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1/33. 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.
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ranking = 1
keywords = neoplasm
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2/33. 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.
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ranking = 1
keywords = neoplasm
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3/33. 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.
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ranking = 2
keywords = neoplasm
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4/33. 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.
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ranking = 5
keywords = neoplasm
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5/33. 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.
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ranking = 5
keywords = neoplasm
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6/33. Spindle epithelial tumor with thymus-like differentiation (SETTLE): a distinctive malignant thyroid neoplasm with significant metastatic potential.

    Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a very rare tumor of the thyroid believed to be derived from branchial pouch or thymic remnants and showing primitive thymic differentiation. Although this tumor is prone to develop delayed blood-borne metastases, the metastatic risk is unclear because the case reports in the recent literature had very short follow-up periods. We report one case, the oldest patient reported so far, who had been followed up until death. The 59-year-old man had an enlarged thyroid for all of his adult life, and presented with recent rapid enlargement of the thyroid. neck exploration revealed a hard tumor in the right lobe, with adhesion to sternothyroid muscle. Histologic examination showed an invasive biphasic neoplasm traversed by sclerotic septa. Tight to loose fascicles of bland-looking spindly cells were intimately intermingled with tubulopapillary structures, diagnostic of the SETTLE. This patient developed left pulmonary metastases at 2 years and subsequently developed bilateral pulmonary and widespread metastases. He died 8 years after initial presentation. This case illustrates the protracted clinical course of the tumor, and survival for many years despite the occurrence of metastases. review of the literature shows that SETTLE occurs predominantly in young patients with a median age of 15 years and male predominance. There is a significant metastatic rate of 71% for patients with more than 5 years of follow-up in spite of the otherwise indolent nature of the tumor.
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ranking = 5
keywords = neoplasm
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7/33. Solid and papillary epithelial neoplasm arising in heterotopic pancreatic tissue of the mesocolon.

    AIM: Solid and papillary epithelial neoplasm (SPEN) is an uncommon pancreatic tumour. Very rarely it has also been described outside the pancreas, usually arising from heterotopic pancreatic tissue. This report summarises all the published extrapancreatic SPENs and documents the sixth such case arising from heterotopic pancreatic tissue of the transverse mesocolon in a 15 year old girl. methods/RESULTS: Histological and immunohistochemical examination revealed typical papillary and solid areas composed of columnar, cuboidal, and round cells, which were focally positive for vimentin, cytokeratin, neurone specific enolase, carcinoembryonic antigen, alpha1-antitrypsin, alpha1-antichymotrypsin, and negative for neuroendocrine markers (neurofilament, PGP 9.5, chromogranin a, synaptophysin, and S100), p53, and oestrogen and progesterone receptors. Electron microscopy showed scant zymogen but no neurosecretory granules. In agreement with the flow cytometric result s of diploidy, comparative genomic hybridisation (CGH) did not reveal loss or gain of genetic material, and the in situ hybridisation analysis of the RB1 and p53 genes revealed no abnormality in the 13q and 17p arms. CONCLUSIONS: Immunohistochemical and electron microscopic data support exocrine differentiation. The CGH and the flow cytometric results suggest a subtle, yet unknown genetic change, rather than a large genetic alteration. RB1 and p53 in situ hybridisation ruled out the role of deletion at these sites in the pathogenesis of SPEN. Interestingly, review of the published and the present heterotopic pancreatic SPENs identified the mesocolon as the most common anatomical site (four of six), despite the very rare occurrence of ectopic pancreatic tissue at this site.
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ranking = 5
keywords = neoplasm
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8/33. Epithelial ovarian cancer in female pseudohermaphroditism. A diagnosis by computed tomography and review of the literature.

    We describe the case of a patient proven to be a female pseudohermaphrodite with a complete virilization syndrome who developed an epithelial tumor of the ovary of borderline malignancy. The tumor appeared as an abdominal mass with cystic features on ultrasonography. The diagnosis of the intersexual condition and adnexal cancer was made by computed tomography (CT).
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ranking = 0.0009555011948217
keywords = malignancy
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9/33. Spindle epithelial tumor with thymus-like differentiation (SETTLE) of the thyroid.

    BACKGROUND: Tumors of the so-called intrathyroidal epithelial thymoma type are a rare group of thyroid neoplasm tumors. Of this type of tumor, spindle epithelial tumor with thymus like differentiation (SETTLE) has been reported only 17 times in English literature. methods: An 18-year-old woman was initially seen with a 6-cm left thyroid mass that was resected with a left thyroidectomy. Histopathologic features of the excised left thyroid tumor together with an immunohistochemistry and electron microscopy led us to a diagnosis of SETTLE. RESULTS: Most of the specimen was composed of highly cellular spindle cells that formed intersecting and streaming fascicles. The spindle cells showed both vimentin immunoreactivity and cytokeratin immunoreactivity but no immunoreactivity for thyroglobulin or calcitonin. Electron microscopic examination of the spindle cells demonstrated prominent cytoplasmic tonofilaments, desmosomes, and basal lamina consistent with epithelial cell origin. dna content analysis by flow cytometry revealed dna diploidy. CONCLUSIONS: SETTLE of thyroid gland is an extremely rare entity. A review of the literature reveals that SETTLE has distinctive morphologic features and an immunohistochemical profile.
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ranking = 1
keywords = neoplasm
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10/33. Epithelial-myoepithelial carcinoma of the lung. A case report and review of the literature.

    Primary lung tumors mimicking the salivary gland-type neoplasms are extremely rare. These types of tumors originate from submucosal bronchial glands. Epithelial-myoepithelial carcinoma is an uncommon tumor in this group, and only 7 cases have been reported. It is considered to be a low-grade malignant neoplasm. We report a case of epithelial-myoepithelial carcinoma of bronchial gland origin in a 73-year-old man who presented with coughing and dyspnea. The bronchus of the left lower lobe contained a tumor mass that had a polypoid growth pattern. The tumor also extended into the pulmonary parenchyma, forming a well-circumscribed mass with a pushing margin. The tumor consisted of epithelial and myoepithelial cells. The epithelial cells were positive for cytokeratins and epithelial membrane antigen, while the myoepithelial cells were positive for S100 protein and muscle-specific actin. According to these findings, we diagnosed epithelial-myoepithelial carcinoma. After undergoing pneumonectomy, the patient has been disease free for 34 months.
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ranking = 2
keywords = neoplasm
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