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Mucosa-associated lymphoid tissue (MALT) lymphoma arising from the thymus is extremely rare. Only 33 cases of thymic MALT lymphoma have been reported to date. We present the case of a 53-year-old Japanese woman with Sjögren's syndrome who was diagnosed with thymic MALT lymphoma. In addition, the patient had the characteristic clinical and pathological features of thymic MALT lymphoma, as found in most of the 33 previous cases, except that there was an immunoglobulin G (IgG) phenotype, i.e. Sjögren's syndrome, epithelial cysts, lymphoepithelial lesions, and marked plasmacytic differentiation. The serum IgA levels were also elevated with IgA kappa M protein. This hypergammaglobulinemia remained unchanged after operation. The serological abnormalities may not arise from MALT lymphoma itself and may arise from the immune system hyper-reactivity evoked by Sjögren's syndrome. Of further interest were marked accumulations of CD68-positive histiocytes containing abundant eosinophilic globular inclusions in their cytoplasm. These inclusions were immunopositive for IgG-kappa, suggesting immunoglobulin inclusion bodies. The globular immunoglobulin inclusion bodies have been reported in non-crystallized immunoglobulin-storing histiocytosis in only one patient with multiple myeloma. To our knowledge, this is the first case of thymic MALT lymphoma with marked accumulation of histiocytes with immunoglobulin inclusions in a patient with Sjögren's syndrome.

Host defense mechanisms play important roles in suppressing the development and growth of tumors. It is known that S-100 protein-positive immature dendritic cells (S100DC), as antigen presenting cells (APC), and macrophages have roles in the immune responses to tumor growth. Mediators such as nitric oxide are also important in the surveillance against cancer. We examined the distribution of S100DC and CD68-positive macrophages (CD68MØ) immunohistochemically to compare the condition of apoptotic tumor cells in 69 patients with human non-small cell lung carcinoma. The expression of inducible nitric oxide synthases (iNOS) in tumors was also studied. Unlike macrophages, S100DC were distributed predominantly in cancer nests. In the areas with infiltration of 'many' S100DC (i.e. more than 10 DC/HPF), we found two distinct patterns of tumor infiltration: scattered and aggregated infiltration of DC in tumor nests. In areas of scattered S100DC distribution, only a few apoptotic tumor cells could be detected. However, in the areas of DC aggregations, apoptotic tumor cells were significantly more abundant (P = 0.0491). In contrast to S100DC, the distribution and density of CD68MØ were associated with iNOS expression of tumor cells (P < 0.0001), but not with distribution of apoptotic tumor cells. These findings reveal differences in the in vivo condition between S100DC and CD68MØ in tumors, and suggest there is a relationship between tumor-infiltrating S100DC aggregation and apoptosis in in vivo non-small cell lung cancers.

Seminoma arising in patients with Klinefelter's syndrome is extremely rare; to our knowledge, only three cases have been reported in the English language literature. We report a case of intrapelvic seminoma in a 39-year-old man with Klinefelter's syndrome. Gross examination revealed that the tumor was a solid and irregular mass measuring 90 mm in diameter. The cut surfaces of this ill-defined tumor were yellow-white with necrotic foci. Histologically, the tumor cells were separated into lobules by branching, fibrous septa containing lymphocytes. In some parts of the tumor, a cord-like arrangement of tumor cells was present. Immunohistochemically, the tumor cells were strongly and diffusely positive for antiplacental alkaline phosphatase antibody along their cytoplasmic membranes, but negative for both chorionic gonadotrophin and alpha-fetoprotein. Based on these findings, we diagnosed this tumor as a seminoma. The testes when examined were found to be atrophic bilaterally, but with no tumor lesions. Chromosomal analysis yielded a 47XXY karyotype, compatible with Klinefelter's syndrome. These findings indicate a case of primary intrapelvic seminoma in Klinefelter's syndrome. The patient underwent intensive radiation therapy postoperatively, and he demonstrated no evidence of recurrence or metastasis during the 13-month period following surgery.

