Tadashi Kagawa

Surgery and chemotherapy for intrahepatic cholangiocarcinoma.

Cholangiocarcinoma, arising from bile duct epithelium, is categorized into intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC), including hilarcholangiocarcinoma. Recently, there has been a worldwide increase in the incidence and mortality from ICC. Complete surgical resection is the only approach to cure the patients with ICC. However, locoregional extension of these tumors is usually advanced with intrahepatic and lymph-node metastases at the time of diagnosis. Resectability rates are quite low and variable (18%-70%). The five-year survival rate after surgical resection was reported to be 20%-40%. Median survival time after ICC resection was 12-37.4 mo. Only a small number of ICC cases, accompanied with ECC, gall bladder carcinoma, and ampullary carcinoma, have been reported in the studies of chemotherapy due to the rarity of the disease. However, in some reports, significant anti-cancer effects were achieved with a response rate of up to 40% and a median survival of one year. Although recurrence rate after hepatectomy is high for the patients with ICC, the residual liver and the lung are the main sites of recurrence after tentative curative surgical resection. Several patients in our study had a long-term survival with repeated surgery and chemotherapy. Repeated surgery, combined with new effective regimens of chemotherapy, could benefit the survival of ICC patients.

Plexiform neurofibroma in the hepatic hilum associated with neurofibromatosis type 1: a case report

We present an extremely rare case of plexiform neurofibroma involving the hepatic hilum. A 24-year old woman who had been diagnosed with neurofibromatosis type 1 was referred to our hospital for evaluation of an abdominal mass found on computed tomography and progressive aggravation of intermittent abdominal pain. Abdominal computed tomography revealed a multilobulated non-enhancing mass involving the celiac trunk and hepatic artery, that extended to the hepatic hilum through the hepatoduodenal ligament. Magnetic resonance imaging showed the lesion extending along the intrahepatic Glissons sheath. Based on the imaging findings, the patient was diagnosed to have a neu-rofibroma, although sarcomatous differentiation could not be excluded. The tumor was resected, leaving behind the intrahepatic extension, with the aim of alleviating the abdominal pain and preventing obstructive jaundice. Histopathological examination revealed the diagnosis of plexiform neurofibroma. At present, three years after the surgery, the patient remains symptom-free, without any evidence of recurrence.

Patient with advanced intrahepatic cholangiocarcinoma with long-term survival successfully treated with a combination of surgery and chemotherapy

Intrahepatic cholangiocarcinoma (ICC) is a relatively rare malignancy arising from the biliary epithelium. Prognosis is typically poor. Currently, aggressive surgical resection is the only treatment modality that offers patients any chance of long-term survival. Here, we present the case of a 57-year-old woman in whom we diagnosed ICC, with the tumor occupying the entire left and caudate hepatic lobes and daughter nodules in the right lobe. She underwent hepatectomy of segments I to VI, combined with intraoperative microwave coagulation therapy for nodules in the residual liver. Three months after the surgical resection, she had recurrence of the disease. The patient subsequently received weekly intraarterial chemotherapy with irinotecan (CPT-11), and a partial response was observed which persisted for 18 months. Subsequent computed tomography revealed the regrowth of three tumors, and she therefore underwent a repeat resection 24 months after the first surgical operation. In postoperative-month (POM) 32, she received systemic chemotherapy with tegatur/gimeracil/oteracil potassium (S-1)/cisplatin for multiple small nodules in her lung. Following three cycles of chemotherapy with a stable disease response, partial resections of the lung were performed. Third and fourth hepatectomies were performed in POMs 46 and 59, respectively. Five years and 5 months after the first hepatectomy, she is alive with small lesions in her lung. This multimodal approach may be effective for ICC.

Malignant Triton tumor in the retroperitoneal space associated with neurofibromatosis type 1: a case study

We report an extremely rare case of malignant Triton tumor developing in the retroperitoneal space in a patient with neurofibromatosis type 1. A 21-year old man who had been diagnosed with neurofibromatosis type 1 was admitted to our hospital with the chief complaint of a palpable abdominal mass. Abdominal computed tomography revealed a huge heterogeneous tumor measuring approximately 17 cm in diameter occupying the left retroperitoneal space, and numerous metastatic lesions between the left psoas muscle and the left thigh with dissolution of the left hip joint. After the diagnosis of a retroperitoneal malignant neurogenic tumor, resection of the tumor with reconstruction of the abdominal aorta was conducted, followed by postoperative transarterial infusion chemotherapy. The histopathological diagnosis was malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation, namely malignant Triton tumor. Postoperative chemotherapy was in vain and the patient died 14 months after the surgery as a result of lung metastasis.