0
ARTICLE |

Von Hippel-Lindau Disease and Pheochromocytoma-Reply FREE

Hartmut P.H. Neumann, MD; Charis Eng, MD, PhD; Lois M. Mulligan, PhD
JAMA. 1996;275(11):840-840. doi:10.1001/jama.1996.03530350021026
Text Size: A A A
Published online

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 11.2Prior 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

REFERENCES

Crossey PA, EngC, Ginalska-Malinowska M, et al.  Molecular genetic diagnosis of von Hippel-Lindau disease in familial pheochromocytoma. J Med Genet . 1995;;32:885-886.
Heutink P, van der Mey AG, Sandkuijl LA, et el.  A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 1 1q23-qter. Hum Mol Genet . 1992;;1:7-10.
Richard S, Beigelman C, Duclos JM, et al.  Pheochromocytoma as the first manifestation of von Hippel-Lindau disease. Surgery . 1994;;116:1076-1081.
Neumann HPH, Berger DP, Sigmund G, et al.  Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med . 1993;;329: 1531-1538.

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

Crossey PA, EngC, Ginalska-Malinowska M, et al.  Molecular genetic diagnosis of von Hippel-Lindau disease in familial pheochromocytoma. J Med Genet . 1995;;32:885-886.
Heutink P, van der Mey AG, Sandkuijl LA, et el.  A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 1 1q23-qter. Hum Mol Genet . 1992;;1:7-10.
Richard S, Beigelman C, Duclos JM, et al.  Pheochromocytoma as the first manifestation of von Hippel-Lindau disease. Surgery . 1994;;116:1076-1081.
Neumann HPH, Berger DP, Sigmund G, et al.  Pheochromocytomas, multiple endocrine neoplasia type 2, and von Hippel-Lindau disease. N Engl J Med . 1993;;329: 1531-1538.
CME Course for:


You need to register in order to view this quiz.


To understand the clinical management of acute heart failure syndromes.
Accreditation Information The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
To view and print your certificate and access a summary of your CME courses go to My CME.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Submit a Response

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.