Hepatic mucosa-associated lymphoid tissue lymphoma and hepatocellular carcinoma in a patient with hepatitis B virus infection

Primary hepatic mucosa-associated lymphoid tissue (MALT) lymphoma is an extremely rare disease. A 65-year-old female patient with chronic hepatitis B presented with multiple solid masses in segment (S) 4, S5, and S6 of the liver. The nodule in S5 was diagnosed preoperatively as hepatocellular carcinoma by computed tomography, magnetic resonance imaging, and angiography. The nodule in S4 was initially interpreted as lymphoid follicles by needle biopsy. Segmentectomy of S5 and partial resection of S6 were performed. Microscopic examination of the S5 nodule revealed moderately differentiated hepatocellular carcinoma. The nodule from S6 showed nodular proliferation of atypical intermediate to medium-sized lymphoid cells in the portal area and lymph epithelial lesions of bile ducts. The atypical lymphoid cells were positive for LCA, L-26 and bcl-2 and negative for UCHL-1. These features were consistent with the diagnosis of MALT lymphoma. This is the first case report of synchronous hepatic MALT lymphoma and hepatocellular carcinoma associated with chronic hepatitis B.     

Polymorphic lymphoproliferative disorders in patients with rheumatoid arthritis are associated with a better clinical outcome

Objectives: The characteristics of lymphoproliferative disorders (LPD) in patients with rheumatoid arthritis (RA) remain unclear. Therefore, we retrospectively analyzed the clinical characteristics of these patients in our department.

Methods: Twenty RA patients who developed LPD between April 2003 and August 2016 in our department were analyzed.

Results: All of the RA patients who developed LPD had been treated with methotrexate (MTX). The median weekly and total dosages of MTX were 6.8?mg/week and 2530?mg, respectively. The median duration of MTX administration was eight years. Nineteen patients (95%) achieved complete remission (CR) and 15 (75%) achieved CR with MTX cessation alone. Based on the pathological findings, we divided MTX-associated LPD patients into two groups (n?=?16); polymorphic LPD (31%) and other groups. CR with MTX cessation alone was achieved in 5 (100%) and 6 (54.5%) patients in the polymorphic LPD and other groups, respectively (p?=?.12). Moreover, the duration from the cessation of MTX to CR was significantly shorter in the polymorphic LPD group than in the other group (5.3 months vs 12.6 months, p?=?.01, respectively).

Conclusion: Polymorphic LPD, which was the most frequent pathological diagnosis in this cohort, was associated with a higher incidence of CR and a significantly shorter duration to CR.     

Junctophilins: molecular components contributing junctional membrane complexes between the cell-surface membrane and endoplasmic/sarcoplasmic reticulum

The junctional membrane complex between the plasma membrane (PM) and endoplasmic/sarcoplasmic reticulum (ER/SR) is an important structural foundation for functional crosstalk between ionic channels. In cardiac myocytes, functional coupling between cell-surface and intracellular Ca(2+) channels produces Ca(2+) transients for contraction. Junctophilins, a novel family of junctional membrane complex proteins, contribute to the stabilization of the junctional membrane complex by anchoring the ER/SR and interacting with the PM. Mutant mice lacking the cardiac-type junctophilin exhibited embryonic lethality due to heart failure, and the mutant cardiac myocytes showed deficiency of the junctional membrane complexes and abnormal Ca(2+) signaling.     

GASTRIC SUBMUCOSAL HETEROTOPIA OF THE IMMATURE GASTRIC GLANDS WITH ENDOSCOPIC FEATURE MIMICKING CARCINOID TUMOR

We describe a rare case of gastric submucosal heterotopia of the immature gastric glands mimicking carcinoid tumor shown by endoscopic examination, which was successfully treated by endoscopic tumor resection. A 66‐year‐old woman was admitted to our hospital for further examination of the gastric abnormality. Endoscopic examination of the upper gastrointestinal tract revealed a flat, rounded elevated lesion with a central erosion in the anterior wall of the upper gastric body. Endoscopic ultrasonography revealed a poorly demarcated, slightly hypoechoic lesion located in the third layer of the gastric wall. Based on these findings, a submucosal tumor, particularly a carcinoid tumor, was suspected. Endoscopic tumor resection, which provides a significant benefit for accurate final diagnosis and eradication of submucosal lesion, was performed. Histological study showed the gastric gland heterotopia of immature type in the submucosa.     

