Carcinosarcoma of Gall Bladder

Faiz Akram Ansari; Mohit Bhatnagar; DC Katiyar; Munish Gairola; Vaneet Gupta; A Princee

doi:10.7869/tg.325

Carcinosarcomas of the gall bladder are rare neoplasms that constitute less than 1% of gall bladder malignancies[1]. The diagnosis of carcinosarcoma requires the presence of both malignant epithelial and mesenchymal components. The other uncommon sites are the uterus, lung, oesophagus, kidney and pancreas.[2]

Case report

A 50 year old lady presented with the complaint of pain in the right upper quadrant of the abdomen. Complete blood counts and liver and renal function tests were unremarkable. Serum CA 19-9 and CEA levels were within normal limits. An ultrasound abdomen showed a well distended gall bladder with thickened walls (7.2mm) with the presence of sludge. An abdominal CECT showed asymmetrical mural thickening predominantly in the body and neck region of the gall bladder with prominent intrahepatic biliary radicles and subcentimeter lymphadenopathy in the pre- aortic and para-aortic regions. In view of the radiological findings, the patient was planned for radical cholecystectomy. Per- operatively, the gall bladder was found to be totally replaced by a hard mass involving its fundus, body and neck but sparing the cystic duct and this was confirmed by a frozen section examination . A radical cholecystectomy was performed. The histopathology report was suggestive of carcinosarcoma of the gall bladder (pT2N0M0). Grossly there was a 5 x 4 cm tumor involving the body and fundus of the gall bladder with a large area of central necrosis. On microscopic examination, the tumor showed poorly differentiated epithelial and sarcomatous components infiltrating the perimuscular connective tissue with large areas of necrosis but no lymphovascular or perineural invasion. The serosa and peripheral rim of liver tissue were not involved. Immunohistochemistry supported the diagnosis of carcinosarcoma with the carcinomatous epithelial component expressing cytokeratin and the stromal component positive for vimentin and SMA. The patient had a steady, uneventful recovery in the post-operative period and was discharged on the sixth post-operative day. Keeping in mind the rarity of carcinosarcoma of the gall bladder and the fact that there was no established optimal adjuvant treatment defined in literature for the same, the case was discussed by a multidisciplinary tumor board. Following the board’s approval, the patient was subjected to postoperative concurrent chemo-radiotherapy with weekly leucovorin and 5-flurouracil. The dose of radiation was 50.4Gy/ 28 fractions which was given 5 days/week for 5.5 weeks. A follow up PET-CT after completion of chemoradiotherapy showed hypermetabolic hypodense liver lesions and an FDG-avid nodular lesion in the right lung. The patient was then started on palliative chemotherapy with gemcitabine and cisplatin but was lost to follow up.

Discussion

Carcinosarcoma of the gall bladder is very difficult as imaging studies cannot differentiate it from carcinoma.1-3 The diagnosis of carcinosarcoma requires histopathology and immunohistochemical staining. Microscopically, the diagnosis requires the presence of both a malignant epithelial and a mesenchymal component. Currently, there are no recommended treatment guidelines for carcinosarcoma. It is treated in the same way as carcinoma gall bladder. No optimal post-operative adjuvant therapy has been established. Previous studies have reported that there is no clear advantage of chemo- radiotherapy after surgery. The overall prognosis is very poor.[3,4] The prognostic factors are size of the tumor and invasion of surrounding tissues by the tumor. Patients with tumors smaller than 5 cm have a longer survival. The 5-year survival rate after a curative resection of carcinosarcoma gall bladder is 88.9 % when the invasion is restricted to the muscularis propria.[3,4]

References

Yan W, Xiaodong G, Zhenyang L, Jianbin X, Zongyou C. Gallbladder carcinosarcoma accompanied with bile duct tumor thrombi: A case report. Oncol Lett. 2013;5:1809-12.
Sung BP, Yang HK, Hye LR, Gi BC, Seong KH. Primary carcinosarcoma of the gallbladder. J Korean Surg Soc. 2012;82:54-58.
Al-Sheneber IF, Jaber T, Huttner I, Arseneau J, Loutfi A. Carcinosarcoma of the gallbladder: A case report and review of literature. Saudi J Gastroenterol 2002;8:22-4.
Huguet KL, Hughes CB, Hewitt WR. Gallbladder carcinosarcoma : a case report and literature review. J Gastrointest Surg. 2005;9:818-21.

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