Retrospective analysis of prognostic factors for WaldenstrÓ§m macroglobulinemia: a multicenter cooperative study in Japan.

Although population-based cancer registries have reported lower incidence of WaldenstrÓ§m macroglobulinemia (WM) in East Asia than in Western countries, previous retrospective analyses have found the clinical features of WM to be similar in these two populations. To clarify the characteristics of Japanese WM patients, we retrospectively analyzed clinical and laboratory characteristics, treatments, outcomes, and prognostic factors in 93 patients with WM. Based on the Second International Workshop on WM (IWWM-2) criteria, symptomatic WM was found in 73 (78.5%) and asymptomatic WM in 20 (21.5%) of cases examined. The median overall survival (OS) was similar to that in reports from Western countries. Patients receiving treatment regimens including rituximab exhibited significantly better survival than those not given rituximab. Although prognostic factors for WM in Western countries may not apply to Japanese patients, our finding that newly diagnosed WM patients with pleural effusion have a poorer prognosis suggests that this may be a novel predictor of adverse prognosis in symptomatic WM.

Animal-type malignant melanoma associated with nevus of Ota in the orbit of a Japanese woman: a case report.

We present a patient with an animal-type malignant melanoma associated with the nevus of Ota in the orbit who showed a good prognosis after a combination of orbital extirpation, chemotherapy, stereotactic radiotherapy, and gamma knife. A 42-year-old Japanese woman presented with two tumors, one pathologically diagnosed as right-sided intraconal animal-type malignant melanoma and the other intracranially, presumed to be of the same pathogenesis and both were considered to have arisen from the nevus of Ota. She underwent an extirpation of the orbit, chemotherapy (DAV therapy, which is a combination of dacarbazine, nimustine, and vincristine), stereotactic radiotherapy (54 Gy in 27 fractions), and gamma knife (marginal dose was 17 Gy, target volume was 0.2 ml). She has been alive for 33 months since the extirpation, with no sign of local recurrence, new metastasis, nor enlargement of the intracranial tumor. Not just combination therapy but also the low malignancy of animal-type melanoma may have contributed toward the good prognosis.

Isolated cutaneous manifestation of IgG4-related disease.

AIMS: The cutaneous manifestation of IgG(4)-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG(4)-positive plasma cell infiltration in the skin. METHODS: The authors investigated two cases of IgG(4)-related disease with solitary skin lesions and compared the immunohistochemical characteristics of infiltrating cells among IgG(4)-related disease, Kimura's disease and cutaneous pseudolymphoma. RESULTS: IgG(4)-related disease manifested as an indurated plaque on the anterior chest in one case and a nodule on the toe in the other case. Histopathologically, skin lesions of IgG(4)-related disease showed a dense, mixed-cell infiltrate containing lymphocytes, plasma cells and eosinophils along with fibrosis. Plasma cells stained positively with anti-IgG and anti-IgG(4) antibodies, and the ratio of IgG(4)+/IgG+ cells was more than 50%. Serum levels of IgG and IgG(4) were not elevated and no lesions were found in other organs. Skin samples taken from Kimura's disease showed histopathological features similar to those of IgG(4)-related disease. The proportion of IgG(4)+/IgG+ was high in Kimura's disease, but not in cutaneous pseudolymphoma. CONCLUSIONS: The solitary skin lesions of IgG(4)-related disease were similar histologically and immunohistochemically to the skin lesions of Kimura's disease. The concept of IgG(4)-related disease may help clarify the pathomechanism of diseases of unknown aetiology that possess features of IgG(4)-related disease.

Thoracoscopic resection of a mesenchymal cystic hamartoma of the lung.

Mesenchymal cystic hamartomas are uncommon tumors originating from nodules of primitive mesenchymal cells, and their occurrence in the lung is extremely rare. We present a case of surgically resected mesenchymal cystic hamartoma of the lung in a 49-year old woman who presented without any symptoms. Chest computed tomography showed a solitary cystic lesion in the right lower lobe. The patient underwent preoperative marking by 0.2 ml lipiodol 4 days before surgery. Thoracoscopic partial resection of the right lower lobe was performed. The histological picture and immunohistochemical profile were compatible with this tumor.

Primary thymic mucosa-associated lymphoid tissue lymphoma: diagnostic tips.

Mucosa-associated lymphoid tissue (MALT) lymphoma arising in the thymus is extremely rare and little is known regarding its clinicopathological features. This study examined the clinicopathological features of nine cases of thymic MALT lymphoma. Most patients had autoimmune disease or hyperglobulinemia, and they also had cysts in the tumors. Both increased serum autoantibody levels and polyclonal serum immunoglobulin levels remained essentially unchanged after total thymectomy in all patients. Thymic MALT lymphoma needs to be included in the differential diagnosis in Asian patients with a cystic thymic mass accompanied by autoimmune disease or hyperglobulinemia.

