Pancreatic endocrine tumors or apudomas.

INTRODUCTION AND OBJECTIVE: pancreatic endocrine tumors (PET) are difficult to diagnose. Their accurate localization using imaging techniques is intended to provide a definite cure. The goal of this retrospective study was to review a PET series from a private institution. PATIENTS AND METHODS: the medical records of 19 patients with PETs were reviewed, including 4 cases of MEN-1, for a period of 17 years (1994-2010). A database was set up with ten parameters: age, sex, symptoms, imaging techniques, size and location in the pancreas, metastasis, surgery, complications, adjuvant therapies, definite diagnosis, and survival or death. RESULTS: a total of 19 cases were analyzed. Mean age at presentation was 51 years (range: 26-67 y) (14 males, 5 females), and tumor size was 5 to 80 mm (X: 20 mm). Metastatic disease was present in 37% (7/19). Most underwent the following imaging techniques: ultrasounds, computed tomography (CT) an magnetic resonance imaging (MRI). Fine needle aspiration punction (FNA) was performed for the primary tumor in 4 cases. Non-functioning: 7 cases (37%), insulinoma: 2 cases [1 with possible multiple endocrine neoplasia (MEN)], Zollinger-Ellison syndrome (ZES) from gastrinoma: 5 (3 with MEN-1), glucagonoma: 2 cases, 2 somatostatinomas; carcinoid: 1 case with carcinoide-like syndrome. Most patients were operated upon: 14/19 (73%). Four (4/14:28%) has postoperative complications following pancreatectomy: pancreatitis, pseudocyst, and abdominal collections. Some patients received chemotherapy (4), somatostatin (3) and interferon (2) before or after surgery. Median follow-up was 48 months. Actuarial survival during the study was 73.6% (14/19). CONCLUSIONS: age was similar to that described in the literature. Males were predominant. Most cases were non-functioning (37%). Most patients underwent surgery (73%) with little morbidity (28%) and an actuarial survival of 73.6% at the time of the study.

[Oncocytoma of the pancreas. A case report]. / Vurkhu edin sluchai s onkotsitom na pankreasa.

For the first time in the bulgarian literature a case with pancreatic oncocytoma is described, initially diagnosed as carcinoma. The patient survived for 13 years being nowadays in perfect condition.

[Ultrastructural phenotypes of tumor cells of endocrine-cellular neoplasms of hepatopancreatoduodenal organs and their role in determining the degree of malignancy and prognosis of these diseases]. / Ul'trastrukturnye fenotipy opukholevykh kletok éndokrinno-kletochnykh novoobrazovanii organov gepatopankreatoduodenal'noi zony i ikh znachenie v opredelenii stepeni zlokachestvennosti i prognoza étikh zabolevanii.

78 tumors of the hepatopancreoduodenal system were studied clinically, cytologically and ultrastructurally. Hormonal disturbances were observed in 36% of the patients. The 5-year survival after radical surgery did not depend on the tumor size, tumor cell atypia or metastases to the regional lymph nodes. The prognosis was worse when the tumor was located in the liver. Ultrastructural features of the tumor cells were reliable criteria of the malignancy degree and tumor prognosis. The prognosis, recurrence-free interval and survival improve with an increase in the number of ultrastructurally differentiated cells and organoids in cytoplasm, and with a reduction in nuclear polymorphism and number of dark cells. The degree of histologic and ultrastructural differentiation of tumor cells may not coincide.

El sistema APUD en la oftalmología: II. tumores de la cresta neural. Derivados del sistema pigmentario (melanogénico): nevi y melanomas palpebrales y conjuntivales / APUD System in opthalmology: II. neural crest tumors: Pygmentary system derives (melanogenic): nevi and palpebral and conjunctival melanomas

Los tumores de la cresta neural en la oftalmología inclyen entre otros, los derivados del sistema melanogénico de los que sobresalen los siguientes: Nevi de la piel y de la conjuntiva que se clasifican según su localización en superficiales y profundos; dentro de los primeros están los de unión, intradérmico (subepiteliales), mixtos y como profundos los nevi azul fusocelular y celular. Las formas malignas corresponden a los melanomas cutáneos y conjuntivales. Estas neoplasias son tumores de comportamiento biológico muy variable y morfología compleja, el diangnóstico clínico presuntivo es difícil en algunas variedades histológicas. El tratamiento depende de las variantes clínicas y/o histológicas. El objetivo de este trabajo es proporcionar una revisión actual de estos neurolofomas

[Mucinous breast carcinomas with neuroendocrine differentiation]. / Mutsinozni kartsinomi na g'rdata s nevroendokrinna diferentsiatsiia.

Mucinous breast carcinomas, denominated also gelatinous, mucoid and colloid (collomas), represent a heterogeneous group of neoplasms. More than half of them exhibit signs of neuroendocrine differentiation. Eighteen mucus producing carcinomas of the breast are subjected to morphological study. On the ground of demonstrating argyrophilia by the methods of Grimelius and Churukian--Shenk, and presence of secreting granules during electron microscopic study, they are assigned under the heading apudomas, i.e. tumors of the diffuse endocrine system, or the so-called APUD-system. The important practical implications of demonstrating neuroendocrine cells with a special reference to the biological patterns of this particular type of tumors are discussed.

Apudoma vesical / Bladder apudoma

Se presenta un caso de paraganglioma vesical, un tumor poco frecuente del sistema APUD

[Apudoma of the prostate]. / Apudomy predstatel'noi zhelezy.

