metastasis , imatinib , presentation (obstetrics) , trunk , pathology , biopsy , cancer , platelet derived growth factor receptor , genetics , receptor , stem cell , myeloid leukemia , growth factor , biology , ecology
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, tyrosine‐kinase inhibitor imatinib can provide additional treatment options for unresectable metastatic DFSP.