1/35. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/35. Composite cervicofacial flap for reconstruction of complex cheek defects.The authors present the reconstructive technique for complex cheek defects using the composite cervicofacial flap and study the possibilities, advantages, disadvantages, and results that can be expected. The design follows the classic outline of Mustarde's flap. The skin is undermined for 2 cm anterior to the ear, then after incision of the superficial musculoaponeurotic system (SMAS), undermining is continued below the plane of the SMAS, level with the facial nerve branches. It is continued forward to the facial vessels, which give rise to branches that ensure the blood supply of this composite flap and contribute to its high reliability. In the cervical region, undermining is done beneath the platysma, which is transected transversely in the lower cervical region to allow good upward mobility and satisfactory transposition of the flap. The flap is adapted to the defect and the medial suture line is placed as near as possible to the medial limit of the cheek aesthetic unit. The authors carried out a retrospective study of 7 patients with complex facial reconstruction after excision of malignant lesions. The defects measured from 4x4 cm to 9x7 cm. In 4 patients excision included the periosteum, and in 1 patient excision involved the entire thickness and removed the entire anterior half of the cheek. In 4 patients reconstruction involved the cheek and eyelid. In spite of the advanced age of the patients (88, 69, 91, 67, 70, 82, and 59 years), there was no distal edge necrosis. The only complication was a single case of facial paresis, which resolved spontaneously. The results were considered very good in all 7 patients. The authors conclude that the composite flap increases the possibilities of the cervicofacial flap. It is more mobile, more reliable, thicker, and more adaptable. It can be used in complex cheek defects that involve the periosteum, or even in full-thickness defects. The quality of the results obtained using this flap represents a considerable advance in facial reconstruction.- - - - - - - - - - ranking = 0.12597574998876keywords = complex (Clic here for more details about this article) |
3/35. FNAC in the diagnosis of recurrent dermatofibrosarcoma protuberans of the forehead. A case report.BACKGROUND: dermatofibrosarcoma protuberans is a rare cutaneous soft tissue tumor of intermediate malignant potential with a characteristic tendency for recurrence. Metastases are unusual. This tumor usually occurs in the trunk and extremities and, infrequently, on the face and scalp. Its cytologic appearance on fine needle aspiration has only been rarely reported. It is characterized by numerous fibroblastlike cells that arrange as single cells or in clusters of spindle cells arrayed in a storiform pattern. CASE: A 42-year-old male presented with a one-year history of an enlarging left forehead mass (lateral brow) that was adjacent to an old surgical scar. Fine needle aspiration revealed a low grade spindle cell neoplasm morphologically identical to a dermatofibrosarcoma protuberans excised 15 years earlier, indicating tumor recurrence. CONCLUSION: Distinguishing dermatofibrosarcoma protuberans from other spindle cell tumors and fibrohistiocytic lesions may pose significant challenges to the pathologist. However, in the appropriate clinical setting and applying strict diagnostic criteria, fine needle aspiration cytology is a reliable tool in establishing the diagnosis of this neoplasm.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
4/35. Radial forearm free tissue transfer for head and neck reconstruction: versatility and reliability of a single donor site.Since its description as a free flap, the radial forearm flap has undergone numerous modifications for reconstruction of various defects in the head and neck region. Fasciocutaneous, adipofascial, osteocutaneous, tendinofasciocutaneous, or osteotendinofasciocutaneous flaps may be designed and transferred from the radial forearm. This article illustrates the versatility and reliability of this donor site in 15 patients with a variety of head and neck oncologic defects who underwent immediate (12 patients) and delayed (3 patients) reconstruction using different free flaps from the radial forearm. skin flaps were used in 11 patients (73.3%) with floor of mouth (4 cases), hemiglossectomy (2 cases) and partial maxillectomy (2 cases) defects, and for scalp (1 case), lower lip (1 case) and a central face (anterior maxilla/upper lip/nasal) (1 case) defect. Osteocutaneous flaps were used in four patients (26.6%) for reconstruction of bilateral subtotal maxillectomy defects (2 cases), a complex forehead and nasal defect (1 case), and for mandible reconstruction (1 case). In addition, the palmaris longus tendon was included with the flap in the two patients that required oral sphincter reconstruction. One patient required reexploration due to vein thrombosis, and no flap failures were detected in this series. The donor site healed uneventfully in all patients, except one, who had partial skin graft failure. Because of their multiple advantages, free flaps from the radial forearm have a definite role for reconstruction of head and neck defects. New applications of composite flaps from this donor site may continue to emerge, as illustrated in some of our patients.- - - - - - - - - - ranking = 0.017996535712681keywords = complex (Clic here for more details about this article) |
