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1/242. Esophageal small cell carcinoma with ectopic production of parathyroid hormone-related protein (PTHrp), secretin, and granulocyte colony-stimulating factor (G-CSF).

    A patient with primary small cell carcinoma of the esophagus is reported, in whom we have studied the secretion of a variety of hormones and cytokines. The tumor was an intermediate cell type of small cell carcinoma and had either epithelial and neuroendocrinological characteristics. Furthermore, hypercalcemia and neutrophilia were present, and the tumor was shown to produce PTHrp, secretin, and G-CSF. The present case is the first report of primary small cell carcinoma of the esophagus with ectopic production of PTHrp, secretin, and G-CSF. ( info)

2/242. recurrence of adrenal aldosterone-producing adenoma.

    Conn's syndrome (adrenal aldosterone-producing adenoma) and bilateral adrenal hyperplasia are the most common causes of primary aldosteronism. The treatment of choice for patients with aldosterone-producing adenoma is unilateral total adrenalectomy. recurrence after adequate surgery is exceptional. We present a patient with recurrence of an aldosterone-producing adenoma in the right adrenal gland 9 years after adenomectomy of a aldosterone-producing adenoma in the same adrenal gland. We conclude that adenomectomy is not an adequate therapy for patients with adrenal aldosterone-producing adenoma. ( info)

3/242. 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. ( info)

4/242. 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. ( info)

5/242. hypokalemia, metabolic alkalosis, and hypertension: Cushing's syndrome in a patient with metastatic prostate adenocarcinoma.

    Ectopic adrenocorticotropic hormone (ACTH) and/or corticotropin-releasing hormone (CRH) are associated with a growing list of tumors. We report a 69-year-old white man with a history of high-grade prostate carcinoma and widely metastatic adenocarcinoma who presented with metabolic alkalosis, hypokalemia, and hypertension secondary to ectopic ACTH and CRH secretion. Laboratory values were consistent with hypokalemia and metabolic alkalosis. Markedly elevated serum cortisol (135 microg/dL), ACTH (1,387 pg/dL), CRH (69 pg/dL), and urine free cortisol (16,276 microg/24 h) levels were found. Chest computed tomographic (CT) scan showed small noncalcified parenchymal densities; however, bronchoscopy and bronchoalveolar lavage washings were unremarkable for a neoplastic process. Abdominal CT scan and magnetic resonance imaging showed multiple small liver lesions and multiple thoracic and lumbar intensities consistent with diffuse metastatic disease. Histological analysis of a biopsy specimen from the thoracic spine showed an undifferentiated adenocarcinoma consistent with a prostate primary tumor. The severe metabolic alkalosis secondary to glucocorticoid-induced excessive mineralocorticoid activity was treated with potassium supplements, spironolactone, and ketoconazole. In this case report, we describe an unusual tumor associated with ectopic ACTH and CRH production and the pharmacodynamic relationship of plasma cortisol levels and urinary cortisol excretion with ketoconazole treatment. ( info)

6/242. 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. ( info)

7/242. Prostatic carcinoma producing syndrome of inappropriate secretion of antidiuretic hormone.

    The syndrome of inappropriate secretion of antidiuretic hormone was recognized in a sixty-year-old man with a poorly differentiated metastatic adenocarcinoma of the prostate gland. Elevated levels of antidiuretic hormone were found in the patient's serum and in the prostatic tumor but not in the cerebrospinal fluid. The patient's clinical course is detailed, and the pathophysiology of this syndrome is discussed. ( info)

8/242. hypercalcemia induced by parathyroid hormone-related peptide after treatment of carcinoma.

    Squamous cell carcinoma of the head and neck is a rare cause of humoral hypercalcemia of malignancy. This paraneoplastic syndrome is usually one of the presenting symptoms of the disease. We report a case of squamous cell carcinoma of the oral cavity that presumably elaborated parathyroid hormone-related peptide (PTH-rP) and caused hypercalcemia only after radiotherapy and chemotherapy. ( info)

9/242. acromegaly caused by a growth hormone-releasing hormone-secreting carcinoid tumor: case report.

    OBJECTIVE AND IMPORTANCE: We describe a patient with acromegaly and pituitary hyperplasia secondary to a growth hormone-releasing hormone-secreting gastrointestinal carcinoid tumor. This case report illustrates the importance of including this rare clinical syndrome in the differential diagnosis of acromegaly for patients with suspected or known neuroendocrine tumors. CLINICAL PRESENTATION: A 19-year-old, Asian-American, male patient with a 2-year history of a nonresectable, metastatic, intestinal carcinoid tumor presented with complaints of headaches, arthralgias, sweats, and changing features. The examination revealed a young subject with acromegalic features, without visual field deficits. magnetic resonance imaging revealed a diffuse sellar mass that extended suprasellarly to compress the optic chiasm. Endocrinological studies demonstrated a growth hormone level of more than 100 ng/ml and an inappropriately elevated growth hormone-releasing hormone level. INTERVENTION: The patient underwent transsphenoidal resection of the pituitary mass for diagnostic and decompressive purposes. The pathological examination revealed pituitary hyperplasia, without evidence of an adenoma. Therapy with long-acting repeatable octreotide (Sandostatin LAR; Novartis AG, Basel, switzerland) was initiated postoperatively, to further control the acromegaly and carcinoid tumor. The soft-tissue swelling resolved, and the patient remained free of headaches, arthralgias, and sweats at the 6-month follow-up examination. CONCLUSION: Ectopic acromegaly is a rare syndrome that must be recognized by neurosurgeons because its treatment differs from that of classic pituitary acromegaly. We describe a patient for whom this syndrome was documented with magnetic resonance imaging, endocrinological testing, and pathological examinations. ( info)

10/242. January 2002: 59-year-old woman with an intrasellar lesion.

    An intrasellar lesion resembling a hormone-inactive pituitary adenoma was resected in a 59-year-old woman. The well-differentiated benign tumor was composed of ganglion-like cells, clusters of adenohypophyseal tissue as well as a GH-expressing adenoma. In addition, small cells exhibited an intermediate neuronal and epithelial immunoreactivity. Surrounding the tumor was a spindle cell component that histologically resembled Antoni A areas of a schwannoma, but showed a mixed immunohistochemical profile. Final diagnosis was intrasellar glioneuronal hamartoma with GH-cell pituitary adenoma. ( info)
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