Cases reported "Carcinoma, Adenoid Cystic"

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1/9. Solitary cylindroma (dermal analog tumor) of the breast: a previously undescribed neoplasm at this site.

    The authors report a previously undescribed small, well-demarcated breast tumor similar to a dermal cylindroma in a 63-year-old woman. The tumor was an incidental finding in a lumpectomy specimen for infiltrating lobular carcinoma. The cylindroma was surrounded by normal-appearing breast parenchyma and had the typical "jigsaw" pattern of epithelial basaloid islands. The islands showed focal squamous and myoepithelial differentiation. A notable number of reactive dendritic langerhans cells permeated the epithelial cell islands, a feature considered to be characteristic of dermal cylindroma. There was also ductal differentiation. Thick bands of hyaline periodic acid-Schiff (PAS) stain and collagen IV-positive basement membrane material bordered the cell islands, and PAS-collagen IV-positive hyaline globules were seen within the cell islands. There was no nuclear pleomorphism or mitotic figures. The cylindroma did not express gross cystic disease fluid protein 15, carcinoembryonic antigen, estrogen and progesterone receptors, or cytokeratin 20 (CK20). There was diffuse and strong immunoreactivity to CK AE1/AE3, and focal reactivity for CK7 and smooth muscle actin. Cylindroma of the breast should be distinguished from adenoid cystic carcinoma and basal cell carcinoma. Although clearly epithelial, the exact histogenesis and cell phenotype of this unusual dermal type cylindroma of the breast are unknown.
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2/9. Spiradenocylindroma of the kidney: clinical and genetic findings suggesting a role of somatic mutation of the CYLD1 gene in the oncogenesis of an unusual renal neoplasm.

    We describe the morphology and comparative genomic hybridization findings in a tumor for which we propose the term "spiradenocylindroma" of the kidney. The tumor arose in the wall of a renal cyst in an otherwise healthy male patient who had a favorable clinical course after nephrectomy. Tumor cells formed either large nodules exhibiting a solid or trabecular architecture with conspicuous perivascular spaces or cylindromatous small tumor cell islands arranged in a jigsaw pattern. Focally, there were interspersed tubular structures and tumor cell rosettes with central deposits of periodic acid-Schiff-positive material. A minor tumor component showed epidermoid differentiation. The tumor cells were strongly positive for cytokeratins 5/6, high molecular weight cytokeratins 34betaE12 and AE1/3, and E-cadherin, but only weakly positive for cytokeratins 7, 8, 18, 19, and epithelial membrane antigen. Focal reactivity for actin, vimentin, and S-100 protein or lysozyme and alpha 1 -antichymotrypsin within tubular and cylindromatous areas suggested myoepithelial and apocrine differentiation, respectively. By comparative genomic hybridization, the only abnormality was loss of the long arm of chromosome 16 and gain of genetic material on the short arm of chromosome 16, suggesting isochromosome i(16p). This finding is unique among renal neoplasms and implies loss of heterozygosity at 16q12-13 of the CYLD1 gene that is critically involved in the oncogenesis of familial cylindromatosis and some sporadic spiradenocylindromas. We conclude that somatic mutation of the CYLD1 gene outside the skin can have a role in the oncogenesis of tumors with cylindromatous features.
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3/9. Primary cutaneous adenoid cystic carcinoma treated with Mohs micrographic surgery toluidine blue technique.

    A patient with primary cutaneous adenoid cystic carcinoma treated with mohs surgery is presented. This tumor is characterized clinically by frequent local recurrences and infrequent metastases. Histologically it demonstrates cribiform islands of tumor cells with an abundance of mucin. Because toluidine blue stains this mucin metachromatically, it may be superior to hematoxylin and eosin for identifying the presence of this tumor. We recommend Mohs micrographic surgery with toluidine blue staining technique for the treatment of adenoid cystic carcinoma.
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4/9. Solid adenoid cystic carcinoma of the maxilla.

    Seven cases of solid variant of adenoid cystic carcinoma of the maxilla are reported. Clinical and radiographic characteristics suggest origin within the maxillary alveolar bone. Swelling was minimal and disproportionate to the extensive, diffuse bone destruction universally present. Histologic features were typical of this neoplasm occurring in other sites and consisted of diffusely infiltrating islands of small, closely packed monomorphous cells with sparse cytoplasm, indistinct borders, and small hyperchromatic nuclei. Four of five patients with follow-up data died of their disease. This confirms the lethality of the solid variant. Rationale is presented for considering these malignancies to be of primary intraosseous origin.
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5/9. basement membrane components and keratin in the dominantly inherited form of cylindroma.

