A case of simple type Caroli's disease confined to one segment of the liver / 대한내과학회지

Caroli's disease is a rare congenital anomaly of the intrahepatic biliary tree characterized by multiple, saccular and cystic dilatation of the bile ducts that are connected with the biliary drainage system. It is frequently associated with congenital hepatic fibrosis, resulting in portal hypertension. The complications are stone formation, recurrent cholangitis and occasionally cancer. Less than 20% of all cases reported describe monolobar or segmental invovement. In the pure form or simple type, the patient presents without hepatic fibrosis or portal hypertension. We report a case of simple type Caroli's disease confined to the one hepatic segment in a 22 year-old woman. She was admitted because of cystic mass, associated with right upper abdominal pain. Radiologic findings including abdominal ultrasonography, computerized tomography, magnetic resonance imaging demonstrated that the cystic lesions involving segment VIII of the liver. The lesion was treated with hepatic segmentectomy.

Extrahepatic Metastasis of Hepatocellular Carcinoma to the Nasal Cavity Manifested as Massive Epistaxis: A Case Report / 대한간학회지

Extrahepatic metastasis of hepatocellular carcinoma (HCC) is not infrequently found during the later stage, regarding that the autopsy report described its prevalence to be up to 50%. The most frequent sites are known to be the abdominal lymph nodes, lung and bone. However, metastasis to the nasal cavity and paranasal sinuses has been seldom reported, and to out knowledge, there is no Korean report describing extrahepatic metastasis of HCC to these sites. Recently we experienced a case of extrahepatic metastasis of HCC to the nasal cavity in a 50 year-old man with massive epistaxis refractory to conservative treatment. He was found to have a mass of soft tissue attenuation occupying the right nasal cavity at CT, which was biopsy-proven as metastatic HCC. Epistaxis was successfully treated by transcatheter arterial embolization.

A Case of Primary Leiomyosarcoma of the Inferior Vena Cava Treated by Surgical Resection and Reconstruction with Synthetic Graft Replacement / 대한소화기학회지

Leimyosarcoma of the inferior vena cava (IVC) is a rare tumor, with about 200 cases reported in the world literature. There were 5 cases reported in Korea. We report a case of primary leiomyosarcoma of the inferior vena cava in a 50 year-old woman who presented with a heterogenous enhancing mass within IVC on CT scan and MRI scan. The tumor expanded the IVC with displacement of the adjacent structures. The patient underwent ultrasound guided percutaneous biopsy which was consistent with leiomyosarcoma. The patient was treated by surgical resection and synthetic graft replacement of the inferior vena cava and postoperative external beam radiation therapy. We report the case of leiomyosarcoma of the inferior vena cava with a review of relevant literatures.

Implications of Serum Levels of Basic Fibroblast Growth Factor and Vascular Endothelial Growth Factor in Chronic Liver Diseases and Hepatocellular Carcinoma / 대한간학회지

BACKGROUND/AIMS: Angiogenesis occurs in response to tissue damage, and is of vital importance for tumor growth and metastasis. Basic fibroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF) are potent angiogenic factors, and have been suggested to be useful diagnostic markers in certain hypervascular tumors. However, little is known of serum bFGF and VEGF in patients with hepatocellular carcinoma (HCC). We attempted to measure serum bFGF and VEGF in patients with chronic liver diseases (CLD) and HCC to assess their pathogenetic role and usability as tumor markers. METHODS: Serum bFGF and VEGF were measured in 8 patients with chronic hepatitis (CH), 15 patients with liver cirrhosis (LC), and 49 patients with HCC. bFGF was measured in 33, and VEGF was measured in 50, healthy blood donors. RESULTS: Serum bFGF was 3.8+/-1.9, 2.0+/-1.4, 4.2+/-6.0, 17.4+/-30.0 pg/mL in normal control, CH, LC, HCC, respectively. The serum bFGF level was significantly increased in patients with HCC when compared with normal control or patients with CLD. No difference, however, was observed in serum VEGF levels among the four groups. The serum levels of bFGF and VEGF were not significantly different in patients with HCC according to tumor type, size and stage. Serum bFGF showed good sensitivity (90%), specificity (87%), and positive predictive value (94%) in differentiating patients with HCC from those with CLD at the cut-off value of 4.6 pg/mL. CONCLUSIONS: bFGF might play a role in the growth of HCC and its serum level might be used as a tumor marker. On the other hand, serum VEGF does not seem to be an adequate tumor marker.

