Segment Of Right Hepatic Lobe Research Articles

SAT-073 A Case of Williams Syndrome Associated with Aldosterone Producing Adrenal Adenoma Mimicking Hepatic Adrenal Rest Tumor

Background: Williams syndrome (WS) is a microdeletion disorder caused by heterozygous loss of approximately 1.5-Mb pairs of DNA from chromosome 7. Patients with WS have a characteristic constellation of medical and cognitive findings, with a hallmark feature of generalized arteriopathy presenting as stenoses of elastic arteries and hypertension. We present here a case of WS associated with primary aldosteronism (PA), whose source of aldosterone excess was difficult to locate. Clinical case: A 31-year-old man suspected having PA was referred to our Hospital. He was diagnosed as having Williams syndrome at 2 months of age. He had been hypertensive for 9 years. At the age of 31 years old, low serum potassium (K) and high aldosterone to renin ratio were pointed out. His plasma renin activity (PRA) was 0.2 ng/ml/h, plasma aldosterone concentration (PAC) was 456 pg/ml, serum K level was 3.1 mEq/l at admission. Diagnosis of PA was confirmed according to positive captopril challenge test. CT scan revealed a 13mm tumor with low density in posterosuperior segment of right hepatic lobe, but adrenal tumor was not detected. Venography of adrenal vein sampling (AVS) showed an extended tributary of the right adrenal vein to the liver that surrounded the tumor. Segmental AVS was performed for right adrenal vein and showed a high value of PAC (377000 pg/ml) in superior tributary which was drained from the tumor. PAC in other tributaries of right adrenal vein and left adrenal vein was suppressed. Laparoscopic partial hepatectomy was performed as a liver tumor. After surgery, serum K level was normalized, PAC decreased to 67 pg/ml. Pathological examination revealed a golden-yellow tumor separated from the liver by fibrous septum. The tumor was attached to an adrenal cortex embedded in the liver. The tumor was composed of clear cells, positive for 3βHSD2 and CYP11B2 using immunohistochemistry, diagnosed as aldosterone producing adrenal adenoma. Conclusion: To the best of our knowledge, this is the first report of WS associated with PA. Since WS is a disease with high prevalence of hypertension, measurement of PRA and PAC for screening of PA is recommended for patients with WS associated with hypertension. According to CT findings, hepatic adrenal rest tumor was suspected because the tumor was not continuous with the right adrenal gland, but pathological findings demonstrated that an adrenal adenoma was originated from right adrenal cortex rather embedded in the liver. Segmental AVS was useful to distinguish adrenal tumor from hepatic adrenal rest tumor.

Eosinophilic Cholangiopathy: A Case Report

Purpose: A 56-year-old African-American man presented with a 1-week history of fatigue, nausea and vomiting, scleral icterus, light stools, and dark urine. He denied abdominal pain, fever, chills, or recent weight loss. Past medical history was significant for hypertension, asthma, and questionable lupus. He did not use alcohol, tobacco, or drugs, and had no recent travel events. His physical exam was relatively unremarkable aside from icterus. His white blood cell count was 51,000/mm3, with 82% eosinophils. Liver function tests were significant for an albumin of 2.4g/dL, total protein 8.7 g/dL, total bilirubin 15.3mg/dL, aspartate aminotransferase 248 IU/L, alanine aminotransferase 284 IU/L, alkaline phosphatase 845 IU/L, and lipase 322 IU/L. His INR was 1.35. CT of the abdomen showed a 2.4 cm x 1.5 cm enhancing lesion in the posterior segment of right hepatic lobe. Magnetic resonance cholangiopancreatography had non-visualization of a 1-cm segment of anterior branch of the right hepatic duct with mild dilation. A liver biopsy revealed marked portal mixed inflammation with numerous eosinophils, bile duct proliferation, and edematous fibrous stoma consistent with an inflammatory process and bile duct obstruction. No malignancy was seen. Endoscopic retrograde cholangiopancreatography revealed intra-hepatic duct beading with pruning, consistent with primary sclerosing cholangitis (PSC) or autoimmune cholangiopathy, with no extra-hepatic duct filling defects. Common bile duct brushings were negative for malignancy. A broad differential of infectious, allergic, and auto-immune causes were ruled out. Endoscopy did not reveal a GI malignancy. Bone marrow biopsy revealed hypercellular (˜70%) marrow with marked eosinophilia (˜36% of marrow cellularity), but no evidence of neoplastic causes. The patient remained stable throughout his hospitalization. He was discharged from the hospital with an indeterminate origin of hypereosinophilia. He was started on 60 mg oral prednisone daily, and had normalization of his symptoms and laboratory values within 2 months. This case illustrates the rare clinical entity of hypereosinophilia causing eosinophilic cholangiopathy. Clinical features were suggestive of a variety of disease processes, including primary sclerosing cholangitis (PSC), idiopathic hypereosinophilia syndrome (HES), and auto-immune cholangiopathy. Based upon literature review and further workup, this case likely represents a rare cholestatic manifestation of HES caused by eosinophilic infiltration of the biliary system. Treatment with steroids was both diagnostic and therapeutic.

