Cases reported "Neuroendocrine Tumors"

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1/11. Middle ear adenoma with neuroendocrine differentiation.

    The lining of the middle ear cleft is normally a thin modified respiratory type mucosa. Normal mucosa of the middle ear is devoid of salivary type seromucous glands. Middle ear adenoma is a rare tumor that seems to be derived from the middle ear mucosa. This tumor has been previously described under a variety of names because of its different nature and biological behavior. We herein report a case of middle ear tumor that shows adenomatous and neuroendocrine features. A 64-year-old woman presented with a history of hearing loss, tinnitus and stuffy feeling of the right ear. The patient was treated two times for ear polyp. She finally underwent a tympanomastoidectomy and there was no evidence of recurrence 18 months after the procedure. Histopathological examination displayed cuboidal and columnar cells, arranged as glands, trabeculae and solid sheets. Neuroendocrine differentiation was revealed by immunohistochemical staining with polyclonal antibodies against neuron specific enolase and chromogranin antigens.
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2/11. Recurrent prostate carcinoma presenting as omental large cell carcinoma with neuroendocrine differentiation and resulting in bowel obstruction.

    Neuroendocrine differentiation in the neoplastic prostate varies from foci of adenocarcinoma showing immunoreactivity to the pure small cell carcinoma, which correlates with poor prognosis. Widely metastatic disease in unusual sites is reported for small cell carcinoma, and rarely is the serum prostate-specific antigen level elevated. We report a case of recurrent prostate adenocarcinoma presenting as bowel obstruction due to widespread metastatic disease in the omentum and peritoneum. The histopathology of the omental metastasis was that of a large cell neuroendocrine carcinoma, without evidence of an adenocarcinoma. The absence of a clinically evident second primary tumor, the concomitant elevated serum prostate-specific antigen level, and the positive tissue immunoreactivities to prostatic markers all supported the prostatic origin of the omental tumor. review of the importance of prostatic neuroendocrine differentiation and its unusual metastatic patterns is presented.
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3/11. Dendritic cell immunotherapy in a neuroendocrine pancreas carcinoma.

    OBJECTIVE: Metastatic neuroendocrine carcinomas of the pancreas frequently fail to respond to conventional therapies, including radiation and chemotherapy. We therefore tested a dendritic cell-based immunotherapy in an attempt to eradicate residual tumour masses in a patient suffering from a metastatic insulin-producing pancreatic carcinoma. DESIGN: Autologous dendritic cells (DCs) were generated from peripheral blood monocytes in the presence of granulocyte/macrophage colony-stimulating factor, interleukin-4 and tumour necrosis factor alpha. DCs were loaded with tumour-derived lysate (TL), and were delivered by subcutaneous injections in 4-week intervals. RESULTS: Three weeks after first treatment, the patient developed a strong delayed-type hypersensitivity (DTH) skin reaction with an erythema and induration after the challenge with TL-pulsed DCs, which indicates the efficient generation of antigen-specific memory T-cells. Immunohistochemical analysis of skin biopsy demonstrated a strong perivascular and epidermal infiltration by T-helper (CD4 positive) and cytotoxic T cells (CD8 positive). Stimulation with TL revealed a dose-dependent T-cell proliferation with a stimulation index of 1.1-5.7 compared to 1.1-1.4 before vaccination (P < 0.01). Most strikingly, DC-based vaccination was accompanied by a steady decrease of the tumour marker chromogranin a from 2.93 umol/l initially to below the detection limit of 0.15 umol/l within 9 months of therapy. The ultrasound examination revealed a tumour regression of the metastasis in the right lobe of the liver. CONCLUSIONS: Our data indicate that vaccination with tumour lysate-pulsed DCs induced a significant antitumour immune response in a neuroendocrine carcinoma of the pancreas. This approach represents an alternative strategy for the treatment of advanced neuroendocrine carcinomas that are resistant to conventional therapy.
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4/11. Rare case of neuroendocrine small cell carcinoma of the endometrium with paraneoplastic membranous glomerulonephritis.

