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A Case of Extramedullary Plasmacytoma of the Biliary Tract with a Poor Prognosis
Eiko Sakurai1, Kazunori Nakaoka2, Seiji Yamada1
1Department of Diagnostic Pathology, Graduate School of Medicine, Fujita Health University, 1-98, Dengakugakubo, Kutsukake-cho, Toyoake 470-1192, Japan.
Abstract:
Extramedullary plasmacytoma (EMP) is a rare disease consisting of the presence of monoclonal plasma cells in tissues other than the bone. Most EMPs are located in the head and neck region. We present an extremely rare case of an EMP originating from the biliary tract in a 76-year-old male. This is the fifth report of a primary EMP arising from the biliary tract. He was diagnosed with jaundice, and he was referred for an additional examination. Abdominal ultrasonography revealed a tumor in the gallbladder and bile ducts, and a bile duct biopsy was performed via endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The pathological and immunohistochemical examination revealed that the tumor was a plasmacytoma originating in the biliary tract. Although endoscopic biliary drainage was performed, the bile duct infection was not well controlled due to obstructive jaundice caused by the tumor. Furthermore, the bleeding from the tumor during chemotherapy was uncontrolled. Pancreaticoduodenectomy and cholecystectomy were performed to control the infection and bleeding. Although chemotherapy was continued after surgery, the tumor of the intrahepatic bile duct enlarged. He died seven months after the diagnosis because of the treatment-resistant tumor.
Insights
Extramedullary plasmacytoma (EMP), a rare plasma cell tumor outside bone, can occur in the biliary tract. This case highlights a treatment-resistant biliary EMP in a 76-year-old male, emphasizing diagnostic and management challenges.
Area of Science:
- Oncology
- Gastroenterology
- Pathology
Background:
- Extramedullary plasmacytoma (EMP) is a rare neoplastic proliferation of monoclonal plasma cells occurring outside the bone marrow.
- While most EMPs manifest in the head and neck, primary biliary tract involvement is exceptionally rare.
Observation:
- A 76-year-old male presented with jaundice, leading to the discovery of a biliary tract tumor.
- Diagnostic imaging revealed a gallbladder and bile duct tumor, confirmed as plasmacytoma via endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and immunohistochemistry.
- This represents the fifth reported case of primary extramedullary plasmacytoma originating in the biliary tract.
Findings:
- The patient experienced obstructive jaundice and uncontrolled bile duct infection despite endoscopic biliary drainage.
- Chemotherapy led to uncontrolled tumor bleeding, necessitating surgical intervention with pancreaticoduodenectomy and cholecystectomy.
- Despite aggressive treatment, the intrahepatic bile duct tumor progressed, leading to death seven months post-diagnosis due to treatment resistance.
Implications:
- This case underscores the diagnostic and therapeutic challenges posed by rare biliary tract malignancies like extramedullary plasmacytoma.
- Effective management strategies for biliary tract EMP require further investigation due to its rarity and aggressive nature.
- Early diagnosis and novel therapeutic approaches are crucial for improving outcomes in patients with this rare condition.
