ISOLATED PEUTZ JEGHERS GALL BLADDER POLYP

PRASHANTH KOYYODA, G R Mallikarjuna, B Moksha Prasuna, Ch Madhusudhan, R Pratap Reddy

Osmania Medical College, Hyderabad

INTRODUCTION : Hamartomatous polyp is an extremely rare polypoidal lesion of the gallbladder, with the first case having been reported in 1998(1). Fewer than 10 hamartomatous gallbladder polyps have been reported in patients with Peutz-Jeghers syndrome (PJS)(2). Only a single case report of isolated peutz jeghers polyp in gall bladder has been reported in a patient without PJS like in the present case.

Absent perioral pigmentation

MRCP showing polypoidal mass in the Gallbladder , with filling defects in Common Bile Duct

CASE REPORT : 64year old female presented with abdominal pain and was found to have a polypoid mass in the gall bladder and multiple filling defects in the CBD. Patient underwent extended cholecystectomy in view of suspected carcinoma gall bladder. On examination there was a large friable polyp in the gall bladder and multiple CBD stones. Microscopic examination revealed a hamartomatous (peutz jegher) polyp. Post operatively screening was done by endoscopy, colonoscopy and CT enteroclysis to look for polyps elsewhere and no such lesions were found. There was no significant family history in this case

DISCUSSION : Peutz-Jeghers syndrome (PJS) is an autosomal-dominant inherited syndrome characterized by hamartomatous polyps in the gastrointestinal tract, pigmented macules of mucous membranes and skin, and a predisposition to gastrointestinal and other tumors . The small bowel is the most common affected site followed by colon, stomach, duodenum, and appendix. Extra intestinal polyps may occur rarely and the possible sites include the respiratory tract, urogenital tract and biliary tract.Fewer than 10 cases of gall bladder hamartomatous polyps are described in peutz jeghers syndrome. The largest series evaluating extraintestinal polyps in patients with PJS revealed 3 patients with a gallbladder polyp among 72 patients with 1 pathologically proven hamartomatous polyp(2). Only 1 case of isolated peutz jeghers polyp in non syndromic case has been reported till date(3). Solitary PJP do not carry a risk of gastrointestinal cancer and are not an indication for specific high-risk screening(4) .

CONCLUSION : Extra intestinal isolated hamartomatous peutz jeghers polyps have been described in biliary tree in PJS. This case is an isolated peutz jeghers polyp in gall bladder in a non PJS patient.

Resected specimen of extended cholecystectomy showing distended gall bladder

Polypoid pedunculated growth inside the resected gall bladder

4x view showing arborizing pattern of smooth muscle surrounding the lobules of hyperplastic mucosal glands

40x view showing splaying of smooth muscle bundles and hyperplastic glandular epithelium

REFERENCES:1. Guzmán P, Roa I, Villaseca M, Roa JC, Araya JC. Peritoneal pseudomyxoma in a child with a gallbladder Peutz-Jeghers like hamartomatous polyp: a case report. J PediatricsSurg 1998; 33:1320–1322 2. Vogel T, Schumacher V, Saleh A, et al. Extraintestinal polyps in Peutz-Jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe.Int J Colorectal Dis.

2000 Apr;15(2):118-23.3. A.DevrimKaraosmaniglu,MD,Michael Blake, MD, Hamartomatous polyp of the gall bladder with an associated choledochal cyst by American Institute of Ultrasound in Medicine.J Ultrasound Med

2010;29:1663-1666.4. Oncel M, Remzi FH, Church JM, Goldblum JR, Zutshi M, Fazio VW. Course and follow-up of solitary Peutz-Jeghers polyps: a case series. Int J Colorectal Dis. 2003 Jan;18(1):33-5.