Dermatofibrosarcoma Protuberans: Report of Two Cases

Mopper, Coleman; Pinkus, Hermann

doi:10.1093/ajcp/20.2.171

Cited by 24 publications

(10 citation statements)

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“…Darier and Ferrand, in 1924, are credited with first describing dermatofibrosarcoma protuberans, although earlier reports of the lesion appear to exist . Tumors have long been recognized for their distinct clinical presentation and locally aggressive course; the latter initially prompted debate as to whether this merited designation as a true malignancy .…”

Section: Discussionmentioning

confidence: 99%

“…Darier and Ferrand, 18 in 1924, are credited with first describing dermatofibrosarcoma protuberans, although earlier reports of the lesion appear to exist. 19 Tumors have long been recognized for their distinct clinical presentation and locally aggressive course 2,3 ; the latter initially prompted debate as to whether this merited designation as a true malignancy. 3 While the characteristic storiform morphology, adnexal-sparing, and "honeycomb" pattern of subcutaneous infiltration has long-been appreciated, 2,3 it was not until the early 1990s that immunoreactivity for CD34 was recognized as distinguishing dermatofibrosarcoma protuberans from related neoplasms.…”

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma protuberans with a novel COL6A3‐PDGFD fusion gene and apparent predilection for breast

Dickson

Hornick

Fletcher

et al. 2018

Genes Chromosomes & Cancer

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Dermatofibrosarcoma protuberans is a locally aggressive superficial mesenchymal neoplasm. It typically occurs in adulthood, and has been reported to have a slight male predilection. Tumors have a characteristic histopathologic appearance, including: storiform architecture, infiltrative "honeycomb" growth within subcutaneous adipose tissue, and immunoreactivity for CD34. Virtually all molecularly characterized cases to date have been found to harbor a COL1A1-PDGFB fusion product. Following identification of an index patient with a novel COL6A3-PDGFD fusion gene, we undertook a molecular investigation, using a combination of RNA sequencing and fluorescence in situ hybridization (FISH), to assess the prevalence of PDGFD rearrangement in dermatofibrosarcoma protuberans (N = 63). Three additional patients were found to have balanced PDGFD rearrangements. Interestingly, all 4 tumors arose on the breast of females. As a result, we subsequently examined 16 additional cases of primary breast dermatofibrosarcoma protuberans, identifying 2 additional tumors with PDGFD rearrangement. The morphology and immunophenotype of all 6 cases was analogous to those with the canonical COL1A1-PDGFB fusion; none of the cases showed fibrosarcomatous transformation. This study illustrates that the COL6A3-PDGFD fusion product is rare in dermatofibrosarcoma protuberans, and associated with an apparent predilection for breast. An awareness of this variant is important for pathologists, as it will not be detected using conventional reverse transcription polymerase chain reaction or FISH-based diagnostic assays for dermatofibrosarcoma protuberans.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Dermatofibrosarcoma protuberans with a novel COL6A3‐PDGFD fusion gene and apparent predilection for breast

Dickson

Hornick

Fletcher

et al. 2018

Genes Chromosomes & Cancer

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“…Dermatofibrosarcoma protuberans is an unusual, locally invasive, destructive neoplasm of uncertain origin [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15], which occasionally metastasizes to local lymph nodes or to distant sites [7,. It frequently recurs after surgery, which should entail a wider excision than would otherwise appear necessary.…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma non‐protuberans: Description and report of five cases of a morpheaform variant of dermatofibrosarcoma

Abramovits

Gonzalez-Sevra

et al. 1985

Journal of Surgical Oncology

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Five cases of dermatofibrosarcoma are reported. All showed features typical of dermatofibrosarcoma protuberans except that in four cases, and a portion of the fifth case, no protusion of the tumor was noted clinically despite the rather advanced stage of growth of the tumor. These lesions resembled morphea or a morpheaform basal cell carcinoma clinically but could be recognized as "dermatofibrosarcoma non-protuberans" by physicians who had observed a previous case.

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“…FS areas may be present equally in the recurrences as well as in the metastases. The recurrent and metastatic lesions can, in addition, be composed of the moie pleomorphic areas (1,5,10,11). To our knowledge, thtfre ha.s never been described a primary tumor containing both DFSP and MFS components within one tumor.…”

Section: Discussionmentioning

confidence: 91%

Composite tumor consisting of dermatofibrosarcoma protuberans and myxoflbrosarcoma

1998

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A unique case of a composite tumor consisting of dermatofibrosarcoma protuberans and low-grade myxofibrosarcoma is described. The myxofibrosarcoma component formed 70% of the lesion volume. Both components protruded above the skin, and equally both infiltrated the subcutaneous tissue in the base of the lesion. The interface between them was mostly sharp but some small areas of gradual transition were also seen. Beside fibrosarcoma arising in the dermatofibrosarcoma protuberans, myxofibrosarcoma seems thus to be another possible manifestation of the change into a more malignant tumor rarely seen in the dermatofibrosarcoma protuberans.

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Dermatofibrosarcoma Protuberans: Report of Two Cases

Cited by 24 publications

References 0 publications

Dermatofibrosarcoma protuberans with a novel COL6A3‐PDGFD fusion gene and apparent predilection for breast

Dermatofibrosarcoma protuberans with a novel COL6A3‐PDGFD fusion gene and apparent predilection for breast

Dermatofibrosarcoma non‐protuberans: Description and report of five cases of a morpheaform variant of dermatofibrosarcoma

Composite tumor consisting of dermatofibrosarcoma protuberans and myxoflbrosarcoma

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