Cases reported "Carcinoid Tumor"

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1/13. Pancreatic carcinoid: an unusual tumour in an uncommon location.

    Primary pancreatic carcinoid is an extremely rare pancreatic neoplasm. It differs from other primary pancreatic tumours in cytoarchitecture, immunocytochemistry and biologic behaviour. Recognition of this rare entity is of vital importance having considerable therapeutic and prognostic implications. We report a case of an exophytic, pancreatic body carcinoid tumour in a man who presented with abdominal pain. The diagnosis was established by histopathological examination of the core biopsy specimen. A surgical resection of the lesion was done successfully and the patient made a satisfactory recovery from the operation.
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2/13. growth hormone-releasing hormone (GHRH) and GHRH receptor (GHRH-R) isoform expression in ectopic acromegaly.

    Bronchial endocrine neoplasms causing acromegaly due to ectopic production of growth hormone (GH)-releasing hormone (GHRH) have been reported. We describe the case of a 39-year-old man with clinical and biochemical acromegaly. magnetic resonance imaging revealed an enlarged pituitary, which was confirmed histologically to harbour somatotroph hyperplasia. Further investigations identified a circumscribed central mass in the right lung which was surgically resected and histologically confirmed to be an endocrine tumour with strong immunopositivity for GHRH, synaptophysin and chromogranin; the lesion also exhibited mild positivity for peptide yy, calcitonin gene-related peptide (CGRP), glucagon-like peptide (GLP)-1, corticotrophin-releasing hormone (CRH), tyrosine hydroxylase, vasoactive intestinal peptide (VIP) and enkephalin. S100 protein was identified in stellate cells surrounding nests of epithelial tumour cells. The MIB-1 antibody labelled about 10% of the tumour cells. We established that the tumour not only produced GHRH but the GHRH-receptor (GHRH-R) as well. GHRH and GHRH-R mRNA were identified and the latter was characterized as two variants, a full-length transcript and a truncated splice variant that has been described in human pituitary somatotroph adenomas. We suggest that GHRH expression by this tumour and the presence of its receptor may be responsible for enhanced growth. The expression of a truncated splice variant that is unable to transduce GHRH signalling may be implicated in the less aggressive behaviour of well-differentiated endocrine tumours that produce GHRH compared with small-cell lung carcinomas that are very responsive to GHRH growth stimulation.
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3/13. Primary ovarian trabecular carcinoid tumour: a case report with an immunohistochemical study and a review of the literature.

    INTRODUCTION: Primary ovarian carcinoid tumours are uncommon neoplasias. There are distinct histological types with different behaviours: insular, trabecular, mucinous and mixed. The trabecular subtype is very rare and unlike other carcinoid subtypes, it is characterised by the absence of a clinical carcinoid syndrome and has been related with a better prognosis than the others. No distant metastases have yet been reported. CASE REPORT: We present a case of a 76-year-old woman diagnosed with a left ovarian tumour. She underwent a radical hysterectomy with bilateral salpingo-oophorectomy and regional lymph node clearance. histology revealed an ovarian trabecular carcinoid tumour and no adjuvant treatments were performed. The patient is alive and free of disease 70 months following diagnosis. DISCUSSION: Immunohistochemical staining for p53 protein, and a comparative study with other subtypes of ovarian carcinoid tumours (insular and mucinous) is discussed in this report.
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4/13. gallbladder carcinoid: a case report.

    Carcinoids are neuroendocrine tumours that rarely originate in the biliary tract. We report a case of gallbladder carcinoid associated with cholelithiasis extending to hepatic segments IV and V. The mass was detected by ultrasound in a 71-year-old-man investigated for recurrent right upper quadrant pain irradiating to the back and associated with dyspepsia. cholecystectomy, locoregional lymphadenectomy and 4th and 5th hepatic segment resection were performed. The patient underwent chemotherapy and was also treated with somatostatin analogues. Despite this, he died after 12 months. We stress the rarity and aggressive behaviour of gallbladder carcinoid.
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5/13. Presacral carcinoid tumour. review of the literature and report of a clinically malignant case.

    Carcinoid tumours arising in the presacral region are extremely rare and they are usually benign. We report the case of a 37-year-old black man with a clinically malignant carcinoid tumour (well differentiated endocrine carcinoma) occurring in a sacrococcygeal teratoma and already metastasised to pelvic nodes, liver and bone at the time of the initial diagnosis. Such an aggressive behaviour of the presacral carcinoid tumours has never been described. The derivation of these tumours from hindgut rests with reference to embryological development of the tailgut cysts is discussed.
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6/13. Adeno-carcinoid or amphicrine tumors of the middle ear a new entity?

