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Malignant pheochromocytoma with ganglioneuroblastomatous elements in a patient with von recklinghausen's disease
Author(s) -
Nakagawara Akira,
Ikeda Keiichi,
Tsuneyoshi Masazumi,
Daimaru Yutaka,
Enjoji Munetomo
Publication year - 1985
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19850615)55:12<2794::aid-cncr2820551213>3.0.co;2-l
Subject(s) - medicine , pheochromocytoma , disease , pathology
A 14‐year‐old girl with numerous café‐au‐lait spots in her skin was hospitalized because of fever, weight loss, and a mass of the right upper quadrant of the abdomen. Despite intensive chemotherapy, she died 6 months after admission. The autopsy revealed a right adrenal tumor with metastases to liver, lungs, vertebrae, and lymph nodes. Histologically the tumor was a pheochromocytoma with small foci of ganglioneuroblastoma. The catecholamine contents of the tumor were markedly elevated, as confirmed by the catecholamine fluorescence technique. Electron microscopically, the tumor cells contained intracytoplasmic membrane‐bound chromaffin granules of varying sizes and shapes. This may be the first report of the concomitant occurrence of malignant catecholamine‐secreting pheochromocytoma with ganglioneuroblastomatous elements in a patient with von Recklinghausen's disease.