Thymic large cell neuroendocrine carcinoma: report of a resected case - a case report

Thymic large cell neuroendocrine carcinomas (LCNECs) are very rare. We here describe a case in which the tumor could be completely resected. A 55-year-old male was admitted to our hospital for treatment of an anterior mediastinal tumor found at a regular health check-up. The patient underwent an extended thymectomy of an invasive thymoma of Masaoka's stage II that had been suspected preoperatively. The tumor was located in the right lobe of the thymus and was completely resected. Final pathological diagnosis of the surgical specimen was thymic LCNEC. The patient underwent adjuvant chemotherapy with irinotecan and cisplatin in accordance with the diagnosis of a lung LCNEC, and is alive without recurrence or metastasis 16 months after surgery.     

Combined large cell neuroendocrine carcinoma

We report a case of combined large cell neuroendocrine carcinoma. A 78-year-old man with vertigo was referred to our hospital where chest X-ray revealed a tumor shadow in the right lung. A transbronchial lung biopsy specimen verified a diagnosis of non-small cell lung carcinoma (cT1N0M0). Right lower lobectomy with mediastinal lymph node dissection (#7,8,9) was performed. A postoperative histological diagnosis was combined large cell neuroendocrine carcinoma of a component of squamous cell carcinoma [pT4 (pm) N2M0]. The patient received concurrent chemoradiotherapy due to upper mediastinal lymph node metastasis 4 months after surgery. The chemoradiotherapy well responded and the patient remains well 9 months after surgery.     

The prognostic role of c-kit protein expression in resected large cell neuroendocrine carcinoma of the lung

BACKGROUND: Large cell neuroendocrine carcinoma (LCNEC) is a high-grade neuroendocrine tumor of the lung that shares some clinicopathologic and molecular features with small cell lung carcinoma (SCLC). Optimal treatment has not yet been standardized and significant prognostic factors are lacking. Because c-kit protein overexpression has been recently reported as a negative prognostic factor in SCLC we investigated its expression and prognostic value in a series of LCNEC. METHODS: Resected LCNEC fulfilling the morphologic criteria of the 1999 World Health Organization classification of lung tumors and showing neuroendocrine differentiation by appropriate immunohistochemical markers were retrospectively reviewed. Immunostaining for c-kit protein expression was performed using the polyclonal antibody CD117. Clinical and pathologic characteristic were reported and analyzed and a survival study was performed. RESULTS: Thirty-three patients underwent radical resection. Thirty-one were male (94%) and 32 were smokers (97%). Ten (30.3%), 11 (33.3%), 5 (15.2%), and 7 (21.2%) were at stage IA, IB, IIB, and IIIA respectively. Overall 1-, 3-, and 5-year survival rates were respectively 79%, 58%, and 51%. Survival analysis showed no differences for any of the clinicopathological features except for CD117 immunostaining: 1-year and 3-year survival rates were respectively 91% and 82% for CD117-negative LCNEC, and 72% and 44% for CD117-positive ones (p = 0.046). Positivity of CD117 was significantly related to recurrence rate: 60% versus 23% for CD117 positive and negative LCNEC respectively (p = 0.037). CONCLUSIONS: Radical resection of large cell neuroendocrine carcinoma achieves poor outcomes. The c-kit protein is frequently expressed in this neoplasia and its expression represents a negative prognostic factor. This immunohistochemical marker may represent the basic rationale to select LCNEC for novel targeted therapy.     

Fast-growing large cell neuroendocrine carcinoma of mediastinum

Neuroendocrine carcinomas combine a heterogeneous group of tumors occurring in lungs on a rare occasion, and in some cases, they appear to have extraordinary quick growth and extrapulmonary localization. In this case we present a 42-year-old patient who underwent a right upper lobectomy for emphysema, and 6 months later, the tumor developed again into a giant neuroendocrine carcinoma of the mediastinum.     

Combined large cell neuroendocrine carcinoma and squamous cell carcinoma of the lung; report of a case

An 80-year-old man was admitted to our hospital because a routine chest X-ray had revealed a nodular shadow in the right lower lung field. Transbronchial lung biopsy (TBLB) failed to give at definitive diagnosis, therefore open lung biopsy was performed because of suspected lung cancer. Rapid intraoperative pathological examination diagnosed the tumor as large cell carcinoma. However, bloody pleural effusion was classified as class V. It was judged difficult to perform a curative operation, so the operation was interrupted. Pathological diagnosis was combined large cell neuroendocrine carcinoma and squamous cell carcinoma. Pleurodesis was done, and the patient is under observation at 7 months after the operation.     

