mart-1-antigen and Dermatofibrosarcoma

To investigate the clinical pathological features, diagnosis and differential diagnosis of pigmented dermatofibrosarcoma protuberance (PDFSP).. The clinical history, histopathological features, immunohistochemical characteristics, treatment and prognosis were analyzed in seven cases of PDFSP. Fluorescence in situ hybridization (FISH) was used to detect the expression of COL1A1/PDGFB fusion gene, and related literature was reviewed.. The median age of the seven patients (4 females, 3 males) was 47 years with the tumors involving mostly the trunk (four cases). Histologically, PDFSP showed a cellular lesion composed of spindle cells arranged in short fascicles that form a distinct storiform pattern, and the pigmented bipolar or multipolar dendritic cells were present with tentacle like processes emanating from a nucleus containing zone. One case showed fibrosarcomatous change. The pigment was tinctorially similar to melanin. The spindle cells were positive for CD34 and vimentin, but negative for HMB45, Melan A, S-100, desmin, CD68 or α-SMA. HMB45, Melan A, S-100 and vimentin were expressed in the melanin containing cells in 4, 4, 5 and 7 cases, respectively. The labeling index of Ki-67 was 1%-8%. Among the 4 cases successfully examined by FISH, 3 showed t(17;22)(q21;q13) which suggested COL1A1/PDGFB fusion gene. Three patients were treated by wide local excision and four were treated by simple surgical excision. Two patients developed recurrences during the follow-up period of 12 to 123 months. Of those treated by wide local excision, none developed recurrence. No patient died in the follow-up period.. PDFSP is a rare pigmented variant of DFSP and an intermediate grade malignant tumor. The orgin of the tumor cells is still controversial. Surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of PDFSP as there is a risk of misdiagonsing it as either pigmented tumors associated with neurocutaneous syndromes or a highly malignant melanocytic neoplasm.

Topics: Adult; Aged; Antigens, CD34; Child, Preschool; Dermatofibrosarcoma; Diagnosis, Differential; Female; Follow-Up Studies; gp100 Melanoma Antigen; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Male; MART-1 Antigen; Melanoma; Melanoma-Specific Antigens; Middle Aged; Neoplasm Recurrence, Local; Neurilemmoma; Neurofibroma; Oncogene Proteins, Fusion; Prognosis; Retrospective Studies; S100 Proteins; Skin Neoplasms; Vimentin

With the advent of incorporating the immunoperoxidase staining technique into the processing of frozen tissue, the use of Mohs micrographic surgery (MMS) has been expanded to include several high-risk tumors such as lentigo maligna, malignant melanoma, and dermatofibrosarcoma protuberans.. To thoroughly review the English medical literature pertaining to the use of immunohistochemical staining techniques on frozen sections during MMS and to summarize the basic relevant outcomes from the different relevant studies.. Medline search was conducted, with the following words used in the search criteria: "Mohs surgery,""staining,""immunostaining," and "immunoperoxidase." RESULTS Generally, all immunostains showed advantage over the traditional hematoxylin and eosin approach. Studies of MART-1 in melanoma chemosurgery indicated that it is typically crisp and has less background staining than MEL-5 and better staining consistency than HMB-45. In cases of desmoplastic melanomas, S100 is the stain of choice.. Immunostaining offers an advantage in MMS. Large, randomized, prospective studies comparing the different immunostains are still lacking in the literature. The authors have indicated no significant interest with commercial supporters.

Topics: Antigens, Neoplasm; Dermatofibrosarcoma; Frozen Sections; Humans; Hutchinson's Melanotic Freckle; Immunoenzyme Techniques; Immunohistochemistry; MART-1 Antigen; Melanoma; Melanoma-Specific Antigens; Mohs Surgery; Neoplasm Proteins; S100 Proteins; Sensitivity and Specificity; Skin Neoplasms

Topics: Adult; Antigens, CD34; Biopsy; Cell Differentiation; Dermatofibrosarcoma; Diagnosis, Differential; Female; Humans; MART-1 Antigen; Melanocytes; Microphthalmia-Associated Transcription Factor; Rare Diseases; S100 Proteins; Skin Neoplasms; SOXE Transcription Factors; Thorax

Atypical fibroxanthoma (AFX) is a distinctive clinicopathologic entity presenting on sun-damaged skin of the elderly. Its behavior is benign if strict diagnostic criteria are applied. Tumors showing invasion of deeper structures or perineural/lymphovascular invasion are best regarded as undifferentiated pleomorphic sarcoma of the skin. The diagnosis requires immunohistochemical studies to exclude melanoma, squamous cell carcinoma, angiosarcoma and leiomyosarcoma.. Two AFX and one undifferentiated pleomorphic sarcoma showing aberrant expression of Melan-A were identified. Clinical data were obtained and histopathological features, immunohistochemical profile and electron microscopy were assessed.. All tumors arose on sun-damaged skin of elderly males. Two AFX showed pushing growth into superficial subcutis only. The undifferentiated pleomorphic sarcoma was characterized by infiltrative growth into galea as well as perineural invasion. Multifocal expression of Melan-A and MART-1 was largely limited to tumor giant cells in the absence of S100 or HMB-45 labeling. No melanosomes or premelanosomes were identified by electron microscopy.. Aberrant expression of Melan-A and MART-1 in AFX and undifferentiated pleomorphic sarcoma of the skin represents an important diagnostic pitfall with potential for misdiagnosis as melanoma.

Topics: Aged; Dermatofibrosarcoma; Diagnosis, Differential; Fibroma; Gene Expression Regulation, Neoplastic; Humans; Male; MART-1 Antigen; Skin Neoplasms; Xanthomatosis

Topics: Antigens, CD34; Antigens, Neoplasm; Biomarkers, Tumor; Biopsy; Cell Proliferation; Dermatofibrosarcoma; Dermis; Diagnosis, Differential; Female; Humans; MART-1 Antigen; Melanoma; Middle Aged; Mohs Surgery; Neoplasm Proteins; S100 Proteins; Skin Neoplasms