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1/93. Linear melanonychia due to subungual keratosis of the nail bed: a report of two cases.

    Longitudinal melanonychia displaying features of keratinized acanthoma is described in two patients. In both cases, a pigmented band consisting of a subungual keratinized epithelial ridge originated in the nail bed. We have been able to show, using the clinical and histological findings, that the origin of the pigment is linked to its synthesis within the acanthoma of the nail bed. These lesions are reminiscent of pigmented seborrhoeic keratosis. The horny cysts are replaced, because of the special physiological longitudinal arrangement of the ridges in the nail bed, by a single, prominent, longitudinal, keratinized and pigmented mass. ( info)

2/93. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid with prominent mitotic activity and focal necrosis.

    Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a rare, apparently low-grade spindle cell tumor of the thyroid gland occurring in young individuals and thought to be derived from thymic or branchial pouch remnants. Spindle epithelial tumor with thymus-like differentiation has little to no mitotic activity, and focal necrosis has been reported in one case. We present a case of SETTLE in a 29-year-old man that was initially evaluated by fine-needle aspirate biopsy and ultimately found to be consistent histologically with SETTLE. In this case, there were numerous mitotic figures among the spindle cells and focal necrosis. Spindle epithelial tumor with thymus-like differentiation has been considered to be a tumor of low malignant potential with metastases developing some years after diagnosis. This is the first case in which prominent mitotic activity and necrosis is reported perhaps representing an aggressive variant. ( info)

3/93. Sialoblastoma and epithelial tumors in children: their morphologic spectrum and distribution by age.

    This commentary addresses the histologic spectrum of salivary gland neoplasms in children with emphasis on perinatal tumors. Histopathologically, perinatal tumors fall into four categories: 1) histologically benign with adult counterpart, 2) hamartomas 3) embryomas-sialoblastomas, and 4) histologically and biologically malignant adult analogue tumors. Although the criteria to serrate benign from malignant sialoblastomas are not well-established, the following histologic features would favor an aggressive clinical course: perineural and/or vascular spaces invasion, necrosis, and a cytologic atypia beyond the expected for embryonic epithelium. ( info)

4/93. Outer root sheath acanthoma.

    The term "outer root sheath acanthoma" is proposed to specify a distinctive keratosis exhibiting outer root sheath differentiation. This lesion features anastamosing columns of keratinocytes emanating from the undersurface of the epidermis that respectively demonstrate, with progressive descent into the upper dermis, outer root sheath differentiation characteristic of the infundibulum, isthmus, stem and anagen bulb. In our patient, the lesion was solitary, and no associated systemic disease was evident. Outer root sheath acanthoma is a unique keratosis displaying all four types of tricholemmal differentiation, thus representing a true neoplasm of the entire outer root sheath. ( info)

5/93. Disseminated epidermolytic acanthoma probably related to trauma.

    Epidermolytic acanthoma is a rare benign tumour, which may occur in both isolated and disseminated forms. Only seven cases of disseminated epidermolytic acanthoma (DEA) have been described. This entity should be distinguished from other hereditary or acquired conditions which involve epidermolytic hyperkeratosis and other benign acanthomas. On the basis of the clinical history and the histological findings, we diagnosed a case of DEA which was probably secondary to repeated trauma. ( info)

6/93. Primary invasive signet-ring cell melanoma.

    The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells. ( info)

7/93. Leiomyosarcomas of the oral cavity: an unusual topographic subset easily mistaken for nonmesenchymal tumours.

    AIMS: Oral leiomyosarcoma is rare and poorly documented. We aimed to characterize these lesions clinicopathologically in order to facilitate their distinction from other spindle cell neoplasms in the oral cavity. methods AND RESULTS: Ten cases of oral leiomyosarcoma were retrieved and studied histologically and immunohistochemically. Clinical data were obtained from referring pathologists and prior literature concerning 46 comparable cases was reviewed. Nine out of 10 cases occurred in adults; 50% arose in the jaws and four showed bone involvement. Histological appearances were similar to leiomyosarcomas elsewhere. In addition to myogenic markers, two cases were also keratin-positive. Four patients developed local recurrence or metastatic disease and three died of tumour (median follow-up 37 months). CONCLUSIONS: leiomyosarcoma is under-recognized in the mouth, often being mistaken for a spindle-celled epithelial neoplasm. Aside from an unusual but infrequent tendency to spread to lymph nodes and a location-specific differential diagnosis, its clinicopathological features are comparable to leiomyosarcomas at other locations. ( info)

8/93. adult mesoblastic nephroma.

    We report a case of asymptomatic mesoblastic nephroma in a 54-year-old woman. The tumor showed immunohistochemical reactions similar to developing nephrons. Electron microscopy showed immature tubules with numerous intracytoplasmic intermediate filaments. Recent studies support the concept of pathogenesis of the mesoblastic nephroma originating from collecting ducts. However, this case exhibited a complex pattern of antigenic expression not restricted to the collecting ducts, but including the glycoprotein CD24 and the neural cell adhesion molecule (NCAM). The following differential diagnoses will be discussed: benign mixed epithelial and stromal tumor, metanephric adenoma, and nephrogenic adenofibroma. ( info)

9/93. Solid and papillary epithelial neoplasm. Ultrasound and CT features of an unusual pancreatic tumour.

    The ultrasonographic and computed tomography features of four cases of solid and papillary epithelial neoplasm (SPEN)--a rare pancreatic tumour--are described. Although not diagnostic, the presence of these imaging features in the typical clinical setting may permit pre-operative radiological diagnosis and facilitate planning for curative surgery for this malignant tumour which has an excellent prognosis even without further adjuvant therapy. ( info)

10/93. FNAC of papillary and solid epithelial neoplasm of pancreas--a case report.

    A case of solid papillary epithelial neoplasm (PSEN) of pancreas in a young woman is reported in which the nature of tumour was recognised pre-operatively by ultrasound guided Fine needle aspiration. The pre-operative cytologic diagnosis enabled prompt and appropriate surgical treatment. FNAC revealed large cell clumps in the aspirate showing branching papillary appearance in which multiple layers of tumour cells surrounded central vascular stalks. The above was confirmed on histopathological examination of the excised tumour tissue. ( info)
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