Prognosis of surgically treated large cell neuroendocrine carcinoma

Large cell neuroendocrine carcinoma (LCNEC) is a relatively rare tumor in malignant lung neoplasms. The prognosis of LCNEC is poor and there is no consensus on the treatment for LCNEC. We report our retrospective assessment of 11 patients of LCNEC from 1999 to 2008. Three of 11 patients had malignant exudate at thoracotomy. Seven patients received limited resection. There was a recurrence even after complete surgical resection in its early stage. Four patients received platinum-based chemotherapy for adjuvant therapy or recurrence. The response to platinum-based chemotherapy was relatively good and may be comparable to that of small cell lung cancer. The overall 5-year survival rate was 30.3%. Pulmonary LCNEC represents an aggressive tumor and multimodal treatment is required.     

Metastatic carcinoma of the adrenal gland from large cell neuroendocrine carcinoma; report of a case

A 69-year-old man had undertaken left upper lobectomy (ND 2 a) with partial resection of the left lower lobe under the diagnosis of a primary lung cancer, T2N0M0, stage IB in June, 2002. The histopathological diagnosis was large cell neuroendocrine carcinoma (LCNEC), T3N0M0, stage IIB. The patient was discharged on postoperative day 25. Abdominal computed tomography (CT) revealed an enhanced tumor in the left adrenal lesion, 3 cm in diameter, in October, 2003. Magnetic resonance imaging (MRI) showed a slightly low intensity mass on T1-weighted imaging and slightly high intensity on T2-weighted imaging without invasion to other organs. Tumor marker, NSE was slightly elevated on blood examination. Left adrenalectomy was performed with a surgical margin. Histopathologically, the tumor was diagnosed as metastasis of LCNEC. There is no evidence of recurrence for a year after surgery. Although a prognosis of LCNEC is poor in general, we should consider the resection of metastatic carcinoma of the adrenal gland from LCNEC for long-term survival expectantly.     

前列腺小细胞神经内分泌癌一例报告

分析1例前列腺小细胞神经内分泌癌临床资料。患者60岁,因排尿困难1个月入院,直肠指检发现前列腺质硬肿块。前列腺穿刺活检病理诊断为小细胞神经内分泌癌。行化疗和放疗联合治疗有效,患者症状明显改善,患者确诊后15个月死亡。前列腺小细胞神经内分泌癌预后差,化疗在治疗中占有重要地位。     

Double primary lung carcinoma consisting of large cell neuroendocrine carcinoma and squamous cell carcinoma: report of a case.

We report a rare case of double primary lung carcinoma including large cell neuroendocrine carcinoma (LCNEC). A 67-year-old man underwent an annual medical checkup in 2000, pulmonary carcinoma was strongly suspected by sputum cytology and radiological images. Preoperative diagnosis was double primary lung carcinoma with a squamous cell carcinoma in the right lower lobe and non-small cell carcinoma in the right upper lobe. The histological carcinoma type in the right upper lobe could not be determined preoperatively. The patient underwent a right lower lobectomy and wedge resection of the right upper lobe. Histologically, the tumor in the right upper lobe was LCNEC and the tumor in the right lower lobe was a moderately differentiated squamous cell carcinoma. The patient had right supraclavicular lymph node metastases of LCNEC and died of multiple pulmonary metastases 10 months after the operation.     

Primary large cell neuroendocrine carcinoma of the urinary bladder

Large cell neuroendocrine carcinoma (LCNEC) of the urinary bladder is rare. It is a type of neuroendocrine carcinoma morphologically distinct from small cell carcinoma. We report here a case of primary LCNEC of the urinary bladder. We observed a very large invasive tumor, which was not able to be detected three months previously by cystoscopy or computed tomography. The tumor cells morphologically and immunohistochemically resembled that of pulmonary LCNEC. With prompt cystoprostatectomy and chemotherapy, the patient is free of disease 16 months after diagnosis. Although LCNEC is usually very aggressive, it may be controlled by early diagnosis and treatment.     

肾原发性大细胞神经内分泌癌1例报告并文献复习

目的提高对肾神经内分泌癌的认识。方法报告1例肾原发性大细胞神经内分泌癌的临床资料并结合文献讨论有关临床症状、诊断和治疗。结果肾神经内分泌癌临床表现和影像学与常见。肾癌相比无明显特异性,需通过病理学和免疫组织化学结果肯定诊断。结论肾原发性大细胞神经内分泌癌罕见,其临床和影响学表现难与肾癌区别,主要靠病理学和免疫组织化学确诊,该病恶性程度高、预后差,只有早期发现,早期手术治疗以提高疗效。     

A resected case of renal cell carcinoma with metastasis to pancreas]

We report a case of renal cell carcinoma with metastasis to the pancreas, treated by radical nephrectomy and total pancreatectomy. A 56-year-old man visited our hospital because of macrohematuria and right low backache. An intravenous pyelography, ultrasonography and a CT scan of the abdomen revealed right renal tumor at the upper portion, about 11 cm in diameter, but no abnormal findings of the pancreas. Aortic and celiac angiograms demonstrated multifocal lesions, 1 or 2 cm in size, compatible with a metastatic tumor in the region of the pancreas. The patient underwent right radical nephrectomy and open biopsy of the pancreas. The right renal tumor was histologically revealed to be renal cell carcinoma without nodal or venous extension. Histological examination of the pancreas biopsy specimen confirmed it to be a renal cell carcinoma metastatic to the pancreas. Therefore, he underwent total pancreatectomy 1 month after the previous surgery. Three months after the second surgery, a CT scan of the brain revealed metastasis to the pituitary gland. He is still under therapy.     

