Case Of Neuroendocrine Carcinoma Research Articles

腺癌成分を伴う胆嚢原発神経内分泌癌の1例

腺癌成分を伴う胆嚢原発神経内分泌癌の1例

乳腺神経内分泌細胞癌の6例

神経内分泌細胞への分化を伴う乳癌は比較的稀な腫瘍である．WHO 2003年分類ではepithelial tumorsの中でneuroendocrine tumorsに分類されている．今回われわれが経験した6例の乳腺神経内分泌細胞癌を報告する．年齢34歳～68歳（平均46.5歳），5症例は単孔性の血性乳頭分泌を主訴に来院した．超音波検査では乳管拡張や限局性の腫瘤像を認めたが，マンモグラフィ検査ではいずれも異常所見を認めなかった．乳頭分泌細胞診では良性の所見であった．2症例は超音波下針組織生検を行い，4症例は乳管腺葉区域切除の診断的治療を行い，病理組織学的検査ではH-E染色にて神経内分泌細胞癌が疑われた．免疫組織学的染色にてchromogranin Aおよびsynaptophysin染色が陽性であったことより，乳腺神経内分泌細胞癌との診断を得た．

A Case of Neuroendocrine Carcinoma of the Ascending Colon without Recurrence Treated by Adjuvant Chemotherapy Using FOLFOX

A Case of Neuroendocrine Carcinoma of the Ascending Colon without Recurrence Treated by Adjuvant Chemotherapy Using FOLFOX

A very rare case of neuroendocrine carcinoma of stomach

We report an extremely rare case of neuroendocrine carcinoma (NEC) of the stomach, which is known for its aggressive behavior and poor prognosis. To the best of our knowledge, only a few cases have been reported in the world till date and our case may be one of the very few of this rare pathology. A 42-year-old male presented with vague abdominal pain and significant weight loss. Upper gastrointestinal endoscopy revealed ulceroproliferative growth in the pylorus. Computed tomography of the abdomen showed thickening of the pylorus with locoregional lymphadenopathy. Positron emission tomography scan showed localized disease. A distal gastrectomy with D1 dissection was performed. Postoperative histopathology revealed high grade NEC of the stomach with cytokeratin-20, epithelial membrane antigen, chromogranin, and CDX-2 positivity on immunohistochemistry. He was treated with adjuvant chemotherapy followed by locoregional radiotherapy with significant response to treatment. Presently on regular follow-up without any evidence of recurrence or distant metastasis.

RARE-17NEUROENDOCRINE CARCINOMA IN THE BRAIN

OBJECT: Neuroendocrine carcinoma (NEC) is a malignant tumor that usually arises in the lung, pancreas and gastrointestinal tract. Brain metastasis of NEC is merely reported. Herein, we report two cases of NEC in the brain mimicking glioblastoma (GBM). CASES: Both patients were in their 60s, cystic lesions with peritumoral edema affected the temporal lobe. Cyst walls were thin but hyperintense in diffusion weighted images implying increased cellularity. Positron emission tomography (PET) revealed malignant nature by strong uptake of methionine (MET), fluorothymidine (FLT) and fluoromisonidazole likewise GBM. However, fluorodeoxyglucose (FDG) indicated relatively weak uptakes compared as GBM. Both tumors were resected under the preoperative diagnosis of GBM, however, pathological diagnoses were NEC by immunohistochemical finding of negative GFAP and positive markers of neuroendocrine differentiation. Systemic examination failed to reveal the origin. Both cases underwent radiotherapy. One case was lost by spinal dissemination 11 months after the operation. DISCUSSION: FDG-PET of neuroendocrine tumor in the extracranial lesion does not always demonstrate strong uptake. FDG-PET for NEC in the brain could be less accumulated than GBM, while it should be evaluated keeping in mind that the background level of FDG would be high in the brain tissue. In case of malignant tumors endorsed by other tracer such as MET and FLT, inconsistent FDG-PET finding might be useful for the diagnosis of NEC in the brain.

