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Hartmut P.H. Neumann, MD
Albert-Ludwigs University Freiburg, Germany

Charis Eng, MD, PhD
Dana-Farber Cancer Institute Harvard Medical School Boston, Mass CRC Human Cancer Genetics Research Group University of Cambridge Cambridge, England

Lois M. Mulligan, PhD
Queens University Kingston, Ontario

JAMA. 1996;275(11):840.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

In Reply.

—The findings of Drs Walther and Linehan are consistent with the findings in our article. These authors also call attention to another entity, namely, pheochromocytoma-only families. These families are different from the two reported in our article since both of our families have well-documented C-cell tumors, the hallmark of MEN-II. We suspect that the majority of pheochromocytoma-only families will turn out to have VHL mutations¹ and those without might be due to the as yet unidentified PGL locus on the long arm of chromosome 11.²

Prior to the observations made by Walther and Linehan and those in the cited French study,³ we reported a high incidence of VHL in patients with symptomatic pheochromocytoma and documented that 38% of VHL patients identified in our series had only pheochromocytoma even after intensive investigation.⁴ . . . [Full Text PDF of this Article]

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