ACCESSION NO:  89-90-1755
       TITLE:  Genomic Imprinting in Hereditary Glomus Tumors: Evidence of 
               New Genetic Theory
      AUTHOR:  VAN DER MEY, A.G.L.; MAASWINKEL-MOOY, P.D.; CORNELISSE, C.J.; 
               SCHMIDT, P.H.; VAN DE KAMP, J.J.P.
     JOURNAL:  Lancet
    CITATION:  December 2, 1989(8675): 1291-1294.
        YEAR:  1989
    PUB TYPE:  Article
 IDENTIFIERS:  GENOMIC IMPRINTING; HEREDITARY DISEASES; GLOMUS TUMORS; HEAD 
               AND NECK; TUMORS; BENIGN TUMORS; TUMORS/HEREDITARY
ABSTRACT: Glomus tumors derive from glomus body tissue and are usually located in the head and neck. The most common types, are the carotid body tumor, the glomus jugulare tumor, and the vagal body tumor. In general, these slow-growing tumors are benign and usually present as an enlarging cervical mass or cranial nerve palsy.

The familial occurrence of glomus tumors was first noted in 1933. The current study, based on fifteen pedigrees, showed that familial glomus tumors are inherited almost exclusively via the paternal line, a finding inconsistent with autosomal dominant transmission which was demonstrated by earlier investigators. These results can be explained in terms of the genomic imprinting hypothesis--the maternally derived gene is inactivated during female oogenesis and can be reactivated only during spermatogenesis.

Genetic imprinting may have considerable implications for genetic counselling with respect to glomus tumors and also for the understanding of other hereditary diseases such as retinoblastoma and Wilms tumor.