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PubMed1982

Malignant melanoma and dermatofibrosarcoma in a 60-year-old patient with lifelong acrodermatitis enteropathica.

The life course of acrodermatitis enteropathica is recorded in a 62-year-old white man. Initially saved in infancy by breast-feeding and good medical care, later in his twenties he responded well to diiodohydroxyquinoline (Diodoquin) therapy, his only residua being dermatitis, hoarseness, and short stature. Subsequently untreated, this patient years later developed not only a dermatofibrosarcoma but also a large amelanotic malignant melanoma. Both were successfully excised. Subsequently, oral zinc therapy initiated for the first time cleared his acrodermatitis, which had been present for 60 years. It is suggested that this patient's malignancies developed as a result of an immune deficiency state typically found in acrodermatitis enteropathica. On this basis, acrodermatitis enteropathica may be viewed as having a malignant potential over the long term. The zinc-dependent nature of the immune deficit, however, suggests that lifelong daily zinc supplementation is an appropriate prophylactic measure.

Acrodermatitis↗
PubMed1989

Dermatofibrosarcoma protuberans of the scalp: a series of cases.

Three cases of dermatofibrosarcoma protuberans on the scalp of young women are presented. The scalp is a rare but important anatomic site for this tumor because of the possibility of direct invasion into the skull and brain. The use of DNA analysis to predict biologic aggressiveness of a tumor is discussed.

Adult↗
PubMed1991

Dermatofibrosarcoma protuberans. A histological and ultrastructural study of 11 cases with emphasis on the study of recurrences and histogenesis.

Eleven cases of dermatofibrosarcoma protuberans (DFSP) (6 male: 5 female; mean age +/- SD: 48.7 +/- 16.1 years) were reviewed in an attempt to correlate the histological and ultrastructural findings with the recurrence pattern of these tumours and to shed light on their histogenesis. Six (54.5%) of the tumours recurred: 3 with a similar appearance to the primary tumours, 2 with fibroblastic malignant fibrohistiocytic pattern (F-MFH), and one with fibrosarcomatous features (F), confirmed ultrastructurally. In one primary tumour with associated F features there were two recurrences. The ultrastructural findings of 6 primary tumours (2 with and 4 without recurrences) showed a predominance of primitive mesenchymal cells. The comparison between the features (sex, age, site, necrosis, mitotic rate size) of recurring DFSP vs DFSP without recurrences did not yield any significant difference. At follow-up (range: 11-108 months) all patients were alive and well. These findings support the assumption that DFSP originates from primitive mesenchymal cells that are able to show, at least, F and F-MFH differentiation, and corroborate the low-grade malignancy of DFSP regardless of their macroscopic, histologic and ultrastructural characteristics.

Adult↗
PubMed1991

[Dermatofibrosarcoma protuberans of the face. A case report].

Report of a dermatofibrosarcoma protuberans (DFSP) of the face with a follow-up of more than 40 years in a 63-year-old women. A total of 64 operations and a radiotherapy were performed to treat the recurrences, which increased in number in the last 9 years. Defects were mostly closed primarily, with free grafts or local flaps. Two metastases, probably of yatrogenic origin, were found at the skin of the clavicula and the breast one year after a Bakamjian flap at the former site of the drains. Histology revealed mature fibroblasts growing in bundles, cart-wheel or storiform with destructive infiltration of the dermis. The DFSP is mainly a locally malignant tumor, growing slowly and painless as a flesh-coloured multinodular mass localised in the dermis. Local recurrence occurs in more than 50% of the cases, whereas metastases are very rare.

Combined Modality Therapy↗
PubMed1976

The histiocytic nature of dermatofibrosarcoma protuberans. Tissue culture and electron microscopic study.

Specimens from four cases of dermatofibrosarcoma protuberans when explanted in vitro exhibited a typical ameboid outgrowth, confirming the histiocytic nature of these tumors. The cultures displayed the same evolution from macrophages to fibroblast-like cells observed previously in cultures of other types of histiocytomas. Electron microscopic study of the cultures confirmed the light microscopic findings and suggested that the fibroblast-like cells were histiocytes with some secondarily acquired fibroblastic features. The possibility that neoplastic histiocytes may transform into true fibroblasts was deemed unlikely, but could not be excluded.

Adult↗
PubMed1992

Immunohistochemical characterization of atypical fibroxanthoma and dermatofibrosarcoma protuberans.

