Cases reported "Peutz-Jeghers Syndrome"

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1/191. Total gastrointestinal endoscopy in the management of peutz-jeghers syndrome.

    peutz-jeghers syndrome was diagnosed in a 51-year-old woman presenting with iron deficiency anaemia. Upper gastrointestinal endoscopy and colonoscopy revealed several hamartomatous polyps in the stomach, duodenum and colon, which were removed. At a combined surgical-endoscopic procedure, 42 hamartomatous polyps were removed from the small intestine by snare polypectomy. This enteroscopic procedure reduces symptoms, may protect against future intestinal obstructive episodes and their associated surgery, and may reduce the risk of developing gastrointestinal malignancy. ( info)

2/191. A new simple technique for performing intraoperative endoscopic resection of small-bowel polyps in patients with peutz-jeghers syndrome.

    We describe herein a simple method for performing intraoperative endoscopic resection of small-bowel polyps associated with peutz-jeghers syndrome, using a corrugated anesthetic tube. A 34-year-old man with peutz-jeghers syndrome underwent emergency surgery for an ileo-ileo-colic intussusception. A sterile corrugated anesthetic tube was inserted into the small-bowel, proximal to the affected lesion. The small bowel was then telescoped sequentially over the tube using a pleating technique. Consequently, a colonoscope inserted through the tube was easily able to reach the duodeno-jejunal junction, and ten small-bowel polyps were removed using a wire snare and electrocauterization. All resected specimens were washed out by the instillation of saline through a nasogastric tube, then collected on gauze placed near the outlet of the tube. Our technique has the following merits: it is feasible even in emergency surgery; it prevents contamination of the surgical field; and it facilitates the easy collection of polypectomized specimens. ( info)

3/191. intussusception in peutz-jeghers syndrome: sonographic findings.

    An intussusception can easily occur in patients with peutz-jeghers syndrome (PJS), and its early detection is crucial. We present a PJS patient in whom sonography (US) helped in detecting intussusception at a time when there was only a very vague symptom and provided successful conservative treatment. Close clinical and US follow-up of PJS patients should improve management of this condition. ( info)

4/191. Misplacement of dysplastic epithelium in Peutz-Jeghers Polyps: the ultimate diagnostic pitfall?

    peutz-jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Small bowel polyps in the syndrome may exhibit epithelial misplacement, into the submucosa, the muscularis propria, and even the subserosa. The authors demonstrate two patients in whom there is also misplacement of dysplastic epithelium into the submucosa and muscularis propria of the small bowel. Epithelial misplacement is recognized to mimic invasive malignancy. Such mimicry is heightened substantially when the misplaced epithelium is dysplastic. Correct interpretation of the histologic changes is aided by the use of special stains, which demonstrate the associated lamina propria and the lack of a desmoplastic response, and immunohistochemistry, which shows that the misplaced dysplastic epithelium is accompanied by non-neoplastic mucosa. There is an increased prevalence of gastrointestinal malignancy in peutz-jeghers syndrome. However, the presence of perplexing histologic features, caused by epithelial misplacement, especially when some of that epithelium is dysplastic, in small bowel polyps at least has the potential for the overdiagnosis of malignancy in the syndrome. ( info)

5/191. serum inhibin B concentration in a prepubertal boy with gynecomastia and peutz-jeghers syndrome.

    gynecomastia in boys with peutz-jeghers syndrome and Sertoli cell tumors of gonadal origin results from increased estrogen production due to increased aromatase activity within the testicular tumor. We present a prepubertal boy with peutz-jeghers syndrome, gynecomastia and bilateral neoplastic Sertoli cell proliferation in whom the only abnormal hormonal profile was increased concentration of inhibin B and Pro-alpha C in serum. ( info)

6/191. peutz-jeghers syndrome--a case report.

    A case of peutz-jeghers syndrome presenting acutely with ileo-ileal intussusception is described. Management issues including follow up protocols are reviewed with reference to the current literature. ( info)

7/191. Sporadic peutz-jeghers syndrome in a Nigerian.

    peutz-jeghers syndrome is a rare condition of muco-cutaneous pigmentation and polyposis of the gut. Reports of its occurrence from the Black world have been infrequent. A case is presented of an 18 year old Nigerian girl with pigmentation of the inner lips and soles of both feet, and recurrent attacks of abdominal pain necessitating two surgical procedures for intestinal obstruction with removal, in both cases, of polyps. Problems of complications and therapeutic modalities are discussed. ( info)

8/191. peutz-jeghers syndrome: case report.

    A case of peutz-jeghers syndrome (PJS) in an African girl is presented to document the clinical presentation, management and follow up of this condition. The patient who presented with black buccal mucosal hyperpigmentation and clinical features of jejuno-jejunal intussusception was successfully managed by operative reduction of the intussusception and polypectomy. She is being followed up for evidence of malignant transformation in associated intestinal polyps and development of extra-intestinal malignancies at other sites. The management of PJS in light of recent trends is discussed, especially with reference to suggested protocols for screening and surveillance of sites at supposed risk of tumour development. ( info)

9/191. Malignant ovarian sex cord tumor with annular tubules in a patient with peutz-jeghers syndrome: a case report.

    The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. In patients who have peutz-jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding. Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear. ( info)

10/191. Extraintestinal polyps in peutz-jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe.

    peutz-jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. Extraintestinal polyps have rarely been reported. Possible sites include the respiratory tract, urogenital tract, and gallbladder. We here describe four cases of extraintestinal polyps in PJS patients and review the literature on the need for operative therapy of extraintestinal polyps in PJS. Three nonrelated patients were examined who had PJS and polyps in the gallbladder; the fourth patient had PJS and recurrent choanal polyps. Surgery has so far been performed only for symptomatic polyps: one laparoscopic cholecystectomy and removal of the choanal polyps for recurrent infections of the respiratory tract. The remaining two patients reported no symptoms from the extraintestinal polyps. No malignant transformation was found in these patients, nor has such been reported in the literature on PJS. The frequent observation of this manifestation in our patients raises the question of clinical management: Is prophylactic surgery indicated? Since malignant transformation of PJS polyps in the intestine is extremely rare we see no reason for operative therapy as long as the polyps are small and asymptomatic. Regular sonographic controls are recommended since the risk of malignant transformation cannot be ruled out at present. ( info)
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