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1/51. Sinonasal teratocarcinosarcoma ("mixed olfactory neuroblastoma-craniopharyngioma") presenting with syndrome of inappropriate secretion of antidiuretic hormone.

    Sinonasal teratocarcinosarcoma (SNTC) is a rare, aggressive, histologically heterogeneous neoplasm of the paranasal sinuses and nasopharnyx of adults that is composed of variably benign or malignant neuroepithelial, epithelial, and mesenchymal elements. Occasional cases show intracranial extension and may be operated on by neurosurgeons and encountered by neuropathologists who may not be familiar with the entity. STNCs have not previously been associated with functional hypersecretory status. We report a 59-year-old male who presented with headache and syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and was subsequently found to have a bulky tumor of the frontal and ethmoid sinuses with focal dural invasion. The tumor was predominantly composed of olfactory neuroblastoma areas (90% of tumor) admixed with unusually well-developed craniopharyngioma-like mature squamous epithelium and ghost cells ( 10% of tumor). Scattered neuroblastoma tumor cells showed strong immunoreactivity with antibodies to arginine vasopressin, supporting ectopic hormone secretion by the tumor. While the coexistence of neuroectodermal and oral ectodermal-like differentiation in SNTCs is characteristic, in our case it was developed to an extreme functional and morphologic degree and was unassociated with other mesenchymal or epithelial elements often found in these complex tumors. SNTCs with limited differentiation have prompted controversy in classification.
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ranking = 1
keywords = neoplasm
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2/51. adenocarcinoma of the prostate with ectopic antidiuretic hormone production: a case report.

    An 88-year-old patient with a poorly differentiated adenocarcinoma of the prostate gland was found to have all cardinal findings of syndrome of inappropriate antidiuretic hormone secretion (SIADH). Elevated levels of antidiuretic hormone were found in the patient's serum and in the prostatic tumor and the cytoplasms of the tumor was positive for prostate specific antigen and was faintly positive for antidiuretic hormone (ADH). He responded well to combination therapy of androgen blockade with leuprorelin acetate and flutamide, and laboratory findings of SIADH and serum ADH level returned to normal. However, he died of sudden profuse bleeding caused by gastric ulcers 6 months after the therapy. Ten cases of SIADH caused by prostatic cancer have been reported including the present case.
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ranking = 0.19901395479798
keywords = cancer
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3/51. Metastatic carcinoma of penis complicated by hypercalcemia.

    A fifty-three-year-old man with epidermoid carcinoma of the penis metastatic to the right inguinal lymph nodes and adjacent areas was found to have persistent hypercalcemia. Associated with this biochemical abnormality was an elevated parathormone activity in the absence of any bony metastases. Other than a transient response to furosemide-inducded diuresis he was refractory to treatment with oral inorganic phosphates and mithramycin. Ablation of the primary tumor did not affect his hypercalcemia. However, when therapy using external irradiation and parenteral bleomycin was directed to the metastases, his serum calcium stabilized and became normal and remained so until further progression of his humor. We postulate that the penile cancer metastases were elaborating parathyroid hormone-like substances responsible for the hypercalcemia and suppression of normal parathyroid activity.
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ranking = 0.19901395479798
keywords = cancer
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4/51. Mediastinal polyembryoma associated with klinefelter syndrome.

    A 4.5-year-old boy presented with isosexual precocious puberty and an anterior mediastinal mass. Surgical resection demonstrated a teratoma with foci of malignant mixed germ cell tumor elements of polyembryoma. On further investigation he was found to have klinefelter syndrome. Most mediastinal germ cell tumors are treated with adjuvant therapy. He was managed with surgical excision alone and is well at 2 years follow-up. The rationale for this approach is discussed.
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ranking = 45.638517799455
keywords = germ cell, germ
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5/51. EPO-producing gastric carcinoma in a hemodialysis patient.

    A case of erythrocytosis caused by gastric cancer that produced erythropoietin is described. To the authors' knowledge, no case of erythropoietin-producing gastric cancer has been reported until now. A 73-year-old man with a 4-year history of maintenance hemodialysis for diabetic nephropathy required phlebotomy. serum erythropoietin level was 181 mU/mL (181 IU/L). gastroscopy results showed rough mucosa with hemorrhaging caused by gastric cancer. The patient underwent distal gastrectomy, and serum erythropoietin level decreased to 27.1 mU/mL (27.1 IU/L) by postoperative day 8. Existence of erythropoietin in the tumor tissue was confirmed immunohistochemically. The presence of severe acquired cystic disease of the kidney, renal cell carcinoma, and other malignant tumors should be investigated in hemodialysis patients displaying erythrocytosis.
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ranking = 0.59704186439395
keywords = cancer
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6/51. gynecomastia attributable to human chorionic gonadotropin-secreting giant cell carcinoma of lung.

