The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma was found in two young female Mayo Clinic patients, and two of these tumors occurred in another two. Review of the world literature disclosed nine patients who probably had two or more of the three neoplasms. We are also aware of two other patients with two of the three tumors. The association of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma may well constitute a syndrome because of 1) the improbability of the coincidental occurrence of this triad of tumors, 2) the multicentricity of the tumors in the organs or system affected, and 3) the development of the tumors at a young age. Because two of the components of the triad are potentially lethal, it is important that patients less than 35 years of age who have any one of the three tumors be examined periodically to search for the others.
Cancer 43~374-382, 1979.
HE OCCURRENCE OF multiple primary T dissimilar tumors in a member of the general population is an unusual phenomenon. However, among certain heredofamilial disorders, multiple specific neoplasms develop in different organs and tissues. In fact, the combination of such tumors may be distinctive and constitute a syndrome. In a recent preliminary report we described the association of three uncommon neoplasms-gastric leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma-in two young female patients, and we adduced supportive evidence from five other cases (two Mayo Clinic and three non-Mayo Clinic) that this unusual triad of tumors might constitute a syndrome.