Chordoid glioma of the third ventricle: a report of two cases, one with ultrastructural findings.

Chordoid glioma, which generally occurs in adults, is a rare CNS tumor arising in the anterior part of the third ventricle. We report two cases of chordoid glioma of the third ventricle in a 42-year-old woman and a 51-year-old man, respectively. Both tumors showed essentially the same histological and immunohistochemical features; the tumors were composed of cords and nests of epithelioid, GFAP-immunoreactive cells in a mucinous stroma with lymphoplasmacytic infiltrates at the tumor periphery. Ultrastructural examination in one case revealed that the tumor cells were characterized by the presence of hemidesmosomes and associated focal basal lamina formation, intermediate junctions, microvilli and cilia, and intercellular microrosettes with microvilli. Of interest was that small blood vessels with fenestrated endothelial cells were present in the stroma. In the brain, the presence of fenestrated endothelial cells is a feature of the circumventricular organs (except the subcommissural organ), among which the organum vasculosum of the lamina terminalis is located in the anterior part of the third ventricular floor that is lined by specialized ependymal cells known as tanycytes. These findings further strengthen the hypothesis that chordoid glioma may represent a peculiar clinicopathological subtype of ependymoma (chordoid ependymoma) originating from the lamina terminalis area.

[A case of idiopathic pulmonary alveolar proteinosis with multiple localized ground-glass opacities].

A 58-year-old Japanese female consulted our staff with multiple localized ground-glass opacities in chest CT. She underwent video assisted thoracoscopic surgery for diagnosis. Histopathologic finding from surgery specimen in one of ground-glass opacities revealed bronchioloalveolar carcinoma. Six months later, we performed second video assisted thoracoscopic surgery, and histopathologic finding of all other ground-glass opacities revealed pulmonary alveolar proteinosis. Serum anti GM-CSF antibody elevated, and she was diagnosed as having idiopathic pulmonary alveolar proteinosis. A case of idiopathic pulmonary alveolar proteinosis presenting multiple localized ground-glass opacities is rare. And, differentiating ground-glass opacities of pulmonary alveolar proteinosis and bronchioloalveolar carcinoma by chest CT is difficult.

Clinicopathological significance of antinuclear antibodies in non-alcoholic steatohepatitis.

AIM: Serum antinuclear antibodies (ANA) are occasionally noted in patients with non-alcoholic steatohepatitis (NASH). We examined the significance of ANA in NASH. METHODS: We compared clinicopathological features in patients with ANA-positive NASH (n = 35) and ANA-negative NASH (n = 36). Inflammatory cell profiles and the distribution of oxidative stress markers were also examined immunohistochemically. RESULTS: ANA-positive NASH was significantly associated with female gender (P = 0.005), high degree of portal inflammation (P = 0.039), interface activity (P = 0.036) and hepatocellular ballooning (P = 0.0008). In addition, ANA of high titer (320-fold or more) was significantly associated with the histological grade and stage of NASH (P = 0.02). The degree of steatosis wais rather mild in the high-titer ANA group(P = 0.01). The analysis of inflammatory cell profiles revealed that CD3-positive T cells were predominant and plasma cells were rather few in the portal area and hepatic lobules in both ANA-positive and ANA-negative groups. There was no difference in the distribution of oxidative stress markers between ANA-positive and ANA-negative groups. CONCLUSION: These findings suggest that the presence of ANA may be related to the progression of NASH and that a different type of autoimmune mechanism may be involved in the pathogenesis of NASH with ANA, compared to the pathogenesis of autoimmune hepatitis.

Cryofibrinogenemia associated with polyarteritis nodosa.

We report the case of a 77-year-old woman with biopsy-confirmed polyarteritis nodosa (PAN) associated with cryofibrinogenemia presenting with polyarthralgia and digital gangrene induced by cold exposure. The clinical manifestations and parameters measured by laboratory markers including cryofibrinogen responded well to corticosteroid therapy. To our knowledge, the case of the combination of PAN and cryofibrinogenemia has not been reported. Our case indicates that cryofibrinogenemia might be associated with PAN. The PAN patients with cold-induced symptoms should be screened for cryofibrinogen.

IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?

