Cases reported "Facial Neoplasms"

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1/31. Microcystic adnexal carcinoma arising in the setting of previous radiation therapy.

    While there are several reports of microcystic adnexal carcinoma developing in patients within sites of previous therapeutic irradiation, this relationship is not well described in the dermatologic literature. We report a case of a 42-year-old man with a remote history of therapeutic irradiation following surgical resection of periorbital rhabdomyosarcoma. Subsequently, he developed multiple basal cell carcinomas and a microcystic adnexal carcinoma within the field of irradiation. The histologic features were those of a classic microcystic adnexal carcinoma, with well differentiated nests and cords of keratinocytes displaying follicular and ductular differentiation infiltrating diffusely into the reticular dermis. Dense fibrosis was present surrounding the neoplastic keratinocytes. Nuclear atypia and mitotic figures were not identified. A carcinoembryonic antigen (CEA) stain demonstrated glandular differentiation. It is important for dermatologists to be aware of the apparent relationship between the rare microcystic adnexal carcinoma with its innocuous scar-like clinical appearance and prior local radiation therapy.
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2/31. The association of latent Epstein-Barr virus infection with hydroa vacciniforme.

    patients with hydroa vacciniforme (HV)-like eruptions and malignant potential have been reported from Asia and mexico, and those patients frequently had an associated latent Epstein-Barr virus (EBV) infection. In order to elucidate the association of latent EBV infection with HV, we studied six children with typical manifestations of HV by detection of EBV genes and EBV-related RNAs in biopsy specimens from cutaneous lesions. Cutaneous lesions of all six children with typical HV contained EBV-encoded small nuclear rna (EBER) cells in 3-10% of the dermal infiltrates, whereas no Bam HI-H, l-fragment (BHLF) mRNA, or transcripts encoding EA-D antigen, were detected. No EBER cells were detected in other inflammatory or benign lymphoproliferative skin disorders tested. polymerase chain reaction amplification confirmed the presence of EBV DNA sequences in five of six biopsy specimens from the patients. Latent EBV infection is associated with the development of cutaneous lesions of HV.
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3/31. Tissue-specific expression of SV40 in tumors associated with the li-fraumeni syndrome.

    Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in li-fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. dna tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals.
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4/31. Signet ring cell basal cell carcinoma: a basal cell carcinoma with myoepithelial differentiation.

    Basal cell carcinoma (BCC) can show a variety of routes of differentiation, but myoepithelial differentiation has rarely been described. We describe a case of BCC showing histologic and immunohistochemical features of myoepithelial differentiation. Histologically, the lesion showed well-demarcated tumor nodules composed of two different components. One component was typical of BCC, and the other component was composed of tumor cells containing abundant cytoplasm, eccentric nuclei, and no peripheral palisading, with scattered signet ring-shaped cells. Immunohistochemically, the tumor cells in the typical BCC component stained with CKAE1/AE3 and smooth muscle actin (SMA), but not with S-100 protein. They stained weakly with CAM5.2, epithelial membrane antigen, and glial fibrillary acidic protein (GFAP). The tumor cells in the other component stained strongly with CKAE1/AE3 and SMA, moderately with epithelial membrane antigen and GFAP, and weakly with CAM5.2. In a small area, the tumor cells stained with S-100 protein.
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5/31. Ectopic extramammary Paget's disease arising on the cheek.

    BACKGROUND: Extramammary Paget's disease (EMPD) is a rare neoplasm affecting apocrine gland-bearing skin. patients usually present with a long-standing unilateral, ill-defined, erythematous or whitish-gray, crusted patch or plaque. This tumor almost never occurs outside of the anogenital or axillary areas. OBJECTIVE: We report the first case of "ectopic" extramammary Paget's neoplasm afflicting the face. methods: The excisional specimen from a 67-year-old man was reviewed. histology and immunohistochemistry studies were performed on the specimen. RESULTS: histology revealed hyperkeratosis, parakeratosis, acanthosis, and proliferation of cytologically atypical epithelioid cells permeating the epidermis and the cutaneous adnexae. Special stains showed reactivity to cytokeratins (AE1/AE), epithelial membrane antigen, and carcinoembryonic antigen in the atypical cells. There was no evidence of apocrine glands in the area. CONCLUSION: Herein we present the first case of truly "ectopic" extramammary Paget's disease on the face.
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6/31. Cytotoxicity reactions during immunotherapy of melanoma with neuraminidase altered autologous tumor cells.

