Right upper quadrant calcification: porcelain gallbladder disease - Radiographic Highlights

Author: Eddie L. Hoover, James Hassett
Date: May, 1992

EDDIE L. HOOVER, M.D., and RAMANTHAPUR K. NATESHA, M.D. State University of New York at Buffalo School of Biomedical Sciences, Buffalo, New York MARC COOPERMAN, M.D., St. Anthony Medical Center, Columbus, Ohio JAMES HASSETT, M.D., State University of New York at Buffalo School of Biomedical Sciences Buffalo, New York Large solitary calcification in the right upper quadrant is rarely seen in the United States. It may indicate disease in the gallbladder, adrenal glands, kidneys, pancreas, lungs or chest wall. Disease processes associated with calcification in these organs include echinococcal cysts, calcified renal cysts, chest wall masses and degenerative cystic lesions of the pancreas and adrenal glands. However, if calcification is associated with porcelain gallbladder, the incidence of carcinoma is high. Treatment consists of cholecystectomy with a careful search for malignancy.

Relatively few diseases in the Western Hemisphere progress to the point of producing large, solitary calcified masses in the right upper quadrant. Echinococcal cysts are commonly found on radiographs of patients living in the Far East. In the United States, the differential diagnosis of a calcified mass in the right upper quadrant includes calcified renal cysts, chest wall masses, a large solitary gallstone, porcelain gallbladder and, very rarely, an adrenal or pancreatic degenerative cystic lesion. The majority of these disease processes are benign and are likely to be detected only during diagnostic evaluation of other symptoms. Because of the high incidence of carcinoma in patients with porcelain gallbladder, primary care physicians as well as surgeons should always include porcelain gallbladder in the differential diagnosis of opacified right upper quadrant lesions.

Porcelain gallbladder is rare, and patients are usually asymptomatic. Cholecystectomy is required, as well as a diligent pathologic search for malignancy. The unique character of this disease and the treatment implications of the diagnosis comprise the basis of this report.

Illustrative Case

A 61-year-old woman with hypertension and diabetes presented with an eightmonth history of pain in the right upper quadrant. She had a long history of intermittent intolerance to fatty foods. On physical examination, a hard palpable mass, 5 x 4 cm, was discovered in the right upper quadrant.

Plain film of the abdomen revealed a clearly outlined oval calcification in the right upper quadrant (Figure 1). Computed tomographic (CT) scans demonstrated circumferential calcification in the gallbladder wall (Figure 2). Ultrasound examination of the abdomen demonstrated stones in the gallbladder. Liver function tests, bilirubin level, serum amylase level, electrolytes, glucose level, urinalysis and complete blood count were all within normal limits.

At surgery the gallbladder was found to be enlarged, white and rock-hard. Cholecystectomy was performed, and a cholangiogram was normal. No stones were seen, and no evidence of malignancy was found on light microscopy. The postoperative course was uneventful, and the patient was discharged in five days. She remained asymptomatic 15 months later.

Discussion

Intramural calcification of the gallbladder has been referred to by the terms "calcifying cholecystitis," "china gallbladder," "cholecystopathy chronic calcanea," "simple calcified gallbladder', and "porcelain gallbladder." The latter term describes the brittle consistency and bluish discoloration of the diseased organ. [1,2] The pathogenesis of the calcification is unknown, but it occurs as either a broad, continuous band in the muscularis or as multiple, punctate areas in both the glandular spaces and the Rokitansky-Aschoff sinuses. [3]

The mechanism of injury appears to be related to chronic irritation by stones; this is supported empirically by the observation that more than 95 percent of patients with porcelain gallbladder also have stones. [2,4] It is hypothesized that cystic duct obstruction results in the deposition of calcium carbonate salts in the gallbladder mucosa, followed by stagnation of bile. [5] Alternatively, the disorder may be seen as a dystrophic process secondary to chronic infection and compromised circulation from cystic duct obstruction, resulting in scarring, hyalinization and, finally, the deposition of lime salts. [6]

The disease is five times more common in women between the ages of 38 and 84 years (with a mean age of 54 years) than in the general population. The first pediatric case, reported in 1990, occurred in a 10-year-old girl. [7] Predisposing factors in the pediatric age group include anatomic abnormalities, hemolytic diseases and disorders affecting bile composition. The girl in this case had no evidence of stones or malignancy, but chronic cholecystitis was demonstrated microscopically. Although some evidence of function was demonstrated after infusion of a synthetic cholecystokinin, the authors recommended surgery despite the patient's young age.