Sclerosing cholangitis (SC) is a heterogeneous disease entity. Different etiologies such as choledocholithiasis, biliary tumor, or pericholangitis can manifest as SC. Hepatic inflammatory pseudotumor (IP) is rarely associated with SC (sclerosing cholangitis associated with hepatic inflammatory pseudotumor; SC-hepatic IP), but sclerosing pancreatitis (SP) is not infrequently associated with bile duct lesions (sclerosing pancreatitis-associated sclerosing cholangitis; SP-SC). In this study, we compared the histologic changes of hepatic hilar and extrahepatic bile duct lesions of SC (7 cases), SC-hepatic IP (5 cases), SP-SC (5 cases), and typical primary sclerosing cholangitis (PSC) (5 cases). Histologically, all SP-SC cases showed extensive and dense fibrosis with marked lymphoplasmacytic infiltration, many eosinophils, and obliterative phlebitis. Four cases of SC showed bile duct lesions similar to those of SP-SC, whereas other three cases of SC showed milder lymphoplasmacytic infiltration, scant eosinophilic cell infiltration, and no obliterative phlebitis. All SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC. Immunohistochemically, many IgG4-positive plasma cells were found in the bile duct lesions of all SP-SC cases, 4 SC cases with marked lymphoplasmacytic infiltration, and all SC-hepatic IP cases. By contrast, IgG4-positive plasma cells were scarce or hardly found in the remaining 3 SC cases and all PSC cases. In conclusion, 4 SC cases and all SC-hepatic IP cases showed bile duct lesions identical to those of SP-SC, suggesting that these three conditions may be a single disease entity. Their pathogenesis may be similar or closely related to that of SP, and in that respect they may represent an IgG4-related biliary disease. They may respond to steroid therapy as SP does.

Ets-1 and integrin beta3 for lung metastasis from colorectal cancer.

Ets-1 functions as tissue-specific transcription factor and plays an important role in cell proliferation, differentiation, angiogenesis, and apoptosis. To elucidate the involvement of Ets-1 in lung metastasis from colorectal cancer, immunohistochemical analysis was performed on 51 colorectal cancer patients (22 with lung metastasis and 29 with advanced colorectal cancer showing no recurrence for at least 5 years after surgery). Tumorous and stromal Ets-1 immunoreactivity was evaluated. Ets-1 protein was demonstrated within stromal fibroblasts, myofibroblasts and capillaries, and also within carcinoma cells. Stromal Ets-1 immunoreactivity in primary colorectal cancer was statistically correlated with lung metastasis (p<0.05). In primary colorectal cancer, a significant association was observed between stromal Ets-1 immunoreactivity and vascular integrin beta3 expression (p<0.01). In the cases with lung metastasis, vascular integrin beta3 index in lung metastases was significantly diminished compared with primary tumors or liver metastases (p<0.01). In contrast, stromal or tumorous Ets-1 expression did not change. In a multivariate model using logistic stepwise regression analysis, vascular integrin beta3 and stromal Ets-1 overexpression were significantly and independently related to lung metastasis. Stromal Ets-1 and/or vascular integrin beta3 may be useful markers for risk of lung metastasis from colorectal cancer.

Integrin alpha v, c-erbB2 and DNA ploidy in lung metastases from colorectal cancer.

BACKGROUND/AIMS: We analyzed integrin alpha v and c-erbB2 expression and DNA ploidy in primary tumors and pulmonary metastases from colorectal carcinoma. METHODOLOGY: Fifty primary tumors and 21 lung metastases were examined for immunohistochemical detection of integrin alpha v, c-erbB2 and DNA ploidy. RESULTS: In integrin alpha v-positive tumors, integrin alpha v was strongly recognized in the cytoplasm of colorectal carcinoma. In total cases, in a multivariate model using logistic stepwise regression analysis, venous invasion was significantly and independently related to lung metastasis in patients who underwent curative resection. Integrin alpha v, c-erbB2 and DNA ploidy in primary tumors were not predictable for lung metastasis. On the other hand, in the 21 patients with lung metastases, integrin alpha v overexpression was identified in 52.4% (11/21) of primary tumors and in 85.7% (18/21) of lung metastases, respectively. The positive ratio was significantly higher in lung metastases than in primary tumors (p < 0.05). There was no significant difference between primary tumors and lung metastases in DNA ploidy or c-erbB2 expression. CONCLUSIONS: Integrin alpha v might be one of the important signals for formation of lung metastases from colorectal cancer as a late event.

Correlation between expression levels of thymidine phosphorylase (dThdPase) and clinical features in human gastric carcinoma.

