1/5. Feminizing sertoli cell tumor associated with peutz-jeghers syndrome.peutz-jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the association of mucocutaneous pigmentation and multiple gastrointestinal hamartomatous polyps and with an increased risk of developing gonadal sex tumors besides other malignancies. We describe a 7 1/2 year-old boy with PJS and bilateral gynecomastia. He has had buccal pigmentation since 1.5 years and had been operated for rectal polyp excision at 3.5 years. On physical examination, his height was at the 90th percentile, and his height age and bone age were 9 and 10 1/2 years, respectively. Increased melanotic buccal pigmentation of the lips and bilateral gynecomastia were noticed. Both of the testes were firm, non-tender and smooth on the surface, and each measured 8 ml. Hormonal measurements were all in the prepubertal range. testis ultrasonography showed bilateral hyperechogenic areas within the glands. When he was operated for invagination and an ileum segment full of polyps was resected, bilateral testicular biopsies were also performed. Histopathological evaluation of the testes revealed bilateral multicentric benign Sertoli cell tumors. The aromatase inhibitor testolactone was started to slow skeletal maturation. On the basis of this and previous reports, PJS associated with sex-cord tumors is increasingly recognized in males as well as in females.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/5. Localized giant pseudopolyposis in ulcerative colitis.A case of localized giant pseudopolyposis in ulcertative colitis is discussed in which a mass effect mimicked tumor. The fulminant collection of pseudopolyps was palable in the epigastrium on physical exam and caused a partial obstruction to the retrograde flow of barium. carcinoma is a worrisome possibility in ulcerative colitis, but localized giant pseudopolyposis may also present as a mass.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/5. Total colectomy and anal ileostomy in multiple polyposis coli.Two patients with familial polyposis coli were treated by total abdominal colectomy, endorectal mucosal stripping, and ileoanal anastomosis. About 24 months after operation, both patients had four to five soft-formed stools daily with good anal sphincter control. Because of its physical and emotional advantages and the fact that possible precancerous lesions are treated completely, we believe that this operation is superior to those generally used today.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/5. Deletion 10q23.2-q23.33 in a patient with gastrointestinal juvenile polyposis and other features of a Cowden-like syndrome.A cytogenetically visible interstitial deletion of chromosome band 10q23 was found in a 6-year-old boy with mental retardation, dysmorphic features, and juvenile polyposis coli. In order to map this patient's deletion physically, we performed fluorescence in situ hybridization by using yeast artificial chromosomes (YACs) in the vicinity of the deletion. Five YACs that span an 11-15 cM region within the deletion were identified. This patient's deletion contains the putative locus for Cowden syndrome and a recently discovered candidate tumor suppressor gene (MMAC1 or PTEN) that has been implicated in the progression of a variety of human malignancies. Furthermore, the deletion is near and possibly overlaps a locus associated with juvenile polyposis. The findings in this patient with a constitutional 10q23 deletion raise the issue of whether there are separate genes in this region that are involved in Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, juvenile polyposis, and tumor progression, or whether all of these entities could be due to a single gene.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/5. Neonatal ileoileocolic intussusception associated with ileal polyp: report of one case.Neonatal intussusception is an uncommon disease. We report a case of neonatal ileoileocolic intussusception led by an ileal polyp in a female neonate. The patient presented with irritable crying, bilious vomiting and frank bloody stool on the 26th day of life. On physical examination, a mobile abdominal mass was palpated. Abdominal sonography demonstrated a long segment intussusception; associated with a low echogenic mass. At laparotomy, ileoileocolic intussusception led by an ileal polyp was found. pathology confirmed the diagnosis of polyp. Because intestinal obstruction is the primary manifestation, neonatal intussusception is initially indistinguishable from obstructions due to other reasons like intestinal atresia, congenital bands, necrotizing enterocolitis or midgut volvulus. Our experience showed that although uncommon, intussusception should be considered in the differential diagnosis of intestinal obstruction during the newborn period.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |