Introduction: Proteus syndrome (PS) is a rare genetic disorder usually caused by mutations in AKT1 or PTEN genes, characterized by multiple, asymmetric tissue overgrowth with high clinical variability. Sinonasal neuroendocrine carcinomas (SNEC) are exceptionally rare tumors encountered in the ethmoid sinus, nasal cavity, or maxillary sinus. Case Report: We report a 35-year-old patient with PS, who underwent successful surgical removal of a well-differentiated SNEC obstructing his nasal cavity and highlight the role of the otolaryngologist for safe airway management, minimal surgical intervention and coordination of the multidisciplinary care. Histologically, focally hyperplastic mucosal epithelium of respiratory type of the nasal chamber was noticed along with seromucinous glands and capillary congestion of the subepithelial fibrovascular tissue. The limited presence of neoplastic tissue with histomorphological and immunophenotypic features of a neuroendocrine neoplasm was focally observed. Tumor cells grow in the form of islets within a vascular stroma,these neoplastic cells are immunohistochemically positive for synaptophysin, CD56, EMA, Ki67 (low expression, cell proliferation rate: 2%), CD31, chromogranin and pancytokeratin AE1 / AE3 as well as for S-100 protein (weak intensity) Conclusions: This first description of a SNEC in a PS patient, might hint towards a common basis between the two conditions, due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway.

Introduction: Neuroendocrine carcinoma is one of the neoplasms of neuroendocrine system which involves organs sparing of APUD cells. The most common sites of this carcinoma are gastrointestinal and pulmonary system. From a histologic standpoint, these tumors are reactive to silver stains and specific markers like NSE (Neuron Specific Enolase), synaptophysin, and chromogranin A. Case Report: A 74-year-old woman with a complaint of 6-month nasal stuffiness, epistaxis, anosmia, and hearing loss was referred to the hospital. In her nasal examination, a vascular polypoid mass in nasopharynx was noticed. CT-scan showed a homogeneous, isodense mass in nasopharynx without bony erosion.Conclusion: After confirming the diagnosis of carcinoid tumor using specific markers and special stains, there are some factors to predict its malignancy. Systemic chemotherapy(esp. cisplatin & etuposide) is effective to treat malignant neuroendocrine tumors especially aggressive variants. Radiotherapy has palliative effect.

Objective: Small cell neuroendocrine cervical carcinoma is a neuroendocrine tumor with the great aggravation that comprises 0. 5 to 3 percent of cervical tumors and progresses rapidly with early lymphogenous and hematogenous metastases. Case report: We reported a 40 years old woman with cervical cancer in stage IB2 that had radical hysterectomy with mistaken diagnosis of squamous cervical cancer. The disease has progressed after 50 days of surgery with a 6 cm tumor in vaginal cuff,review of pathology demonstrated small cell neuroendocrine cervical carcinoma. Conclusion: Recognition of this separate histopathological entity with IHC analysis is important. Chemoradiotherapy and multimodality therapeutic approaches could improve the survival rates.

Gastric neuroendocrine carcinoma is a rare tumor which has a poor prognosis. Herein, we present a 55-year-old woman who presented with complaints of recurrent vomiting, hematemesis and weight loss. Endoscopic examination showed a large ulcerated mass in the antrum. Microscopic evaluation of the specimen taken through biopsy was compatible with a small round cell tumor. However, definitive histopathological diagnosis was made after surgical resection which revealed a neuroendocrine neoplasm immunohistochemically positive for Chromogranin A and Neuron specific enolase. As a result a diagnosis of neuroendocrine carcinoma of stomach was made for the patient.