5/35. Bednar tumor of the foot: a case report.Pigmented dermatofibrosarcoma protuberans (Bednar tumor) is a rare neoplasm accounting for approximately 1-5% of all cases of dermatofibrosarcoma protuberans. The majority occurs on the trunk, and the remainders are more or less equally distributed in the upper and lower extremities and the head and neck. Microscopically it 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 that distinguish this lesion from conventional dermatofibrosarcoma protuberans. We report here a case of Bednar tumor occurring on the dorsal aspect of the foot in a young female.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/35. Giant cell fibroblastoma: a report of three cases with histologic and immunohistochemical evidence of a relationship to dermatofibrosarcoma protuberans.PROBLEM CONSIDERED: Giant cell fibroblastoma (GCF) is a rare mesenchymal neoplasm, which is classified as a fibrohistiocytic tumor of intermediate malignancy owing to its propensity for local recurrence, although metastasis has not been documented. Prior reports have linked GCF to dermatofibrosarcoma protuberans (DFSP), given overlapping clinical and histologic features. methods: This report documents three additional cases of GCF that further support the contention that this lesion is histogenetically related to DFSP. RESULTS: All three lesions occurred on the trunk of patients whose ages were 4, 28, and 38 years. One case that histologically resembled a GCF on initial excision recurred with areas of both GCF and DFSP. A second recurrence was composed entirely of DFSP. Another case contained areas of both GCF and DFSP, as well as a focus that was felt to be undergoing fibrosarcomatous change. The third case consisted entirely of GCF. Immunohistochemically, all three lesions showed intense immunoreactivity for CD34 in the GCF component. CD34 also strongly marked the cells in those cases with a DFSP component. CONCLUSIONS: Although GCF may not represent the "juvenile form" of DFSP, as previously suggested, the evidence strongly supports a histogenetic relationship between these two lesions, even though the cell of origin remains obscure.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/35. Fine needle aspiration cytology of dermatofibrosarcoma protuberans presenting as a breast mass. A case report.BACKGROUND: dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous soft tissue neoplasm with a tendency to recur but rarely metastasize. It occurs at almost any site but usually in the trunk and extremities. DFSP mimicking a primary breast lesion has not been reported before. CASE: A 30-year-old female presented with an eight-month history of a breast mass that was aspirated, revealing a spindle cell neoplasm. The diagnosis of DFSP was made on excisional biopsy. CONCLUSION: The diagnosis of DFSP may be problematic, especially when it presents clinically as a primary breast lesion.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
8/35. Pediatric pigmented dermatofibrosarcoma protuberans (Bednar tumor): case report and review of the literature with emphasis on the differential diagnosis.dermatofibrosarcoma protuberans (DFSP) is a fibrous tumor of intermediate malignant potential that usually affects the trunk of young to middle-aged adults. On histological examination, it is characterized by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. Bednar tumor (BT), formerly known as storiform pigmented neurofibroma, is currently considered the pigmented variant of DFSP due to the histological and cytogenetic similarities between these two lesions. There are very few reports on BT affecting pediatric patients. We describe a case of BT affecting the dorsal aspect of the left forearm of a 6-year-old-male patient and emphasize the diagnostic clues to distinguish this unusual cutaneous neoplasm from other pigmented lesions, including pigmented (melanotic) neurofibroma (PMN), psammomatous melanotic schwannoma (PMS), neurocristic cutaneous hamartoma (NCH), and desmoplastic malignant melanoma (DMM). We would like to stress that surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of BT as there is the risk of misdiagnosing it either as pigmented tumors associated with neurocutaneous syndromes, such as PMN and PMS, or as a highly malignant melanocytic neoplasm (DMM).- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
9/35. Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as Bednar tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma.We report an unusual case of composite giant cell fibroblastoma-dermatofibrosarcoma protuberans (DFSP) that, in its second recurrence, contained a pattern of Bednar tumor (BT) and giant cell fibroblastoma (GCF). The recurrent tumor showed extreme myxoid change with creation of mucoid lakes, which mimicked a pattern of myxoid liposarcoma. One area in the recurrent lesion contained amputation neuroma overgrown with neoplastic spindle cells, which simulated a nerve sheath neoplasm. This case demonstrates common histogenesis of GCF, DFSP and BT, and it shows how broad morphological spectrum can be produced by a composite tumor, especially when the tumor includes unconventional growth pattern or additional non-neoplastic lesion.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/35. dermatofibrosarcoma protuberans and breast cancer: genetic link or coincidental association?dermatofibrosarcoma protuberans is a low-grade cutaneous neoplasm originating from the dermis with a high propensity for local recurrence. These malignancies are classified as soft tissue sarcoma although regional or distance metastatic spread is rare. Germ-line mutations of p53 have been described with a rare autosomal dominant syndrome in patients with soft tissue sarcoma and breast adenocarcinoma (Li Fraumeni's syndrome). The described case illustrates the rare occurrence of dermatofibrosarcoma protuberans (DFSP) and breast adenocarcinoma with an emphasis on the possibility of a molecular or genetic association.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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