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Muscular dystrophy associated with extra-abdominal desmoid tumor showing aberrant chromosome 1 [46,XX,add(1)(p36)]

✍ Scribed by Katsuura, Michihiko; Kato, Mitsuhiro; Sendo, Dai; Akiba, Kaori; Honma, Akira; Takahashi, Yumiko; Numakura, Chikahiko; Yokoyama, Shinkichi; Nonaka, Ikuya; Shibata, Takanori; Hayasaka, Kiyoshi


Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
7 KB
Volume
76
Category
Article
ISSN
0148-7299

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✦ Synopsis


We report on a 2-year-old girl with probable limb-girdle muscular dystrophy associated with an extra-abdominal desmoid tumor of the right mandible. This association is previously undescribed. The tumor was totally removed. Cytogenetic analysis of the tumor showed a clonal karyotypic abnormality: 46,XX,add(1)(p36) in 3 of 20 cells analyzed. Since an association of a neoplasm with limb-girdle muscular dystrophy has previously been reported in 3 cases, the two abnormalities are likely related causally. The chromosome abnormality in our patient may play a role in the occurrence of her desmoid tumor. Am.