Paraganglioma
A neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion, such as the carotid body, or medulla of the adrenal gland (usually called a chromaffinoma or pheochromocytoma). It is more common in women than in men. (Stedman, 25th ed; from Segen, Dictionary of Modern Medicine, 1992)
Paraganglioma, Extra-Adrenal
A relatively rare, usually benign neoplasm originating in the chemoreceptor tissue of the CAROTID BODY; GLOMUS JUGULARE; GLOMUS TYMPANICUM; AORTIC BODIES; and the female genital tract. It consists histologically of rounded or ovoid hyperchromatic cells that tend to be grouped in an alveolus-like pattern within a scant to moderate amount of fibrous stroma and a few large thin-walled vascular channels. (From Stedman, 27th ed)
Succinate Dehydrogenase
A flavoprotein containing oxidoreductase that catalyzes the dehydrogenation of SUCCINATE to fumarate. In most eukaryotic organisms this enzyme is a component of mitochondrial electron transport complex II.
Pheochromocytoma
A usually benign, well-encapsulated, lobular, vascular tumor of chromaffin tissue of the ADRENAL MEDULLA or sympathetic paraganglia. The cardinal symptom, reflecting the increased secretion of EPINEPHRINE and NOREPINEPHRINE, is HYPERTENSION, which may be persistent or intermittent. During severe attacks, there may be HEADACHE; SWEATING, palpitation, apprehension, TREMOR; PALLOR or FLUSHING of the face, NAUSEA and VOMITING, pain in the CHEST and ABDOMEN, and paresthesias of the extremities. The incidence of malignancy is as low as 5% but the pathologic distinction between benign and malignant pheochromocytomas is not clear. (Dorland, 27th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1298)
Carotid Body Tumor
Glomus Jugulare Tumor
A paraganglioma involving the glomus jugulare, a microscopic collection of chemoreceptor tissue in the adventitia of the bulb of the jugular vein. It may cause paralysis of the vocal cords, attacks of dizziness, blackouts, and nystagmus. It is not resectable but radiation therapy is effective. It regresses slowly, but permanent control is regularly achieved. (From Dorland, 27th ed; Stedman, 25th ed; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1603-4)
Abdominal Neoplasms
Cauda Equina
Retroperitoneal Neoplasms
3-Iodobenzylguanidine
A guanidine analog with specific affinity for tissues of the sympathetic nervous system and related tumors. The radiolabeled forms are used as antineoplastic agents and radioactive imaging agents. (Merck Index, 12th ed) MIBG serves as a neuron-blocking agent which has a strong affinity for, and retention in, the adrenal medulla and also inhibits ADP-ribosyltransferase.
Electron Transport Complex II
Germ-Line Mutation
Peripheral Nervous System Neoplasms
Normetanephrine
Metanephrine
Para-Aortic Bodies
Small masses of chromaffin cells found near the SYMPATHETIC GANGLIA along the ABDOMINAL AORTA, beginning cranial to the superior mesenteric artery (MESENTERIC ARTERY, SUPERIOR) or renal arteries and extending to the level of the aortic bifurcation or just beyond. They are also called the organs of Zuckerkandl and sometimes called aortic bodies (not to be confused with AORTIC BODIES in the THORAX). The para-aortic bodies are the dominant source of CATECHOLAMINES in the FETUS and normally regress after BIRTH.
Cervicoplasty
Aortic Bodies
Small clusters of chemoreceptive and supporting cells located near the ARCH OF THE AORTA; the PULMONARY ARTERIES; and the CORONARY ARTERIES. The aortic bodies sense PH; CARBON DIOXIDE; and OXYGEN concentrations in the BLOOD and participate in the control of RESPIRATION. The aortic bodies should not be confused with the PARA-AORTIC BODIES in the abdomen (which are sometimes also called aortic bodies).
Somatostatinoma
A SOMATOSTATIN-secreting tumor derived from the pancreatic delta cells (SOMATOSTATIN-SECRETING CELLS). It is also found in the INTESTINE. Somatostatinomas are associated with DIABETES MELLITUS; CHOLELITHIASIS; STEATORRHEA; and HYPOCHLORHYDRIA. The majority of somatostatinomas have the potential for METASTASIS.
Hypoglossal Nerve Diseases
Diseases of the twelfth cranial (hypoglossal) nerve or nuclei. The nuclei and fascicles of the nerve are located in the medulla, and the nerve exits the skull via the hypoglossal foramen and innervates the muscles of the tongue. Lower brain stem diseases, including ischemia and MOTOR NEURON DISEASES may affect the nuclei or nerve fascicles. The nerve may also be injured by diseases of the posterior fossa or skull base. Clinical manifestations include unilateral weakness of tongue musculature and lingual dysarthria, with deviation of the tongue towards the side of weakness upon attempted protrusion.
Tomography, X-Ray Computed
Spermatic Cord
Either of a pair of tubular structures formed by DUCTUS DEFERENS; ARTERIES; VEINS; LYMPHATIC VESSELS; and nerves. The spermatic cord extends from the deep inguinal ring through the INGUINAL CANAL to the TESTIS in the SCROTUM.
Thoracic Neoplasms
Iodobenzenes
Head and Neck Neoplasms
Soft tissue tumors or cancer arising from the mucosal surfaces of the LIP; oral cavity; PHARYNX; LARYNX; and cervical esophagus. Other sites included are the NOSE and PARANASAL SINUSES; SALIVARY GLANDS; THYROID GLAND and PARATHYROID GLANDS; and MELANOMA and non-melanoma skin cancers of the head and neck. (from Holland et al., Cancer Medicine, 4th ed, p1651)
Carney Complex
Autosomal dominant syndrome characterized by cardiac and cutaneous MYXOMAS; LENTIGINOSIS (spotty pigmentation of the skin), and endocrinopathy and its associated endocrine tumors. The cardiac myxomas may lead to SUDDEN CARDIAC DEATH and other complications in Carney complex patients. The gene coding for the PRKAR1A protein is one of the causative genetic loci (type 1). A second locus is at chromosome 2p16 (type 2).
Cranial Nerve Neoplasms
Vanilmandelic Acid
Vanilmandelic acid (VMA) is a major metabolite of catecholamines, specifically normetanephrine and metanephrine, which are produced by the adrenal gland and can be measured in urine to help diagnose pheochromocytoma or neuroblastoma.
Synaptophysin
Chromogranin A
A type of chromogranin which was first isolated from CHROMAFFIN CELLS of the ADRENAL MEDULLA but is also found in other tissues and in many species including human, bovine, rat, mouse, and others. It is an acidic protein with 431 to 445 amino acid residues. It contains fragments that inhibit vasoconstriction or release of hormones and neurotransmitter, while other fragments exert antimicrobial actions.
Paraganglia, Nonchromaffin
Several clusters of chemoreceptive and supporting cells associated with blood vessels and nerves (especially the glossopharyngeal and vagus). The nonchromaffin paraganglia sense pH, carbon dioxide, and oxygen concentrations in the blood and participate in respiratory, and perhaps circulatory, control. They include the CAROTID BODY; AORTIC BODIES; the GLOMUS JUGULARE; and the GLOMUS TYMPANICUM.