Mediastinal Neuroendocrine Carcinoma of Unknown Origin Presenting with Superior Vena Cava Syndrome (SVCS): A Case Report

Primary neuroendocrine carcinoma (NEC) of the mediastinum is a rare type of carcinoma. According to the literature, only five cases of this condition have been reported so far. In this paper, we present a rare case of mediastinal NEC of unknown primary site. The patient was a 34-year-old man with mediastinal NEC, who presented with chronic dry cough and a right-sided mediastinal mass one year prior to hospital admission (winter 1391). His condition was confirmed by cervical lymph node biopsy. The patient refused to undergo chemotherapy treatment. During the hospital admission, the patient presented with severe dyspnea and signs of superior vena cava syndrome. Contrast-enhanced CT scan of the chest revealed a large heterogeneous mass extended from the right superior mediastinum to the right lung base with the encasement of the superior vena cava. After radiotherapy, the patient′s symptoms subsided and he underwent etoposide and cisplatin chemotherapy. After a 20-month patient follow-up, the subject remained alive and symptom-free.

Neuroendocrine Carcinoma of Gall Bladder: A Series of 19 Cases with Review of Literature.

Neuroendocrine neoplasms of the gall bladder (GB) are rare tumors classified as grade 1 and 2 neuroendocrine tumor (NET), neuroendocrine carcinoma (large cell or small cell type), and mixed adeno-neuroendocrine carcinoma (MANEC). Primary neuroendocrine carcinomas (NEC) of GB are rare with grave prognosis not withstanding radical surgical treatment. Chemotherapy forms the primary management due to the advanced stage at presentation, as complete surgical resection is generally not possible. The overall median survival is 4-6 months despite aggressive management. Only 73 cases of small cell carcinoma (SCC) and 10 cases of large cell neuroendocrine carcinoma (LCNEC) have been reported in English literature till now. We present a series of 19 cases of NEC of GB with their pathological and clinical features and response to treatment. The objective of this study was to evaluate NECs of the GB for their clinical behavior, prognosis, and treatment outcome in terms of survival and to segregate NECs into further subcategories, that is 20-50 % and >50 %, based on MIB-1 index. We retrospectively searched the clinical and pathological database over 2.5 years (January 2012 to June 2014) through existing electronic hospital medical records to collect cases of NEC arising in GB. A total of 447 cases of gall bladder pathologies were analyzed, of which 19 cases were diagnosed as NEC. All relevant investigations were documented and response to treatment/therapy was evaluated clinically and radiologically. Tumor specimen was obtained by fine-needle aspiration cytology and/or biopsy and classified using the latest World Health Organisation (WHO) classification (2010) via cytopathologic, histopathologic, and immunohistochemical visage. Nineteen patients diagnosed as NEC of GB were evaluated in the study, with 16 having SCC, 2 having LCNEC, and 1 with MANEC. All patients presented in advanced clinical stage III or IV, along with distant metastasis and showed progressive disease during therapy. Ki67 index was between 20 and 50 % in 5 cases and >50 % in 8 cases. Follow-up was available in 14 cases with a median survival of 3 months, and 2 of these patients capitulated to their illness, before commencement of treatment. The remaining 5 cases were lost to follow-up. Out of these, 2 had deranged liver function test and no chemotherapy could be administered, and the other 3 refused treatment. NECs are aggressive with generally poor prognosis, characterized by insidious onset and advanced clinical stage of presentation. A radical approach to treatment with chemotherapy is the best form of palliation. Role of radiotherapy remains undefined due to paucity of data.

Bronchial neuroendocrine neoplasms: A Surveillance Epidemiology and End Results (SEER) database review of demographics and survival in 187,991 cases.

4097 Background: Bronchial neuroendocrine neoplasms (NENs) are a biologically heterogeneous group of malignancies whose differences are incompletely understood. We aimed to study the demographics and survival of patients with these neoplasms by reviewing the SEER database. Methods: We identified 945,331 cases in the SEER database with malignancies of the lung and extracted 187,991 bronchial NENs to examine patient characteristics & clinical outcomes by histology and stage. Results: By histology and grade, there were 130,092 cases of small cell carcinoma, 44,520 cases of large cell carcinoma, 3,349 cases of neuroendocrine carcinoma (grade 3/4), 9,135 cases of typical bronchial carcinoid (grade 1), and 895 cases of atypical bronchial carcinoid (grade 2). The ethnic predilection was Caucasian (87%, with 9% black and 4% other). Overall there were more cases in men (55% of the entire cohort), but women accounted for 66% of the carcinoid cases. Mean age at diagnosis for all NENs was 66, with typical carcinoids ...