Recent cytogenetical studies have indicated that trisomy 12 is a feature of ovarian tumors in the thecoma-fibroma group. Ten cases of these ovarian tumors were studied in total, including two thecomas, two fibrothecomas, four fibromas, one cellular fibroma and one fibrosarcoma, to clarify the relationship between polysomy 12 and proliferative activity in these tumors. Each formalin-fixed, paraffin-embedded tumor tissue was examined by fluorescence in situ hybridization to determine copy numbers of chromosome 12 and by immunohistochemical staining of Ki-67 for evaluation of tumor cell proliferation. Gains of trisomy 12 were found in seven of the 10 cases, and the percentage of cells with tetrasomy 12, but not that of cells with trisomy 12, was significantly and positively correlated with percentage of Ki-67-positive cells, but significantly and inversely correlated with patient age. These findings suggest that tetrasomy 12 is an age-related aberration of chromosome 12 in ovarian tumors of the thecoma-fibroma group, and that such tumors exhibit more active proliferation in younger patients.

Primary gall-bladder carcinoma producing granulocyte-colony stimulating factor (GCSF) is extremely rare. Only four cases, histologically investigated, have been reported to date in the English literature. We report a case of a 48-year-old female with primary gall-bladder carcinoma, associating with leukocytosis (15 700/mm3) and a high level of serum GCSF (54.0 pg/mL). The tumor was, histologically, a poorly differentiated adenocarcinoma with marked interspersed neutrophils invading into the primary tumor itself and the right lobe of the liver. Tumor cells distinctly showed positive immunoreaction in the cytoplasm with anti-GCSF antibody, and in the nucleus for anti-p53 antibody. After surgery, the leukocytosis and serum level of GCSF began to decrease. These findings confirmed the present case of GCSF-producing gall-bladder carcinoma, exhibiting leukocytosis. A total of five cases, including our case, reported as a GCSF-producing gall-bladder carcinoma were clinicopathologically reviewed.

Primary ovarian angiosarcoma is extremely rare. Only 16 cases have histologically been reported to date in the literature. A case of angiosarcoma arising in the right ovary of a 46-year-old female is presented. Grossly, the resected right ovary was completely replaced by a solid tumor mass, which revealed multiple necrotic and/or hemorrhagic foci. This case revealed the typical histological features of angiosarcoma with sinusoidal and solid patterns of anaplastic tumor cells. Immunohistochemically, tumor cells were strongly and diffusely positive for CD31 and CD34, in particular, along the cytoplasmic membrane of the tumor cells. Ultrastructurally, tumor cells possessed the intermediate junctions between tumor cells, discontinuous basal laminae attached to the irregularly shaped blood vessels and occasional cytoplasmic pinocytotic vesicles. These findings confirmed the case as being one of angiosarcoma of the ovary. The patient died 9 months after surgery as a result of developed multifocal brain metastases. A total of 17 cases reported as primary ovarian angiosarcoma, including this presented case, are clinicopathologically reviewed.

An autopsy case of a 58-year-old woman with massive cardiac involvement of adult T cell leukemia/lymphoma (ATLL) is reported. She developed cardiac failure due to aortic and mitral regurgitation with cardiac infiltration of ATLL cells, and underwent replacement of both aortic and mitral valves. Studies of the cut-surfaces revealed diffuse thickening of the subendocardial wall of the left chamber with widespread whitish-brown tumor infiltrates. In the regions surrounding the replaced aortic and mitral valves there was also massive tumor cell infiltration. The tumor cells infiltrating the cardiac muscle wall were T cell in origin and exhibited Leu-3a (CD4)-positive immunoreaction. Ultrastructurally, tumor cells contained markedly indented nuclei and some were attached directly to the muscle cells. These findings suggest that this was an unusual form of ATLL with widespread involvement of the heart.