Since most patients are asymptomatic and the lesion is detected only by newer imaging modalities, investigators have correlated ultrasound and CT findings to assist in the diagnosis. In 1984, Kane and colleagues [8] reported three distinct sonographic patterns in nine patients. The type I pattern was identified by a hyperechoic semilunar structure with a posterior shadow and no gallstones. The type II pattern had a biconvex, curvilinear echogenic structure with acoustic shadowing and stones, and the type III pattern had irregular echoes with posterior shadowing.

In 1989, Shimizu and co-authors [9] reviewed the 30 cases in the world's literature in which ultrasound features of porcelain gallbladder were described. They collated the presence of gallbladder mucosal epithelium, stones and malignancy, and compared their groups with Kane's classification. Half of the patients had the type I pattern, with no stones or malignancies. A 42 percent incidence of cancer was found in patients with the type II pattern (14 of 30 patients).

Shimizu and colleagues [9] recommended a simple classification: complete (replacement of the mucosa with dense connective tissue and calcification) and incomplete (some mucosa remaining). Their "complete" group corresponded to Kane's type I pattern, and their "incomplete" group corresponded to types II and III. Shimizu and colleagues assumed that cancer arose from the mucosal epithelium, which meant that malignancy was improbable in type I or complete groups.

The correlation between carcinoma and porcelain gallbladder was emphasized in a 1962 review of 1,786 cholecystectomy specimens, which included 78 cases of carcinoma. [10] Twenty-six of the 78 patients had a porcelain gallbladder, of whom 16 had carcinoma--an incidence of 61 percent, the highest reported in the literature. In another review of 45 cholecystectomies performed because of carcinoma, 16 patients had porcelain gallbladder. [6] Only two of these patients had adenocarcinoma, an incidence of 12.5 percent.

Scirrhous carcinoma is the most common malignancy encountered; 80 percent are adenocarcinomas, with isolated reports of mucinous adenocarcinornas and one reported case of squamous cell carcinoma. [2] Other researchers demonstrated that carcinoma occurs in both the calcified. and noncalcified areas of the gallbladder. [11] This observation may provide support for Shimizu's classification system, in which the probability of carcinoma is based on the presence of mucosal epithelium.

The writing of this article was supported in part by the Russell and Cora PhiferFoundation, Charlotte, N.C.

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The Authors

EDDIE L. HOOVER, M.D. is professor and chairman of the Department of Surgery and chief of the Division of Cardiothoracic Surgery at State University of New York at Buffalo School of Biomedical Sciences. Dr. Hoover graduated from Duke University School of Medicine, Durham, N.C., and completed a residency in general surgery and thoracic surgery at Duke University School of Medicine and Cornell University Medical College, New York City.

RAMANTHAPUR K. NATESHA, M.D. is chief resident in general surgery at State University of New York at Buffalo. Dr. Natesha is a graduate of Mysore Medical College, Mysore, India, and a fellow of the Royal College of Surgeons of Edinburgh and Glasgow, Great Britain.

MARC COOPERMAN, M.D. is a clinical associate professor of surgery at Meharry Medical College, Nashville, Tenn., and chief of surgery at St. Anthony Medical Center, Columbus, Ohio. Dr. Cooperman graduated from Stanford (Calif.) University School of Medicine, and completed advanced training in general surgery at the University of Chicago Pritzker School of Medicine and Ohio State University College of Medicine, Columbus.

JAMES HASSETT, M.D. is an associate professor of surgery and director of surgical education at State University of New York at Buffalo, After graduating from Loyola University of Chicago Stritch School of Medicine, Maywood, Ill., he completed a residency in general surgery at State University of New York at Buffalo.

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