    OBJECTIVE: To describe a patient with human chorionic gonadotropin (hCG)-secreting giant cell carcinoma of the lung manifesting as painful gynecomastia, which resolved after surgical treatment. methods: We present the clinical, radiologic, and clinical course of our patient, who presented with painful gynecomastia. RESULTS: A 51-year-old man presented with a lung mass and rapidly progressing bilateral painful gynecomastia. Hormonal evaluation revealed high levels of hCG beta subunit, estradiol, and total and free testosterone and suppressed levels of luteinizing hormone and follicle-stimulating hormone. The patient underwent resection of the right upper and middle lobes of the lung. After the surgical procedure, the levels of hCG and estradiol rapidly decreased, and the gynecomastia resolved completely within 3 months. Fluctuation in the levels of serum hCG paralleled the clinical course of the lung tumor. CONCLUSION: Although gynecomastia is a common disorder, hormonal work-up including hCG beta subunit should be done in an adult male patient presenting with rapidly progressing or recent onset of painful gynecomastia. lung cancer should be considered in the differential diagnosis of gynecomastia attributable to ectopic production of hCG beta subunit.
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ranking = 0.19901395479798
keywords = cancer
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7/51. Small cell carcinoma with two paraendocrine syndromes.

    Simultaneous elevated levels of ectopic arginine vasopressin (AVP) and ectopic adrenocorticotropin (ACTH) were found in a patient with small cell carcinoma (SCC). The finding of one of these paraendocrine syndromes at the time of diagnosis is common; however, the simultaneous presence of both syndromes has been reported in the literature only on four occasions in the past 25 years. This is the only report in which elevated plasma levels of both hormones are documented in a patient who simultaneously fulfills the criteria for the syndrome associated with each ectopically produced peptide. In the English-language literature, this is the first case that demonstrates by immunohistochemical staining the presence of both of these hormones in the patient's neoplasm. In addition to the use of radiographs, the presence of paraendocrine disorders can provide a method of monitoring the patient's response to therapy. The levels of ACTH and AVP were assayed during this patient's course and correlated with disease refractory to therapy, resulting in poor survival.
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ranking = 1
keywords = neoplasm
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8/51. hypercalcemia and breast cancer related to parathormone-like secretion by liver metastases.

    A premenopausal woman developed hypercalcemia 30 months after treatment for infiltrating breast cancer. After bone metastases had been excluded, primary hyperparathyroidism was suspected. A parathyroid adenoma was removed and histologically confirmed. hypercalcemia, associated with low plasma phosphate and severely depressed plasma parathormone (PTH) levels, persisted. Further investigations showed liver metastases from the primary breast cancer and also secretion of a PTH-like substance. Antitumoral treatment was effective on the liver metastases and also normalized calcemia and the PTH-like substance, demonstrating the existence of a paraneoplastic syndrome related to the secretion of a PTH-like substance by disseminated liver metastases of primary breast cancer.
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ranking = 1.3930976835859
keywords = cancer
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9/51. Ovarian non-small cell neuroendocrine carcinoma with paraneoplastic parathyroid hormone-related hypercalcemia.

    Ovarian tumors associated with hypercalcemia due to ectopic secretion of parathyroid hormone (PTH) are extremely rare. A 33-year-old woman presented with a pelvic mass and profound hypercalcemia accompanied by an elevated serum level of PTH. laparotomy demonstrated a left ovarian tumor that on histological examination was a neuroendocrine carcinoma of non-small cell type admixed with a component of endometrioid adenocarcinoma. After left salpingo-oophorectomy, the serum calcium and PTH levels normalized. The cells of the neuroendocrine carcinoma were positive for neuron-specific enolase, synaptophysin, chromogranin A, and PTH. hypercalcemia and elevated serum PTH levels recurred during tumor relapse, and the patient died of disease 6 months postoperatively. This is the eleventh case of neuroendocrine carcinoma of non-small cell type associated with surface epithelial neoplasm of the ovary, and the first such tumor to be associated with hypercalcemia.
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ranking = 1
keywords = neoplasm
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10/51. Large cell carcinoma with calcitonin and vasoactive intestinal polypeptide-associated Verner-Morrison syndrome.

    Verner-Morrison syndrome, characterized by diarrhea, hypokalemia, and hypochlorhydria, is caused most commonly by vasoactive intestinal polypeptide-secreting islet cell tumors of the pancreas. Verner-Morrison syndrome has not been described as a paraneoplastic syndrome in non-small cell lung cancer. We describe a 38-year-old man with metastatic non-small cell lung cancer of large cell carcinoma with neuroendocrine differentiation who presented with bone metastasis and intractable secretory diarrhea that was unresponsive to pharmacological treatment, including octreotide. Laboratory evaluation indicated elevated serum calcitonin and vasoactive intestinal polypeptide levels. Chemotherapy resulted in a transient response in the patient's diarrhea and neuroendocrine markers. The patient did not respond to further therapy and died 5 months after onset of back pain. To our knowledge, this is the first published case of large cell carcinoma with neuroendocrine differentiation associated with treatment-responsive paraneoplastic Verner-Morrison syndrome.
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ranking = 0.39802790959597
keywords = cancer
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