    Small cell carcinomas are well-recognized tumors known to occur predominantly in the lung. These neoplasms are occasionally associated with a variety of paraneoplastic syndromes. Four cases of paraneoplastic glomerulopathy associated with small cell lung carcinoma have been reported. However, there have been no reports in the literature indicating an association between endometrial small cell carcinoma and paraneoplastic glomerulopathy. We report a case of neuroendocrine small cell carcinoma of the endometrium associated with membranous glomerulonephritis (MGN), which appeared to be a component of an unusual paraneoplastic syndrome. A 33-year-old multiparous woman presented with abnormal vaginal bleeding and abdominal bloating. Endometrial biopsy revealed neuroendocrine small-cell carcinoma of the endometrium. On the eighth day of hospitalization the patient suddenly developed renal failure. Renal biopsy revealed MGN, probably due to tumor-antigen-related immune complex deposition. Small cell carcinoma of the endometrium may be associated with paraneoplastic MGN. medical staff should take into account the possibility of a preexisting glomerular injury when managing a patient with small cell carcinoma of the endometrium.
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5/11. Small cell carcinoma of the prostate expressing prostate-specific antigen and showing syndrome of inappropriate secretion of antidiuretic hormone: an autopsy case report.

    An autopsy case of primary small cell carcinoma (SCC) of the prostate in a 68-year-old man is reported. The patient was admitted to hospital because of a bloody stool and suspected rectal cancer. However, a diagnosis of prostate cancer was made on the basis of a digital rectal examination, the serum level of prostate-specific antigen, and a needle biopsy of the prostate. The patient also experienced a syndrome of inappropriate secretion of antidiuretic hormone. He died 29 days after admission. At autopsy, the tumor had invaded the rectum, bladder and pelvic peritoneum. Metastases to the heart, vertebrae and lymph nodes were observed. Microscopically, the tumor was composed of small round cells that showed a solid growth pattern. Rosette formations were observed. Immunohistochemically, the tumor cells were positive for a prostatic epithelial marker and neuroendocrine markers. A high level of antidiuretic hormone was detected in the tumor tissue. To our knowledge, this is the first reported case of SCC of the prostate in which both a prostatic epithelial marker and neuroendocrine markers have been found in the same tumor. This finding supports the hypothesis that SCC of the prostate originates from a multipotential stem cell of the prostatic epithelium.
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6/11. Metastatic conventional prostatic adenocarcinoma with diffuse chromogranin a and androgen receptor positivity.

    Conventional prostate adenocarcinomas consist mainly of tumour cells of luminal immunophenotype with scattered neuroendocrine (NE) cells. NE cells are defined by chromogranin a (CGA) immunoreactivity. Unlike luminal cells, NE cells lack androgen receptor (AR) and prostate specific antigen (PSA) immunoreactivity. This report describes the first case of conventional prostate adenocarcinoma expressing CGA, PSA, and AR as determined by immunohistochemistry. A 64 year old man was diagnosed with conventional prostate adenocarcinoma in 1993; he underwent cystoprostatectomy in 1994; he developed an iliac bone metastasis in 1997 and mediastinal lymph node metastases in 1999. All specimens obtained during the progression of the disease consisted primarily of luminal cells with only scattered NE cells. In contrast, in samples of non-osseous and osseous metastases obtained at necropsy in 2001, greater than 80% of tumour cells were shown to express PSA, AR, and CGA. This suggests that during tumour progression, conventional prostate adenocarcinomas may evolve into an NE cell phenotype.
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7/11. von hippel-lindau disease presenting as pancreatic neuroendocrine tumour.

