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Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans.

ABSTRACT: Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosis of DFSP was 40.2 years. Chest wall was the dominant location. The histopathological diagnosis was ordinary DFSP (O-DFSP) in 46 cases and fibrosarcomatous DFSP (FS-DFSP) in 11 cases. No differences were noted between FS-DFSP and conventional DFSP in age at presentation and diagnosis, tumor size, as well as CD34 and SMA expression (P > 0.05). However, the time course to recurrence in FS-DFSP group was less than that in O-DFSP group (P = 0.038). All of the patients were treated with Mohs micrographic surgery (MMS). On follow-up, none of the patients had tumor recurrence. Our data demonstrated that the clinical features of recurrent DFSP are non-specific and variable. Our current practice suggests that MMS is very useful treatment choice for recurrent DFSP.

SUBMITTER: Li Y

PROVIDER: S-EPMC5463448 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature

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Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans.

Li Yanan Y Wang Chuan C Xiang Bo B Chen Siyuan S Li Li L Ji Yi Y

Journal of Cancer 20170512 7

Dermatofibrosarcoma protuberans (DESP) is a relatively rare and low-grade tumor of the skin and soft tissue. We review the clinical features, pathological findings and management of recurrent DESP with the aim to improve our understanding of this rare tumor. Fifty-seven patients were diagnosed with recurrent DFSP. The mean age at the presentation of DFSP was 30.9 years. The mean age at diagnosis of DFSP was 40.2 years. Chest wall was the dominant location. The histopathological diagnosis was ord ...[more]

PMID: 28607608

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Project description:The aim of this study was to analyze the computed tomography (CT) and magnetic resonance imaging (MRI) findings of dermatofibrosarcoma protuberans (DFSP), with a view to improving the diagnosis of this kind of tumor. A total of 27 cases of histopathologically confirmed DFSP were analyzed retrospectively. Of these, 18 patients underwent a CT scan and 9 patients underwent an MRI. All patients underwent unenhanced and contrast-enhanced examinations; 1 patient underwent multiphrase CT enhancement examination. Imaging characteristics, including location, shape, size, number, edge, and attenuation or intensity of each lesion, both unenhanced and contrast enhanced, were analyzed. Of the 27 cases, 24 were solitary, 2 had 2 nodules, and 1 had multiple confluent tumors. The lesion with multiple confluent tumors was ill defined and irregular; the other lesions were oval or round, well-defined nodules or masses. The unenhanced CT images showed 19 homogenous isodense lesions. There was no calcification in any of the patients. The contrast-enhanced CT images showed intermediate and marked nonhomogeneous enhancement in 13 lesions, intermediate homogeneous enhancement in 4 lesions, and a mild heterogeneous enhancement in 2 lesions. MR T1-weighted images revealed 1 ill-defined and 9 well-defined homogeneous isointense lesions. T2-weighted images showed homogeneous hyperintensity to the muscles in 6 lesions, 3 mild hyperintense lesions with hypointense lesions, and 1 mixed, mild hyperintense and isointense lesion. Contrast-enhanced T1-weighted images demonstrated intermediate and marked nonhomogeneous enhancement in 9 lesions and intermediate homogeneous enhancement in 1 lesion. DFSP is characterized by a subcutaneous well-defined soft tissue nodule or mass on plain CT/MR scans, and shows intermediate-to-marked enhancement on contrast-enhanced CT/MR scans. The imaging findings for DFSP are nonspecific, but may help to define the diagnosis in an appropriate clinical setting.

Dataset Information

Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans.

Publications

Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans.

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