B0071 - Primary Squamous Cell Biliary Carcinoma With Liver Metastasis Is Rare but Malicious

Mohamad Khaled Almujarkesh, MD¹, Anirudh R. Damughatla, DO¹, Hiba Zeid, MD², Dana LaBuda, BS³, Samer Alkassis, MD⁴, Zaid Kaloti, MD⁴, Mohammed Najeeb Al Hallak, MD, MS^5
1Wayne State University/Detroit Medical Center, Detroit, MI; ²Yale-Waterbury Internal Medicine Residency Program, Waterbury, CT; ³Wayne State University School of Medicine, Detroit, MI; ⁴Wayne State University/ Detroit Medical Center, Detroit, MI; ⁵Karmanos Cancer Institute, Detroit, MI

Introduction: Primary squamous cell biliary carcinoma (SCBC) with liver metastasis is quite rare, and to our knowledge, very few cases have been reported in the literature. The exact pathogenesis of the disease is unestablished; however, it’s believed that chronic inflammation predisposes to malignant transformation of squamous metaplasia in biliary glandular epithelium. We report a case of a middle-aged woman who was initially diagnosed with adenocarcinoma of the biliary tree that later transformed into SCBC.



Case Description/Methods: A 50-year-old woman with no prior medical history initially presented with postprandial epigastric & right upper quadrant pain. Her symptoms continued to worsen and were associated with early satiety, nausea, and weight loss of 25 pounds over two months, which prompted further evaluation by her primary care physician. CT and MRI (figure 1) examination a month later revealed a large heterogeneous area measuring 8.5 x 2.4 x 7.4 cm in the inferior right hepatic lobe with heterogeneous enhancement and involvement of the gallbladder concerning cholangiocarcinoma. Given radiographic findings, she underwent a CT-guided core biopsy of the liver, which showed a necrotic malignant tumor, favoring adenocarcinoma, and was also found to have germline BRCA mutated. PET scan revealed a large partially necrotic FDG avid mass possibly arising from the gallbladder fossa with an invasion of both lobes of the liver and probable involvement of a portion of the ascending colon. There was no gross evidence of distant metastatic disease. The patient underwent staging laparoscopy before initiating chemotherapy, and another biopsy was done, which returned in favor of squamous cell carcinoma, with immunohistochemical stains being positive for CK19, Ber-EP4, & P40; while negative for CK7, CK20, CDX-2, PAX-8, & Mucicarmine. The patient was started on platinum-based chemotherapy due to germline BRCA mutation. However, due to her poor performance status and recurrent cholangitis related to her cancer and chemotherapy, she could not stay on treatment for an adequate period of time to assess for a response.

Discussion: Primary SCBC remains an unexplored aggressive malignancy with a poor prognosis. Diagnosis can be challenging and requires high clinical suspicion due to the scarcity of specific laboratory workup. Pathological diagnosis remains the gold standard; however, it also carries its own challenges. Treatment is usually case-oriented, and definitive protocols have yet to be established.



Disclosures:


Mohamad Khaled Almujarkesh, MD¹, Anirudh R. Damughatla, DO¹, Hiba Zeid, MD², Dana LaBuda, BS³, Samer Alkassis, MD⁴, Zaid Kaloti, MD⁴, Mohammed Najeeb Al Hallak, MD, MS⁵. B0071 - Primary Squamous Cell Biliary Carcinoma With Liver Metastasis Is Rare but Malicious, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.