1/223. Small cell neuroendocrine carcinoma of the cervix with involvement of multiple pelvic nodes--A successfully treated case by multimodal approach. Small cell neuroendocrine carcinoma of the cervix is very rare and is usually associated with dismal prognosis if treated by conventional surgery and radiotherapy even in early stage disease. This tumor is characterized by early lymphatic and hematogenous spread. Only one successfully treated case of small cell neuroendocrine carcinoma of the cervix with dissemination to the pelvic nodes had been reported before in the literature. We are reporting a case of small cell neuroendocrine carcinoma of the cervix with multiple pelvic nodal metastases including the common iliac nodes, which had been successfully treated with a multimodal approach including radical hysterectomy, pelvic/para-aortic lymphadenectomy, and postoperative chemotherapy using cisplatin-etoposide combinations and pelvic irradiation. ( info) |
2/223. The effectiveness of chemotherapy with cisplatin and 5-fluorouracil for recurrent small cell neuroendocrine carcinoma of the rectum: report of a case. We report herein the case of a 46-year-old-man with small cell neuroendocrine carcinoma (NEC) concomitant with large villous adenoma of the rectum, who underwent abdominoperineal resection with regional lymphnode dissection. The resected specimen was histologically found to contain a small lesion of NEC confined to the submucosa in the large adenoma. A computed tomography scan done 4 months postoperatively revealed recurrences in the liver, lymph nodes, and bone. Therefore, two cycles of sequential intravenous combined chemotherapy with standard doses of cisplatin and 5-fluorouracil (5-FU) were administered, after which the size of each tumor decreased remarkably. Nevertheless, the patient died 8 months after the operation. As there was a fair response of this tumor to the combined chemotherapy of cisplatin and 5-FU, this regimen against NEC of the colon and rectum should be given consideration. ( info) |
3/223. A case report of gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation. We report herein an unusual gastric carcinosarcoma with rhabdomyosarcomatous and neuroendocrinal differentiation in a 63-year-old Japanese male. The tumor was a pedunculated large polypoid tumor (7 x 6.5 x 3.5 cm) located in the pylorus. Histologically, it invaded to the subserosa and was composed of both adenocarcinomatous and sarcomatous components. Adenocarcinomatous foci generally showed tubular to solid patterns and occupied the parts facing the gastric lumen, while the sarcomatous components showed a generally irregular and solid arrangement. There were transitions between the sarcomatous and carcinoma elements. In addition, carcinoma cells with a cord-like or trabecular arrangement similar to that seen in endocrine carcinoma expressed chromogranin a, and were mainly observed in an intermediate area between the adenocarcinomatous and sarcomatous foci. The sarcomatous areas were mainly composed of spindle cells and occasionally contained a sarcomatous component showing rhabdomyosarcomatous differentiation. This is an interesting case to consider how the variety of cell type appeared in such a type of tumor in the stomach. ( info) |
4/223. 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. ( info) |
| We report four cases of lambert-eaton myasthenic syndrome (LEMS) or myasthenia gravis (MG) associated with pulmonary neuroendocrine carcinoma having prolonged survival. The tumors were atypical carcinoid or large cell neuroendocrine carcinoma. LEMS is associated with several neuroendocrine carcinomas. Because some neuroendocrine carcinomas have a better prognosis, aggressive tissue diagnosis of lung cancer in LEMS is warranted. Whether the association between MG and atypical carcinoid is a significant co-occurrence is uncertain. ( info) |
6/223. 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. ( info) |
7/223. Angiomatoid neuroendocrine carcinoma of the thymus: report of a distinctive morphological variant of neuroendocrine tumor of the thymus resembling a vascular neoplasm. Three cases of primary thymic neuroendocrine tumors characterized by prominent angiomatoid features that resembled a vascular neoplasm are presented. The patients were all men between 52 and 59 years of age who presented with chest pain and shortness of breath attributable to a large anterior mediastinal mass. The lesions ranged in size from 6 cm to 15 cm in greatest diameter, and were grossly soft and well circumscribed, but not encapsulated. The cut surface was remarkable for multiple blood-filled cyst-like spaces admixed with focal solid, hemorrhagic areas. Histologically, the tumors contained multiple cystically dilated spaces filled with blood which imparted the lesion with a striking angiomatoid appearance. The walls of the cysts were lined by a monotonous proliferation of round to oval cells with distinct cell borders, round central nuclei, and abundant eosinophilic cytoplasm. Mitotic activity was present in all cases and varied from 3 to 8 mitoses per 10 high-power fields. Immunohistochemical studies performed in two cases showed positivity of the tumor cells for keratin, Leu 7, and synaptophysin, and focal chromogranin positivity in one. Follow-up information obtained in two patients showed that both had died of tumor 4 and 8 years after initial diagnosis. The present cases show an unusual morphological appearance of thymic neuroendocrine tumors that may be mistaken for a vascular neoplasm. Immunohistochemical stains may be of importance in such instances in arriving at the correct diagnosis. ( info) |
8/223. 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. ( info) |
| Large cell neuroendocrine carcinomas (LCNECs), one of the four newly categorised endocrine tumors of the uterine cervix, are unusual and aggressive tumors. The present report describes a case of LCNEC diagnosed at an early stage and associated with cervical intraepithelial neoplasia (CIN). The LCNEC showed organoid and trabecular growth patterns and was positive for chromogranin and synaptophysin. The CIN lesion was of a high grade and was negative for these neuroendocrine markers. polymerase chain reaction (PCR) using genomic dna extracted from archival tissue demonstrated human papillomavirus (HPV) type 16 dna in both the LCNEC and CIN lesions. These histological, immunohistochemical and PCR findings suggested that the LCNEC lesion was distinct from the CIN lesion and that both resulted from the carcinogenic field effect of HPV 16. ( info) |
10/223. CEA-producing mucin-negative gastric signet-ring cell carcinoma with neuroendocrine markers: a case report. biopsy and autopsy materials excised from a 69-year-old woman were investigated. serum carcinoembryonic antigen (CEA) showed a high value of 955 ng/mL. A plateaulike tumor was located in the gastric cardia and fundus to the entire gastric body. It showed severe proliferation and infiltration from the mucosa to the serosa. The tumor was comprised of signet-ring cells and poorly differentiated adenocarcinoma cells, which spread into the submucosa of the pylorus, duodenum, and jejunum. Signet-ring cells had a large, eccentric vesicular nucleus and a pale cytoplasmic inclusion. Poorly differentiated adenocarcinoma cells had a pleomorphic nucleus, small eosinophilic nucleolus, and abundant eosinophilic cytoplasm. Both neoplastic cells were positive for CEA, epithelial membrane antigen, Leu-7 (CD57), and neuron-specific enolase, and were negative for cytokeratin, vimentin, and periodic acid-Schiff, alcian blue, and mucicarmine stains. Electron microscopy showed endocrine granules with a limiting membrane measuring approximately 238 nm in diameter in the cytoplasm. The authors diagnosed this patient as having mucin-negative gastric signet-ring cell carcinoma with neuroendocrine markers, which is suggested to exist among poorly differentiated adenocarcinoma, undifferentiated carcinoma, and signet-ring cell carcinoma. ( info) |