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The patient in the above case(paraganglioma) was found to have a gastric stromal sarcoma. In light of the
new findings, what is the best clinical diagnosis for this patient? A. Von Hippel-Lindau disease B. Multiple endocrine neoplasia C. Carneyʼs triad D. Carneyʼs syndrome E. None of the above |
D. The combination of paraganglioma and gastric stromal sarcoma inherited in an
autosomal dominant pattern is characteristic of Carneyʼs syndrome. The association of extraadrenal paraganglioma, gastric malignant stromal tumors, and pulmonary chondroma characterizes carneyʼs triad. It is only rarely seen in a familial setting. Paragangliomas can be associated with von Hippel-Lindau disease and multiple endocrine neoplasia, but are not associated with gastric stromal sarcomas. ( |
