1/180. Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy. Bednar tumor is a rare pigmented variant of dermatofibrosarcoma protuberans (DFSP). Because of its rarity, information is lacking regarding the optimal therapy and potential utility of immunohistochemistry in diagnosis. We report a case of Bednar tumor in which the diagnosis was aided by immunohistochemistry for CD34, an antigen known to be expressed in DFSP but not previously reported in Bednar tumor. Our case was also striking because it represents the first reported appearance of a Bednar tumor at a site of prior immunization, a phenomenon previously noted in some cases of DFSP. The patient was treated effectively with mohs surgery and is without recurrence at 9 months. ( info) |
2/180. The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases. dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment. ( info) |
3/180. Acral dermatofibrosarcoma protuberans with metastases. dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor that occurs rarely on the extremities. This tumor has a particular propensity for local recurrence following seemingly adequate excision. Metastases are rare, and usually follow repeated local recurrences. On rare occasions, fibrosarcomatous change may arise in a DFSP, and appears to affect adversely the prognosis. The adequacy of the initial resection is the most important prognostic factor; however, suggested margins vary between 1.5 and 5 cm. The ideal margin of resection on the foot is unknown. We report a patient with recurrent, acral DFSP with fibrosarcomatous change and pulmonary parenchymal metastases in a 48-year-old black male. ( info) |
| We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor. ( info) |
5/180. A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences. dermatofibrosarcoma protuberans (DFSP) is a cutaneous tumour of borderline malignancy, the cytogenetic features of which include the translocation t(17;22)(q22;q13) or, more commonly, supernumerary ring chromosomes containing material from 17q22 and 22q13. These rearrangements result in the COL1A1/PDGFB fusion gene. Here, we describe a case of DFSP displaying a ring chromosome 5 together with a large marker chromosome composed of chromosome 22 alphoid dna, material from distal 12q and amplified COL1A1 and PDGFB sequences. This is the first case of DFSP with multiple copies of COL1A1 and PDGFB not confined to ring chromosomes, showing that DFSP is similar to other borderline malignant mesenchymal tumours, where rings and giant markers are alternative vehicles for amplified material. ( info) |
6/180. dermatofibrosarcoma protuberans: an early non-protuberant phase of the tumour. A 40-year-old female patient presented with a flat scar like plaque on the upper chest. The patient's perception of subtle change in the lesion was of paramount importance in the decision to biopsy this lesion of innocuous clinical appearance. Histopathological findings were those of dermatofibrosarcoma protuberans. This case illustrates the early clinical features of the tumour and a recommendation for taking a biopsy of any scar-like plaque where there is no clear history of preceding trauma. ( info) |
7/180. Adjuvant therapy with hyaluronidase prior to excision of dermatofibrosarcoma protuberans. BACKGROUND: dermatofibrosarcoma protuberans (DFSP) are uncommon, low grade cutaneous malignancies, which rarely metastasize. Although surgery is the main therapy for these tumors, they commonly recur in spite of wide excisions. Hyaluronidase (HD) has been used both intralesionally as well as intravenously as an adjuvant in therapy of both epithelial and mesenchymal malignancies to decrease local spread, to potentiate differentiation induced apoptosis, and to potentiate the tissue penetration of chemotherapeutic agents. In addition, there is a long history of intralesional HD use without significant complications. The biologic characteristics of DFSP including the observation that hyaluronic acid is commonly present and sometimes is increased in these tumors and the known effects of HD therapy in other malignancies suggested to us that HD pretreatment may be a helpful adjuvant in the surgical management of DFSPs. Following diagnostic biopsy of a recurrent DFSP, the patient was treated for 4 weeks with intralesional HD injections. RESULTS: Based on margins necessary for complete excision from past studies, this patient had decreased margin width and thus had a postoperative wound size less than was expected. The patient has had no evidence of recurrence at 24 months follow-up. ( info) |
8/180. dermatofibrosarcoma protuberans harboring t(9;22)(q32;q12.2). More than 20 cases of dermatofibrosarcoma protuberans (DFSP) exhibiting chromosomal abnormalities have been reported. Approximately three fourths of these tumors have harbored supernumerary ring chromosomes, which have been suggested to be specific for this tumor. However, a small number of DFSPs with translocations such as t(2;17), t(X;7), and t(17;22) have recently been reported. We report a DFSP arising in a 23-year-old woman which unexpectedly exhibited the balanced translocation, t(9;22)(q32;q12.2) as the only anomaly with G-band technique. Dual-color fluorescence in situ hybridization (FISH) confirmed these cytogenetic findings. Similar to that previously reported for DFSPs with translocations, the present tumor also lacked ring chromosomes. ( info) |
9/180. An ultrastructural and immunohistochemical study of pigmented dermatofibrosarcoma protuberans (Bednar tumor). A case of Bednar tumor on the right shoulder of a 47-year-old Japanese woman is reported. Histological examination showed plump, spindle cells arranged in a storiform pattern in central areas of the tumor and a diffuse infiltration of the dermal stroma, which was frequently extended into the subcutis at the periphery of the tumor. The tumor contained a fairly identified population of dendritic pigmented cells. Ultrastructurally, most cells had folded nuclei, were spindle-shaped and had long, slender cytoplasmic projections. Dendritic pigmented cells, which were dispersed among neoplastic cells, contained premelanosomes and mature melanosomes. Immunohistochemically, tumor cells exhibited positive reactions for vimentin and CD 34 and failed to show a positive reaction for neuron specific enolase, HMB-45 or S-100 protein. factor x IIIa was only expressed on tumor cells around melanin-containing cells, which reacted positively with antibodies to S-100 protein and vimentin. These results indicate that the phenotype of tumor cells around melanin-containing cells differs from other tumor cells and that this difference may be caused by the relationship of tumor cells and melanin-containing cells. ( info) |
10/180. dermatofibrosarcoma protuberans. A rare case involving the pediatric foot. dermatofibrosarcoma protuberans is a rare, slow-growing, locally destructive, intermediate-grade malignancy that originates in the dermal layer of the skin. dermatofibrosarcoma protuberans rarely metastasizes and has a marked tendency to recur following local surgical excision. The lesion is found most commonly on the trunk, on the proximal aspect of both the lower and upper extremities, and in the head and neck region. dermatofibrosarcoma protuberans occurs most commonly in people between the ages of 20 and 50. This article describes the clinical and pathologic features of this rare, malignant neoplasm and describes a rare case of dermatofibrosarcoma protuberans involving the pediatric foot. ( info) |