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PubMed1978

Histogenesis of dermatofibrosarcoma protuberans. An ultrastructural study.

Ultrastructural study of five typical lesions of dermatofibrosarcoma protuberans revealed that the basic cell is fusiform and has a somewhat indented nucleus and an even distribution of cytoplasmic organelles. Fragments of basal lamina, intercellular junctions, abundant intercellular fibers, and a tendency to form cellular sheaths also were found. The tumor cells had a strong resemblance to pericytes and perineural cells, as well as marked ultrastructural similarities to neurofibroma. The authors conclude that dermatofibrosarcoma protuberans arises from a primitive fibroblastic cell with a "sheath forming cell" differentiation that has a closer relationship to the perineural cell than to the pericyte.

Fibrosarcoma↗
PubMed1983

Myxoid variant of dermatofibrosarcoma protuberans.

We describe two cases of dermatofibrosarcoma protuberans that were entirely myxoid when excised initially. The tumors had the following distinctive histologic features: 1) a monotonously haphazard arrangement of spindle-shaped and stellate tumor cells; 2) little variation in cellularity from field to field; 3) pale basophilic to clear stroma containing hyaluronic acid; 4) minimal cytologic pleomorphism and a low mitotic rate; 5) a dispersed, nonpatterned vasculature; 6) eccrine structures and fat cells trapped and surrounded by tumor tissue; and 7) areas where spindled cells had a parallel arrangement evocative of neurofibroma. A cutaneous tumor with the foregoing features should be carefully examined for subtle, radial arrangements of tumor cells and a tentacular or course, web-like infiltration of any attached fat. Use of the foregoing criteria will allow distinction between myxoid dermatofibrosarcoma protuberans and other myxoid tumors of the skin.

Adipose Tissue↗
PubMed1985

Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study.

Described by Bednar as a "storiform neurofibroma," pigmented dermatofibrosarcoma protuberans is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans (DFSP). The lesion commonly presents as an exophytic, multinodular neoplasm of the dermis or subcutaneous tissue. It occurs predominantly in blacks. The majority are located on the trunk, and the remainder are more or less equally distributed in the upper and the lower extremities and the head and neck. Microscopically the lesion is characterized by spindled cells arranged in a tight storiform pattern and admixed with a small population of melanin-containing dendritic cells. The dendritic cells are the primary feature distinguishing this lesion from conventional DFSP. Three cell populations are identifiable by electron microscopy. The majority of cells resemble fibroblasts. A second population of cells exhibits long slender cell processes partially or completely invested by basal lamina. The third population of cells, also invested by basal lamina, contains both melanosomes and premelanosomes. The histogenesis of this neoplasm remains controversial. Although Bednar considered these lesions as variants of neurofibroma, S-100 protein could not be identified, and this finding contrasts significantly from the description of conventional neurofibroma, which almost always contains this antigen. Follow-up information available in nine cases indicates that this lesion may recur locally. Although distant metastases were not observed in our material, complete excision in conjunction with close follow-up care is indicated for this neoplasm of probable intermediate malignant potential.

Adolescent↗
PubMed1990

Dermatofibrosarcoma protuberans of the toe.

Dermatofibrosarcoma protuberans is an unusual soft-tissue tumor with a propensity for local recurrence and occasional metastatic spread. Given its indolent course, it may be mistaken for atypical scarring or a keloid growth. Most lesions are trunkal or on the proximal extremities with digital lesions being extremely rare. There is only one previous reference of the tumor arising on a toe. Early diagnosis and prompt wide local resection of this entity are required to prevent a local recurrence, which would necessitate a wider, more debilitating resection. The management of a patient with a dermatofibrosarcoma protuberans of the second toe is presented along with a review of the literature.

Adult↗
PubMed1988

Treatment of dermatofibrosarcoma protuberans with Mohs micrographic surgery.

Ten patients with dermatofibrosarcoma protuberans, all of whom had been treated previously by conventional excisional surgery without success, were treated with Mohs micrographic surgery. A team approach utilizing margin control by Mohs-trained physicians and reconstruction by surgical specialists was employed. Average follow-up exceeds 3 1/2 years, with no recurrences. Microscopically controlled excision appears to be the treatment of choice for dermatofibrosarcoma protuberans.

