bromochloroacetic-acid and Cushing-Syndrome

Pituitary carcinomas, currently defined as primary adenohypophyseal neoplasms with evidence of either brain invasion or metastatic spread, are exceptionally rare. A case of corticotroph pituitary carcinoma is reported. A 17-year-old female first presented with an invasive and clinically non-functioning pituitary macroadenoma. The primary pituitary lesion lacked atypical histological features and retrospective immunohistochemical studies confirmed its corticotrophic nature. Repeated episodes of local recurrence followed together with the acquisition of severe Cushing's disease. Local disease control was not obtained despite repeated surgical decompression and courses of radiation therapy. Systemic dissemination with multiple bone metastases became manifest eleven years after the first presentation. Atypical histological features and Crooke's hyaline change were evident in both the recurrent and metastatic disease. She remains alive at last follow-up with severe complications relating to persisting sellar and metastatic disease and ongoing endocrine dysfunction. A further 32 cases of pituitary corticotroph carcinoma reported in English are reviewed to highlight the clinicopathological features of this rare form of pituitary neoplasm. Difficulties associated with the diagnosis and management of pituitary carcinoma are also discussed.

Topics: Adenoma; Adolescent; Adrenocorticotropic Hormone; Cushing Syndrome; Female; Humans; Immunohistochemistry; Keratins; Magnetic Resonance Imaging; Neoplasm Invasiveness; Neoplasm Recurrence, Local; Pituitary Gland, Anterior; Pituitary Neoplasms

In a 52-year-old Caucasian man osteopoikilosis had been misdiagnosed roentgenologically 2 years before his death. Gradually he developed Cushing's syndrome and ultimately superior vena caval obstruction. At autopsy a primary thymic carcinoid with extensive osteoblastic bone metastasis was found. Immunohistochemically the tumor was shown to be positive for adrenocorticotropic hormone (ACTH), cytokeratin (KL1), neuron-specific enolase, synaptophysin, chromogranin and glucagon. Remarkably the tumour was negative for serotonin despite high urinary hydroxyindolacetic acid levels. Bilateral hyperplasia of the adrenal cortex was found. The adenohypophysis showed a considerable reduction of ACTH-producing cells and numerous Crooke's cells with a characteristic immunohistochemical pattern.

Topics: Adrenal Cortex; Adrenocorticotropic Hormone; Bone Neoplasms; Carcinoid Tumor; Cushing Syndrome; Diagnostic Errors; Humans; Keratins; Male; Middle Aged; Osteopoikilosis; Phosphopyruvate Hydratase; Radiography; Thymus Neoplasms; Vena Cava, Superior

Oncocytoma is a neoplasm that can arise in several organs, and it has been more commonly described in the kidney, salivary gland and thyroid. Oncocytoma arising in the adrenal gland is a rare finding. Moreover, functioning adrenocortical oncocytoma is exceptionally rare. A 47-yr-old man was incidentally discovered to have a right adrenal mass. The patient had no clinical features suggestive of increased adrenal function. However, hormonal evaluation showed a disturbed cortisol circadian rhythm, supranormal urinary cortisol excretion, a low level of ACTH, and a lack of suppressibility of cortisol secretion after dexamethasone. Right adrenalectomy was performed, and this revealed a well-circumscribed dark-brown tumor that measured 2.4x2.2 cm. The tumor consisted almost exclusively of large eosinophilic and epitheloid cells whose cytoplasm was packed with eosinophilic granulations, which corresponded to the numerous mitochondria confirmed on electron microscopy. This is a rare case of subclinical Cushing's syndrome that was caused by adrenocortical oncocytoma.

Topics: Adenoma, Oxyphilic; Adrenal Cortex Neoplasms; Cushing Syndrome; Dexamethasone; Glucocorticoids; Humans; Inhibins; Keratins; Male; Middle Aged; Synaptophysin

A 58-year-old woman with a history of Cushing's syndrome for three years presented with a mediastinal mass and received the diagnosis of small cell neuroendocrine carcinoma of the thymus invading the pericardium. On immunohistochemical study, the neoplastic cells reacted with antibodies against cytokeratin, epithelial membrane antigen, neuron-specific enolase, chromogranin, synaptophysin, and ACTH. Clinicopathologic findings of this rare case of ectopic adrenocorticotropic hormone (ACTH) syndrome are discussed with a literature review.

Topics: Adrenocorticotropic Hormone; Carcinoma, Neuroendocrine; Carcinoma, Small Cell; Chromogranins; Cushing Syndrome; Dexamethasone; Female; Humans; Immunoenzyme Techniques; Keratins; Middle Aged; Mucin-1; Phosphopyruvate Hydratase; Radiography, Thoracic; Synaptophysin; Thymus Neoplasms

A 36-year-old Russian patient had been exposed to severe nuclear radiation during the Tschernobyl accident and developed Cushing's syndrome in September 1991. After bilateral adrenalectomy a small centrally localized lung tumor in the left segment S6 was diagnosed, and the syndrome was correlated to primary lung cancer. Thoracotomy and resection of the segments S6 revealed a primary ACTH-producing lung tumor of the cell type of a paraganglioma. The patient lost his symptoms after curative tumor resection. The case is discussed under the aspects of tumor etiology, its clinical course and immunohistochemical findings.

Topics: Adrenocorticotropic Hormone; Adult; Cushing Syndrome; Humans; Keratins; Lung Neoplasms; Male; Neoplasms, Radiation-Induced; Paraganglioma; Phosphopyruvate Hydratase; Power Plants; Radioactive Hazard Release; Tumor Suppressor Protein p53; Ukraine; Vimentin