Abstract

In Reply.— Greene et al presented a historic account of observations of hereditary cutaneous malignant melanoma (CMM) that has led to the simultaneous elucidation of a unique subset by Lynch and colleagues, 1,2 designated by them as the FAMMM syndrome , and by Greene and associates, who refer to it as DNS but have also called it the B-K mole syndrome and the large atypical nevus syndrome (LANS) . With respect to the contributions of other early investigators, our reports are replete with their efforts. Our concern with Rahbari and Mehregan's 3 study in our recent communication 4 is that they assigned sole credit for the delineation of the subset to only one group (Greene and associates), and thus did not mention the serious concern for the presence of other types of primary cancers in the FAMMM syndrome. More importantly, they used unsubstantiated historic genetic data to support the concept of their report of five cases of sporadic FAMMM syndrome. The statement of Frichot et al, 1 quoted by Greene et al, and interpreted as our admission of prior discovery by them, was taken out of context. In our yearly follow-up of patients with cancer at the Institute for Familial Cancer Management and Control at Creighton University, Omaha, we recognized, in the mid-70s, the existence of the FAMMM syndrome in the CMM pedigree reported by Lynch and Krush5 in 1968, before Dr Clark's oral presentation at the American Academy meeting in December 1976.

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