Dermatofibrosarcoma protuberans: report of a case with a variant ring chromosome and metastases following pregnancy

Bigby, Susan M.; Oei, Paul; Lambie, Neil; Symmans, Pennie

doi:10.1111/j.0303-6987.2006.00404.x

Cited by 21 publications

(19 citation statements)

References 33 publications

(62 reference statements)

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“…The characteristic fusion gene of DFSP has been found in only one out of three cases tested; however, a limited number of primers were used. 11 Prominent myxoid stromal changes, together with the unusual multinodular growth and the prominent vascular pattern seen in the majority of the reported cases, may cause considerable diagnostic problems, especially in the distinction of more or less aggressive myxoid mesenchymal neoplasms. Superficial angiomyxoma may show an infiltration of the subcutis and an expression of CD34 by neoplastic cells as well; however, typical cases of superficial angiomyxoma are characterized by a lobular growth without a diffuse infiltration, often contain neutrophils surrounding vascular structures, and may contain epithelial structures in a number of cases.…”

Section: Discussionmentioning

confidence: 94%

Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic, Immunohistochemical, and Molecular Analysis of Eight Cases

Mentzel¹,

Schärer²,

Kazakov³

et al. 2007

The American Journal of Dermatopathology

106

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Dermatofibrosarcoma protuberans (DFSP) represents a locally aggressive mesenchymal neoplasm of skin and subcutis with characteristic clinicopathologic, immunohistochemical, and molecular findings. In addition to typical cases, morphologic variants such as pigmented, fibrosarcomatous, myofibroblastic, and granular cell DFSP have been described. Purely or predominantly myxoid DFSP is extremely rare, and may cause considerable diagnostic problems. Eight cases of predominantly myxoid DFSP were studied. Paraffin-embedded blocks and slides were retrieved from the files of the authors. Clinical data were obtained from the referring pathologists and dermatologists. Immunohistochemistry was performed using the ABC method, and three cases were studied by polymerase chain reaction technique. There were six male and two female patients (age range: 29 to 74 years). Locations included the inguinal area (three cases), thigh, upper arm, shoulder, abdominal wall, and back (one each). The patients were treated by wide excision as well as reexcision. Tumor size ranged from 1.5 to 12 cm. Histologically, a nodular growth with peripheral diffuse infiltration, as well as a diffusely infiltrating growth of relatively uniform spindled and stellated tumor cells containing slightly enlarged nuclei, was noted. Three cases were entirely myxoid, and in five cases more than 80% of the tumor area showed myxoid stromal changes. In two cases each, focal fibrosarcomatous and focal giant cell fibroblastoma-like changes were present. At least focally, hypocellular areas were evident in one case. Scattered enlarged tumor cells were seen in two cases. The mitotic rate ranged from 1 to 10 mitoses in 10 high-power fields. Numerous blood vessels with slightly fibrosed vessel walls were seen in seven cases. Immunohistochemically, tumor cells in all cases stained positively for CD34, and in one case each a focal expression of alpha-smooth muscle actin and epithelial membrane antigen (EMA) was noted. The remaining antibodies (CD99, CD31, S-100, Factor XIIIa) were all negative. Polymerase chain reaction technique showed in one case the characteristic COL1A1-PDGFB fusion gene. Follow-up information in seven cases (range: 2 months to 10 years; mean: 62 months; median: 48 months) revealed a local recurrence at 5 years. In conclusion, myxoid DFSP represents a very rare morphologic variant with characteristic changes that has to be distinguished from benign and malignant myxoid mesenchymal neoplasms as superficial angiomyxoma, superficial acral fibromyxoma, myxoid solitary fibrous tumor, myxoid perineurioma, low-grade myxofibrosarcoma, low-grade fibromyxoid sarcoma, myxoid liposarcoma, and myxoid synovial sarcoma.

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Section: Discussionmentioning

confidence: 94%

Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic, Immunohistochemical, and Molecular Analysis of Eight Cases

Mentzel¹,

Schärer²,

Kazakov³

et al. 2007

The American Journal of Dermatopathology

106

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“…We identified 23 case reports [19,20,23-25,27,29-32,35,38,39,41,42,50-52], [55-59], 15 case series [16,26,33,34,36,37,45,47,53,60-65], 6 phase II studies [18,40,46,48,49] and 4 pediatric studies [28,43,44,54] where Imatinib was used for the treatment of patients with DFSP. These studies include a total of 199 patients.…”

Section: Resultsmentioning

confidence: 99%

Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature

et al. 2014

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Dermatofibrosarcoma Protuberans (DFSP) is a rare skin tumor, characterized by frequent local recurrence but is seldom metastatic. It is histologically characterized by storiform arrangement of spindle cells. Cytogenetically, most tumors are characterized by translocation 17:22 leading to overexpression of tyrosine kinase PDGFB which can be targeted with tyrosine kinase inhibitor, Imatinib. We describe the first case of unresectable pancreatic metastases from DFSP treated with neoadjuvant Imatinib and subsequently R0 metastectomy. Additionally, a comprehensive systematic review of DFSP pancreatic metastases and the current published data on the use of Imatinib in DFSP is summarized.

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“…None of these cases presented with two separate primary DFSP . The accelerated growth seen in pregnancy could be related to the increased production of PDGF, progesterone, or both …”

Section: Discussionmentioning

confidence: 99%

“…We believe this is the first presentation of two primary DFSP in one patient. Our patient also noted both tumors in close proximity to two separate pregnancies, which is an important documented association . The tumors presented on the upper back and left flank, making an in‐transit metastasis highly unlikely.…”

Section: Discussionmentioning

confidence: 99%

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Two Primary Dermatofibrosarcoma Protuberans Associated with Different Pregnancies in a Single Patient

Anderson

Vidimos

2012

Dermatologic Surgery

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Dermatofibrosarcoma protuberans: report of a case with a variant ring chromosome and metastases following pregnancy

Abstract: Cryptic rearrangement of chromosomes 17 and 22 should be suspected in variant ring chromosomes and translocations. Pregnancy may contribute to accelerated growth of DFSP, and delay in surgical resection should be avoided.

Cited by 21 publications

References 33 publications

Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic, Immunohistochemical, and Molecular Analysis of Eight Cases

Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic, Immunohistochemical, and Molecular Analysis of Eight Cases

Neoadjuvant treatment of Dermatofibrosarcoma Protuberans of pancreas with Imatinib: case report and systematic review of literature

Two Primary Dermatofibrosarcoma Protuberans Associated with Different Pregnancies in a Single Patient

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