Saudi Journal of Gastroenterology
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CASE REPORT
Year : 2012  |  Volume : 18  |  Issue : 6  |  Page : 388-391

An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome


1 Department of Medicine, Aga Khan University Hospital, Karachi, Pakistan
2 Shifa International Hospital, Islamabad, Pakistan
3 Department of Surgery, Aga Khan University Hospital, Karachi, Pakistan
4 Department of Pathology, Aga Khan University Hospital, Karachi, Pakistan
5 Department of Radiology, Aga Khan University Hospital, Karachi, Pakistan

Correspondence Address:
Nazish Butt
Resident Gastroenterology, Aga Khan University Hospital, Stadium Road, Karachi
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-3767.103432

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Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by mucocutaneous melanin pigmentation and gastrointestinal (GI) tract hamartomatous polyps and an increased risk of malignancy. In addition to polyposis, previous studies have reported increased risk of GI and extraGI malignancies in PJS patients, compared with that of the general population. The most common extraintestinal malignancies reported in previous studies are pancreatic, breast, ovarian and testicular cancers.We report the case of a 17-year-old boy who presented with generalized weakness, recurrent sharp abdominal pain and melena, had exploratory laparotomy and ileal resection for ileo-ileal intussusception. Pigmentation of the buccal mucosa was noted. An abdominal computed tomography scan (CT) revealed multiple polyps in small bowel loops. Gastroscopy revealed multiple dimunitive polyps in stomach and pedunculated polyp in duodenum. Colonoscopy revealed multiple colonic polyps. Pathological examination of the polyps confirmed hamartomas with smooth muscle arborization, compatible with Peutz-Jeghers polyps. CT scan guided left para-aortic lymph node biopsy revealed the characteristic features of extra-adrenal para-aortic paraganglioma. Although cases of various GI and extra GI malignancies in PJS patients has been reported, the present case appears to be the first in literature in which the PJS syndrome was associated with asymptomatic extraadrenal para-aortic paraganglioma. Patients with PJS should be treated by endoscopic or surgical resection and need whole-body screening.


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