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SHR Neuro Cancer Cardio Lipid Metab Microb
Saxena, AK; van Tuil, C; Schultze-Everding, A.
Frasier syndrome in a pre-menarchal girl: laparoscopic resection of gonadoblastoma.
Eur J Pediatr. 2006; 165(12):917-919
Doi: 10.1007/s00431-006-0225-x
(- Case Report)
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- Leading authors Med Uni Graz
- Saxena Amulya Kumar
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- Abstract:
- Frasier syndrome (FS) is characterized by male pseudohermaphroditism, slowly progressing nephropathy, and frequent development of gonadoblastoma. These patients are, however, often diagnosed when evaluated for primary amenorrhea. We report the case of FS in a pre-menarchal girl at the age of 6 years. Ultrasound examinations were performed and were inconclusive as to the presence or size of the gonads. Diagnostic laparoscopy was performed and the presence of bilateral streak gonads was documented and a bilateral salpingo-oophorectomy was performed. The postoperative course was uneventful. Histological examination of the streak gonads confirmed the presence of gonadoblastoma. To our knowledge, this is the first case in the literature of a pre-menarchal patient diagnosed with FS and a laparoscopic bilateral gonadoblastoma resection. Laparoscopic aspects regarding safe streak gonad removal in the pediatric population have been elaborated.
- Find related publications in this database (using NLM MeSH Indexing)
- Child -
- Fallopian Tubes - surgery
- Female - surgery
- Frasier Syndrome - diagnosis
- Gonadoblastoma - surgery
- Humans - surgery
- Laparoscopy - surgery
- Ovarian Neoplasms - surgery
- Ovariectomy - surgery
- Find related publications in this database (Keywords)
- gonadoblastoma
- Frasier syndrome
- laparoscopy
- pre-menarchal