Correspondence

Germ-Line Mutations in Nonsyndromic Pheochromocytoma

N Engl J Med 2002; 347:854-855September 12, 2002

Article

To the Editor:

The article on germ-line mutations in nonsyndromic pheochromocytoma, by Neumann et al. (May 9 issue),1 contains a common point of confusion: the terms “paraganglioma” and “glomus tumor” are used interchangeably. They are not interchangeable. The glomus tumor is a tumor of modified perivascular smooth muscle, which frequently presents as a painful subungual mass, and is unrelated to tumors of the adrenal and extraadrenal paraganglia. The tumor to which the authors are actually referring is the jugulotympanic paraganglioma, which has been mistakenly referred to as a “glomus jugulare tumor” in the past. This neoplasm, which was recognized as a distinct pathologic entity by Sadao Otani at Mount Sinai Hospital in the 1940s and is still sometimes referred to as Otani's tumor, arises from minute, anatomically dispersed paraganglia located at the base of the skull and temporal bone and is closely related to similar tumors of the carotid body and other extraadrenal paraganglia.2 Though still sometimes referred to as glomus jugulare tumor, it is unrelated to the much more common glomus tumor of skin and soft tissue.

James A. Strauchen, M.D.
Mount Sinai School of Medicine, New York, NY 10029
james.strauchen@msnyuhealth.org

2 References

1. 1

   Neumann HPH, Bausch B, McWhinney SR, et al. Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 2002;346:1459-1466
   Full Text | Web of Science | Medline

2. 2

   Lack EE. Tumors of the adrenal gland and extra-adrenal paraganglia. Atlas of tumor pathology. 3rd series. Fascicle 19. Washington, D.C.: Armed Forces Institute of Pathology, 1997.
   

Author/Editor Response

The authors reply:

To the Editor: As Dr. Strauchen points out, the term “glomus tumor” was originally used to refer only to glomus tumors of the skin. Nevertheless, there is also widespread use of this term to refer to paragangliomas of the neck and skull base, both in clinical practice and in standard textbooks of otolaryngology.1,2 Furthermore, at the time of this writing, a Medline search for the term “glomus” yielded 53 citations for the years 2000, 2001, and 2002, and about 60 percent of the cited articles pertain to humans. Of the latter citations, the majority refer to glomus tumors and paragangliomas interchangeably. Because the term “glomus” is the Latin word for ball, it naturally enough has been used to refer to several entities. We believe the term “glomus jugulare” evolved because some have referred to the neuroendocrine cells of carotid-body paragangliomas as glomus cells.3

Hartmut P.H. Neumann, M.D.
Jörg Schipper, M.D.
Albert-Ludwigs University, D-79106 Freiburg, Germany
neumann@med1.ukl.uni-freiburg.de

Charis Eng, M.D., Ph.D.
Ohio State University, Columbus, OH 43210

3 References

1. 1

   Cummings CW, Fredrickson JM, Harker LA, Krause CJ, Schuller DE, Richardson MA, eds. Otolaryngology — head & neck surgery. 3rd ed. St. Louis: Mosby, 1998:3305.
   

2. 2

   Zenner HP, ed. Hals-Nasen-Ohren-Heilkunde. Weinheim, Germany: Chapman & Hall, 1997:62.
   

3. 3

   Pardal R, Lopez-Barneo J. Carotid body thin slices: responses of glomus cells to hypoxia and K(+)-channel blockers. Respir Physiolo Neurobiol 2002;132:69-79
   CrossRef | Web of Science | Medline

Citing Articles (1)

Citing Articles

1. 1

   F.S. Lozano Sánchez, M. Núñez Lozano, P. Santos Gorjón, A. Masegosa Medina, Á. Muñoz Herrera, R. González Sarmiento. (2010) Estudio genético del complejo enzimático succinato deshidrogenasa en los paragangliomas carotídeos. Implicaciones diagnósticas. Angiología 62:6, 214-218
   CrossRef