BACKGROUND/AIMS: dThdPase (thymidine phosphorylase) is an enzyme that is involved in pyrimidine nucleoside metabolism. It has been recently reported that dThdPase is identical to platelet-derived endothelial cell growth factor. Previous studies demonstrated that the immunohistochemical expression of dThdPase was related with disease progression. The purpose of this study was to examine the expression levels of dThdPase and their prognostic significance in gastric carcinoma, prospectively. METHODOLOGY: We obtained fresh samples (tumors and adjacent normal mucosa) from 263 patients with gastric cancer who underwent gastrectomy between April 1998 and March 2000. dThdPase was analyzed by the enzyme-linked immunosorbent assay method. RESULTS: The expression levels of dThdPase in tumors (98.4 +/- 62.5 U/mg protein) was significantly higher than that in normal mucosa (46.5 +/- 27.8 U/mg protein) (P < 0.0001). Intratumoral dThdPase levels were significantly higher in Borrmann types II and III macroscopically (P < 0.01), in poorly differentiated type histologically (P < 0.05), and in the tumor-invading serosa (P < 0.001). The expression levels of dThdPase were also significantly higher in tumor tissue from patients with lymph node metastasis (P < 0.01) and in that from patients with lymphatic invasion (P < 0.05). The survival rate of patients with tumor of high dThdPase (> or = 100 U/mg protein) was significantly poorer than that of patients with tumor of low dThdPase (< 100 U/mg protein) (P < 0.05). CONCLUSIONS: These findings indicate that the measurement of dThdPase may provide useful information for predicting prognosis in gastric carcinomas.

[A case of hypersensitivity pneumonitis caused by Lyophyllum karst].

A 52-year-old woman who has worked at factory to check pack Lyophyllum karst for six years consulted another doctor for dry cough as common cold. After a month, low grade fever, dyspnea on effort, and productive cough brought her to our hospital. Her chest radiographs showed multiple small nodular shadows and ground glass opacities in entire lung fields. Histopathological examination of transbronchial lung biopsy specimen revealed granuloma and thickening of alveolar septa with lymphocytes infiltration. She was admitted to our hospital for hypersensitivity pneumonitis and prescribed predonisorone 30 mg/day. Her symptoms and the finding of chest radiograph improved in two weeks. The precipitation antibodies to Lyophyllum karst were positive and the symptoms exacerbated by going to work. Those finding suggests that hypersensitivity pneumonitis in this case. After quitting her job, she has had no relapse by tapering steroids.

[A patient with advanced gastric cancer in the gastric tube whose QOL was improved by TS-1].

We present the case of a 72-year-old man with gastric tube cancer accompanied by multiple liver metastases, after esophagectomy for esophageal cancer, whose quality of life (QOL) was improved with a small dosage of TS-1. The patient's high serum AFP level suggested alpha-fetoprotein-producing gastric cancer. He was treated with half the standard dose of TS-1, because the patient's poor general condition necessitated chemotherapy with low toxicity and high efficacy. The daily dose was 40 mg for the first three courses and 50 mg for the last two. Each treatment course consisted of a four-week administration followed by two drug-free weeks. The patient received five courses of chemotherapy at our outpatient clinic before his death from re-progression of liver metastasis. No serious side effect except temporary stomatitis was observed. A decrease in tumor markers, alpha-fetoprotein and carcinoembryonic antigen, was obtained after 4 weeks. After 2 cycles, computed tomography and endoscopy examinations showed regression of the primary tumor and liver metastases, and tumor markers were decreased remarkably. The patient's QOL improved gradually after the treatment. His performance status before the chemotherapy was 3, and improved to 1 after two cycles. The small dosage of TS-1 was effective without any adverse effects, and improved the patient's QOL, for 6 months.

Thyroglobulin gene mutation with cold nodule on thyroid scintigraphy.

Thyroglobulin gene mutation is a rare cause of congenital hypothyroidism, but thyroglobulin gene mutations are thought to be associated with thyroid cancer development. A 21-year-old Japanese man treated with levothyroxine for congenital hypothyroidism had an enlarged thyroid gland with undetectable serum thyroglobulin despite elevated serum TSH level. The patient was diagnosed with thyroglobulin gene mutation, with compound heterozygosity for Gly304Cys missense mutation and Arg432X nonsense mutation. Ultrasonography showed a hypovascular large tumor in the left lobe that appeared as a cold nodule on thyroid scintigraphy. He underwent total thyroidectomy, but pathological study did not reveal findings of thyroid carcinoma, but rather a hyperplastic nodule with hemorrhage. Strong cytoplasmic thyroglobulin immunostaining was observed, but sodium iodide symporter immunostaining was hardly detected in the hyperplastic nodule. The clinical characteristics of patients with thyroglobulin gene mutations are diverse, and some patients are diagnosed by chance on examination of goiter in adults. The presence of thyroid tumors that appear as cold nodules on thyroid scintigraphy should consider the potential for thyroid carcinoma, if the patient has relatively low serum thyroglobulin concentration in relation to the degree of TSH without thyroglobulin autoantibody.