Bronchial neuroendocrine neoplasms: A Surveillance Epidemiology and End Results (SEER) database review of treatment outcomes.

4098 Background: Bronchial neuroendocrine tumors (NENs) are a biologically heterogeneous group of malignancies with varied approaches to treatment. We queried the SEER database about outcomes of surgery and radiation. Methods: We identified 945331 cases in the SEER database with malignancies of the lung and extracted 187991 bronchial NENs – 130092 cases of small cell carcinoma, 44520 cases of large cell carcinoma, 3349 cases of neuroendocrine carcinoma (grades 3/4 [G3/4]), 9135 cases of typical bronchial carcinoid (grade 1 [G1]), and 895 cases of atypical bronchial carcinoid (grade 2 [G2]) – to examine clinical outcomes by treatment modality, including radiation in 82157 cases and surgery in 21352. Results: Surgical intervention on the primary NEN became less frequent as SEER stage become more extensive; operations were performed in 56.0% of cases of localized disease, 19.9% of regional disease, and 4.5% of distant disease. Proportionately more surgery was performed on lower-grade histologies (e.g. 84% of...

CARCINOMA NEUROENDOCRINO: REPORTE DE CASO

Se comunica un raro caso de carcinoma de cérvix neuroendocrino. Se presenta la sintomatología comunicada en la consulta inicial, el informe del estudio anatomopatológico de la biopsia, la revisión bibliográfica, así como algunos comentarios pertinentes.

A rapidly evolving carcinoma of the thymus discovered in a patient with multiple endocrine neoplasia type 1 and operated on for primary hyperparathyroidism

Introduction Thymic neuroendocrine (NE) tumors associated with multiple endocrine neoplasia type 1 (MEN-1) are rare, variably documented in 1–8% cases. They are malignant and aggressive tumors and constitute a major cause of mortality in MEN-1. We report here a case of neuroendocrine carcinoma of the thymus rapidly developing after parathyroidectomy in a patient with MEN-1. Case A 45-year-old man was admitted for headaches. He had hypercalcemia, a hypophosphatemia and high PTH. Imaging procedures favor parathyroid tumor and pituitary adenoma with slightly high prolactinemia = 1768 mU/mL. Abdominal MRI, FDG PetScan, and scintigraphy with octreotide completed the evaluation. A tumor of the thymus with a necrotic and atypical aspect and high metabolic activity without evidence of metastases was observed, together with 5 pancreatic and small bilateral adrenal nodules. The patient was operated on: in the same time resection of a retrotracheal parathyroid adenoma and of a malignant thymoma type P3 with invasion of pericardium and mediastinal fat, finally pituitay adenomectomy. Surgery was completed by mediastinal radiotherapy. Genetic evaluation confirmed MEN 1. Comment and conclusion NE tumors of thymus in MEN-1 are more common in males and smokers, quite always hormonally inactive, and incidentally diagnosed. They are malignant, aggressive tumors and are widely invasive and metastatic at presentation (affecting usually bone). They traditionally never are the initial feature of MEN-1, and almost always occur after hyperparathyroidism, thus providing an opportunity for prophylaxis at the time of parathyroid surgery. Usually tumors of the thymus occur 15–20 years after discovering g of MEN1. In contrast in our patient, the malignant tumor of the thymus was rapidly developed, that does not exclude precession of an unrecognized primary hyperparathyroidism.

A Case of Neuroendocrine Carcinoma in the Hilar Bile Duct

A Case of Neuroendocrine Carcinoma in the Hilar Bile Duct

Spontaneous Rupture of Thymic Neuroendocrine Carcinoma: A Case Report

Thymic neuroendocrine carcinoma (NEC), also known as thymic carcinoid tumors, are uncommon thymic neoplasms with neuroendocrine differentiation. Among primary thymic malignancies, NEC is the least common, accounting for less than 5% of thymic tumors (1). Thymic NEC tends to be locally invasive towards adjacent structures, such as mediastinal fat, lung, pericardium, and great vessels (2). Metastatic spread can occur by either hematogenous or lymphatic routes, and mediastinal lymph node metastasis is found in about 30 percent of patients at presentation (3). Although thymic NEC is typically identified as a large, usually invasive, anterior mediastinal mass with hemorrhage and necrosis (4, 5), it is extremely rare for it to present rupture. Herein, we report a case of the thymic NEC that was initially presented as a potentially fatal hemorrhage by capsular rupture. CASE REPORT