Retrospective Analysis of Treatment Outcome and Prognostic Factors in Multiple Myeloma / 대한혈액학회지

BACKGROUND: The prognostic outlook for multiple myeloma has markedly improved in recent decades, which is probably related to the introduction of chemotherapy and the development of supportive care for various complications. In the present work we analysed retrospectively the therapeutic outcomes of newly diagnosed patients with multiple myeloma treated at Korea Cancer Center Hospital (KCCH). And we studied to identify prognostic factors influencing the therapeutic outcome of the disease. METHODS: Between January 1987 and December 1998, eighty three patients were diagnosed as multiple myeloma by the criteria of Southwestern Oncology Group at KCCH. Of these patients, clinical analysis was performed retrospectively for sixty-one patients who were treated with combination chemotherapy. RESULTS: The median age at diagnosis was 55 years of age, which was lower than that of western countries and 48% of patients were in their 4th decade. Male to female ratio was 1: 1.1. The main chief complaint at diagnosis was bone pain. Ninety-one percent of the patients were clinical stage III. Serum immuno-electrophoresis revealed M-protein as IgG in 41%. The ratio of micro to lambda light chain was 1.5:1. The response rate to initial chemotherapy was 75% (95% C. I.=63.4~86.6%) and median progression free interval was 20.9 months. The median overall survival of total 61 patients was 28.5 months. The patients' age at diagnosis and the response to initial chemotherapy were statistically significant prognostic factors influencing the overall survival of patients. CONCLUSION: This study showed that we could get relatively high response rate with conventional chemotherapy for the patients with multiple myeloma, but, that most patients eventually progressed and the cure was nearly impossible. To obtain cure or to have much longer survival time, we need more delicate strategies including more intensive chemotherapy with stem cell transplantation for the treatment of multiple myeloma patients.

A Case of Idiopathic Mediastinal Fibrosis Presenting with Vocal Cord Palsy / 결핵

An idiopathic mediastinal fibrosis is a rare disease with an unknown etiology. It is a benign condition in which a fibrosis of the soft tissue and chronic inflammation occurs within the mediastinum. This leads to a constriction and obliteration of the adjacent mediastinal structures, particularly the great veins. This can result in a variety of clinical conditions depending on the anatomic location of the disease. Here, we report a case of an idiopathic mediastinal fibrosis with vocal cord palsy, which was confirmed by a biopsy with a thoracotomy. Postoperative medical treatment using prednisolone and tranilast was performed.

Necessity and Safety of Fine-needle Aspiration Cytology for Diagnosis of Hepatocellular Carcinoma / 대한간학회지

BACKGROUNDS/AIMS: The fine-needle aspiration (FNA) is a useful method for diagnosis of hepatocellular carcinoma (HCC). The aims of our study are to assess diagnostic accuracy of FNA, to define proper indications of FNA for diagnosis of HCC, and to evaluate the complications of FNA. SUBJECTS AND METHODS: To assess diagnostic accuracy we compared the results of preoperative FNA with postoperative pathology in 38 resected cases with primary liver cancer. To define proper indications and complications of FNA, we prospectively followed 138 patients received FNA for their liver tumors which were suspicious of primary liver tumor. RESULTS: The sensitivity, specificity, positive and negative predictive values of FNA were 100%, 97%, 100% and 66% respectively. All patients with serum alpha-fetoprotein (AFP) level over 1000 ng/ml were having HCC on FNA result. Among 36 patients with AFP level ranged 15-1000 ng/ml and hypervascular mass on angiography, 96% were having HCC. Among 50 patients with normal AFP level and hypervascular mass on angiography, 92% were having HCC. The major complications after FNA such as hemoperitoneum, pneumothorax, and iatrogenic arterioportal shunt developed in 2%, 2%, and 7% of subjects, respectively. We did not find any case of needle-tract seeding of cancer during a mean 4.7 months of follow-up. CONCLUSIONS: Although the FNA is an accurate method for diagnosis of HCC, FNA was usually not indicated for patients with serum AFP level over 1000 ng/ml or patients with hypervascular mass on angiography when they were suspected of having primary liver cancer. Major complications were hemoperitoneum, pneumothorax and iatrogenic arterioportal shunt. Iatrogenic arterioportal shunt may influence the efficacy of subsequent transcatheter arterial embolization.