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No abstract available.
Assuntos
Feminino , Humanos , Dermatofibrossarcoma , Diagnóstico DiferencialRESUMO
Dermatofibrosarcoma protuberans (DFSP), the most common dermal sarcoma, is a malignant fibroblastic tumor most frequently arising in middle-aged adults. It is typically a low-grade sarcoma that grows slowly but has a high rate of local recurrence with low metastatic potential. Dermatofibrosarcoma protuberans is characterized by a specific translocation t(17;22)(q22;q13) leading to the formation of COL1A1-PDGFB fusion transcripts. Histologically, DFSP has characteristic morphology, of storiform islands of bland spindle cells, and immunohistochemically, it shows diffuse expression of CD34. However, the morphology and immunoprofile can overlap with a variety of other soft tissue neoplasms. The preferred management of localized disease is wide surgical resection or Mohs micrographic surgery, whereas radiotherapy may be used for margin-positive disease where reexcision is not possible, or for inoperable disease. Dermatofibrosarcoma protuberans is generally regarded as refractory to conventional chemotherapy. Treatment options for systemic disease have been previously limited, but the PDGFßR, KIT, and ABL inhibitor imatinib is now an option for effective systemic therapy. Continued insight into the tumorigenic molecular changes generated by the fusion oncogene may lead to further specific targeted treatments. We review DFSP, discussing the morphologic spectrum and variants, immunohistochemistry, molecular genetic findings, potential targeted treatments, and the differential diagnosis.
Assuntos
Biomarcadores Tumorais/análise , Dermatofibrossarcoma , Recidiva Local de Neoplasia , Neoplasias Cutâneas , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/genética , Dermatofibrossarcoma/patologia , Dermatofibrossarcoma/terapia , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica/métodos , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Patologia Molecular/métodos , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Translocação GenéticaRESUMO
Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.
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Dermatofibrosarcoma protuberans (DFSP) is a relatively rare low-grade sarcoma that occasionally exhibits melanin-containing spindle cells within the tumor. Fibrosarcomatous DFSP (FS-DFSP) is a variant that is characterized by areas which are histopathologically indistinguishable from fibrosarcoma. In the present study, we describe a case of fibrosarcomatous pigmented DFSP and review the clinicopathological features of this extremely rare lesion. A 51-year-old male presented with a slow-growing nodular cutaneous mass in his left upper arm. Histopathologically, the resected tumor was comprised of pigmented DFSP in approximately 20% of the tumor, with the remaining area further covered by a fibrosarcomatous component. A review of the clinicopathological features of the five previously reported cases as well as the present case indicated that this lesion mainly affects middle-aged males and occurs mostly in the extremities. Melanin-containing spindle cells are present only in the conventional DFSP component. The prognosis appears to be poor; in the six cases reviewed, four demonstrated multiple metastases and three succumbed to the disease. Our analyses revealed that the presence of a fibrosarcomatous component in pigmented DFSP is associated with aggressive behavior; therefore, careful assessment for the presence of a fibrosarcomatous component is necessary in the diagnosis of this disease.
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A Bednar tumor is a rare pigmented subtype of dermatofibrosarcoma protuberans (DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP, except for the presence of melanin-containing cells within the lesion. We present a case of a Bednar tumor on the left anterior chest of a 58-years-old woman. It was presented as an asymptomatic, firm, hyperpigmented 1.8x2.7cm sized nodule with surrounding erythematous patch which had been present for over 35 years. The tumor was treated by wide local excision of the lesion. There has been no evidence of recurrence during a 1 year follow-up period.
Assuntos
Feminino , Humanos , Dermatofibrossarcoma , Seguimentos , Recidiva , TóraxRESUMO
Bednar tumor is a variant of dermatofibrosarcoma protuberans (DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP except for the presence of melanin- containing cells scattered within the lesion, so called pigmented DFSP. There are no known precipitating or predisposing factors associated with this tumor. A 30-year-old woman was presented with a tumor on the right upper arm arising after trauma at the site of prior vaccination. Histologically, it shows large uniformed spindle shaped cells arranged in a cartwheel or storiform pattern mixed with scattered pigmented cells. On immunohistochemical staining, the tumor cells were positive for vimentin and CD34, but negative for factor XIIIa and S-100 protein. The patient was treated with a wide excision and skin graft. We report a case of Bednar tumor occuring after trauma at the site of prior vaccination.