胸腺神経内分泌腫瘍との鑑別が困難であった中縦隔神経内分泌癌の1例

胸腺神経内分泌腫瘍との鑑別が困難であった中縦隔神経内分泌癌の1例

A case of neuroendocrine carcinoma of the cystic duct surviving two years after surgical resection

A case of neuroendocrine carcinoma of the cystic duct surviving two years after surgical resection

A case of neuroendocrine carcinoma of the gallbladder with localized liver metastases

A case of neuroendocrine carcinoma of the gallbladder with localized liver metastases

Neuroendocrine carcinoma of the urinary bladder:clinicopathologic features analysis of 17 cases

To investigate the clinicopathological characteristics and the prognosis of bladder neuroendocrine carcinoma (NEC). Clinicopathological data from 17 NEC of the bladder cases were collected, and immunohistochemical staining was performed with follow-up analysis and literature review. The recruited included 13 male and 4 female patients, aged from 48 to 86 years old (average 61 years; 14 patients >60 years). Gross hematuria of the whole urination course or intermittent was the initial symptom. Macroscopically, the outer surface of the tumor presented with polypoid, lobulated, fungating or ulcerous structures. Histologically, according to the criteria of WHO classification of neuroendocrine tumor of the lung, our NEC cases were divided into three histological types: 13 cases of small cell carcinoma, 3 cases of large cell neuroendocrine carcinoma and 1 case of atypical carcinoid. The urothelial carcinoma was concurrent with NEC in 6 cases, and adenocarcinoma was concurrent with NEC in 2 cases. Most tumor tissue infiltrated to the muscular layer, some infiltrated to the outer membrane. Immunohistochemically, the positive expression rates of CD56, Syn and CgA were 16/17, 16/17 and 12/17, respectively. The epithelial markers, including CK7 and CKpan, were also expressed with positive rates of 12/17 and 15/17, respectively. TTF-1 was positively expressed in 11 cases. The follow-up data were available in 14 cases, of which 9 patients died of the tumor 1-34 months after surgery (average, 11 months). Five patients lived uneventfully for 1-12 months after surgery. NEC is a rare malignant tumor of the bladder. Immunohistochemical markers such as CD56, Syn, CgA and CKpan could be helpful in determining the diagnosis and differential diagnosis of the tumor. NEC is a highly invasive malignant tumor with poor prognosis. Based on its biological behavior, radical cystectomy is the preferred method of treatment for the tumor.

Next-generation sequencing of salivary high-grade neuroendocrine carcinomas identifies alterations in RB1 and the mTOR pathway

Salivary gland high-grade neuroendocrine carcinomas are rare, aggressive tumors that are morphologically and immunohistochemically similar to cutaneous high-grade neuroendocrine (Merkel cell) carcinomas. The majority of Merkel cell carcinomas harbor Merkel cell polyomavirus, while the virus is rare or absent in salivary high-grade neuroendocrine carcinomas. Inactivation of retinoblastoma 1 (RB1) has been implicated in the pathogenesis of both virus-positive and -negative Merkel cell carcinomas but by different mechanisms. In virus-positive tumors, a portion of the viral genome, the large T antigen, may inactivate RB1, and in virus-negative Merkel cell carcinomas truncating mutations in the RB1 gene have been identified. The molecular genetics of salivary high-grade neuroendocrine carcinomas are not well understood. Here, we performed targeted next-generation sequencing of 151 cancer-related genes on 4 four Merkel cell polyomavirus-negative primary salivary gland high-grade neuroendocrine carcinoma cases. Somatic mutations were predominantly found within tumor suppressor genes [TP53 (3 cases), PTEN (2 cases), RB1 (1 case)]. Truncating RB1 mutations, as seen in virus-negative Merkel cell carcinomas, were not identified. However, 3 of 4 cases had RB1 deletions by copy number variation analysis. The 4th case had loss of heterozygosity for RB1. Fluorescence in situ hybridization confirmed RB1 deletions in 2 of 3 cases, and the absence of RB1 deletion in the 4th case that had loss of heterozygosity. All 4 cases showed loss of RB1 protein expression by immunohistochemistry, indicating that RB1 inactivation is important. However, the mechanism of RB1 inactivation appears different than that seen in Merkel cell carcinomas. In addition, copy number variation consistent with activation of the PI3KCA/AKT/mTOR pathway was also observed in all 4 cases. The mTOR pathway may be a potential therapeutic target in these tumors as mTOR inhibitors are currently used to treat other tumor types.

