What Is This? Gastroenterology Case Study
February 1, 2023
This article was written by Jacqueline Estevez, MD, Gastroenterology and Hepatology Fellow, Year III.
A 46-year-old female with a history of hypothyroidism was transferred from an outside hospital for respiratory and cardiac failure and extensive clot burden with concern for malignancy. She was found to have a large right-sided pleural effusion, multifocal pneumonia, a new decrease in her left ventricular ejection fraction which is 15%, and extensive clots bilaterally from her wrist to her internal jugular vein and left popliteal artery. At the outside hospital, she was intubated, and was started on norepinephrine, antibiotics, and heparin drip. Prompt transfer to UPMC Presbyterian ensued.
A computed tomography (CT) scan of the chest, abdomen, and pelvis was performed with intravenous contrast, which showed a partially calcified gallbladder wall, gallstones, and splenic infarcts. Porcelain gallbladder (PGB) is very rare with an incidence of <1%. Found predominantly in females in their sixth decade of life,1 most cases are found incidentally.2,3 There are two types of PGB: complete (or diffuse) intramural calcification characterized by a continuous band of calcification within the muscularis, and selective (or partial) mucosal calcification characterized by punctate calcifications in the glandular spaces of the mucosa.4
The rate of gallbladder carcinoma in PGB is about 0% to 5% based on recent studies.5 Of the two types of PGB, selective mucosal calcification holds a higher malignancy risk. Gallbladder cancer is usually adenocarcinoma. In selective mucosal calcification, some glands are still present and have the potential to transform into malignant cells.4,5 Gallbladder carcinoma has a five-year survival of <5%6,7 with median survival of nine months.8
Should we recommend cholecystectomy for every PGB case? There are no guidelines to answer this question currently, but published expert opinion recommends cholecystectomy if the patient is symptomatic or has concurrent gallbladder disease, complications, or selective mucosal calcification type.1,4,5,9,10
The pleural effusion was found to be an empyema, likely from her multifocal pneumonia. The etiology of her heart failure was unclear, with unremarkable left and right heart catheterization. Regarding her extensive venous and arterial clot burden, she underwent a thorough hypercoagulable workup (including factor V Leiden, JAK2 mutation, etc.) that was unremarkable, as well. The patient never had an esophagogastroduodenoscopy or colonoscopy and denied any family history of gastrointestinal malignancies. An esophagogastroduodenoscopy, colonoscopy, and cholecystectomy were offered, but she refused gastrointestinal evaluation. At home colorectal cancer screening tests were not pursued in the setting of anticoagulation for her extensive thrombi and reported hemorrhoidal bleeding. She was discharged on life-long anticoagulation with apixaban.
Recommended Reading
1. Morimoto M, Matsuo T, Mori N. Management of porcelain gallbladder, its risk factors, and complications: A review. Diagnostics (Basel, Switzerland). 2021;11(6) doi: 10.3390/ diagnostics11061073 [published Online First: Epub Date].
2. Chen GL, Akmal Y, DiFronzo AL, Vuong B, O’Connor V. Porcelain gallbladder: No longer an indication for prophylactic cholecystectomy. Am Surg. 2015;81(10):936-40.
3. 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):1064-69 doi: 10.1016/j. jamcollsurg.2017.11.026[published Online First: Epub Date].
4. Stephen AE, Berger DL. Carcinoma in the porcelain gallbladder: A relationship revisited. Surgery. 2001;129(6):699-703 doi: 10.1067/ msy.2001.113888[published Online First: Epub Date].
5. Schnelldorfer T. Porcelain gallbladder: A benign process or concern for malignancy? J Gastrointest Surg : Official journal of the Society for Surgery of the Alimentary Tract. 2013;17(6):1161-8 doi: 10.1007/s11605-013- 2170-0[published Online First: Epub Date].
6. Sheth S, Bedford A, Chopra S. Primary gallbladder cancer: Recognition of risk factors and the role of prophylactic cholecystectomy. Am J Gastroenterol. 2000;95(6):1402-10 doi: 10.1111/j.1572-0241.2000.02070.x[published Online First: Epub Date].
7. Wullstein C, Woeste G, Barkhausen S, Gross E, Hopt UT. Do complications related to laparoscopic cholecystectomy influence the prognosis of gallbladder cancer? Surg Endosc. 2002;16(5):828-32 doi: 10.1007/s00464-001- 9085-7[published Online First: Epub Date].
8. Rana SS, Bhasin DK. Porcelain gall bladder on endoscopic ultrasound. Endosc Ultrasound. 2013;2(2):112-3 doi: 0.4103/2303-9027.117702 [published Online First: Epub Date].
9. EASL Clinical Practice Guidelines on the Prevention, Diagnosis and Treatment of Gallstones. JHepatol. 2016;65(1):146-81 doi: 10.1016/j.jhep.2016.03.005[published Online First: Epub Date].
10. Khan ZS, Livingston EH, Huerta S. Reassessing the need for prophylactic surgery in patients with porcelain gallbladder: Case series and systematic review of the literature. Arch Surgery. (Chicago, Ill. : 1960) 2011;146(10): 1143-7 doi: 10.1001/archsurg.2011.257 [published Online First: Epub Date].
