Primary adrenal leiomyosarcoma is extremely rare tumor. We report a case with adrenal leiomyosarcoma. Our case was a 48-year-old man who presented with lower urinary tract symptoms. Ultrasonography and magnetic resonance imaging revealed approximately 9 cm solid mass originating from right adrenal gland. He underwent right adrenalectomy. Pathology of the specimen showed histologic and immunohistochemical features of adrenal leiomyosarcoma.
Neonatal tumors are reported to occur in approximately 17-121 per million live births worldwide. They are often diagnosed by ultrasonography after mid-pregnancy. Teratomas are the most frequent solid neoplasms, accounting for between one-quarter and one-third of cases. Here, we describe the prenatal diagnosis of a fetal face teratoma located on the right temporal side at 26 weeks of gestation. Besides 2D and 4D ultrasound imaging, fetal magnetic resonance imaging provides substantial support in perinatal management and promotes the perception of fetal malformations by the family. Extreme intrauterine growth of the tumor with remarkable pressure to the surrounding facial structures and good perinatal prognosis following complete tumoral resection are reviewed.
Complete androgen insensitivity syndrome (CAIS) associated with Müllerian remnant is rare during childhood. The Müllerian system usually regresses because of the presence of the anti-Müllerian hormone (AMH) originating from the Sertoli cells of the gonads. Rarely, residual Müllerian structures may exist. We present two cases from the same family, raised as females. They were 12 and 18 years old, respectively, and they had Tanner V breast development but Tanner I-II pubic hair. The older patient had primary amenorrhea. Both have a 46,XY genotype. Pelvic ultrasonography revealed no uterus and ovaries. The patients underwent bilateral laporoscopic gonadectomy. Both had residual Müllerian structures. Mutation analyses were performed, and both patients were found to be carrying a point mutation in exon 4 of the AR gene consisting of a G nucleotide deletion at position c.1890delG, followed by a frame-shift mutation and a stop codon. This mutation has not been described yet in the literature. Although the association with CAIS and Müllerian remnant is rare, no genetic defect specific to androgen insensitivity with Müllerian remnants has been identified so far.
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