Prophylactic Cholecystectomy for Porcelain Gallbladder

AUTHORS:
Preslav Valchev, MD, RN¹ • Sejal Patel, MD²

AFFILIATIONS:
¹St. James School of Medicine, Park Ridge, Illinois
²Nephrology Department, AMITA Health St. Alexius Medical Center, Hoffman Estates, Illinois

CITATION:
Valchev P, Patel S. Prophylactic cholecystectomy for porcelain gallbladder. Consultant. 2022;62(7):e6-e8. doi:10.25270/con.2021.09.00004

Received March 23, 2021. Accepted May 10, 2021. Published online September 24, 2021.

DISCLOSURES:
The authors report no relevant financial relationships.

CORRESPONDENCE:
Preslav Valchev, MS4, RN, St. James School of Medicine, 1480 Renaissance Drive, Suite 300, Park Ridge, IL 60068 (pvalchev@mail.sjsm.org)

A 41-year-old man presented to our emergency department (ED) with weight loss, abdominal pain, nausea, and vomiting over a 6 month period.

History. His medical history was significant for chronic intermittent postprandial biliarycolic pain secondary to cholelithiasis. The patient had no other relevant medical history and reported no known family history of gastrointestinal cancer. He reported a prior occupation as a textile worker for a 6-year period.

Approximately 6 months prior to his presentation to our ED, the patient had reported postprandial nausea and right upper quadrant abdominal pain. As a result of his symptoms, he had undergone an ultrasonography scan, results of which had showed evidence of gallstones. A computed tomography (CT) scan had also been conducted at that time, results of which had confirmed the presence of calcifications in the gallbladder wall, which were suspicious to be premalignant. At that time, it was advised that the patient undergo cholecystectomy, but he decided to postpone surgery because of an upcoming vacation.

Physical examination. Upon arrival to our ED, the patient’s vital signs were stable and within normal limits. Upon examination, the patient’s skin appeared to be jaundiced, and an evaluation of the abdomen showed abdominal guarding and right upper quadrant tenderness. Additionally, a large palpable mass was noted in the right upper quadrant. The rest of the physical examination was unremarkable.

Diagnostic testing. Initial laboratory results showed elevated liver enzymes, with an aspartate aminotransferase level of 432 U/L, an alanine aminotransferase level of 241 U/L, and a total bilirubin level of 1.1 mg/dL. Moreover, the patient’s total protein level (6.2 g/dL) and albumin level (3 g/dL) were low, signifying possible liver dysfunction. To investigate for malignancy, blood tumor markers were also evaluated, results of which were positive for an elevated carcinoembryonic antigen level of 11.3 ng/mL. The patient’s α-Fetoprotein level was also examined to investigate for liver carcinoma, but results were unremarkable.

In addition, a CT scan and magnetic resonance imaging (MRI) of the abdomen were conducted. Results revealed a large mass measuring 9 × 7 cm originating from the gallbladder and extending into the liver, with mild extrahepatic and intrahepatic biliary duct dilation. Ultimately, the mass was biopsied via ultrasound-guided fine-needle core biopsy, results of which revealed adenocarcinoma. Additionally, immunohistochemistry of the biopsy specimen was performed, results of which were focally positive for carbohydrate antigen 19-9, further confirming the diagnosis of adenocarcinoma of gallbladder origin.

Treatment. The patient underwent extended right hepatic lobectomy, cholecystectomy, myofascial wall flap, omentectomy, and portal lymphadenectomy (Figure). After the procedures, the patient was given time to recover and eventually was started on adjuvant chemotherapy with intravenous (IV) cisplatin, 55 mg; IV gemcitabine, 2190 mg; and IV dexamethasone, 12 mg, for 3 weeks with a 2-week recovery interval. The complications and risks of chemotherapy were explained to the patient and his family, and appropriate premedication was given to the patient.

Figure. The resected metastatic tumor from the patient’s liver measuring 9 × 7 cm.

Discussion. Porcelain gallbladder—also referred to as, “cholecystopathiachronica,” calcified gallbladder, or calcifying cholecystitis—is a rare condition, with an incidence rate of roughly 0.06% to 0.8%, and women are more often affected than men.¹ It is characterized by bluish discoloration of the gallbladder and calcification of the gallbladder wall.¹ The etiology of porcelain gallbladder is not well known, but it is strongly associated with cholelithiasis, which is present in 95% of cases of porcelain gallbladder.² Another risk factor for porcelain gallbladder is stenosis of the biliary tree. In the past, porcelain gallbladder had been strongly associated with gallbladder cancer. However, recent research indicates that the malignancy risk of porcelain gallbladder may vary from 5% to 22%.¹ Although current research points to a variable increase in malignancy potential of porcelain gallbladder, identification and treatment should still be considered. Complications caused by an unsuccessfully treated porcelain gallbladder, or a delay in treatment, can be devastating.

