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PubMed1995

Ultrastructure of composite tumours consisting of giant cell fibroblastoma and dermatofibrosarcoma protuberans.

We present here ultrastructural findings in three composite tumours having giant cell fibroblastoma--dermatofibrosarcoma protuberans components occurring in adult patients. We further hypothesize that these 3 tumours belong to the group of neoplasms that can show giant cell fibroblastoma, dermatofibrosarcoma protuberans, dendritic pigment cell, myxoid, and fibrosarcomatous differentiation. We consider the giant cell fibroblastoma areas of the tumours to represent a well-differentiated component of these neoplasms.

Adult↗
PubMed1995

Ring 22 chromosomes in dermatofibrosarcoma protuberans are low-level amplifiers of chromosome 17 and 22 sequences.

Ring chromosomes have been found with some regularity as solid tumors have come increasingly under cytogenetic study. The full genetic content and significance of these rings remain unclear. Dermatofibrosarcoma protuberans, a tumor of the deep dermis, consistently has supernumerary ring chromosomes, sometimes as the sole detectable cytogenetic change. Using a modified method for comparative genomic hybridization and fluorescent in situ hybridization with a panel of various probes, we found that these ring chromosomes consistently contain the chromosome 22 centromere along with interstitial sequences from chromosomes 17 and 22, specifically from regions 17q23-24 and 22q11-12. The ring chromosomes in dermatofibrosarcoma protuberans are vehicles for a particular pattern of relatively low-level genomic amplification of selected sequences.

Adult↗
PubMed1993

Myxoid variant of dermatofibrosarcoma protuberans with fibrosarcomatous areas.

A case of dermatofibrosarcoma protuberans in a 39-year old woman excised from the right subclavicular region is described. Beside the typical storiform cellular pattern characteristic of dermatofibrosarcoma protuberans, a considerable part of the tumour displayed a myxoid change. Another part of the tumour exhibited large areas with a fibrosarcoma-like pattern. The transitional areas between these myxoid and fibrosarcoma-like areas resembled a poorly differentiated spindle cell sarcoma. Two years later the tumour recurred. The remarkable feature of the recurrent tumour was the occurrence of multiple giant cells identical to those seen in giant cell fibroblastoma.

Adult↗
PubMed1995

A case of dermatofibrosarcoma protuberans on the right first toe.

Reports of dermatofibrosarcoma protuberans on a toe are extremely rare. It frequently occurs on the trunk and extremities. A Japanese woman presented with a dark-brownish hyperkeratotic plaque on the dorsal skin of her first toe. The initial clinical diagnosis of verruca vulgaris prompted treatment with cryotherapy. After that a glossy milky-white tumor appeared. Only the results of the histopathologic examination resulted in a diagnosis of dermatofibrosarcoma protuberans. The unusual macroscopic finding was considered to be due to repetitive stimulation by foot movement.

Dermatofibrosarcoma↗
PubMed1995

Dermatofibrosarcoma protuberans in a renal transplant patient.

Dermatofibrosarcoma protuberans in an unusual soft tissue tumour with a propensity for local recurrence and rarely metastatic spread. Given its indolent course it may be mistaken for atypical scarring or a keloid growth. Early diagnosis and prompt wide local resection of this entity are required in order to prevent a local recurrence. We report a patient who developed locally invasive dermatofibrosarcoma protuberans 4 years after a successful renal transplantation. Although other forms of sarcoma have been reported as a complication of organ transplantation and immunosuppression, this pathology has, so far, not been reported in a transplant recipient.

Adult↗
PubMed1996

[Dermatofibrosarcoma protuberans--a clinico-anatomical and immunohistochemical study].