Atypical fibroxanthoma (AFX) and dermatofibrosarcoma protuberans (DFSP) have generated undue interest regarding their histogenesis, biological behavior, and differentiation from other forms of spindle cell tumors of the skin, including spindle cell squamous carcinomas and desmoplastic melanomas. To identify characteristic immunophenotypes, 12 AFXs and 15 DFSPs were examined with a panel of antibodies against cytokeratin; vimentin; desmin; proteolytic enzymes (alpha-1-antitrypsin and alpha-1-antichymotrypsin); melanoma-associated antigens defined by HMB-45, HMB-50, and NKI/C3; muscle-specific actin (HHF-35); and S-100 protein. The staining patterns of these two tumors were nearly identical. All cases tested negative for cytokeratin, desmin, and S-100 protein and strongly positive for vimentin. Six (50%) AFXs and 12 (80%) DFSPs tested focally positive for muscle-specific actin. None of the cases were reactive with melanoma antibodies HMB-45 and HMB-50; NKI/C3 strongly stained 26 of 27 tumors. Compared to HMB-45 and HMB-50, NKI/C3 cross-reacted with nonmelanocytic neoplasms. Two AFXs stained for alpha-1-antitrypsin and alpha-1-antichymotrypsin. This study confirms (1) the immunophenotypic similarity of AFX and DFSP, (2) the presence of myofibroblastic differentiation in both tumors, as reflected by HHF-35 staining, and (3) that AFX and DFSP are easily distinguished from spindle cell squamous carcinoma and desmoplastic melanoma by the absence of cytokeratin, HMB-45, and HMB-50 staining.

Adult↗
PubMed1983

Dermatofibrosarcoma protuberans metastatic to lymph nodes and showing a dominant histiocytic component.

Dermatofibrosarcoma protuberans is a neoplasm composed of cells that have morphologic characteristics of fibroblasts and histiocytes. We report the eighth documented case of dermatofibroma protuberans metastatic to regional lymph nodes. The metastases consisted predominantly of cells that appeared to be atypical histiocytes. This case is considered to be an example of dermatofibroma protuberans in which primitive histiocytic cells have not acquired secondarily acquired fibroblastic characteristics.

Adult↗
PubMed1990

An immunohistochemical study of dermatofibrosarcoma protuberans supports its fibroblastic character and contradicts neuroectodermal or histiocytic components.

Paraffin-embedded material from 26 cases of dermatofibrosarcoma protuberans (DFSP) was investigated by the peroxidase-antiperoxidase technique. Antibodies to S-100 protein, Leu-7 antigen, and neuron-specific enolase (neural markers); to lysozyme, alpha-1-antitrypsin, and alpha-1-antichymotrypsin (histiocytic markers); and to cytokeratin, desmin, vimentin, and factor VIII were used. The tumor cells reacted only for vimentin. In addition, 12 cases showed positive reactions with histiocytic markers (1-3% of the cells; in two cases, up to 10%). These results support a fibroblastic and contradict a neural or histiocytic histogenesis of DFSP.

Fibroblasts↗
PubMed1988

Fibrosarcomatous change in dermatofibrosarcoma protuberans.

This report describes six patients with dermatofibrosarcoma protuberans (DFSP) that contained fibrosarcomatous areas (FS). The clinical signs and symptoms, ages of the patients, and anatomic distribution of the tumors were similar to those of uncomplicated DFSP. FS was concentrated in the subcutis in each case and comprised more than 50% of the tumor in four cases. The characteristic storiform cellular arrangement of DFSP was replaced by long, gently sweeping fascicles of spindle cells that intersected at various angles, forming the so-called herringbone pattern. Trapped fat cells, characteristic of DFSP when it infiltrates subcutaneous tissue, were absent in five of the six FS and only focally present in one. Two FS were grade 1; their cytologic features were similar to those of DFSP. Four FS were grade 2 and had cytologic atypia exceeding that of DFSP. There was a statistical difference between the mitotic rates of DFSP and FS. Five patients were alive and well at the time of last follow-up (median, 2 years), and one patient had an unexcised recurrence when last examined. Six similar cases from the literature are reviewed; in one of them, the FS metastasized.

Fibrosarcoma↗
PubMed1994

Deep penetrating dermatofibroma versus dermatofibrosarcoma protuberans. A clinicopathologic comparison.

A study of the clinical, histological, and immunohistochemical features of 20 cases of deep penetrating dermatofibroma (DPDF) and eight cases with 14 specimens (eight primary, one reexcision, five secondary tumors) of dermatofibrosarcoma protuberans (DFSP) showed distinct entities. Clinically, DPDF usually appeared as a nodule (approximately 2 cm) of the (lower) limbs, whereas DFSP affected the trunk (shoulder) with irregularly arranged plaques or nodules (> 5 cm). Histologically, DPDF showed a regular silhouette with a smooth, nodular (four of 20) or scalloped (16 of 20) lower margin and variable sclerosis (nine of 20); DFSP, irregularly infiltrated fatty tissue in a lacelike/honeycomb (eight of 14), multilayered (three of 14), or mixed pattern (three of 14), but without sclerosis. Immunohistochemically, DPDF was mostly negative with QBEnd 10 (CD34; 18 of 20) but positive for factor XIIIa (17 of 20), actin (HHF35; 10 of 20), and metallothionein (MT; 12 of 20). DFSP was positive for CD34 (13 of 14), yet with some sparing of central tumor parts, highly cellular tumor nodules, and myxoid areas; factor XIIIa and MT were consistently negative, as was HHF35 in 11 of 14 cases. In a multivariate analysis of histologic and immunohistochemical criteria, the combination of sclerosis and labeling with MT was most valid (p = 0.0001) for diagnosis: all DPDF showed either labeling with MT in "early" (metabolically active) lesions or sclerosis in "late" lesions, not present in DFSP.