Sjögren's syndrome with multiple cystic lesions and pulmonary arteriovenous fistulae.

A patient presented with Sjögren's syndrome associated with pulmonary multiple cystic lesions and a pulmonary arteriovenous fistulae. A histological examination of the lungs during the autopsy revealed the stenosis of the bronchiole lumens with hyperplasia of goblet cells, proliferation of smooth muscles in the inner wall of the bronchioli and retention of mucus in the airway lumens. These small airway changes were accompanied with chronic inflammatory changes of the airways in Sjögren's syndrome and led to the formation of cystic lesions via a ball-valve mechanism. Arteriovenous fistulae were situated around the cystic lesions. There may have been a correlation between the formation of the fistulae and cysts, but no mechanism was indicated in the histological findings. This report reveals that chronic inflammatory changes of the airways in Sjögren's syndrome are sufficient to cause the formation of cystic lesions.

Thyroid crisis following interstitial nephritis.

A 54-year-old man with Graves' disease had been treated with thiamazole (5 mg/day). His thyroid hormone level was increased after exodontia in February 2006. Although his prescribed dose of thiamazole was increased after exodontia on the fourth day, he developed thyroid crisis on exodontia 52 nd day. Laboratory findings also showed renal dysfunction (from Cr 1.0 mg/dL in July 2005 to Cr 1.8 mg/dL on exodontia 37th day). His thyroid hormone level was normalized after subtotal thyroidectomy; however, serum Cr level was still high. He was diagnosed with interstitial nephritis as a result of renal biopsy, and he was treated with prednisolone 30 mg/day. This present case developed thyroid crisis even though the quantity of thiamazole was increased after exodontia. It seems that interstitial nephritis, as well as exodontia, is an aggravation factor of thyroid function. After a poor response to anti-thyroid drugs, it is necessary to prevent thyroid crisis by determining the aggravating factor and to then provide appropriate treatment.

Preclinical Cushing's syndrome resulting from adrenal black adenoma diagnosed with diabetic ketoacidosis.

A right adrenal tumor was incidentally discovered on abdominal computed tomography performed on a 53-year-old Japanese man, who had been hospitalized with diabetic ketoacidosis. Normal values were obtained for adrenal hormones in the morning after an overnight fast and urinary cortisol excretion after treatment of diabetic ketoacidosis with insulin. However, overnight dexamethasone administration with 1 mg or 8 mg did not completely suppress serum cortisol levels. There were no remarkable physical findings related to Cushing's syndrome. The patient was diagnosed as having preclinical Cushing's syndrome (PCS). Histological examination of the adrenalectomy specimen demonstrated adrenal black adenoma. Blood glucose levels subsequently improved after adrenalectomy, and the patient never developed adrenal insufficiency after hydrocortisone withdrawal. The patient was treated with diet therapy alone, and maintained good glycemic control. However, the patient still showed a diabetic pattern in an oral glucose tolerance test. It seems that the existence of PCS in addition to the underlying type 2 diabetes mellitus contributed to aggravation of blood glucose levels. Although there are many aspects of the natural course of PCS that have not been thoroughly elucidated, it is necessary to remain aware that a PCS patient with abnormal glucose metabolism may develop diabetic ketoacidosis by environmental agents.

Remission of IgA nephropathy after allogeneic peripheral blood stem cell transplantation followed by immunosuppression for acute lymphocytic leukemia.

We report a case with immunoglobulin A (IgA) nephropathy, showing IgA deposition which disappeared after peripheral blood stem cell transplantation (PBSCT) for acute lymphocytic leukemia (ALL). In 1996, a 28-year-old man was diagnosed with IgA nephropathy by renal biopsy. Steroid therapy improved proteinuria from 3 g/day to 1 g/day. In 2003, he received PBSCT following the initial therapy for ALL. After complete remission, urinary protein and hematuria remained at between (-) and (+/-). In 2004, the second renal biopsy specimen revealed no deposit of IgA or C3. These findings suggested that immune reconstruction with PBSCT following immunosuppression therapy was of benefit to IgA nephropathy.