Introduction: Small cell neuroendocrine carcinoma (NEC) that arises from the tonsil is a particularly rare head and neck carcinoma. This kind of neoplasm mainly originated from the bronchopulmonary area,however, there were reported cases of extrapulmonary areas. The prognosis is poor as the tumour is an aggressive tumour and have a high risk of metastasis. Case Report: We experienced a patient presented with painless right neck swelling and hard tonsillar hypertrophy for past six month. Computer tomography showed the tumour extended to the parapharyngeal space and metastasized to the thoracolumbar vertebras. The intraoral biopsy of the tonsil confirmed primary small cell neuroendocrine carcinoma of the tonsil. The clinical presentation, radiological imaging, histopathological investigations, and methods of treatment are discussed. Conclusions: Due to the rarity of this disease, there is no definitive treatment yet for this disease. The physicians must thoroughly understand the nature and characteristic of the disease to find the best treatment. The latest discoveries in chemotherapy drugs and radiotherapy may improve the treatment modalities in the future.

Introduction: Dispersed neuroendocrine system (D.N.S) consists of a wide variety of cells that are present in the central and peripheral nervous system and in many classic endocrine organs and different tissues such as respiratory and gastrointestinal tracts, skin, prostate, breast and also their neoplasm show neuroendocrine differentiation by electron microscopy, immunohistochemistry or biochemical techniques. Materials and Methods: The present study has been carried out by case-series method in order to evaluating the characteristics of all types of neuroendocrine carcinoma: different anatomical locations during 5 years period in immunohistochemistry department of cancer institute. Results: The diagnosis of 109 cases of neuroendocrine carcinoma consisting of neuroendocrine carcinoma, small cell carcinoma, medullary carcinoma of thyroid, carcinoid tumor and merkel cell carcinoma are confirmed that among them the most common diagnosis was related to neuroendocrine carcinoma (50.5 percent ). The most prevalent age group was 40-49 years and male to female distribution were 56 percent and 44 percent respectively. Anatomical distribution of tumor show that about 30 percent of cases were metastatic carcinoma, 30 percent in thyroid, respiratory tract and head and neck region and remainder in a variety of tissues. In over 50 percent of cases one of endocrinoid patterns as trabecular, organoid or mixed of them were seen. Conclusion: Immunohistochemically N.S.E (Neuron Specific Enolase) show high sensitivity with 96 percent positive reaction and more specific endocrine markers as chromogranin A in 80 percent and synaptophysin only in 24 percent because of lesser application of the latter. Also epithelial markers such as cytokeratin and E.M.A. (Epithelial Membrane Antigen) were positive in 69 percent and 74 percent respectively. Mean survival rate of all neuroendocrine carcinoma reached to 4.8 years with lowest survival of 4.3 years among small cell carcinoma and highest in merkel cell carcinoma with 5.5 years.

allbladder neuroendocrine tumors remain relatively rare in the clinical setting. They are generally asymptomatic and reported as incidental findings. The diagnosis is exclusively made by histopathological and immunohistological examination according to the recent WHO guidelines. Here, we present the case of a 58-year-old woman with small-cell gallbladder neuroendocrine carcinoma.

Mixed hepatocellular-neuroendocrine carcinoma (HCC-NEC) is a rare entity with a poor prognosis. We report a case of a 44-yearold Tunisian man who was admitted for diffuse abdominal pain. Body computed tomography showed multinodular hepatomegaly. Pathologic findings concluded to HCC-NEC. Clinicians should be aware about this entity. Further collection of case reports is needed to standardize the optimal treatment.

Mucinous carcinoma of the breast is a well-differentiated type of adenocarcinoma accounting for 2-5% of all breast cancers. Pure mucinous carcinoma of the breast has a favorable prognosis, usually seen in post-menopausal women. Neuroendocrine differentiation has been described in both in-situ and infiltrating breast cancers. Mucinous carcinomas of the breast appear to have the greatest association with neuroendocrine differentiation. Chromogranin A and synaptophysin are specific immunohisto chemical markers of neuroendocrine differentiation. Wereporta case of mucinous carcinoma of the breast with neuroendocrine differentiation in a 67-year-old female who was treated surgically in a classical manner.