Patients with porcelain gallbladder are often asymptomatic.³ However, when symptoms appear, making the diagnosis is often difficult because there are several common differentials, such as large opaque gallstones, acute cholecystitis, or calcified liver cysts.³ Most often, patients report right upper quadrant tenderness that radiates to the right supra scapular area.² These symptoms are usually exacerbated by fatty food intake.² Nausea and occasional vomiting may also be reported by patients, and these symptoms often occur in the evening.² Symptoms observed in our patient, including painless jaundice, unexplained weight loss, and palpable abdominal mass, may be indicative of more progressive disease with transition to gallbladder carcinoma.⁴ In general, patients with porcelain gallbladder are stable, have vital signs within normal limits, and do not appear acutely ill, which may be why they do not seek treatment.² Unfortunately, for some patients, not seeking treatment may ultimately lead to gallbladder cancer and death.

Porcelain gallbladder may be formed when materials in bile become over saturated.² When bile acid sits in the gallbladder without proper drainage, it accumulates these materials, which over a long period of time can contribute to the formation of small crystals.² The crystals become embedded in the mucous of the gallbladder mucosa, which then forms biliary sludge. The sequela of this process is the formation of large stones, or “cholelithiasis.” This may explain why patients often remain asymptomatic for a long period of time. Progression of the crystals is what may be the cause of the calcification of the gallbladder wall seen commonly in porcelain gallbladder, but further investigation is needed.² It is important to mention that the most prevalent stones in patients with porcelain gallbladder are calcium bilirubinate and cholesterol stones.² This is important because a cholesterol-rich diet may precipitate the formation of cholesterol stones in the gallbladder and further aggravate biliary colic pain.

The process of porcelain gallbladder advancing to carcinoma is reported to be a result of chronic degeneration and regeneration within the gallbladder epithelium due to biliary stasis and exposure to offensive chemicals in the stagnant bile, leading to mucosal dysplasia.³ This is an important point since our patient had an occupational history of industrial exposure to textile chemicals for 6 years, with chronic symptomatic cholelithiasis.

Histopathology of porcelain gallbladder often shows thickening of the gallbladder wall.² The wall may display a complete or patchy area of calcification.² The patchy area of calcifications may show inflammatory changes, which carry the greatest risk of malignancy.² Therefore, it is critical that all porcelain gallbladders be sent for pathology after the resection.

Often porcelain gallbladder is diagnosed incidentally on routine imaging. CT scanning is the most superior imaging modality for accurately identifying and differentiating porcelain gallbladder from other, similar conditions such as calcified renal cysts, cholelithiasis, and calcified lymph nodes.⁴ The classic finding on CT imaging is a partial or completely calcification of the neck or body of the gallbladder.⁴ Plain film radiographs can also show porcelain gallbladder by demonstrating the characteristic curvilinear wall calcification in the gallbladder fossa. Abdominal ultrasonographs may show a highly echogenic acoustic shadowing with curvilinear structure in the gallbladder fossa, but it may be difficult to differentiate it from emphysematous cholecystitis.⁴ Regardless of the imaging preference, it is critical that this condition is identified early so that appropriate surgical intervention can be implemented.

Laparoscopic cholecystectomy is the preferred treatment of choice for porcelain gallbladder. In the past, open cholecystectomy was favored because of the perceived higher incidence of gallbladder cancer.² However, recent reports recommend conservative therapy with repeat gallbladder ultrasonography scanning in patients who are asymptomatic.² Most surgeons, however, would recommend prophylactic cholecystectomy over ultrasonographic surveillance.²

Patient outcome. The patient showed only transient improvement after surgery and chemotherapy. He followed up periodically with his oncologist for routine surveillance imaging and laboratory work and was in remission for about 1 month. After multiple cycles of chemotherapy over a 6-month period, the patient presented again to the ED with “excruciating” abdominal pain and was found to have widespread metastasis to the vertebral spine and rib cage. Due to the degree of metastasis and patient preference, it was determined that chemotherapy and further surgery would be futile. The patient was started on supportive therapy and provided with hospice care.

Conclusion. The malignancy potential of porcelain gallbladder should not be taken lightly, although most recent research points to a relatively low gallbladder cancer risk. In patients, such as ours, who experience chronic postprandial abdominal pain, early prophylactic cholecystectomy would have been appropriate. However, in patients with limited life expectancy and advanced disease, recent research demonstrates that an operative approach provides no significant difference in adverse events compared with an observational approach.⁵ Ultimately, each individual case of porcelain gallbladder needs to be correctly identified early, and the pros and cons of performing prophylactic laparoscopic cholecystectomy over observation should be weighted accordingly.

References

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2. Jones MW, Weir CB, Ferguson T. Porcelain Gallbladder. In: StatPearls [Internet]. StatPearls Publishing; January 2021. http://www.ncbi.nlm.nih.gov/books/nbk518979/

3. Thakrar R, Monib S, Pakdemirli E, Thomson S. Calcified gallbladder cancer: is it preventable? J Surg Case Rep. 2019;2019(3):rjz069. https://doi.org/10.1093/jscr/rjz069

4. Iqbal S, Ahmad S, Saeed U, Al-Dabbagh M. Porcelain gallbladder: often an overlooked entity. Surg J (N Y). 2017;3(4):e145-e147. https://doi.org/10.1055/s-0037-1606546

5. DesJardins H, Duy L, Scheirey C, Schnelldorfer T. Porcelain gallbladder: Is observation a safe option in select populations? J Am Coll Surg. 2018;226(6):1064-1069. https://doi.org/10.1016/j.jamcollsurg.2017.11.026