Breast cancer with endocrine differentiation: report of two cases showing different histologic patterns.

We report two cases of breast cancer with endocrine differentiation. Case 1 was a 56-year-old woman with a 2-cm tumor in the upper outer quadrant of the right breast and right axillary lymphadenopathy. Excisional biopsy suggested carcinoma and we performed breast-conserving surgery with lymph node dissection. Histologic examination revealed breast cancer with endocrine differentiation resembling small cell carcinoma of the lung, with one nodal metastasis. Case 2 was a 71-year-old woman with a 2.5-cm tumor in the upper outer quadrant of the right breast. Aspiration cytology suggested carcinoma and we performed mastectomy with lymph node dissection. Histologic examination revealed a carcinoid tumor, as one of the breast cancers with endocrine differentiation, but no nodal metastasis. The two patients are now disease-free 26 and 12 months after surgery, respectively.

Myxoid malignant fibrous histiocytoma of the breast: report of a case.

Malignant fibrous histiocytoma (MFH) is the most common type of soft tissue sarcoma, but it rarely develops as a primary tumor in the breast. Furthermore, no case of the myxoid variant of MFH in the breast has ever been documented. We report the case of a 52-year-old woman with a breast tumor that was immunohistochemically confirmed to be myxoid MFH. She underwent a radical mastectomy and is currently well with no evidence of local recurrence or metastatic spread after 3 years of follow-up.

Characteristics of the pathological images of coronary artery thrombi according to the infarct-related coronary artery in acute myocardial infarction.

BACKGROUND: Unstable plaque and coronary arterial thrombi sometimes induce a no-reflow phenomenon after intervention whereby there is sufficient reperfusion. The greater susceptibility of the right coronary artery to development of large thrombi makes successful reperfusion more difficult, therefore the characteristics of the pathological images of coronary arterial thrombi according to the infarct-related coronary artery were investigated. METHODS AND RESULTS: Coronary arterial thrombi were extracted from 77 patients with acute myocardial infarction (AMI) using a thrombectomy catheter. The 36 patients had a thrombus containing atherosclerotic cells. Platelets, fibrin, and neutrophils were seen in all cases. The mean ratios of structural components of thrombi were 51.0 +/- 29.5% (mean +/- SD) of the platelet component, 19.9 +/- 25.7% of the erythrocyte component and 11.9 +/- 22.5% of atherosclerosis component. Erythrocyte-rich thrombi and mixed thrombi mainly composed of erythrocytes were seen in 14 of the 30 cases involving the right coronary artery, 6 of the 35 cases in the left anterior descending artery, 2 of the 11 cases of the left circumflex artery, and in the 1 case of saphenous vein bypass graft. There was significantly more erythrocyte component in the thrombi from the right coronary artery (28.7 +/- 30.1%) than in those from the left coronary artery (12.1 +/- 18.4%). CONCLUSION: Coronary artery thrombi in AMI are composed principally of platelets. Atherosclerotic cells were identified within thrombi from some patients. In the right coronary artery there were many more thrombi that were rich in erythrocytes than in thrombi from the left coronary artery.

Lymphoplasmacytic sclerosing pancreatocholangitis successfully treated by pancreatoduodenectomy.

A 68-year-old man complaining of jaundice was admitted to our hospital in October 1996. Radiological imaging studies, including dynamic computed tomography, endoscopic retrograde cholangiography, and angiography, were highly suggestive of pancreatic head cancer, and laparotomy was performed on October 25, 1996. On gross examination, the pancreas appeared firm, as in chronic pancreatitis, with a mass lesion in the pancreatic head measuring 35 x 35 x 25 mm. A pylorus-preserving pancreatoduodenectomy was carried out. Histological findings were characterized by uniform fibrosis with diffuse lymphoplasmacytic infiltration and lymph follicles in the thickened wall of the bile duct and in and around the pancreas, with acinar atrophy. The histological diagnosis was lymphoplasmacytic sclerosing pancreatocholangitis. Approximately 5 years postoperatively, the patient was alive and well without adjunctive corticosteroid therapy. This positive result suggests that pancreatoduodenectomy may be effective for lymphoplasmacytic sclerosing pancreatocholangitis that is localized in the pancreatic head.