Human herpesviruses infections (I)--EBV infection and immunity in cancer patients]

Human herpesviruses have been known to be reactivated from latent infection iin immunosuppressed individuals. To assess the immunity, antibodies to human herpesviruses (VZV, HSV, CMV and EBV) were measured in patients with gastrointestinal cancer and normal persons ranging from 40 to 80 in age. CF antibodies to herpesviruses except for EBV were negative in normal persons below the age of 60 and cancer patients aged 60 and over. The CF antibody titer higher than 1:64 occurred only in young cancer patients and older normal persons. The titer of VCA antibody to EBV ranged from 1:40 to 1:160 in more than 80% of normal persons and was higher than 1:640 in less than 7%. The ratio those with antibody negative or below 1:40 increased with the age. In contrast, the titer was higher than 1:320 in 70% of the patients, and the antibody was negative in none of them. In addition, the low titer occurred only in less than 5%. The rate of patients having higher VCA antibody titers increased with age, and the titer was high in more than 50% of patients in the 8th decade. In cancer patients, the higher the VCA antibody titer was, the less number of mature B cells, target cells of EBV, and the B cell functions were observed. This suggests that reactivation of EBV in hosts bearing carcinomas results in a fall of production of the specific antibody.     

Crohn＇s disease in Japanese is associated with a SNP-haplotype of N-acetyltransferase 2 gene

AIM: To investigate the frequency and distribution of N-acetyltransferase 2 (NAT2) and uridine 5'-diphosphate (UDP)-glucuronosyltransferase 1A7 (UGT1A7) genes in patients with ulcerative colitis (UC) and Crohn's disease (CO). METHODS: Frequencies and distributions of NAT2 and UGT1A7SNPs as well as their haplotypes were investigated in 95 patients with UC, 60 patients with CD, and 200 gender-matched, unrelated, healthy, control volunteers by PCR-restriction fragment length polymorphism (RFLP), PCR-denaturing high-performance liquid chromatography (DHPLC), and direct DNA sequencing. RESULTS: Multiple logistic regression analysis revealed that the frequency of haplotype, NAT2*7B, significantly increased in CD patients, compared to that in controls (P=0.0130, OR = 2.802,95%CI = 1.243-6.316). However, there was no association between NAT2 haplotypes and UC, or between any UGT1A7 haplotypes and inflammatory bowel disease (IBD). CONCLUSION: It is likely that the NAT2 gene is one of the determinants for CD in Japanese. Alternatively, a new CD determinant may exist in the 8p22 region, where NAT2 is located.     

Successful management of liver metastasis from gastric adenosquamous carcinoma with adjuvant chemotherapy and radiofrequency ablation

A 70-year-old man was given a diagnosis of advanced type 3 gastric cancer and underwent surgery. In addition to total gastrectomy and D2 lymph node resection, partial liver resection was performed due to detection of a solitary liver metastasis which was initially overlooked on preoperative CT. Histopathologically, the tumor was diagnosed as gastric adenosquamous carcinoma (Int, INFβ, pT2 (ss), H1, ly1, v2 (mp), EVG, n (-), pPM (-), pDM (-)). Three metastasic lesions appeared in the liver on abdominal CT scan performed 3 months after the operation. The patient underwent adjuvant chemotherapy with S-1. Since enlargement of the liver metastases was observed following 2 courses of treatment, the patient received combination chemotherapy of irinotecan and cisplatin. Of 3 metastatic lesions, 1 disappeared and 2 decreased to less than 2 cm in diameter after 8 courses of 2nd line treatment. Radiofrequency ablation (RFA) was successfully performed to treat the remaining liver metastases. The patient has been free of recurrence for 41 months.     

