Psammomatous melanotic schwannoma: an additional component of Carney's complex. Report of a case. 1991

D G Martin-Reay, and M C Shattuck, and F W Guthrie
Department of Pathology, Evanston Hospital, Illinois 60201.

The authors report a case of Carney's complex (the complex of myxomas, spotty pigmentation, and endocrine overactivity) in a 32-year-old white man. Psammomatous melanotic schwannoma has recently been included as a component of this disorder. The complex was recognized after a psammomatous melanotic schwannoma was resected from the sacral canal. Subsequent echocardiography revealed ventricular cardiac myxomata. Resection of these lesions was successfully performed. Review of previous history and pathologic material further supported the diagnosis. The authors conclude that recognition of each component as part of the syndrome is of crucial importance, because it may prevent a fatal outcome. A family screening program should be performed in each case.

UI MeSH Term Description Entries
D008297 Male Males
D009232 Myxoma A benign neoplasm derived from connective tissue, consisting chiefly of polyhedral and stellate cells that are loosely embedded in a soft mucoid matrix, thereby resembling primitive mesenchymal tissue. It occurs frequently intramuscularly where it may be mistaken for a sarcoma. It appears also in the jaws and the skin. (From Stedman, 25th ed) Angiomyxoma,Angiomyxomas,Myxomas
D009442 Neurilemmoma A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5) Neurinoma,Schwannoma,Schwannomatosis, Plexiform,Neurilemoma,Neurilemmomas,Neurilemomas,Neurinomas,Plexiform Schwannomatoses,Plexiform Schwannomatosis,Schwannomas
D010859 Pigmentation Disorders Diseases affecting PIGMENTATION, including SKIN PIGMENTATION. Incontinentia Pigmenti Achromians,Ito Syndrome,Schamberg's Disease,Schamberg Disease,Disease, Schamberg,Disease, Schamberg's,Disorder, Pigmentation,Disorders, Pigmentation,Pigmentation Disorder,Schambergs Disease,Syndrome, Ito
D004700 Endocrine System Diseases Pathological processes of the ENDOCRINE GLANDS, and diseases resulting from abnormal level of available HORMONES. Endocrine Diseases,Diseases of Endocrine System,Disease, Endocrine,Disease, Endocrine System,Diseases, Endocrine,Diseases, Endocrine System,Endocrine Disease,Endocrine System Disease,System Disease, Endocrine,System Diseases, Endocrine
D006338 Heart Neoplasms Tumors in any part of the heart. They include primary cardiac tumors and metastatic tumors to the heart. Their interference with normal cardiac functions can cause a wide variety of symptoms including HEART FAILURE; CARDIAC ARRHYTHMIAS; or EMBOLISM. Cardiac Cancer,Cardiac Carcinoma,Cardiac Neoplasms,Cardiac Tumor,Cardiac Tumors,Heart Cancer,Heart Tumor,Intracavitary Tumors of the Heart,Myocardial Tumors (Rhabdomyomas and Fibromas),Neoplasms, Cardiac,Neoplasms, Heart,Primary Cardiac Tumors, Childhood,Cancer, Cardiac,Cancer, Heart,Cancers, Cardiac,Cancers, Heart,Carcinoma, Cardiac,Carcinomas, Cardiac,Cardiac Cancers,Cardiac Carcinomas,Cardiac Neoplasm,Heart Cancers,Heart Neoplasm,Heart Tumors,Myocardial Tumor (Rhabdomyomas and Fibromas),Neoplasm, Cardiac,Neoplasm, Heart,Tumor, Cardiac,Tumor, Heart,Tumor, Myocardial (Rhabdomyomas and Fibromas),Tumors, Cardiac,Tumors, Heart,Tumors, Myocardial (Rhabdomyomas and Fibromas)
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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