Adolescent↗
PubMed1993

Dermatofibrosarcoma protuberans of the chest and the shoulder: wide and deep excisions with immediate reconstruction.

Dermatofibrosarcoma protuberans is a rare malignant tumor of the skin. The only satisfactory treatment is surgical and consists of a wide excision. In our department, we have established the limits of the excision required to be a margin of 5 cm on the surface, whereas in the depth, we excise the first healthy anatomical structure encountered. Latissimus dorsi flap reconstruction was used for the major tissue deficit for 15 patients with dermatofibrosarcoma protuberans of the chest and the shoulder. When compared to skin grafting, this technique results in an improved aesthetic appearance and a stable cover for the periosteumless clavicle. Fourteen patients were followed for an average of 37.6 months and all remained free of disease.

Adolescent↗
PubMed1985

Dermatofibrosarcoma protuberans: altered collagen metabolism in cell culture.

Dermatofibrosarcoma protuberans is a low-grade malignant tumor that grows invasively but rarely forms metastases. Its origin is still controversial. We characterized the synthesis of collagen in detail in cells which were obtained from dermatofibrosarcoma protuberans tumors by enzymatic tissue disintegration. Similar to fibroblasts, all tumor cell strains produced considerable amounts of collagen. However, the rate was reduced compared to normal skin fibroblasts. Cells grown from the tumors synthesized type I collagen, but no type II could be detected. After serial passaging the cultures started to produce type III collagen, which is probably due to a slow overgrowth by normal fibroblasts.

Cells, Cultured↗
PubMed1985

Dermatofibrosarcoma protuberans: a clinicopathological and immunohistochemical study with a review of the literature.

Forty-one cases of dermatofibrosarcoma protuberans are presented. The clinical features and histopathological appearances are described. Immunohistochemical staining of thirteen cases with antisera to lysozyme, alpha 1-antichymotrypsin and S-100 protein has provided no evidence to support either a histiocytic or neuroectodermal origin for these tumours. In reviewing the literature, the histogenetic origin, differential diagnosis and malignant potential of dermatofibrosarcoma protuberans are discussed.

Adolescent↗
PubMed1982

Dermatofibrosarcoma protuberans and its tissue culture study -- ultrastructural, enzyme histochemical and immunological study.

Dermatofibrosarcoma protuberans arizing in the thigh of a 23-year-old female is reported with an investigation of tumor cell character by electron microscopy, enzyme histochemistry, immunological and in vitro tissue culture techniques. The tumor tissue showed histologically a peculiar storiform arrangement of plump spindle cells with dense proliferation. Electron microscope revealed histiocytic cell structures of some tumor cells: lysosomes, phagosomes, and sparse rough endoplasmic reticula. They revealed diffuse positive reactions to acid phosphatase (Ac Pase) and non-specific esterase. Short-time culture of the tumor tissue indicated rather round and polygonal cells admixed with spindle cells, positive to Ac Pase and non-specific esterase with diffuse reaction. Most of the round cells possessed Fc and C3 receptors on their surface and phagocytic activity. Dermatofibrosarcoma protuberans is probably a histiocytic tumor and structural characteristics of schwann cells were not recognized.

Adult↗
PubMed1988

Dermatofibrosarcoma protuberans.

We report the case a 69-year-old woman with dermatofibrosarcoma protuberans, present, apparently, since birth. To our knowledge, this case represents the longest reported duration of a dermatofibrosarcoma protuberans.

Aged↗
PubMed1992

Dermatofibrosarcoma protuberans metastatic to the lung. A case report.

A case is presented of dermatofibrosarcoma protuberans of the gluteal region with metastasis to the lung appearing 7 years after wide excision of the primary lesion. The world literature is reviewed. The clinical and pathologic features of dermatofibrosarcoma are reviewed and treatment is discussed, with the aim of emphasizing the need for long-term follow-up examination of lymph nodes and for metastases following wide excision of these lesions.

Adult↗
PubMedDate not supplied

Dermatofibrosarcoma protuberans: 4 years after local trauma.

