Malignant pheochromocytoma (paraganglioma) of the organ of Zuckerkandl: a study of two cases. 1980

L O Herrera, and Z M Hossain, and H S Rafal, and R W Frelick, and P F Ashley, and G E Lopez

Ten percent of pheochromocytomas are found in extra-adrenal locations and are called paragangliomas. In recent years we have had the opportunity to study two patients with malignant paragangliomas of the organ of Zuckerlandl. Only 13 cases of such neoplasms have been reported previously. Our cases are of interest because of their features of recurrence, distant metastases, response to adjuvant therapy, and long survival.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009423 Nervous System Neoplasms Benign and malignant neoplastic processes arising from or involving components of the central, peripheral, and autonomic nervous systems, cranial nerves, and meninges. Included in this category are primary and metastatic nervous system neoplasms. Neoplasms, Nervous System,Nervous System Tumors,Tumors of the Nervous System,Neoplasm, Nervous System,Nervous System Neoplasm,Nervous System Tumor,Tumor, Nervous System,Tumors, Nervous System
D010235 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, Gangliocytic,Paragangliomata,Gangliocytic Paraganglioma,Gangliocytic Paragangliomas,Paragangliomas,Paragangliomatas
D010673 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) Pheochromocytoma, Extra-Adrenal,Extra-Adrenal Pheochromocytoma,Extra-Adrenal Pheochromocytomas,Pheochromocytoma, Extra Adrenal,Pheochromocytomas,Pheochromocytomas, Extra-Adrenal
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001341 Autonomic Nervous System The ENTERIC NERVOUS SYSTEM; PARASYMPATHETIC NERVOUS SYSTEM; and SYMPATHETIC NERVOUS SYSTEM taken together. Generally speaking, the autonomic nervous system regulates the internal environment during both peaceful activity and physical or emotional stress. Autonomic activity is controlled and integrated by the CENTRAL NERVOUS SYSTEM, especially the HYPOTHALAMUS and the SOLITARY NUCLEUS, which receive information relayed from VISCERAL AFFERENTS. Vegetative Nervous System,Visceral Nervous System,Autonomic Nervous Systems,Nervous System, Autonomic,Nervous System, Vegetative,Nervous System, Visceral,Nervous Systems, Autonomic,Nervous Systems, Vegetative,Nervous Systems, Visceral,System, Autonomic Nervous,System, Vegetative Nervous,System, Visceral Nervous,Systems, Autonomic Nervous,Systems, Vegetative Nervous,Systems, Visceral Nervous,Vegetative Nervous Systems,Visceral Nervous Systems
D013311 Streptozocin An antibiotic that is produced by Stretomyces achromogenes. It is used as an antineoplastic agent and to induce diabetes in experimental animals. Streptozotocin,2-Deoxy-2-((methylnitrosoamino)carbonyl)amino-D-glucose,Streptozotocine,Zanosar
D014642 Vanilmandelic Acid A 3-O-methyl ether of 3,4-dihydroxymandelic acid. It is an end-stage metabolite of CATECHOLAMINES; EPINEPHRINE; and NOREPINEPHRINE. Methoxyhydroxymandelic Acid,4-Hydroxy-3-Methoxymandelic Acid,Vanillylmandelic Acid,4 Hydroxy 3 Methoxymandelic Acid,Acid, 4-Hydroxy-3-Methoxymandelic,Acid, Methoxyhydroxymandelic,Acid, Vanillylmandelic,Acid, Vanilmandelic

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