[Case of Carney complex complicated with pituitary adenoma and Rathke cleft cyst].
Carney complex is a rare autosomal-dominant, familial tumor syndrome first described in the mid 80's. This syndrome is multiple neoplasia syndrome featuring cardiac, endocrine, cutaneous, and neural tumor, in addition to a variety of pigmented lesions of the skin and mucosa. We report the case of a 12-year-old female patient with Carney complex who manifested a high value of serum growth hormone (s-GH), cutaneous angiomyxomas and labial pigmented lesions. Magnetic resonance imaging (MRI) revealed a cystic pituitary tumor. We carried out removal of the pituitary tumor via the transsphenoidal approach. In addition to the pituitary adenoma, pathological examination revealed the presence of a Rathke cleft cyst. So far, approximately 500 cases of this disorder have been described, but there have been no cases similar to our case described here. Therefore, the present case seems to be the first case of Carney Complex complicated with pituitary adenoma and Rathke cleft cyst.
Kamoshima
Yuuta
Y
Department of Neurosurgery, Hokkaido University School of Medicine, 15-7 Kita-ku, Sapporo-shi, Hokkaido 060-8638, Japan.
Sawamura
Yutaka
Y
Iwasaki
Yoshinobu K
YK
Fujieda
Kenji
K
Takahashi
Hiroyuki
H
D000236:Adenoma / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D018229:Angiomyoma / Q000150:complications* / Q000175:diagnosis; D014408:Biomarkers, Tumor / Q000032:analysis; D020863:Central Nervous System Cysts / Q000150:complications* / Q000175:diagnosis; D002648:Child; D005260:Female; D006801:Humans; D017495:Hyperpigmentation / Q000150:complications* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D009377:Multiple Endocrine Neoplasia / Q000150:complications* / Q000175:diagnosis; D009378:Neoplasms, Multiple Primary; D010911:Pituitary Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D012878:Skin Neoplasms / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome
D014408:Biomarkers, Tumor