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Adulto , Feminino , Humanos , Braço , Causalidade , Dermatofibrossarcoma , Fator XIIIa , Proteínas S100 , Pele , Transplantes , Vacinação , VimentinaRESUMO
Bednar tumor is an uncommon pigmented subtype of dermatofibrosarcoma protuberans(DFSP). The histopathological finding is characterized by a typical storiform pattern composed of spindle-shaped cells resembling DFSP except for the presence of melanin-containing cells scattered within the lesion. The origin of the spindle-shaped cells and melanin-containing cells within the tumor remains unknown. We report a case of Bednar tumor in a 26-year-old woman on the right shoulder which was presented as asymptomatic, firm, black-colored, 1x1.5 cm nodule over 2 years. Histologically, the proliferated spindle-shaped cells showed a cartwheel pattern and stained positively with CD34, similar to those of a common DFSP. The tumor was widely excised and there is no evidence of recurrence from follow-up of 6 months.
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Adulto , Feminino , Humanos , Dermatofibrossarcoma , Seguimentos , Recidiva , OmbroRESUMO
Dermatofibrosarcoma protuberans(DFSP) is a cutaneous mesenchymal neoplasm originating in the dermis, which is diagnosed most in adulthood, with a range in age from birth to the ninth decade. Congenital DFSP is extremely rare and the diagnosis is difficult because of its resemblance to vascular birth mark of skin atrophy. We describe a case of congenital DFSP in a 19-year-old female on the left forearm, which was present as a vascular-appearing macule from birth with recent nodular change. Interestingly, the histopatholgy revealed Bednar tumor, a pigmented DFSP and it has not been reported in congenital case yet. The patient was treated with a wide and deep excision with surgical margin of 4cm and STSG, and no recurrence was noted for one year.
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Feminino , Humanos , Adulto Jovem , Atrofia , Dermatofibrossarcoma , Derme , Diagnóstico , Antebraço , Parto , Recidiva , PeleRESUMO
A Bednar tumor is a variant of dermatofibrosarcoma protuberans(DFSP). The clinical and histopathological findings of Bednar tumors are identical to DFSP except for the presence of melanin-containing cells scattered within the lesion, so called pigmented DFSP. The majority of Bednar tumors are seen as DFSP present as multinodular protuberant masses in the skin. They can also present as an atrophic depressed scar-like lesions without any nodularity. We report an uncommon clinical presented case of a Bednar tumor on the back in a 22-year-old female. She presented with an asymptomatic, firm, bluish, chestnut sized, depressed and atrophic lesion on the back which had been present for 6 years. A Histopathological examination revealed massive proliferation of spindle-shaped cells arranged in a tight storiform pattern mixed with scattered pigmented cells. On immunohistochemical staining, the tumor cells were positive for vimentin, a -l-antitrypsin, and CD34, but were negative for cytokeratin, neurofilament, and factor XIIIa. The majority of the tumor cells was negative and the pigment cells were positive for the S-100 protein. The patient was treated by a wide local excision of the lesion. There has been no evidence of recurrence after 20 months post-operative follow up.
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Feminino , Humanos , Adulto Jovem , Dermatofibrossarcoma , Fator XIIIa , Seguimentos , Queratinas , Recidiva , Proteínas S100 , Pele , VimentinaRESUMO
The authors examined a case of Bednar tumor(pigmented dermatofibrosarcoma protuberans). A 35-year-old woman presented with protruding black back mass 3x2.5 cm in surface dimension and 1 cm in heigh, which has grown slowly for 30 years. Microscopically the lesion is characterized by spindle cells arranged in storiform pattern and admixed with melanin containing dendritic cells. Ultrastructural study revealed three cell populations: (1) cells resembling fibroblasts, (2) cells resembling perineural cells, and (3) melanocytes. The immunohistochemical study using S-100 protein alpha-1 antitrypsin, antichymotrypsin and EMA revealed negative reaction in spindle fibroblastic tumor cells. The histogenesis of this neoplasm remains controversial; neuroectoderm, perineural cell, fibroblast and histiocyte. Through the ultrastructural study and immunohistochemical study, the authors suggested that Bedner tumor arises form the neuroectoderm with bidirectional to perineural and fibroblastic differentiation.
