Malignancy of Dermatofibrosarcoma Protuberans and Report of 2 Cases with Lymphgland Metastases

Przybora, L; Wojnerowicz, Czes&lstrok;aw

doi:10.1159/000224014

Cited by 26 publications

(3 citation statements)

References 1 publication

(1 reference statement)

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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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“…Study of these two reports, their similarities and discrepancies, and other case reports published prior to or later than these review articles brings the figure to 15, with the present author's case being the 16th case with lymph node metastasis in a case of DFSP. The articles reporting such cases according to their year of publication are those by Gentele in 1951, 3 Woolridge in 1957, 4 Waldermann et al in 1958, 5 Przybore et al in 1959, 6 Fisher et al in 1966, 7 Brenner et al in 1975, 8 Kahn et al in 1978, 9 Hausner et al in 1978, 10 Volpe et al in 1983, 11 Petoin et al in 1985, 12 Hirabayashi et al in 1989, 13 Mavili et al in 1994 2 and Lal et al in 1999. 14 Of these articles, only the case reported by Hirabayashi et al 13 had involvement of the scalp as the primary site.…”

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans of Scalp With Cervical Lymph Node Metastasis

Lal

Goel

Mandal

2004

Sarcoma

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Dermatofibrosarcoma protuberans (DFSP) is an uncommon, slow growing and locally aggressive tumor of the skin with a high rate of recurrence even after supposedly wide excision. The reports of regional lymph node metastasis and distant metastasis are very rare. Because of the extreme rarity of these cases with metastasis, the experience with management of such patients is very limited. A case of recurrent DFSP of scalp, with metastasis to the regional lymph nodes, in a 17-year-old boy is reported here. This is the second case of DFSP involving scalp and 16th case of DFSP of all sites metastasizing to the regional lymph nodes reported in literature. The patient was treated with wide excision of the lesion and ipsilateral radical neck dissection (including excision of overlying involved skin).

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“…Distant hematogenic metastases are seen in 4-6% of cases, 3,5 while metastasis through lymphatic route may reach 10%. 10,11 Nevertheless, metastasis is not a justification for elective prophylactic lymph node resection in all the cases. It is important, however, that lymph nodes be carefully examined during a long period of follow-up.…”

Section: Metastasismentioning

confidence: 99%

Unusual presentation of dermatofibrosarcoma protuberans

Mattei¹,

Ramina²,

Aguiar³

et al. 2018

jbnc

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Dermatofibrosarcoma protuberans is a rare neoplasm, usually presenting as a low-grade, non-invasive, slow-growing tumor, with a pronounced tendency to local recurrence. We present a rare case of a high-grade, histologically undifferentiated lesion, which eroded underlying bony structures. A wide local excision with careful pathologic analysis of margins was performed with immediate reconstruction of skull bone and musculo-cutaneous flap transposition in order to cover the skin defect. A one-stage surgical procedure, such as presented in this report, is the treatment of choice recommended by the authors.

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Malignancy of Dermatofibrosarcoma Protuberans and Report of 2 Cases with Lymphgland Metastases

Cited by 26 publications

References 1 publication

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

Dermatofibrosarcoma Protuberans of Scalp With Cervical Lymph Node Metastasis

Unusual presentation of dermatofibrosarcoma protuberans

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