Pulmonary large cell neuroendocrine carcinoma demonstrates high proliferative activity

BACKGROUND: In 1999, the World Health Organization classified large cell neuroendocrine carcinoma as a variant of large cell carcinoma and this has been categorized as lying between atypical carcinoid and small cell lung carcinoma in terms of clinical aggressiveness. METHODS: We analyzed the proliferative activity of stage 1 large cell neuroendocrine carcinoma derived from patients with primary lung cancer who underwent surgical resection and compared the results with stage 1 classic large cell carcinoma cases. The mitotic rate was counted in ten high-power fields of light microscope. Immunohistochemical staining using anti-Ki-67 antibody was performed. The Ki-67 labeling index, expressed as a percentage of positive cells, was determined by light microscopy with random counting of at least 1000 tumor nuclei. The expression of P53 and Bcl-2 was examined and compared. RESULTS: The mitotic rate of large cell neuroendocrine carcinoma cases was significantly higher than that of classic large cell carcinoma cases. The Ki-67 labeling index of stage 1 large cell neuroendocrine carcinoma cases was significantly higher than that of stage 1 classic large cell carcinoma cases. Immunohistochemical expression of P53 in large cell neuroendocrine carcinoma and classic large cell carcinoma was comparable. However, large cell neuroendocrine carcinoma exhibited a significantly higher expression of Bcl-2 than classic large cell carcinoma. The disease specific disease-free survival for patients with stage 1 large cell neuroendocrine carcinoma was significantly lower than that for patients with stage 1 classic large cell carcinoma. CONCLUSIONS: Large cell neuroendocrine carcinoma appears to be more clinically aggressive than classic large cell carcinoma with these findings indicating that large cell neuroendocrine carcinoma has a higher level of proliferative activity than classic large cell carcinoma.     

Small cell neuroendocrine carcinoma of the bladder. A new case report

This rare but aggressive bladder tumour presents the morphological and immunohistochemical characteristic common to all neuro-endocrine tumours observed in other organs. The authors report a case of primarymalignant neuro-endocrine bladder tumour. Combined surgery-cisplatin-based adjuvant chematherapy is recommended.     

A resected case of pleomorphic carcinoma of pancreatic head

A 41-year-old man was admitted to the hospital complaining of back pain and progressive jaundice. Ultrasonography, CT and hypotonic duodenography revealed a large and well-defined tumor in the head of pancreas. The tumor was hypervascular on angiography. Total pancreatectomy was performed, and the examination of the resected specimen disclosed that the tumor was 50 X 45 mm in size and extrapancreatic tumor-forming type. Histopathologically, the tumor comprized mostly bizzare mono- and multinucleated giant cells with sarcomatous growth pattern. The patient died 6 months after operation due to liver metastasis and peritoneal dissemination. The pathological feature of the pleomophic carcinoma of the pancreas is well-known, but the clinical feature is vague because the resected cases are rare. The pathological finding of the specimen of this case is typical and it is considered that the clinical findings of this case, such as well-defined and hypervascular tumor, are characteristic of the pleomorphic carcinoma of the pancreas.     

Mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum: Report of a rare case and review of the literature

INTRODUCTIONMixed large cell neuroendocrine neoplasms of the rectum are rare and aggressive neoplasms. Survival is poor due to the high rate of lymph node metastases and distant metastases at the time of diagnosis.PRESENTATION OF CASEWe report a case of a 50-year-old male patient with a mixed large cell neuroendocrine carcinoma with squamous cell carcinoma of the rectum located 8 cm from the anal verge, treated with low anterior resection and total mesorectal excision with free surgical margins. There were lymph nodes metastases but no distant metastases at the time of diagnosis. The patient refused to receive adjuvant chemotherapy and died 6 months later due to liver failure as a result of multiple hepatic metastases.DISCUSSIONThere are not known predisposing factors for the development of neuroendocrine rectal carcinoma. A neuroendocrine carcinoma of the rectum is a rare tumor with an incidence of less than 0.1% of all colorectal malignancies. The median survival ranges from 5 to 10.4 months in several studies and there are not sufficient data in bibliography about ideal adjuvant therapy after resection of mixed squamous large cell neuroendocrine carcinoma of the rectum.CONCLUSIONLow anterior resection and total mesorectal excision with free surgical margins in the presence of lymph nodes metastasis is not a sufficient treatment for rectal neuroendocrine carcinoma. More studies should be done in order to determine the ideal adjuvant treatment of these rare and aggressive tumors.