1/23. family with low-grade neuroendocrine carcinoma of salivary glands, severe sensorineural hearing loss, and enamel hypoplasia.Four sibs in a family on the Isle of Man, two brothers and two sisters ranging in age from 33 to 45 years, presented with low-grade malignant tumors of the submandibular gland in three cases and of the nasal cavities and maxillary sinuses in one. The neoplasms were all of the same histological type, apparently hitherto undescribed, showing well-differentiated neoplastic ducts, surrounded by neoplastic myoepithelial cells, together with sheets of epithelial cells expressing neuroendocrine markers by immunohistochemistry. Cervical neck node metastases have developed in all four cases. In the sib with a primary sinonasal neoplasm, widespread bloodstream metastases also became manifest and a single such metastasis in his brother. All four sibs have severe enamel hypoplasia and the same lesion is present in 5 of their 11 children. In the two male patients, severe sensorineural hearing loss has developed in adult life, unilateral in the left ear in one brother, bilateral in the other. In the brother with bilateral sensorineural hearing loss, magnetic resonance imaging revealed a vestibular schwannoma on the left side, which is currently under treatment. The inherited hearing loss is thought to be unilateral in this case also.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/23. Primary hepatic carcinoid and neuroendocrine carcinoma: clinicopathological and immunohistochemical study of five cases.Primary hepatic carcinoid and neuroendocrine carcinoma (NEC) are rare tumors. We experienced three carcinoids and two NEC originating in the liver during the past 25 years and attempted to elucidate the clinicopathological and immunohistochemical features of these tumors. The patients had no endocrine symptoms despite two of them having elevated plasma serotonin. Three of the five patients died of the tumor after operation with an average survival time of 20.6 months. All tumors were large (up to 26 cm in diameter), four of them solitary and one multinodular, and were not associated with liver cirrhosis. The carcinoid tumors showed insular, trabecular or glandular arrangement of argyrophilic cells, whereas in the NEC this histological pattern was distorted. Immunohistochemically the tumors showed expression of chromogranin a (all cases), chromogranin b (three cases), pancreastatin and chromostatin (four cases, respectively), prohormone convertase PC3 (three cases), carcinoembryonic antigen (CEA) and CA19-9 (two cases), cytokeratin 56 kDa (three cases), 160 kDa neurofilament (two cases) and neuron-specific enolase (two cases). serotonin and glucagon were sporadically detected in two tumors. The most useful marker to confirm the diagnosis was chromogranin a, which was cleaved to pancreastatin and chromostatin in the tumor tissue, and was more reliable than other markers of neuroendocrine differentiation.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
3/23. brain metastases from adenoendocrine carcinoma of the common bile duct: a case report.A 68-year-old man with metastatic brain tumors from adenoendocrine carcinoma of the common bile duct is reported. A common bile duct tumor and a metastatic liver tumor had been resected 6 years and 3 years prior to admission, respectively. Microscopically they showed two components; moderately differentiated tubular adenocarcinoma and neuroendocrine carcinoma. He presented with headache and vomiting and MRI revealed two metastatic brain tumors. They were successfully resected and radiotherapy was carried out. Histological diagnosis of the metastatic brain tumors was neuroendocrine carcinoma, but carbohydrate antigen (CA)-19-9 and carcinoembryonic antigen (CEA)-immunoreactive cells were observed without glandular pattern. Immunohistochemically serotonin and pancreatic polypeptide were detected, but somatostatin was not. As the endocrine cells demonstrated in the normal extrahepatic bile ducts are only somatostatin-containing D cells, these cells are considered to originate as part of a metaplastic process. To our knowledge, this represents the second case of adenoendocrine carcinoma of the common bile duct.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
4/23. Neuroendocrine carcinoma of the posterior mediastinum: a possible primary lesion.A paravertebral mass was noted in the posterior mediastinum in a 47-year-old man. Microscopically, the tumor showed solid and trabecular patterns and consisted of poorly differentiated atypical cells that often formed Flexner-Wintersteiner rosettelike glands. Immunohistochemically, the tumor cells expressed both epithelial and neuroendocrine markers, including cytokeratin (AE1/3), carcinoembryonic antigen, epithelial membrane antigen, neuron-specific enolase, chromogranin a, and synaptophysin, but were negative for CD99 (MIC2). Ultrastructurally, numerous desmosomes and neurosecretory granules were identified in the tumor cells. The present lesion was a primary neuroendocrine carcinoma of the posterior mediastinum-an unusual site for such a lesion.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
