Hepatic sarcoidosis mimicking hilar cholangiocarcinoma: case report and review of the literature.

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Hepatic involvement was reported in about 11% of patients with sarcoidosis. However, cases of sarcoidosis in which the granuloma is solitary and limited in the liver are very rare. A 51-year-old woman with tumors in the liver underwent extended left lobectomy with caudate lobectomy and bile duct resection. The tumor was located between segment 4 and the hilar region. Some daughter nodules were found in the left lobe, which were regarded as intrahepatic metastasis. Our case displayed clinical and radiologically distinct findings, which are very similar to those of hilar cholangiocarcinoma restricted to the liver. This report demonstrates that sarcoidosis can show solitary hepatic involvement in the absence of thoracic lymphadenopathy. In such a case, it is difficult to distinguish the diagnosis from other malignant neoplasms. In conclusion, the diagnosis of hepatic sarcoidosis has to be made through prudent and comprehensive investigations that include a full clinical history of sarcoidosis in other organs. Despite utilizing several detailed diagnostic modalities, the definitive diagnosis of cases of solitary sarcoidosis may remain difficult. In these cases, surgical treatment including liver resection should be considered in order to avoid missing a suitable opportunity for treatment.

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.

Treatment of patients with unresectable advanced carcinoma of biliary tract - chemotherapy and surgical resection.

BACKGROUND: The results of 12 consecutive patients with unresectable advanced biliary tract carcinoma treated with first line chemotherapy of S1/cisplatin, combined surgical resection and second line chemotherapy of gemcitabine are evaluated. PATIENTS AND METHODS: Eight patients with intrahepatic cholangiocarcinoma, 1 with extrahepatic cholangiocarcinoma and 3 with gallbladder carcinoma were included in the study. All patients were treated with S1/cisplatin. Two of the patients underwent combined surgical resection before and 2 after therapy. Second line chemotherapy of gemcitabine was administered in 6 patients. RESULTS: MST of the patients was 14.9 months. With S1/cisplatin therapy, 6 patients had PR and 4 had SD. Two patients with surgical resection after the therapy survived more than 3 years. Second line chemotherapy of gemcitabine with moderate effects and mild adverse effects was well tolerable. CONCLUSION: S1/cisplatin showed considerable anti-cancerous effects. Employing surgical resection for patients with good response may lead to the chance of long-term survival.

Hepatocellular carcinoma with extensive peliotic change.

Peliosis hepatis is a rare lesion histologically characterized by multiple cavities representing dilated sinusoids filled with blood in the liver. Although it has been observed in the liver parenchyma in association with several diseases and medications, there are few reports of nodules of hepatocellular carcinoma (HCC) showing extensive peliotic change. We describe a case of HCC showing extensive peliotic change in the cancer nodule. A 73-year-old man with a liver tumor was referred to our hospital for further investigation. Abdominal ultrasonography revealed an 8-cm hyperechoic lesion with a halo and mosaic pattern in segment 8 (S8) of the liver. Dynamic magnetic resonance imaging of the liver showed early irregular enhancement of the peripheral part of the lesion, and the effect persisted into the late phase, spreading into the central part of the nodule. Hepatic arteriography showed the "cotton-wool" sign, usually observed in cavernous hemangiomas. Fine-needle aspiration biopsy revealed the diagnosis of HCC. Anterior sectionectomy of the liver was conducted. Histological examination of the resected specimen showed that the tumor was a well-differentiated HCC with extensive dilated sinusoid-like structures in the main portion of the nodule, suggestive of peliotic change.

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 Glisson's 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.

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.

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.