Sclerocalcifying Cholecystitis (SclCC) and Associated Carcinomas: Characterization of a Clinicopathologically Distinct and Challenging Entity
S Patel, O Tapia, JC Roa, A Cakir, N Dursun, O Basturk, D Akdemir, S Bandyopadhyay, J Sarmiento, I Coban, NV Adsay. Emory, GA; U de La Frontera, Temuco, Chile; NYU, NY; Ohio Northern, OH; WSU, MI
Background: There are no systematic analyses in the pathology literature of cholecystitis(CC) with extensive sclerosis & calcifications(calcs). We have seen examples of carcinoma(CA) associated with these patterns, which typically presented as a diagnostic challenge.
Design: 3625 consecutive cholecystectomies were systematically analyzed. Separately, consultation & institutional files were also reviewed for cases designated as sclerotic, calcifying or porcelain.
Results: SclCC (n=68): Incidence in cases systematically reviewed=1.5%(53/3625). F/M=4/1. Mean age=57(vs 47 in ordinary CC; p<0.001). Characterized by dense, paucicellular fibrosis transforming GB wall into a relatively thin & uniform band and diffusely replacing most(>80%) of the normal structures such that only minimal/none mucosa or muscularis is identifiable. 75% were associated with calcs. CA arising in SclCC (n=38): F/M=4.4/1. Mean age=62(vs 57 in non-cancerous SclCC; p=0.04). Carcinomatous glands were well-masked by hyalinization; widely separated, typically spanning into a very large zone and often circumferentially involving the GB wall without forming any localized thickening(mean wall thickness=2.64 mm in SclCC-associated CA vs 3.92 mm in usual CA; p=0.002). Clear cytoplasm, dyspolarized atypical nuclei, washed-off chromatin & irregular contours distinguished these from benign glands. Surface epithelium was entirely denuded in most areas with rare clinging CIS cells. The epithelium of inv glands was also commonly attenuated/denuded, leaving only the necrotic luminal debris as the sole sign of invasion. In many areas, this debris imperceptibly transformed into the hyalinized stroma, suggesting a regression phenomenon akin to that in melanomas. 3 and 5-yr surv were 15% and 15%, respectively(vs 36% and 30% for usual CA; p<0.05).
Conclusions: SclCC is the name we propose to employ for this distinctive type of CC, which is seen in patients a decade older than ordinary CC patients, suggesting long-standing injury in its pathogenesis. CAs arising in SclCC are highly subtle and challenging due to their insidious pattern. They are virtually undetectable grossly; careful sampling is warranted. Conventional staging parameters are difficult to apply due to the effaced layering of the GB wall. Overall prognosis appears to be even worse than that of usual CA.
Category: Liver & Pancreas
Monday, March 22, 2010 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 213, Monday Morning