    Lymphocyte cytotoxicity, serum cytotoxicity, and the ability of the serum to inhibit lymphocyte cytotoxicity (blocking effect) were studied in a melanoma patient treated with six monthly injections of her own (autologous) tumor cells incubated with neuraminidase to increase their antigenicity. The same tumor cells grown in tissue culture were used as target cells for the cytotoxicity test. Large fluctuations of blocking effect in the serum were found, which correlated with the clinical course of tumor removal, recurrence, and regression. After the fifth injection of autologous tumor cells, the blocking effect disappeared from the serum (unblocking). In general, changes in serum cytotoxicity corresponded with changes in the amount of blocking effect produced by the serum. The results suggest that active immunotherapy may play a role in the prevention of metastases and, that when used within the autologous system, the cytotoxicity test is valuable in studying response to this type of therapy.
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7/31. Remarkable remission of a follicular lymphoma treated with rituximab and polychemotherapy (CHOP).

    We describe a 50-year-old female patient who developed extensive lymphomatous infiltrates on her forehead, scalp and face within a few months. histology and immunohistochemistry of skin tumours revealed a CD20 positive follicular B-cell lymphoma. Subsequently, extracutaneous manifestations were detected by computed tomography scans and bone marrow biopsy. The patient suffered from a primary nodular malignant lymphoma with extraordinary cutaneous infiltration of the head. Therefore, combination treatment with a monoclonal antibody against the CD20 antigen, rituximab, and polychemotherapy (CHOP scheme) was administered every 3 weeks. After the second course of treatment a complete regression of cutaneous infiltrates was noticed. Follow-up biopsies on the forehead showed no evidence of CD20 positive lymphoma cells, now. Despite mild leucocytopaenia therapy was well tolerated.
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8/31. Extraocular sebaceous carcinoma.

    A 79-year-old man with no history of previous irradiation presented with a large ulcerated tumour of the cheek. The histological features favoured a true sebaceous carcinoma. Neither squamous nor basal differentiation were seen. sudan stainings were positive. Tumour cells expressed suprabasal keratins and were negative for carcinoembryonic antigen, vimentin and S 100 protein. Extraocular sebaceous carcinomas occurring without previous irradiation are rare tumours which behave aggressively. Treatment regimens are discussed.
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9/31. Distribution of adhesion molecules, IgE, and CD23 in a case of angiolymphoid hyperplasia with eosinophilia.

    angiolymphoid hyperplasia with eosinophilia developed in a 13-year-old boy. The immune phenotype of infiltrating cells and the distribution of adhesion molecules of the integrin, immunoglobulin, and selectin families were analyzed with monoclonal antibodies. The infiltrate consisted mainly of helper lymphocytes, monocytes, eosinophils, CD1 dendritic cells, and mast cells. A CD23/Fc epsilon receptor was present on 40% of these cells, and surface bound IgE was present on 30% of these cells. The latter was found on all cell types. A cellular adhesion molecule analysis revealed that the proliferating endothelial cells were expressing constitutive ICAM-1, LFA-3, VLA-1, VLA-3, VLA-5, alpha 6, and beta 3-integrin chain as well as activation-dependent VLA-2, VCAM-1, and human lymphocyte antigen-DR. The infiltrating cells expressed LFA-1 and CD2, the correlating ligands for ICAM-1 and LFA-3. Our studies support previous observations of an unusual IgE-mediated immune reaction, and suggest an additional pathogenic role for adhesion events in the development of the pathognomonic cellular composition in angiolymphoid hyperplasia with eosinophilia.
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10/31. Hereditary heparin cofactor ii deficiency and thrombosis: report of six patients belonging to two separate kindreds.

    We describe six patients belonging to two families with congenital heparin cofactor ii deficiency (HC II). The affected members had low levels of HC II antigen and activity, and no abnormalities in HC II electrophoretic mobility were detected in the presence of heparin or dermatan sulphate during the first migration of crossed immunoelectrophoresis. These data suggested that patients had a type I (true) HC II deficiency. The association of thrombotic manifestations with congenital HC II deficiency has not been completely clarified. In these two families, thrombotic events occurred in two out of six affected members. In addition, there was a high incidence of spontaneous abortion in the affected females. Finally, the association of congenital HC II deficiency with angiomatosis was also observed in one patient.
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