Progression of dermatofibrosarcoma protuberans to malignant fibrous histiocytoma:Report of a case with implications for tumor histogenesis

O'Dowd, J; Laidler, P.

doi:10.1016/s0046-8177(88)80534-3

Cited by 48 publications

(14 citation statements)

References 18 publications

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“…As in previous reports, this phenomenon is uncommon. 15,16,18,19 Review of the institutional files at a major cancer treatment center and the consultation files of 2 soft tissue experts (H.L.E. and C.D.M.F.)…”

Section: Discussionmentioning

confidence: 99%

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Dermatofibrosarcoma Protuberans With Unusual Sarcomatous Transformation: A Series of 4 Cases With Molecular Confirmation

Swaby¹,

Evans

Fletcher

et al. 2011

The American Journal of Dermatopathology

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Dermatofibrosarcoma protuberans (DFSP) is a superficial sarcoma of intermediate malignancy usually composed of monotonous short spindle cells with storiform architecture. The tumor cells are diffusely reactive for CD34 and characterized by a translocation involving chromosomes 17 and 22 or a supernumerary ring chromosome that results in the fusion of exon 2 of platelet-derived growth factor beta (PDGFβ; 22q13) to various exons of collagen type 1 alpha 1 (COL1A1; 17q22). In some tumors, fibrosarcomatous transformation can occur and is characterized by a monotonous spindle cell proliferation arranged in fascicles or a herringbone-type pattern. We report 4 DFSPs with unusual and pleomorphic sarcomatous transformation. They occurred on the back, scalp, shoulder, and forehead of women (ages 31,48, 48, and 27 year). In addition to areas of conventional DFSP that strongly expressed CD34, 2 cases showed pleomorphic areas mimicking undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma: 1 case had a patternless area and 1 case had combined round/spindled cells with myxoid areas. Reverse transcription--polymerase chain reaction was performed in 1 case, confirming the presence of a COL1A1-PDGFβ fusion transcript. The remaining three cases were found to be positive for a PDGFβ gene rearrangement by fluorescence in situ hybridization. This series illustrates that sarcomatous transformation in DFSP may occasionally display areas, which can mimic undifferentiated pleomorphic or unclassified sarcoma. Ancillary diagnostic testing may be helpful to confirm the diagnosis, especially in small biopsies.

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Section: Discussionmentioning

confidence: 99%

“…However, sarcomatous transformation similar to undifferentiated pleomorphic sarcoma (UPS)/malignant fibrous histiocytoma (MFH) or unclassified sarcoma can occur. 15,16,18,19 We examined our files for DFSP with unusual sarcomatous transformation and material available for the molecular confirmation of the characteristic translocation.…”

Section: Introductionmentioning

confidence: 99%

Dermatofibrosarcoma Protuberans With Unusual Sarcomatous Transformation: A Series of 4 Cases With Molecular Confirmation

Swaby¹,

Evans

Fletcher

et al. 2011

The American Journal of Dermatopathology

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“…These variants of DFSP have questionable prognostic relevance. [6][7][8][9][10][11][12][13][14][15][16] Our study investigates the biological behaviour of transformed DFSP by analysing a series of patients with longterm follow up.…”

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confidence: 99%

Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases

Szöllősi

Nemes

2005

J Clin Pathol

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Background: Fibrosarcomatous (FS) or malignant fibrous histiocytomatous (MFH) transformation of dermatofibrosarcoma protuberans (DFSP) is a rare, but well known, entity. DFSPs with sarcomatous areas have questionable biological behaviour. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary DFSPs. One recent study described no difference in the behaviour of conventional and transformed DFSP. Aims: To investigate the biological behaviour of a series of transformed DFSPs. Methods: Eight transformed DFSPs were analysed clinicopathologically. Follow up ranged from four to 36 years. Results: The tumours involved the trunk (six cases) and lower extremity (two cases) and measured 3.5-8 cm (median, 4). Sarcomatous change presented de novo in all cases. The type of sarcomatous change was FS (five cases) and MFH (three cases). The estimated proportion of sarcomatous area in the tumour was 25-70% (median, 43.37%). Mitotic counts ranged from nine to 16 mitotic figures/10 high power fields in the FS and MFH areas (median, 12), and from one to three in the DFSP areas. Six patients were treated by wide local excision with histopathologically negative margins and two were treated by simple surgical excision with positive margins. Three patients developed recurrences and one developed metastasis during follow up. Of those treated by wide local excision, one developed recurrence. All tumours expressed CD34 in the DFSP component, but only three in the sarcomatous area. Conclusions: Although DFSP containing sarcoma may be a more aggressive tumour, its behaviour can be influenced by surgical treatment.

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“…4 DFSP can undergo transformation to fibrosarcoma, although it is not clear whether this morphologic change reflects a more aggressive clinical form. 5,6 Occasionally, DFSP may progression to a malignant fibrous histiocytoma 8 The diagnosis of DFSP is clinically difficult because it resembles a keloid or a hypertrophic scar, which are common in Africans 8 It has been reported as a presentation in the breast as a primary breast lesion 9 .therefore, biopsy of the lesion is needed to make a diagnosis. however, the diagnosis can be made or at least suspected on the basis of fine needle aspiration material.…”

Section: Introductionmentioning

confidence: 99%

A clinicopathological study of Dermatofibrosarcoma Protuberans in Maiduguri, Northeastern Nigeria

Nggada¹,

Gali²,

Na'Aya³

2010

Nigerian Journal of Surgical Research

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Background Dermatofibrosarcoma protuberans (dfsp) is a rare tumour originating from the dermis, it is slow growing, nodular, polypoid neoplasm that invade the subcutaneous tissue and does not usually metastasis.Clinical presentation is not uniform and may mimic common tropical benign skin lesions.We intend to study the behaviour of this tumour in our environment . Materails and method. A Clinicopathologic study of 36 cases of histologically diagnosed Dermatofibrosarcoma protuberans in University Maiduguri Teaching Hospital was carried out.DFSP constitutes 3.05% of all cases of all cancers and 10.2 % of all skin cancers. The peak age incidence was the 4 th and 6 th decades with a median age 37.6 years. The males to female ratio was 2:1. The commonest site was the trunk 17(48.8%), followed by the lower limbs 8 (22.9%); upper limbs 6(17.1%); head and neck 3(8.6%) and buttock 1(2.9%) were not frequently affected.. Conclsusion: DFSP is a common lesion in our environment that warrants proper clinical assessment, and histological characterisation.

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Progression of dermatofibrosarcoma protuberans to malignant fibrous histiocytoma:Report of a case with implications for tumor histogenesis

Cited by 48 publications

References 18 publications

Dermatofibrosarcoma Protuberans With Unusual Sarcomatous Transformation: A Series of 4 Cases With Molecular Confirmation

Dermatofibrosarcoma Protuberans With Unusual Sarcomatous Transformation: A Series of 4 Cases With Molecular Confirmation

Transformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases

A clinicopathological study of Dermatofibrosarcoma Protuberans in Maiduguri, Northeastern Nigeria

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