5/23. Primary large-cell neuroendocrine carcinoma of the parotid gland: immunohistochemical and molecular analysis of two cases.Pulmonary large-cell neuroendocrine carcinoma (LCNEC) is a newly proposed clinicopathologic entity; a few cases of LCNEC have been reported in other sites, such as the uterine cervix and the thymus. In the salivary glands, LCNEC is extremely rare and is not recognized as a specific entity in the world health organization classification. We retrospectively reviewed from our files 1675 cases of surgically resected primary parotid gland tumors and found 2 cases of LCNEC that fulfilled the criteria of pulmonary LCNEC. These cases occurred in 72- and 73-year-old men who had short histories of enlarging parotid gland tumors. The tumors were composed of large cells that exhibited organoid, solid, trabecular, and rosette-like growth patterns with a high mitotic rate and a conspicuous tendency for necrosis. The tumor cells were polygonal and characterized by a moderate nuclear:cytoplasmic ratio, coarse chromatin, and conspicuous nucleoli. Immunohistochemical examination revealed that the tumor cells were positive for six general neuroendocrine markers, cytokeratin, p53, bcl-2, epidermal growth factor receptor, and cyclin d1. Markedly reduced expressions of p21Waf1 and p27Kip1 were also noticed. The Ki-67 labeling index was more than 50% in both cases. One case showed loss of heterozygosity at TP53 accompanied by a p53 gene point mutation. loss of heterozygosity at chromosome 9p21 was detected in both cases; one was accompanied by a p16 gene silent point mutation. Both patients died of the disease, with recurrence 5 months and 4 years after surgery, respectively. These findings indicate that LCNEC is a rare but distinct salivary gland tumor with highly aggressive biologic behavior. Multiple alterations of cell cycle regulators and tumor suppressor genes may play an important role in presenting the biologic characteristics of this rare parotid gland tumor.- - - - - - - - - - ranking = 1.8keywords = gland (Clic here for more details about this article) |
6/23. A case of small polypoid esophageal carcinoma with multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and sarcomatous components.A small composite esophageal carcinoma measuring 1.5 x 1.4 x 1.0 cm is described. The tumor had a polypoid elevation with a superficial extension. Histologic examination revealed invasion of the submucosal layer and multidirectional differentiation, including neuroendocrine, squamous, ciliated glandular, and sarcomatous components. The neuroendocrine component was strongly positive for chromogranin and formed the bulk of the polypoid tumor. The squamous cell carcinoma exhibited a superficial extension. The adenocarcinoma was located in a small region of the tumor and contained ciliated glandular cells. The spindle cell sarcomatous component, which was positive for alpha-smooth muscle actin and negative for cytokeratin, exhibited no specific mesenchymal differentiation. Each component was found in 60%, 10%, 5%, and 25% of the tumor, respectively. Cases of small composite esophageal carcinoma containing various carcinomatous and sarcomatous components are extremely rare.- - - - - - - - - - ranking = 1.2keywords = gland (Clic here for more details about this article) |
7/23. Composite glandular-endocrine cell carcinoma of the common bile duct.A rare case of composite glandular-endocrine cell carcinoma of the common bile duct is presented. Histologically, this tumor consisted of adenocarcinoma and small-cell neuroendocrine carcinoma, with a transition between the two components. The two distinct areas of the tumor were immunohistochemically different, whereas the transitional zone exhibited characteristics of both areas. These features suggest that the tumor arose from a multipotential stem cell. Although it has been reported that the presence of neuroendocrine differentiation in carcinomas indicates a poor prognosis, the patient in the present case was well at the time of writing this report. This may be due to the fact that adenocarcinoma, which characteristically has a low proliferative activity, constituted the majority of the tumor.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