Laparoscopic Anatomic Monosegmentectomy of Hepatocellular Carcinoma of the Right Hepatic Lobe

Laparoscopic resection of lesions at right hepatic lobe is considered difficult. The objective of this study is to describe our institutional techniques of laparoscopic anatomic monosegment resection of hepatocellular carcinoma (HCC) in all segments of the right lobe. Patients with deep-seated solitary tumors in a single segment of right hepatic lobe were considered for laparoscopic anatomic monosegment resection when the planned transection plane could give a 1-cm resection margin from the adjacent nontumour segments. The purpose of this surgery design is to achieve an oncologic resection while preserving the maximal volume of liver parenchyma. Five patients underwent successful laparoscopic anatomic monosegmentectomy with no conversion to open. They were all male with a solitary HCC size ranging from 2 to 3 cm. Cirrhosis was present in 2 (40%) patients. The mean blood loss was 676 mL (range, 280 to 1200 mL) and perioperative blood transfusion was not required. There was no operative mortality or morbidity, and no recurrences were noted. Laparoscopic monosegmentectomy is feasible and safe in selected patients with HCC. It provides the benefit of maximal preservation of nontumour liver parenchyma, while oncologic resection is secured.

Cerebral Lipiodol Embolism after Transcatheter Arterial Chemoembolization of Hepatocellular Carcinoma

Transcatheter arterial chemoembolization (TACE) is the mainstay of treatment for unresectable hepatocellular carcinoma (HCC). Although various complications of TACE have been reported, cerebral lipiodol embolism after TACE is rare. We report a 67-year-old man, who had patent foramen ovale and developed cerebral lipiodol embolism after TACE via the inferior phrenic artery. At 20 months after third TACE of 3 cm sized HCC in the left hepatic lobe, computed tomography (CT) revealed about 1.6 cm newly developed HCC in the anterior superior segment of right hepatic lobe. The angiogram revealed the HCC was supplied from the right inferior phrenic artery. Toward the end of TACE, there were accumulations of the iodized oil in the pulmonary vasculature. Immediately after TACE, he complained of weakness in right upper and lower limbs and sensory decrease in right limbs and right hemitrunk. Magnetic resonance imaging revealed a cerebral lipiodol embolism. Transesophageal echocardiography revealed no visible thrombi but contrast-echocardiography using hand agitated saline revealed an intracardiac right to left shunt consistent with patent foramen ovale. Motor weakness and sensory decrease were gradually improved, and all neurological symptoms disappeared over 4 weeks.

GIANT HEPATOCELLULAR CARCINOMA WITH SPECIFIC METASTASIS IN THE LNER

This is very interesting case of giant hepatocellular carcinoma that has multiple metastases with grape-like shape of 1 to 2cm in the inferior segment of right hepatic lobe, but no metastasis in left hepatic lobe. A 53-year-old female complained of abdominal tumor and abdominal distention. She had a status of central obesity like Baffalo hump, and an apparent elevation of serum cortisol concentration was seen (30.2μg/dl). And when we performed the extended right hepatic lobectomy including giant tumor, serum cortisol concentration returned to normality immediately. After the operation, a gradual weight-loss seen and she returned to standard shape for a year. This operation was a new method of liver resection. Having ligated the right hepatic artery, right hepatic lobectomy was performed without maneuver of the liver. The resected liver including metastatic tumor weighed 2700gr, and the volume of perioperative bleeding was 2750ml. The specimen showed that the main tumor consisting of an elastic soft and yellowish-white tissue, measured 15×13×12cm, and circumscribed multiple metastases in right inferior segment of the liver. Microscopically the tumor was characterized by slight dysplastic cells, producing bile juice. Histological diagnosis was Hepatocellular carcinoma, Edmondson II type. The patient has been well and no replase has been observed yet for 13 months since the operation.