    Specific antibodies against basement membrane associated, connective tissue components: type IV and V collagens, laminin, fibronectin and heparan sulphate proteoglycan were used to study the basement membrane-like structures in cylindroma lesions. All these components were immunohistochemically demonstrated as a band surrounding islands of epithelial cells and all except fibronectin also inside the islands. antibodies to keratin filaments stained most of the cells inside the epithelial islands confirming the epithelial origin of the cells.
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6/9. Type VII collagen is a component of cylindroma basement membrane zone.

    Cylindroma basement membranes (BMs) were studied by electron microscopy and immunofluorescence with antibodies to Types IV and VII collagen in order to correlate the ultrastructure with the biochemical characteristics. Type IV collagen served as a marker for lamina densa structures and Type VII collagen for the anchoring fibrils. Ultrastructurally, the cylindroma BMs were composed of a wide band of BM-like material in which numerous anchoring fibrils were embedded. The BMs surrounding the tumor cell clusters and nodules of BM-like material entrapped within the tumor cell islands stained positively for both Types IV and VII collagen. We conclude, that anchoring fibrils are a major component of cylindroma BM zone. A trichoepithelioma investigated as control showed a BM similar to the one found at the dermoepidermal junction, i.e. a BM distinctly different from the one of cylindroma. The trichoepithelioma BMs and nodules of BM-like material included within the tumor also stained positively for both Type IV and VII collagen.
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7/9. Ultrastructure of adenoid cystic carcinoma arising in the salivary gland of a child.

    A case of adenoid cystic carcinoma which developed in the left parotid gland of an 11-year-old girl was studied by electron microscopy. islands of tumor cells, showing glandular and cribriform appearances, infiltrated the surrounding tissue and nerves. True lumina and pseudocysts were present in the islands of tumor cells. Electron microscopic examination revealed two types of tumor cells; clear cells (secretory epithelial cells) and dark cells myoepitheium-like cells). Some of the tumor cells, showing a transitional form between the two types, formed a part of the luminal wall. There were many crystalline spicules, cellular debris, and fine stellate granules in the lumina. Pseudocysts were composed of replicated basal laminae, fine stellate granules, and a central core of microfilaments. Rarely, a capillary was noted in the center of the pseudocysts. It is speculated that ultrastrutural features of adenoid cystic carcinoma in chidren may differ from the adult cases in the following ways; they contain numerous crystalline spicules in the lumina, dark cells with microvilli forming the luminal wall, and fine capillaries in the pseudocysts.
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8/9. Maxillary adenoid cystic carcinoma with annulate lamellae.

    Of 29 reviewed ultrastructural studies of adenoid cystic carcinoma, only 1 mentions the presence of annulate lamellae. The present report describes a 65-year-old woman who presented with an adenoid cystic carcinoma in her left maxillary sinus. At the light microscopic level, most of the connective tissue was loose and myxomatous and contained fine strands, cords, and islands of epithelium that in places had a cribriform pattern. The ultrastructural appearance was similar to that described by others. Two epithelial cell types predominated. The cytoplasm of one type contained mainly ribosomes, and that of the other type also had peripheral microfilaments. Also present were pseudocysts, lumina with microvilli, and replicated basal lamina. In addition, a number of both types of cells contained parallel arrays of cisternae that had regular small pores. These were annulate lamellae.
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9/9. Thymic basaloid carcinoma: a case report and review of the literature.

    We report a case of thymic basaloid carcinoma in a 65-yr-old man who presented with symptoms referable to the mass effect of the tumor. The tumor arose in association with a multilocular thymic cyst and microscopically was characterized by islands, nests, and cords of basaloid cells. The tumor cells demonstrated prominent palisading around most of the neoplastic islands and nests. The tumor was excised, and there was no evidence of recurrent or metastatic disease in the 6-mo follow-up period. Including the current case, seven cases of basaloid thymic carcinoma have been reported in the English literature, four of which (57%) were associated with a multiloculated thymic cyst. Follow-up was available on three patients. Although no patient has died of tumor, pulmonary metastases developed in two patients. Although basaloid thymic carcinoma is currently considered a tumor of low-grade histology, more cases need to be studied to determine the long-term prognosis of these tumors.
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