    The clinicopathological, ultrastructural and immunohistochemical characteristics of four primary tumors of the middle ear are reported. These neoplasms showed a striking, heterogeneous aspect ranging from solid-trabecular (Type I) to tubulo-glandular (Type II) growth patterns. Secretory activity of the tumor cells was evaluated by immunohistochemistry and electron microscopy. Based on these procedures, three cell types were found, mainly limited to tumors with a tubulo-glandular (Type II) growth pattern. Most frequent were B-cells with an abundant pale cytoplasm containing neuroendocrine granules, both cytokeratin and vimentin as well as several endocrine marker substances. Less frequent were A-cells, which are slender, darkly staining and line the glandular lumina. They showed exocrine activity only and stained strongly with a polyclonal cytokeratin antibody. Finally, least frequent were amphicrine cells, which were characterized by both lumina and neuroendocrine granules in their cytoplasm and were interpreted as the link between A and B cells. Although this morphological description closely resembles that of carcinoids and adenocarcinoids of the respiratory tract and gut, the clinical behaviour of these middle ear tumors nevertheless seems different, with no recurrence or metastasis after a follow-up of 1 to 14 years (median 78 months). Therefore, some authors suggest that these tumors should be classified as middle ear adenomas or adenomatous tumors. However, we strongly feel that these tumours represent a distinct entity and can be classified as adenocarcinoids or amphicrine tumors, i.e. demonstrating both exocrine and endocrine activities. Further work is required to evaluate the exact proportion of neuroendocrine and amphicrine tumors in the heterogeneous group of adenomas and in the rarely described group of adenocarcinomas.
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7/13. Primary carcinoid tumour of the pancreas.

    Pancreatic carcinoid tumours are extremely infrequent. Usually, the biological behaviour is indolent and diagnosis is late and often casual. We present the case of a patient initially diagnosed as having liver metastasis of unknown origin. PET identified a primary pancreatic site and the initial histologic diagnosis was adenocarcinoma. Following an uncertain response to chemo- and radio-therapy the repeat histologic assessment indicated a carcinoid tumour of the pancreas. After complete surgical resection and liver transplantation, patient remains free of disease. CONCLUSIONS: The co-existence of several diseases with similar morpho-structural features makes diagnosis complicated. PET is of uncertain use in the evaluation of carcinoid tumours, and is considered inferior to 111Indium-octreotide scan. The only curative treatment is surgical resection, with liver transplantation as a valid option in the treatment of these tumours.
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8/13. Carcinoids of the kidney: case report and literature review.

    Primary carcinoids of the kidney are very rare, only 10 cases having been reported in the literature. The authors report a case of primary renal carcinoid in a 50-year-old woman. A radical nephrectomy with lymphadenectomy was successfully performed and there was no residual or recurrent tumour at 2-year follow-up. A review of the reported cases revealed a variable, nonspecific presentation. Most laboratory tests were non-contributory except for urinalysis. When a renal carcinoid is diagnosed, a search should be made for a possible primary elsewhere. Primary renal carcinoid does exhibit malignant behaviour. It should be managed by radical nephrectomy with retroperitoneal lymphadenectomy.
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9/13. Goblet cell carcinoid of the appendix.

    Goblet cell carcinoid of the appendix is a relatively uncommon clinical condition. Although its histogenesis remains controversial, its histological characteristics are distinctive enough not to pose a serious problem for diagnosis. The clinical behaviour of the tumor appears to lie between the typical behaviour of an ordinary carcinoid and that of a well differentiated adenocarcinoma of the appendix. Clinical diagnosis of this condition is seldom made pre-operatively, most patients presenting with signs and symptoms of an acute appendicitis. In a number of patients the tumor is identified incidentally during an operation performed for some unrelated entity. Treatment of this tumor is generally by simple appendectomy, although in some patients a more radical procedure is indicated. prognosis for the most part is quite favorable with the exception of those few patients who present with the more virulent form of the disease.
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10/13. Ectopic ACTH production by a thymic carcinoid tumour.

    An ACTH-producing thymic carcinoid tumour was diagnosed in a 10-year-old girl, 8 years after bilateral adrenalectomy for Cushing's syndrome. The peptides produced by the tumour were characterised thoroughly. High circulating levels of beta-endorphin and other peptides may have contributed to mood and behaviour disturbances.
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