Akio Uchiyama

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.

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.

Myxoid Malignant Fibrous Histiocytoma of the Breast : Report of a Case

Abstract.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.

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.

Pulmonary tuberculosis with atypical histopathological manifestations

This report presents a case of pulmonary tuberculosis with atypical histopathological manifestations in an immunocompetent patient. A 37-year-old Japanese man was admitted due to multiple small nodules on chest computed tomography (CT). He was diagnosed with pulmonary tuberculosis following a culture of acid-fast bacterium from suction sputum specimens obtained by bronchoscopy. The histopathological findings from video-assisted thoracoscopy revealed small, sporadically organized, and fibrotic lesions with infiltration of eosinophils, plasma cells, and lymphocytes. The administration of antitubercular drugs eliminated the abnormal shadows on chest CT. Extreme care must be taken in the diagnosis of a patient with inexplicable histopathological findings.

Prostatic Stromal Tumor of Uncertain Malignant Potential Which Was Difficult to Diagnose

Here, we report a case of stromal tumor of uncertain malignant potential (STUMP) that was difficult to diagnose. A 53-year-old male was found to have a hard nodule on digital rectal examination; magnetic resonance imaging revealed a large nodule on the left side of the prostate, indicating prostate cancer. However, pathological diagnosis of the biopsy specimen was benign prostatic hyperplasia. Although a papillary tumor in the prostatic urethra was also seen on urethrocystoscopy, the tumor specimen obtained from transurethral resection was not malignant. The tumor in the prostatic urethra recurred only 3 months after transurethral resection, and pathological findings revealed benign hyperplasia not only in the stromal tissue but also in the epithelium; therefore, the prostate tumor was suspected to be STUMP. It took many prostate pathologists a long time to reach the final diagnosis of STUMP. STUMP is a rare benign tumor, difficult to diagnose, and sometimes transforms into stromal sarcoma. Thus, we should consider radical resection in such cases.