Adult↗
PubMed1993

Clonal origin of dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) is a malignant tumor originating in the dermis. Although it is known to be locally aggressive, it only rarely metastasizes and will recur unless completely excised. The exact cell responsible for the development of a DFSP has been a matter of controversy for several decades; however, most histochemical and electron microscopic studies support a fibroblastic origin, with the tumor cells staining uniformly for vimentin and containing active endoplasmic reticulum synthesizing collagen. Cytogenetic analysis of some of these tumors has demonstrated at least two specific chromosomal abnormalities in DFSP and suggested that this tumor may be polyclonal in origin. To further address the clonal origin of this locally invasive, mesenchymal tumor, we analyzed DNA from two female patients by restriction fragment length polymorphisms and methylation analysis. Our data strongly support the concept that DFSP is monoclonal in origin and that this tumor mass reflects the clonal expansion of a single cell.

Adult↗
PubMed1989

Establishment of a human dermatofibrosarcoma protuberans cell line: cytological characteristics.

We established a dermatofibrosarcoma protuberans (DFSP) cell line derived from a DFSP tumor from a 69-year-old female. The population doubling time of the cells was about 45 hours. The cells were mostly round, although a few triangular or spindle-shaped cells were also found. They could not be transplanted into nude mice. These cells had some histiocyte characteristics which are not observed in fibroblasts: 1) Electron microscopically, there were more lysosomes and phagolysosomes than in fibroblasts. Dense bodies and myelin figures derived from lysosomes were also observed. 2) Staining with acid phosphatase (ACPase), esterase, and alpha-1-antitrypsin was positive. 3) The cells had C3 receptors but no Fc receptors. However, there were two differences between DFSP cells in the established line and those in the early stages: in the former, the capacity for immunophagocytosis (Fc receptors and phagocytosis of Candida albicans) and collagen synthesis were almost entirely absent.

Aged↗
PubMed1992

Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34.

Dermatofibrosarcoma protuberans (DFSP) is a slow growing, locally invasive tumour whose differentiation from other fibrohistiocytic tumours sometimes poses serious diagnostic problems. We investigated CD34 expression immunohistologically in various fibrohistiocytic tumours including dermatofibroma, DFSP, malignant fibrous histiocytoma (MFH), infantile myofibromatosis, fibrosarcoma, hypertrophic scar and keloid. Among these, DFSP was unique in that tumour cells themselves expressed CD34, whereas in other tumours. CD34 expression was observed only on vascular endothelial cells amongst the tumour cells. Until now, there have been no reports of useful immunohistological markers for DFSP. CD34 expression by the tumour cells can be an extremely useful marker in establishing a definitive diagnosis of DFSP.

Antigens, CD↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans.

In view of the important clinicopathologic, evolutional, and ultrastructural characteristics of dermatofibrosarcoma Protuberans, a review was made of 30 patients seen during the last 25 years in Colombia's Instituto Nacional de Cancerologia. The incidence is low, accounting for 0.06% of all malignant tumors and 1.17% of all soft-parts sarcomas. The series comprises 19 women and 11 men, with the highest incidence found between ages of 30 and 50 years, although the study includes two children, ages 8 and 13. The chest was the most common site. Forty percent of the lesions were multinodular, and 23.3% showed ulceration. Histologic appearance is characterized in all cases by fusiform cells arranged like spokes around an empty hub. The fibroblastic origin was ascertained by electron-microscopic studies. The treatment used was broad surgical excision, removing tissue down to the aponeurosis to avoid recurrence.

Adolescent↗
PubMed1975

An autopsy case of metastasizing protuberant dermatofibrosarcoma.

An autopsy case of metastatic dermatofibrosarcoma protuberans (DP) together with the results of four sequential biopsies performed during his illness was reported. In this case, the tumor recurred repeatedly for 38 years in spite of intensive treatments. Finally, the tumor metastasized to the bilateral lungs, retroperitoneum and lumbar vertebrae. The direct cause of death was renal stones which may have been related to paraplegia of the lower limbs due to the vertebral metastasis. The metastasis appeared to have started at the time when the tumor showed marked atypism histologically. It was also recognized that there was an obvious difference in atypism between recurrent and metastatic tumors.

Fibrosarcoma↗