Asymptomatic giant cell hepatitis: a subtype of post-infantile giant cell hepatitis?

Giant cell hepatitis in adults is considered a rapidly progressive and life-threatening disease, but there are few descriptions of a prolonged disease course. A 36-year-old Japanese man was referred to our hospital for further evaluation of abnormal liver function test results. Although asymptomatic, he had undergone follow-up for 9 years with these abnormalities. Because the cause of liver injury was not identified despite extensive noninvasive examinations, the patient underwent needle biopsy. He was finally diagnosed with post-infantile giant cell hepatitis (PIGCH) based on the presence of small numbers of giant multinucleated hepatocytes scattered primarily around the portal area. Necroinflammatory changes were very mild in the portal tracts and hepatic parenchyma. According to the histological findings as well as the accepted international diagnostic scoring system for autoimmune hepatitis (AIH), which is closely related to PIGCH, AIH was unlikely, although antinuclear antibody was positive at a titer of 1:160. The present case may describe an unknown subtype of PIGCH, characterized by insidious disease onset and progression with concurrent, mildly active underlying hepatitis, which is in contrast with the well-documented aggressive nature of PIGCH.

Progressive atrophy in a deformed liver as a contributor to sigmoid volvulus.

The effect of a prior defect on secondary liver atrophy is unknown. We describe a case of sigmoid volvulus that was facilitated by progressive atrophy in a deformed liver. A 75-year-old man with abdominal pain and fullness was referred to our hospital. Computed tomography (CT) revealed reduced left hepatic lobe volume and a whirl sign, characteristic of sigmoid volvulus. The sigmoid volvulus was successfully detorted with endoscopy. Retrospective evaluation of liver morphology on CT and magnetic resonance imaging showed that the portal vein at the liver hilum was denuded due to a parenchymal defect of the medial segment, with compression by the crossing artery. As pulse Doppler ultrasonography demonstrated reduced portal blood flow in the region where liver atrophy developed, compression of the denuded portal vein presumably facilitated secondary atrophy and contributed to sigmoid volvulus. The present case shows that a deformed liver itself can be a cause of secondary atrophy. Therefore, continued monitoring of liver morphology and evaluation of portal blood flow to predict liver atrophy may be required, when an individual with a partial liver defect is encountered.

Acute peri-myocarditis with an unusual initial manifestation of gallbladder edema and a profound eosinophilic surge during convalescence.

A 29-year-old man with fever and right upper quadrant pain was referred to our hospital. Ultrasonography revealed intense gallbladder edema and pericardial effusion. Despite no symptoms and signs related to heart failure, the patient was clinically suspected of cardiac dysfunction related to acute peri-myocarditis based on his symptoms of preceding fever and headache, pericardial effusion, positive troponin T value, elevated B-type natriuretic peptide level, and sequential changes on electrocardiography. With a profound eosinophilic surge (8,022/µL) during convalescence, acute peri-myocarditis and gallbladder edema resolved spontaneously. This case instructively shows that acute peri-myocarditis initially manifests with abdominal symptoms, mimicking acute acalculous cholecystitis. In addition, an extensive review of acute myocarditis cases with peripheral eosinophilia suggests that there is a subgroup characterized by a predilection for young and middle-aged men, concurrence of pericardial effusion, transient eosinophilic surge during convalescence, and favorable outcome.

Spontaneous Celiac and Splenic Artery Dissection.

Dissection of the splanchnic artery unrelated to an aortic lesion is extremely rare. We describe a patient with dissection of the celiac and splenic arteries causing splenic circulatory impairment. A 55-year-old Japanese man was referred to our hospital for left back pain that suddenly occurred 3 days previously and spread to the left flank. He had complicated sleep apnea syndrome well controlled with continuous positive airway pressure, and had been prophylactically taking aspirin (100 mg/day) because of asymptomatic cerebral lacunar infarcts. Contrast-enhanced computed tomography (CT) in the arterial phase revealed dissection from the celiac root extending to the entire splenic artery, the caliber of which was irregularly narrowed, causing malperfusion in the spleen. Because of hemodynamic stability and lack of impending sequelae, the patient was carefully observed with rest, strict blood pressure control, and aspirin administration. One month later, CT revealed restoration of the caliber of the dissected arteries and regression of the organizing false lumen, which confirmed the patient's recovery. Despite the extreme rarity or nonspecific symptoms, splanchnic artery dissection should be considered a potentially life-threatening emergency. This case supports the possible benefit of starting antithrombotic treatment early to prevent thrombotic sequelae such as organ infarction and aneurysmal formation.