This is a report on the clinical, anatomical and immunohistochemical study of thirty patients presenting dermatofibrosarcoma protuberans. Its incidence (4.8 percent), covering a 15-year period in a specialized oncological unit, wider age range and high rate of relapses (66.6 percent) are established as the result of clinical and anatomical revision. The latter findings necessitate radical removal of the neoplasm as early at its primary resection. The practical implications of the so-called "spoke-like" structures, pathognomonic for histological identification of the tumor, are discussed. The immunohistochemical study for S100 [correction of C100] protein and lysozyme is negative, while alpha 1-antitrypsin reaction is positive in single rounded histiocyte-like cells which is by no means a conclusive evidence of the phenotype characteristics of the cell population in dermatofibrosarcoma protuberans.

Adolescent↗
PubMed1978

Dermatofibrosarcoma protuberans with lymph node involvement. A case report of simultaneous occurrence with an atypical fibroxanthoma of the skin.

A 40-year-old man had dermatofibrosarcoma protuberans of the neck with rapid local recurrence, satellite nodules, and a cervical lymph node metastasis, occurring simultaneously with an atypical fibroxanthoma of the scrotum. These two tumors, with distinct clinical and pathologic features, are classified as histogenetically related lesions within the spectrum of the fibrous histiocytomas. The place for the dermatofibrosarcoma protuberans at the malignant end of this spectrum is clinically and ultrastructurally documented further in our patient.

Adult↗
PubMed1993

Dermatofibrosarcoma protuberans of the head and neck. A report of 16 cases.

Dermatofibrosarcoma protuberans of the head and neck is a rare neoplasm. Overall, sarcomas account for less than 1% of all head and neck malignancies. Of 229 patients with sarcomas of the head and neck seen at our institution between 1955 and 1988, 14 (7%) were assigned the diagnosis of dermatofibrosarcoma protuberans after pathologic review. Two additional cases have been seen since 1988, bringing the total to 16. All cases were low-grade lesions. Follow-up ranged from 36 to 198 months, with a median of 114 months. Fifteen patients were managed with surgery alone, and nine (60%) developed local recurrence. Eight were salvaged with further surgery. There were no regional lymph node recurrences or distant metastases. One patient was judged to be a poor surgical candidate and received primary radiation therapy. He died disease free 3 years after treatment. At last follow-up, 15 (94%) of 16 patients were ultimately disease free after salvage treatment. The overall 5-year survival rate was 93% (13/14). We conclude from this series, and from a review of the literature, that wide surgical resection achieving good margins offers excellent probability of cure, and that radiation therapy is a reasonable alternative in patients who have unresectable lesions or who are medically inoperable.

Adolescent↗
PubMed1975

Dermatofibrosarcoma protuberans metastatic to a regional lymph node. Report of a case and review.

A case of dermatofibrosarcoma protuberans of the lower extremity with metastasis to an inguinal lymph node appearing 5 years after wide excision and skin grafting of the primary lesion is presented. The world literature is reviewed. This case is the 24th instance of metastasis and the 7th case of lymphatic metastasis. The other 17 cases were hematogenous metastases. The clinical and pathologic features of dermatofibrosarcoma protuberans are reviewed, and treatment is discussed, with the aim of emphasizing the need for long-term followup examination of the regional lymph nodes following wide and deep local excision.

Fibrosarcoma↗
PubMed1989

Dermatofibrosarcoma protuberans with fibrosarcomatous areas. A clinicopathologic study of nine cases and a comparison with allied tumors.

Nine cases of dermatofibrosarcoma protuberans with fibrosarcomatous areas (DFSP with FS) were analyzed clinicopathologically, and the findings compared with allied tumors comprising 43 cases of ordinary DFSP, ten of superficial fibrosarcoma (SFS), and 11 of storiform variant of malignant fibrous histiocytoma (SV MFH). All but one case of DFSP with FS developed on the trunk of the body. Eight of the nine patients (89%) had one recurrence with a shorter prerecurrent interval than that of the ordinary DFSP, and a hipbone metastasis was identified in one patient. These findings reflect the unfavorable course, in contrast to the ordinary DFSP. Microscopically, the FS areas were presented either in the initial lesion (five cases) or in the recurrent one (four cases), areas showing cellularity, mitotic figures, and cytologic anaplasia that exceeded those in the DFSP areas. Dermatofibrosarcoma protuberans with FS and allied categories such as SFS and SV MFH shared various clinical and histologic features but there was a diversity in the prognosis relative to the grade of malignancy. The relationship between DFSP with FS and the allied tumors was given attention.