    A 21-year-old woman with a family history of von hippel-lindau disease presented with a mass in the head of the pancreas. light microscopic features of the tumour suggested neuroendocrine differentiation and although it displayed positive immunostaining for the antigens expected in a neuroendocrine neoplasm, S-100 staining was also present. This unusual feature prompted further evaluation by routine and post-embedding protein-A gold immunoelectron microscopy, which demonstrated the presence of neuroendocrine granules. Tumour cell dna content was normal by flow cytometry. Although this patient exhibited no other signs of von hippel-lindau disease, the presence of a pancreatic tumour with neuroendocrine differentiation demonstrated that she was affected. Future surveillance and genetic counselling will be influenced by this diagnosis.
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8/11. Neoplastic endocrine cells in prostatic carcinoma: a case report with immunocytochemical and electron microscopic findings.

    A case of a 68-year-old male with prostatic carcinoma which was partially well differentiated but largely poorly differentiated is presented in this report. The poorly differentiated area of the adenocarcinoma seemed to arise from the well-differentiated portion. The former was populated with Grimelius argyrophilic cells, and occasionally contained round, eosinophilic intracytoplasmic inclusions which appeared to be composed of a secretory substance. Immunocytochemistry revealed that endocrine cells were present in the normal portion of the gland as well as in the well-differentiated adenocarcinoma, but were more numerous in the poorly differentiated portion. The latter portion was positive for chromogranin a, endocrine granule constituents, neuroendocrine markers, serotonin, Leu 7, ACTH and neuron specific enolase. In addition, cells of poorly differentiated portion were reactive with CEA, prostatic acid phosphatase and human prostate specific antigen. Ultrastructural features observed in the poorly differentiated adenocarcinoma included five kinds of round neurosecretory granules. This was a rare case of prostatic adenocarcinoma in which the evolution from well differentiated to poorly differentiated tissue containing neuroendocrine constituents could be clearly observed.
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9/11. Spindle cell endocrine carcinoma of the mammary gland.

    We report the pathological characteristics of a variant of mammary endocrine tumour, predominantly formed from cytologically bland spindle cells. This neoplasm grows as a red, well defined mass lacking the usual macroscopical characteristics of breast cancer. Within smoothly contoured aggregates arranged in an insular pattern, delicate capillaries and collagen bundles support the neoplastic epithelial cells. Most of the tumour cells possess a slender spindle shape and form a solid or fenestrated sheet, but a few appear cuboidal and create glands. Immunohistochemical studies demonstrate that the spindle cells and the glandular cells constitute a single population. Both types of cells stain for neuroendocrine markers (chromogranin, synaptophysin, and CD 57), carcinoembryonic antigen, keratin 8/18, S-100 protein, and receptors for oestrogen and progesterone. Many of the tumour cells possess argyrophilic granules, and electron microscopy may reveal dense core granules.
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10/11. Thyroid paraganglioma: a clinicopathologic and immunohistochemical study of three cases.

    We report three cases of intrathyroidal paraganglioma. The patients were adult women without significant personal or family histories that presented with an asymptomatic thyroid nodule. The tumors were single, well-circumscribed solid masses, 2 cm in greatest diameter, located within one thyroid lobe. Microscopically, they were encapsulated and showed the typical nesting (Zellballen) pattern of paraganglioma in other sites. Two of the tumors were composed of small- to medium-sized cells with granular amphophilic cytoplasm, and the third consisted of relatively large cells having a similar staining quality. Immunohistochemically, all tumors showed positivity for neuron-specific enolase, chromogranin a, and synaptophysin. S-100 protein-positive sustentacular cells were demonstrated in each case. negative staining for epithelial markers, thyroglobulin, carcinoembryonic antigen, calcitonin, calcitonin gene-related peptide, serotonin, vimentin, and congo red excluded other tumors that were considered in the differential diagnosis, such as medullary carcinoma, hyalinizing trabecular adenoma, atypical follicular adenoma, Hurthle-cell neoplasm, and metastatic carcinoid tumor. The patients were alive and well without evidence of recurrent disease at the time of the last follow-up. The previous literature on these tumors is discussed. We conclude that intrathyroidal paraganglioma exists and that this tumor can be distinguished from other similar-appearing neoplasms in this organ.
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