Follicular lymphoma-related colitis resembling ulcerative colitis.

Among the various manifestations of colonic involvement in malignant lymphomas, an ulcerative colitis-like appearance is rare. Herein, we describe a case in which extranodal colonic involvement resembled ulcerative colitis in a patient with advanced follicular lymphoma. A 59-year-old Japanese man with diarrhea and body weight loss was referred to our hospital. Computed tomography (CT) revealed systemic lymphadenopathy, splenomegaly, and thickening of the sigmoid colon and rectum walls. 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET/CT) revealed intense tubular 18F-FDG accumulation extending from the rectum to the colon at the hepatic flexure and much weaker accumulation in the systemic lymph nodes, bone marrow, and spleen. The isotope-enriched areas had an ulcerative colitis-like appearance as shown via colonoscopy. The patient was ultimately diagnosed with follicular lymphoma (stage IV A, grade 1) based on a pathological examination of the neck lymph nodes, iliac bone marrow, and colon. After six courses of chemotherapy (R-CHOP), 18F-FDG-PET/CT confirmed complete remission of the lymphoma including the colonic lesion. This is presumably the first case of ulcerative-like colitis caused by a follicular lymphoma. As a novel approach, the lymphoma-related colitis was detected by comparing the pathology results and the 18F-FDG-PET/CT results.

Achilles tendinitis as a rare extraintestinal manifestation of ulcerative colitis.

Patients with inflammatory bowel disease often have extraintestinal manifestations (EIMs) involving almost all organ systems, but little has been reported on Achilles tendinitis. Herein, we present a unique case of Achilles tendinitis, which manifested shortly after initiation of mesalazine therapy for ulcerative colitis. A 26-year-old Japanese woman with bloody diarrhea and abdominal cramps lasting for 7 days was referred to our hospital. The Lichtiger clinical activity index (CAI) score was 9 at the first visit. Based on the clinical symptoms and examination results, she was diagnosed with ulcerative pancolitis in the active phase, and treatment with mesalazine (2.4 g/day) and probiotics was initiated. Her symptoms resolved within 7 days of treatment (CAI 3). However, she then developed bilateral Achilles tendinitis without any apparent cause. The Achilles tendinitis subsided with conservative management within 2 weeks, despite continuation of mesalazine therapy. This case instructively suggests that Achilles tendinitis should be noted as an EIM of ulcerative colitis.

Imaging of acquired cystic disease-associated renal cell carcinoma by contrast-enhanced ultrasonography with perflubutane microbubbles and positron emission tomography-computed tomography.

The preoperative assessment of renal cell carcinoma (RCC) complicated with acquired renal cystic disease in a 63-year-old male patient on long-term hemodialysis (30 years and 8 months) that was difficult because of no or poor contrast enhancement by dynamic CT scan is reported. Contrast-enhanced ultrasonography with perflubutane microbubbles and positron emission tomography-computed tomography (PET-CT) with 18F-fluorodeoxy glucose (FDG) in addition to dynamic CT were effective and useful for preoperative assessment of this patient. The pathological subtype of RCC in this patient was acquired cystic disease-associated RCC (ACD-associated RCC), which has been newly defined by Tickoo et al. (Am J Surg Pathol 30:141-153, 2006).

Primary localized malignant biphasic mesothelioma of the liver in a patient with asbestosis.

We report a case of primary localized malignant biphasic mesothelioma of the liver in a 66-year-old man associated with asbestosis. The tumor was detected as a hepatic nodule, 4 cm in diameter, in the right lobe (S8 segment) on CT scan. Histopathological examination demonstrated an intrahepatic tumor with central necrosis consisting of a papillary epithelioid pattern on the surface of the liver, microcystic (microglandular or adenomatoid) pattern mainly in the subcapsular area and sarcomatoid pattern intermingled with microcystic pattern in the major part of the hepatic nodular tumor. Tumor cells, especially of epithelioid type, showed distinct immunoreactivity for mesothelial markers (WT-1, calretinin, D2-40, CK5/6, mesothelin, thrombomodulin) and no immunoreactivity for epithelial (adenocarcinoma) markers (CEA, CD15, BerEP4, BG8, MOC31). P53 immunoreactivity was detected focally in papillary epithelioid tumor cells and extensively in microcystic and sarcomatoid components, suggesting that the papillary epithelioid mesothelioma arose on the surface of the liver, and tumor cells showing microcystic and sarcomatoid patterns invaded and grew into the liver. To date, this is the first case of primary localized malignant biphasic mesothelioma of the liver, since all three primary hepatic mesotheliomas reported so far were epithelioid type.