Adult↗
PubMed1985

The fibroblastic nature of dermatofibrosarcoma protuberans: morphological investigations in vivo and in vitro.

Six cases of dermatofibrosarcoma protuberans were studied ultrastructurally. Biopsy material from all six cases as well as cell cultures derived from four cases were examined. In all cases, the tumor cells in vivo and in vitro were related to fibroblasts. The two cases with histiocytoid features and a small spiral pattern exhibited numerous lipid vacuoles but no histiocytic cell markers. In these two cases, cultured cells contained a higher number of intracytoplasmic vacuoles than the control fibroblasts. Two other cases exhibited some basement-membrane-like material surrounding tumor cells. All of the investigated cell strains obtained from the tumors showed synthesis of collagenous proteins similar to that found in fibroblasts. However, the level of total collagen was reduced, and type-III collagen was absent. The morphological variations in these cases of dermatofibrosarcoma protuberans appeared to be related to the histological pattern and may reflect the heterogeneity of normal fibroblasts.

Adult↗
PubMed1988

Vulvar dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans of the vulva is an extremely rare neoplasm. This low-grade sarcoma of the dermis clinically appears to be encapsulated but microscopically has tumor projections well away from the central nodules. Wide local excision is the recommended treatment for dermatofibrosarcoma protuberans; however, a more radical surgical approach may be needed to obtain clear surgical margins. The following case study present the fifth reported patient with this unusual neoplasm on the vulva.

Clitoris↗
PubMed1992

Dermatofibrosarcoma protuberans of the scalp.

Dermatofibrosarcoma protuberans is an uncommon tumour of the dermis and rarely occurs on the scalp; there are few known predisposing factors. A case is described of such a tumour that occurred at the site of radiotherapy for a basal cell carcinoma. Histological characteristics, behaviour and management of dermatofibrosarcoma protuberans are discussed.

Aged↗
PubMed1989

An unusual case of dermatofibrosarcoma protuberans.

A dermatofibrosarcoma protuberans involving the thumb of a nondominant hand is reported and represents the first case reported in this location. Dermatofibrosarcoma protuberans is a soft tissue neoplasm. It is a slow growing, locally aggressive fibrous tumor that occurs with equal frequency on the chest, abdomen, back, and proximal aspect of the extremities. Only two cases involving the hand are reported in the literature.

Adult↗
PubMed1988

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

A 79-year-old woman required amputation of her left leg for the control of a rapidly growing malignant fibrous histiocytoma. This tumor had developed at the precise site of repeated recurrence of a previously diagnosed dermatofibrosarcoma protuberans. Biopsy specimens from the recurrences revealed a gradual change in appearance from dermatofibrosarcoma protuberans to malignant fibrous histiocytoma.

Aged↗
PubMed1981

Dermatofibrosarcoma protuberans arising on the hand.

Dermatofibrosarcoma is a relatively common, locally aggressive, soft tissue fibrous histiocytoma. Ignorance of its marked propensity for locally invasive growth can lead to inadequate excision, and patients often suffer multiple recurrences. We present a case of dermatofibrosarcoma arising on the hand, and discuss its natural history and proper surgical therapy.

Adult↗
PubMed1987

Atrophic dermatofibroma and dermatofibrosarcoma protuberans.

Dermatofibroma and dermatofibrosarcoma protuberans usually are seen as firm nodules in the skin. We have observed several patients, all female, with an atrophic or depressed lesion on the chest or back. When biopsy was done, the biopsy punch was often noted to sink into the lesion. In each case the biopsy specimen showed a cellular dermatofibroma or dermatofibrosarcoma protuberans. The correct diagnosis was rarely suspected clinically. This uncommon clinical presentation may be related to the marked cellularity of the fibrous tumors in this series.

Atrophy↗