Katsura Hikotaro, Hori Tomohide, Yamamoto Hidekazu, Harada Hideki, Yamamoto Michihiro, Yamada Masahiro, Yazawa Takefumi, Sasaki Ben, Tani Masaki, Sato Asahi, Kamada Yasuyuki, Tani Ryotaro, Aoyama Ryuhei, Sasaki Yudai, Zaima Masazumi
Department of Surgery, Shiga General Hospital, Moriyama, 5-4-30 Moriyama, Moriyama, Shiga 524-8524, Japan.
Department of Surgery, Shiga General Hospital, Moriyama, 5-4-30 Moriyama, Moriyama, Shiga 524-8524, Japan.
Int J Surg Case Rep. 2021 May;82:105936. doi: 10.1016/j.ijscr.2021.105936. Epub 2021 Apr 30.
The most common liver malignancies are hepatocellular carcinoma, intrahepatic cholangiocarcinoma, and metastatic tumors. Hepatocellular carcinoma and intrahepatic cholangiocarcinoma may invade the portal vein (PV). An association between diffuse large B-cell lymphoma (DLBCL) and primary biliary cholangitis (PBC) remains unclear. We herein report a thought-provoking case of a difficult-to-diagnose liver tumor with PV thrombosis in a PBC patient.
A 66-year-old woman had PBC, systemic sclerosis, diabetes, and osteoporosis. A solitary liver tumor accompanied by macrovascular thrombosis in the PV was detected incidentally. Based on dynamic imaging findings, we considered the tumor to be intrahepatic cholangiocarcinoma, and right lobectomy with lymphadenectomy was performed. Unexpectedly, pathological assessment made a definitive diagnosis of DLBCL that did not invade the vessels and bile duct. In fluorine-18-fluorodeoxyglucose positron emission tomography, abnormal accumulations were clearly observed in the breast tissue and peritracheal, parasternal, mediastinal, and pericardial lymph nodes. The patient achieved complete remission after systemic chemotherapy, and there has been no recurrence 3 years after surgery.
Primary lymphoma in the liver is rare, and we did not consider our patient's tumor as primary liver lymphoma. Our case actually showed no tumor thrombosis in the PV. Although autoimmune disorders may increase the risk of non-Hodgkin's lymphoma, an association between DLBCL and PBC is still unclear, and we must remember that DLBCL may develop rarely in a PBC patient.
Our case report provides a timely reminder for clinicians and surgeons in the fields of hepatology and hematology.
最常见的肝脏恶性肿瘤是肝细胞癌、肝内胆管癌和转移性肿瘤。肝细胞癌和肝内胆管癌可能侵犯门静脉（PV）。弥漫性大B细胞淋巴瘤（DLBCL）与原发性胆汁性胆管炎（PBC）之间的关联仍不明确。我们在此报告一例发人深省的病例，一名PBC患者出现难以诊断的肝脏肿瘤并伴有PV血栓形成。
一名66岁女性患有PBC、系统性硬化症、糖尿病和骨质疏松症。偶然发现一个孤立的肝脏肿瘤并伴有PV大血管血栓形成。基于动态影像学表现，我们认为该肿瘤为肝内胆管癌，并进行了右叶切除术及淋巴结清扫术。出乎意料的是，病理评估明确诊断为DLBCL，该肿瘤未侵犯血管和胆管。在氟-18-氟脱氧葡萄糖正电子发射断层扫描中，在乳腺组织以及气管周围、胸骨旁、纵隔和心包淋巴结中清晰观察到异常积聚。患者在全身化疗后实现完全缓解，术后3年无复发。
肝脏原发性淋巴瘤罕见，我们未将该患者的肿瘤视为原发性肝脏淋巴瘤。我们的病例实际上在PV中未显示肿瘤血栓形成。虽然自身免疫性疾病可能增加非霍奇金淋巴瘤的风险，但DLBCL与PBC之间的关联仍不明确，我们必须记住DLBCL在PBC患者中可能很少发生。
我们的病例报告为肝病学和血液学领域的临床医生和外科医生提供了及时的提醒。