A case of duodenal gangliocytic paraganglioma (DGP) arising in the ampulla of Vater of an 84 year old male is reported. The present case shows the typical histological features with a mixture of epitheliold, ganglion-like and spindle cells. Immunohistochemically, the spindle cells were positive with antibodies to neuron specific enorse, S-100 protein and neurofilament (NF), but negative for anti-glial fibrillary acidic protein (GFAP), synaptophysin and myelin basic protein (MBP). Both anti-Leu-7 and -chromogranin A antibodies gave positive reactions with a fine granular pattern in the epithelioid cells. Most ganglion-like cells showed the cytoplasmic immunoreaction with GFAP and a few with NF and synaptophysin. In these three tumor components, no reactivity was detected in the antibodies to the neurohormonal peptides tested. The present data are fundamentally in keeping with those previously reported, but add the immunohistochemical positivity of ganglion-like cells for GFAP, suggesting different neuroglial differentiation.

An autopsy case of a 61 year old male with primary squamous cell carcinoma of the lung with associated marked leukocytosis and hypercalcemia is reported. High levels of serum parathyroid hormone-related peptide (PTHrP) and granulocyte colony stimulating factor (GCSF) were detected. The tumor cells distinctly showed positive cytoplasmic immunoreactions with anti-PTHrP and anti-GCSF antibodies. Marked granulocytosis and thin bony trabeculae lacking osteoblasts were observed in the vertebral bone. Calcium deposits were found in the proximal tubules of the kidneys. Infarcts were seen as a result of fibrin thrombosis of the splenic artery. The tumor was successfully transplanted into nude mice in which the high levels of serum PTHrP and GCSF were reproduced. These results indicate that the tumor simultaneously produced both PTHrP and GCSF causing the paraneoplastic syndromes of hypercalcemia and leukocytosis.

A granular cell tumor in the prostate of a 72 year old male is reported. The tumor nest was composed of large polygonal tumor cells, possessing cytoplasmic eosinophilic granules which were positive with PAS stain and resistant to diastase digestion. Immunohistochemically, most tumor cells were strongly positive with anti-alpha-smooth muscle actin and -neuron specific enolase antibodies. Only a few scattered tumor cells were reacted with anti-S-100 protein antibody. These findings might suggest smooth muscle differentiation of this case, and provide an additional insight into the histogenesis of granular cell tumors, especially in their heterogeneity.

An autopsy case is presented of a peculiar type of intrahepatic mucinous adenocarcinoma with microcyst formation arising in a 78 year old Japanese man who died of hepatic coma and renal failure 4 months after onset. Macroscopically, the cut surface of the lesion revealed a characteristic honeycomb-like appearance, consisting purely of microcysts, 0.2-0.4 cm in diameter, lined by prolific mucin-producing adenocarcinoma cells. The lesion did not have large cystic space, fibrous capsule, or benign cystadenomatous component other than neoplastic microcyst formation. The carcinoma cells showed various proliferating patterns, such as irregularly shaped nest-like, trabecular, papillary and tubular ones, directly invaded the hepatic parenchyma and portal tract with loose or thick fibrosis, and infiltrated extensively into both intrahepatic and extrahepatic stroma along the vascular structures. From these clinicopathological findings, we consider the present tumor to be a variant of mucinous cholangiocarcinoma with characteristic microcyst formation rather than a type of cystadenocarcinoma.

Periosteal osteosarcoma is an exceedingly rare type of chondroblastic osteosarcoma, showing rather better prognosis, and secondary bone marrow involvement is unusual. A case of a 22 year old male with periosteal osteosarcoma of the right femur with an associated bone marrow lesion is presented. The juxtacortical tumor, 16 x 11 x 9 cm, was located on the bone cortex of the upper diaphysis and extended into the surrounding soft tissues. A minimal bone marrow lesion was present, although the bone cortex was quite intact. Microscopically, the tumor consisted exclusively of atypical chondroblastic cells with a small osteoblastic area. The bone marrow lesion, interestingly, contained both multiple nodules of well-differentiated chondrosarcomatous components and a few demarcated foci of atypical spindle cells producing a fine osteoid matrix. It was reasonable to conclude, therefore, that this tumor was a periosteal osteosarcoma with an unusual secondary bone marrow lesion rather than a conventional (central) chondroblastic osteosarcoma with soft tissue invasion. The patient's good prognosis with no tumor recurrence or metastasis during more than 7 years follow-up after surgery supports this conclusion.