Dermatofibrosarcoma protuberans (DFSP) is a low grade, fibrohistiocytic malignant soft tissue tumor that arises infrequently in the foot and ankle region. It most frequently demonstrates a "storiform" histologic pattern, and if incompletely excised, may be recurrent, more anaplastic and metastatic. Soft tissue plain film radiography, as well as magnetic resonance imaging (MRI) help outline the tumor and involved tissues, which is beneficial in staging, determining surgical approach and evaluation of therapy. Definitive diagnosis is made by biopsy. A dermatofibrosarcoma protuberans is reported in the foot of a 14-year-old female patient, 4 years after trauma to the site.

Adolescent↗
PubMed1990

[A case report of very huge dermatofibrosarcoma protuberans].

A 63-year-old male with dermatofibrosarcoma protuberans of the right hip is presented. The patient noticed a small nontender mass 40 years ago and then the tumor enlarged gradually. The patient was very emaciated and needed to receive blood transfusions. The massive multinodular tumor was pedunculated and on it's surface, there were areas of hemorrhage and secondary infection. The operative specimen weighted 2270 g and measured up to 25 by 20 by 10 cm. To our knowledge, this case represents the largest primary tumor of a dermatofibrosarcoma protuberans in review of English literature.

Fibrosarcoma↗
PubMed1988

[Immunohistochemical studies of the histogenesis of dermatofibrosarcoma protuberans].

Paraffin-embedded tissue sections taken from 16 patients with dermatofibrosarcoma protuberans were stained by means of the peroxidase-antiperoxidase technique using antibodies against S 100 protein, NSE, Leu 7, lysozyme, alpha-1 antitrypsin, alpha-1 antichymotrypsin, cytokeratin, desmin, vimentin, and factor VIII. Most of the tumor cells showed positive reactions to vimentin. Only 1-3% of the cells within the tumor area answered to the histiocytic markers lysozyme, alpha-1 antitrypsin, and alpha-1 antichymotrypsin. The remaining antibodies investigated did not react with the tumor cells. Our results support a fibroblastic, and contradict a neural or histiocytic, histogenesis of dermatofibrosarcoma protuberans.

Antibodies, Monoclonal↗
PubMed1988

[Dermatofibrosarcoma protuberans. Results of radiosurgical therapy by photon and electron rays or fast neutrons].

The dermatofibrosarcoma protuberans, a rare entity of soft tissue sarcomas, is specified as a tumor of intermediate malignancy because of its marked tendency to form local recurrences. Regarding the prevention of local recurrences, the results of combined radiosurgical therapy (n = 13) could be improved in our patients by the application of fast neutrons (n = 6). Despite multiple previous operations for recurrences, a reliable prevention of local recurrences (average follow-up period 5.5 years) was achieved in all patients by neutron irradiation with focal doses of approximately 9 Gy. One patient with multiple macroscopic recurrences received two neutron series with 17 Gy. At seven years, she is still recurrence-free. We consider the postoperative irradiation of the dermatofibrosarcoma protuberans with fast neutrons following to primary tumor excision as method of choice for a reliable prophylaxis of local recurrences, because this method allows to dispense with major radical surgery.

Adult↗
PubMed1988

[Traumatically-induced dermatofibrosarcoma protuberans of the breast].

Dermatofibrosarcoma protuberans is a rare, cutaneous-subcutaneous low grade malignancy. Despite the locally aggressive behaviour, metastases seldom occur. Evidence exists that trauma may be a predisposing factor. Treatment by wide tridimensional excision is recommended. One case of trauma-induced dermatofibrosarcoma protuberans in a 71-year old woman is reported.

Aged↗
PubMedDate not supplied

Fine needle aspiration cytology of metastatic dermatofibrosarcoma protuberans. A case report.

Report is made of the fine needle aspiration (FNA) cytologic detection of a rare pulmonary metastasis of dermatofibrosarcoma protuberans, a locally aggressive tumor with a low metastatic potential but a high propensity for recurrences. The cytologic findings paralleled those seen on tissue sections and were characterized by tissue fragments displaying a storiform pattern and slender, spindle-shaped cells. Histiocytic differentiation and mitotic figures were also detected in the cytologic preparations. It is concluded that metastatic dermatofibrosarcoma may be accurately diagnosed by FNA cytology.

Aged↗