Neuroendocrine carcinoma of the esophagus: clinicopathologic study of 10 cases and verification of the diagnostic utility of mASH1, NeuroD1, and PGP9.5

Neuroendocrine carcinoma (NEC) of the esophagus is a rare histologic type that is aggressive and grows rapidly. Immunohistochemistry with conventional antibodies, such as synaptophysin, chromogranin A, and CD56, is widely performed. Several novel neuroendocrine markers, such as mammalian achaete-scute homolog 1 (mASH1), NeuroD1, and PGP9.5, were recently proposed. From 1115 consecutive cases with esophageal cancer, 10 NEC cases (0.9 %) were enrolled and the clinicopathologic parameters, including prognosis, were compared with those of 32 squamous cell carcinomas (SCC) and 30 basaloid squamous carcinomas (BSC). Immunohistochemistry for mASH1, NeuroD1, PGP9.5 and for conventional antibodies was performed. Both monoclonal and polyclonal antibodies for NeuroD1 and PGP9.5 were evaluated. Six NEC cases were diagnosed as T1b (submucosal invasion), four of which were endoscopically resected. The remaining four cases with T3 or T4 died of the disease within 37 months. Of the six patients with T1b, three are alive with no evidence of disease for more than 50 months, although the prognosis is worse for NEC than for SCC and BSC. The mASH1, NeuroD1-monoclonal, NeuroD1-polyclonal, PGP9.5-monoclonal, and PGP9.5-polyclonal immunohistochemical markers were positive in 8, 8, 1, 7, and 6 NEC cases, respectively. The number of mASH1-positive cases and synaptophysin-positive cases was the same. mASH1 had good concordance with chromogranin A and CD56, and was superior to other novel antibodies. mASH1 had excellent specificity and sensitivity for NEC. Esophageal NEC has a worse prognosis than SCC and BSC, even in T1b patients. mASH1 is an excellent novel marker for diagnosing esophageal NEC.

Multiple scalp metastases from colonic neuroendocrine carcinoma: case report and literature review.

BackgroundColonic neuroendocrine neoplasms (NENs) are relatively rare tumors with an incidence rate of 0.11–0.21/100,000. NENs account for approximately 0.4% of colorectal neoplasms. Cutaneous metastases of colonic neuroendocrine carcinomas (NECs) are very infrequent, while cases of scalp metastasis are even fewer. Cutaneous metastases are more rare than visceral metastases and usually develop later; therefore, cutaneous metastases as initial distant metastases can be easily overlooked. This is the second case report of a colonic NEC with scalp metastasis. Compared with the previous case, in this instance scalp metastasis developed before visceral metastasis, and the cutaneous lesions were confined to the scalp alone.Case presentationA 62-year-old Chinese man, who had undergone radical surgery for a “locoregional” colonic NEC one and half months before, came to our hospital for adjuvant chemotherapy. We found multiple scalp nodules during physical examination. Moreover, these nodules had occurred and had not been detected prior to the patient undergoing radical surgery. The scalp nodules proved to be metastases from colonic NEC as determined using pathological and immunohistochemical examinations following lumpectomy. After one and half months, visceral metastases were detected in this patient. Ultimately, the patient died two months later.ConclusionsIn this report an unusual case of a colonic NEC with initial distant metastasis confined to the scalp is presented. This case is unusual because of the development of cutaneous metastasis before visceral metastasis. The scalp metastasis were initially overlooked, leading to inaccurate staging and radical surgery that was not curative. This demonstrates that distant metastasis can occur during the early phase of tumor growth in these aggressive lesions. Thus, the possibility of distant metastases should be assessed in the initial work up to avoid mistaken clinical staging especially when distant metastases occur only in skin.