Non-familial juvenile polyposis with histological evidence of adenomatous transformation.

A 14-year-old male presented with abdominal pain, diarrhoea and a sensation of something prolapsing through the anus during defecation, and was found to have diffuse colonic polyposis. There was no evidence of mucocutaneous hyperpigmentation and family history was negative, suggesting a diagnosis of non-familial juvenile polyposis. Histological analysis of multiple endoscopic biopsies showed features typical of juvenile or retention type (hamartomatous) lesions: dilated cystic glands lined by mucocus-secreting epithelium and prominent, inflamed and congested lamina propria. However, admixed with these features, focal areas of atypical adenomatous changes were recognized. Even the intervening normal-looking colonic mucosa showed some dysplastic changes. These findings indicate that hamartomatous and atypical adenomatous epithelial changes can co exist in non-familial juvenile polyposis and the latter may confer a risk of malignant transformation in this otherwise non-neoplastic disease.

Cronkhite-Canada syndrome with adenomatous and carcinomatous transformation of colonic polyp.

We describe a 70-year-old woman who presented with watery diarrhea and was found to have gastric and colonic polyposis, cutaneous hyperpigmentation, alopecia and onychodystrophy (Cronkhite-Canada syndrome). Histology of a polyp from the stomach showed features of juvenile or retention type (hamartomatous) polyp. One colonic polyp revealed features of tubular adenoma, with moderate dysplasia. Another large pedunculated colonic polyp showed a tubulovillous adenoma with a focus of well-differentiated adenocarcinoma confined to the submucosa of the stalk. Adenomatous and carcinomatous epithelial changes can occur in Cronkhite-Canada syndrome.

Massive hepatomegaly: a presenting manifestation of multiple myeloma.

We report a 50-year-old woman presenting with isolated massive hepatomegaly. Liver histology showed dilated sinusoids within which some atypical cells, probably of hematopoeitic origin, were identified. Bone marrow was densely packed with similar atypical cells with high nucleo-cytoplasmic ratio, which tested positive for plasma cell markers. Plasma protein electrophoresis showed a distinct M spike in the gamma globulin fraction and skeletal survey revealed multiple lytic lesions in the skull and pelvic bones. Thus, a final diagnosis of multiple myeloma was made. The patient has received six cycles of chemotherapy and is doing well.