Gastric duplication cyst: Evaluation by endoscopic ultrasonography and magnetic resonance imaging

Endoscopic ultrasonography (EUS) and magnetic resonance imaging (MRI) are becoming popular methods for examining tumorous lesions along the upper gastrointestinal tract. Though duplication cysts are uncommon. EUS findings from gastric duplication cysts have accumulated and proven very useful for preoperative diagnosis. There have been few reports, however, concerning MRI findings from these cysts. We report herein the case of a 25-year-old man with a gastric duplication cyst. EUS demonstrated a cystic mass adjacent to the fourth layer of the stomach wall. MRI revealed a cyst containing low signalintensity fluid and high signal-intensity fluid separated by levels. In addition to the characteristic findings from preoperative examinations, the unique histological findings from the cyst are also described.     

Caveolin-1 is a novel immunohistochemical marker to differentiate epithelioid mesothelioma from lung adenocarcinoma

Aims:  The incidence of mesothelioma is increasing in Europe, Japan and other developing countries. There is difficulty in the accurate diagnosis of mesothelioma and its differentiation from lung adenocarcinoma. Mesothelioma shows a complex immunohistochemical profile. Therefore, the use of a immunohistochemical panel that includes both positive and negative mesothelial markers has become a general rule for its accurate diagnosis. However, they are still not sufficient. The aim was to assess the diagnostic utility of caveolin-1 (Cav-1), which is expressed in endothelial cells, alveolar type I pneumocytes and mesothelial cells, as a novel positive marker of mesothelioma.
Methods and results:  An immunohistochemical study of 80 cases of epithelioid mesothelioma and 80 cases of lung adenocarcinoma was performed for the analysis of the expression of Cav-1 and other markers. Cav-1 expression with a membranous and/or cytoplasmic pattern was found in all of the epithelioid mesothelioma. Of these, 42 cases (52.5%) showed Cav-1 expression in >50% of tumour cells, 34 cases (42.5%) in 6–50% of tumour cells, and four cases (5.0%) in <5% of tumour cells. In contrast, only six cases (7.5%) of lung adenocarcinoma showed focal Cav-1 expression in the cytoplasm of the tumour cells. The sensitivity and specificity of Cav-1 expression for the differentiation of epithelioid mesothelioma from lung adenocarcinoma were 100 and 92.5%, respectively. This is comparable or even superior to that of currently available positive markers such as calretinin or D2-40.
Conclusions:  Cav-1 is a novel immunohistochemical marker for the differentiation of epithelioid mesothelioma from lung adenocarcinoma.     

Branched-chain amino acid therapy for spinocerebellar degeneration: a pilot clinical crossover trial.

OBJECT: The potential effects of branched-chain amino acids (BCAAs) on spinocerebellar degeneration (SCD) were explored in eleven patients. METHODS: The patients received 200 ml of BCAA-rich solution, 2 mg of thyrotropin-releasing hormone (TRH; protirelin), or a placebo daily for 7 days each in a random order. An SCD score was used to quantify the severity of symptoms. PATIENTS: Eleven patients with SCD (7 male, 4 female; mean age 60+/-11; mean disease duration 5.5 years) participated in this study. RESULTS: The mean SCD score of the eleven patients improved significantly by the BCAA treatment compared with the baseline. The conditions of five of the eleven patients (45%) were clearly improved by the BCAA treatment. All of the responders manifested predominantly cerebellar symptoms, but no prominent parkinsonian symptoms. Two patients with marked rigidity and akinesia did not respond to the treatment. CONCLUSION: We concluded that BCAAs do have a beneficial effect on functional improvement in patients with SCD, and that further large scale studies are needed.