8/23. Aspiration cytopathology of middle-ear neuroendocrine carcinoma.Primary neuroendocrine carcinoma (carcinoid tumor) is among the rarest of middle-ear (ME) neoplasms, with only a rare case of metastatic disease having been reported. Fine-needle aspiration biopsy (FNAB) was performed in a 51-yr-old male with a two-decade history of multiple local recurrences from a right middle-ear neoplasm, with the most recent surgical excision 2 yr ago. He currently presented with an enlarged right parotid gland, and a right infratemporal mass. Aspirate smears showed a monotonous population of cytologically bland cells with a small to moderate amount of pale granular cytoplasm, round to oval nuclei, inconspicuous nucleoli, and finely granular chromatin. Rare, isolated large cells were occasionally seen. Immunohistochemical staining of the cell block made from the aspirated material showed strong cytoplasmic positivity for chromogranin, synaptophysin, neuron-specific enolase (NSE), serotonin, and cytokeratin cocktail, and negative staining for S100 protein. review of tissue slides from the patient's prior middle-ear tumor showed an identical immunoprofile and morphology, and led to a revision of the original diagnosis of paraganglioma. Middle-ear neuroendocrine carcinoma has a low but definite metastatic potential, which can be diagnosed using FNAB if ancillary immunohistochemical studies are available.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
9/23. Intra-epithelial neuroendocrine carcinoma of the nasal cavity.We describe a distinctive neuroendocrine carcinoma (NEC) that proliferated intra-epithelially. The tumor was 35 mm in diameter and arose from the right superior turbinate of a 46-year-old woman. Histologically, the tumor exhibited papillary growth and the tumor cells were localized in the thickened mucosal epithelium. The tumor cells had round to oval and vesicular or hyperchromatic nuclei, and cohered without any specific structure such as a fibrillary background, rosette or glandular structure. No stromal invasion by the tumor was observed. Immunohistochemically, the tumor cells were positive for neuron-specific enolase focally. In addition, many tumor cells expressed cytokeratin (AE1/AE3 and CAM 5.2), mostly with characteristic perinuclear dot-like patterns. Electron microscopy revealed focal but well-eveloped cytoplasmic processes containing arrays of microtubules and a few dense core granules. The tumor was considered to be a poorly differentiated neuroendocrine carcinomas (NEC) that exhibited exceptional intra-epithelial proliferation. The tumor completely disappeared after the stereotactic radiosurgery and has not recurred for 40 months. It might be difficult to distinguish a poorly differentiated NEC in the sinonasal region from other neuroectodermal tumors, including olfactory neuroblastoma, but the differential diagnosis is important because each tumor has different clinicopathological characteristics.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
10/23. alpha-Fetoprotein-producing liver carcinomas of primary extrahepatic origin.BACKGROUND: alpha-Fetoprotein (AFP)-producing carcinomas, hepatoid or otherwise, are increasingly being recognized at extrahepatic sites. Some of them not only mimic hepatocellular carcinomas (HCCs) in having a proclivity for vascular permeation and distant metastases but also exhibit identical morphology and immunoreactivity for alpha-1-antitrypsin and HepPar1. beta-Human chorionic gonadotropin (hCG) is also detected. This would create diagnostic problems in hepatic fine needle aspiration biopsies (FNABs) from patients with elevated serum AFP. Apart from HCC, its variants and germ cell tumors, one must consider metastatic AFP-producing carcinomas. CASES: A man with gastric adenocarcinoma had a liver mass. Hepatic FNAB revealed an AFP-producing adenocarcinoma. The gastric tumor was positive for AFP, polyclonal carcinoembryonic antigen, HepPar1, CK19, hCG and synaptophysin. A woman with endocervical adenocarcinoma had multiple liver nodules. FNAB revealed an AFP-producing, undifferentiated carcinoma. The cervix showed a large cell neuroendocrine carcinoma coexisting with an adenocarcinoma in situ. The large cells were positive for synaptophysin, AFP, hCG and AE1/3. The glands showed diffuse HepPar1 and focal synaptophysin expression. CONCLUSION: A wide histologic spectrum of extrahepatic carcinomas can produce AFP and other peptide hormones. The true AFP status probably is not recognized at the first presentation. Such carcinomas, whether hepatoid or not, behave aggressively. Their recognition at the initial presentation is crucial to early and appropriate therapy. These entities add a new dimension to the challenges of FNAB diagnosis.- - - - - - - - - - ranking = 0.2keywords = gland (Clic here for more details about this article) |
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