Prostatic apudomas are represented by well-, moderately- and poorly differentiated variants. Histologic differentiation determines various functional properties of tumor cells. Tumor cells of well-differentiated apudomas produce serotonin, less frequently calcitonin, poorly differentiated ones produce only ACTH. Apud cells of moderately differentiated tumors, besides their main function to synthesize biogenic amines and hormones (serotonin, ACTH) possess immunoreactivity to epithelial membrane antigen, carcinoembryonal antigen and prostatic specific antigen.

[Endocrine-cell stomach tumors]. / Endokrinnokletochnye opukholi zheludka.

Screening of the endocrine cell participation in the stomach carcinoma has been performed. Endocrine cells are found in all stomach tumors and those in which these cells occupy more than 75% of the surface are distinguished as endocrine cell carcinomas (ECC). They are subdivided into well (WD), moderately (MD) and poorly differentiated (PD). ECC are more frequently observed in males, their predominant location is cardia and fundus. The growth in the deep parts of mucosa and submucosa (this determines late clinical symptoms) is characteristic for these tumors. Alveolar, trabecular and glandular structural variants are observed in WD ECC and MD ECC, while PD ECC corresponded to small cell carcinoma (iat cell and intermediate types). Prognosis is unfavorable in MD ECC and PD ECC. Apart from this amacrine and combined tumors with an endocrine component are described. The authors emphasize the necessity to single out ECC from whole group of stomach carcinoma.

Cartinoid tumor of the middle ear and mastoid.

Primary cartinoid tumors in the middle ear and mastoid are rare. They are also very difficult to distinguish from adenomas and adenocarcinomas, using conventional histological stains. We present clinical, histological, immunohistochemical and ultrastructural findings of a cartinoid tumor in the middle ear and mastoid in a 40-year-old male. A soft tumor was revealed in the posterior mesotympanum and mastoid cavity, and a radical tympanomastoidectomy was performed. The tumor cells were stained by chromogranin A, and neurosecretory granules were confirmed with electron microscopy. We also review 20 previously reported cases in regard to their presentation, symptoms, signs, tumor extension, treatments, and histopathology.

Carcinoid of the ampulla of Vater. Clinical characteristics and morphologic features.

BACKGROUND: Carcinoid tumors of the gastrointestinal tract are most common localized in the appendix, followed by the small intestine, the rectum, and the stomach. The localization of these tumors at the ampulla of Vater is extremely seldom. METHODS: In the present study the authors describe two patients with carcinoid tumors of the ampulla Vater and review 71 previously published cases. RESULTS: Most patients presented with jaundice, but without carcinoid syndrome. Because the tumor grows submucosally, preoperative diagnosis was correct only in 15%. Most tumors were around 2 cm in size. Metastasis to lymph nodes and/or liver was present in 45%. Standard treatment is Whipple resection or local excision in small tumors. CONCLUSIONS: Carcinoid tumors of the ampulla of Vater are an extremely rare clinical entity. Generally, the prognosis is good with a 5-year survival period of 90%.

Pathology of APUDomas.

The pathology of APUDomas involves two main areas. The first is the biochemical aspects of these tumours and the use of biochemical tests to investigate a patient suspected of having an APUDoma, and the other is the histopathology of these tumours. This review is devoted to the histopathological considerations.

[Cushing syndrome in ACTH-producing neuroendocrine tumor of the hepatic duct]. / Cushing-Syndrom bei ACTH-produzierendem neuroendokrinen Tumor des Ductus hepaticus.

We present a patient with an ACTH producing neuroendocrine tumor of the hepatic bile duct which was detected by chance during abdominal surgery for Cushing's syndrome. Diagnostic strategy and surgical therapy in patients with neuroendocrine tumors are discussed. The diagnostic problems caused by combination with an endocrine function disorder like Cushing's syndrome are pointed out.

[Neuroendocrine system and its tumors]. / Neuroendokrines System mit seinen Tumoren.

The original classification of neuroendocrine tumours proposed by Pearse was based on a common embryologic origin in the neuroectoderm. The term, carcinoid, literally means carcinoma-like, was coined in 1907 to describe the histologic similarity of these tumors to carcinomas on the one hand and their generally indolent biologic behaviour on the other hand. Neuroendocrine tumours represent a group with complex biological, histological, ultrastructural and immunocytochemical properties. This concept was replaced by another classification based on results of modern techniques (electron microscopy, immunocytochemistry, molecular and DNA analyses). This permits a more reliable classification of tumours, that can be used to determine their biological behaviour and prognosis.

[The diagnostic and therapeutic problems of endocrine tumors of the digestive system: our experience]. / Problematiche di diagnosi e terapia dei tumori endocrini dell'apparato digerente: la nostra esperienza.

The authors experience in the surgical treatment of endocrine tumours of the digestive tract is reported. Particularly, they emphasize that in spite of the several syndromes associated with these neoplasms, diagnostic and therapeutic concepts herein analyzed are similar.

Metastasizing neuroendocrine tumor of unknown origin found in cervical lymph nodes: a case report.

Neuroendocrine tumors are a diverse category of tumors that have been known to produce biologically active amines and ectopic hormones of various types. Numerous metastasizing primary lesions have been identified, however, the head and neck region is not a common site for them. Even less common than these are head and neck metastatic sites without an identifiable primary lesion. We report a case of a metastatic neuroendocrine tumor, where the primary site eluded detection despite an extensive battery of investigations.

[A case of carcinoid tumor of the breast]. / Sluchai s kartsinoiden tumor na mlechnata zhleza.

A rare tumor of the mammary gland, built up of argyrophilic cells, with numerous metastases in the axillary lymph nodes is reported. Histochemical and electron-microscopic studied unequivocally demonstrated that this tumor belonged to the APUD system. The possible histogenesis of carcinoid tumor of the mammary gland is briefly discussed.