B-cell lymphoma associated with Sjögren's syndrome among Japanese patients: a clinicopathologic and immunohistochemical study of 15 cases.

To clarify the clinicopathological findings of B-cell lymphoma associated with Sjögren's syndrome (SJS) among Japanese Patients, 15 individuals with this disease were studied. The patients, 14 females and one male, ranged in age from 41 to 73 years with a median age of 56 years. These lymphomas arose not only in the salivary gland (n=4) but also in other mucosal extranodal sites (n=5). Histologically, six cases were marginal zone B-cell lymphoma (MZBL) of the mucosa-associated lymphoid tissue (MALT) type, three cases were diffuse large B-cell lymphoma (DLBCL) + MALT type lymphoma, two cases were nodal MZBL and one case each was small lymphocytic lymphoma, Burkitt's lymphoma, CD10(+) DLBCL and DLBCL + nodal MZBL. Using in situ hybridization, numerous Epstein-Barr virus(+) tumor cells were detected only in the case of Burkitt lymphoma. There were no human-herpes type 8(+) tumor cells in any of the 15 cases. There was no API2-MALT1 fusion transcript in any of the eight cases examined. B-cell lymphoma associated with SJS also frequently affected extranodal organs in patients from Japan as well as from patients in Western countries. The majority of B-cell lymphomas in patients with SJS also appear to be low-grade MZBLs or high-grade lymphomas probably derived from MZBL both in Western countries and in Japan.

Hepatocellular Carcinoma Mimicking Liver Abscesses in a Cirrhotic Patient with Severe Septic Shock as a Result of Salmonella O9 HG Infection.

We describe a case of severe Salmonella O9 HG sepsis with a mass in the liver, which was diagnosed as hepatocellular carcinoma (HCC) by autopsy of the liver. The patient was a 67-year-old man with chronic high blood pressure. In addition, he was an alcoholic and had been drinking every day for many years. He had had a dinner of 'sukiyaki' with a raw egg two days before admission. The next morning, he had developed vomiting, diarrhea, and abdominal pain. Salmonella O9 HG was found in the blood and stool cultures. In the computed tomography (CT) finding of the liver, there was a 2 cm early-enhanced mass with a multilocular structure, with ringed enhancement and daughter nodes. Since we thought that the mass was a liver abscess, we performed needle aspiration from the liver mass and were able to withdraw blood. Despite adequate antibiotic treatment, the patient died as a result of complications on the 55th day after admission. After the patient's death, we conducted an autopsy. There were two HCC masses, a moderately-differentiated and a well-differentiated mass, as a result of alcoholic cirrhosis of the liver. As the HCC had multilocular cyst-like structures, which were fiber- and necrosis-rich, CT images of the liver masses resembled abscesses.

Amplification of the epidermal growth factor receptor gene in glioblastoma: an analysis of the relationship between genotype and phenotype by CISH method.

We examined epidermal growth factor receptor (EGFR) overexpression and EGFR gene amplification using immunohistochemistry (IHC) and chromogenic in situ hybridization (CISH) in 109 glioblastomas, including 98 primary glioblastomas and 11 secondary glioblastomas. EGFR overexpression and EGFR gene amplification were found in 33% and 24% of glioblastoma, respectively, and all of those cases were primary glioblastoma. Large ischemic necrosis was significantly more frequent in primary glioblastomas than in secondary glioblastomas (54% vs. 18%), but pseudopalisading necrosis was not (65% vs. 54%). EGFR gene amplification was detected significantly more frequently in cases with both types of necrosis. Although glioblastomas with EGFR gene amplification invariably exhibited EGFR overexpression at the level of the whole tumor, tumor cells with EGFR gene amplification did not always show EGFR overexpression at the level of individual tumor cells. Cases of "strong" EGFR overexpression on IHC could be regarded as having EGFR gene amplification, and cases without EGFR overexpression could not. Cases of "weak" EGFR overexpression should be tested with CISH to confirm the presence of EGFR gene amplification. We found that 54% of glioblastomas with EGFR gene amplification were composed of areas with and without EGFR gene amplification; however, there were no obvious differences in morphology between tumor cells with and without EGFR gene amplification. Although small cell architecture might be associated with EGFR gene amplification at the level of the whole tumor, it did not always suggest amplification of the EGFR gene at the level of individual tumor cells. In one case, it seemed to suggest that a clone with EGFR gene amplification may arise in pre-existing tumor tissue and extend into the surrounding area. In cases of overall EGFR amplification, CISH would be a useful tool to decide the tumor border in areas infiltrated by tumor cells.