Shigeru Kuramochi

Glomangiosarcoma in a glomus tumor. An immunohistochemical and ultrastructural study

An extremely rare case of glomangiosarcoma (GS) occurring in a glomus tumor (GT) was evaluated ultrastructurally and histochemically. A man 65 years of age who was suffering from back pain underwent resection of a deep cutaneous nodule. Cells of a solid type GT showed numerous subplasmalenimal pinocytotic vesicles, thin filaments with scattered dense bodies, and thick external lamina, but negative desmin staining and a lack of glycogen. Similar findings also were observed in the GS, but were less obvious. The GS compressed the surrounding GT, exhibited many mitotic figures, prominent nucleoli, elongated nuclei and cytoplasm, and reacted more strongly to vimentin staining than the GT. The GT contained S‐100 protein‐positive Schwann cells, a few substance P‐positive nerve fibers, and moderate numbers of infiltrating mast cells. None of these findings were observed in the GS. Results were consistent with the view that GS was transformed possibly from the GT, and that the good prognosis for GS may be due to its small size that may be related to the preexistence of a pain‐causing GT.

Association of a Novel 3-Amino Acid Deletion Mutation of Apolipoprotein E (Apo E Tokyo) with Lipoprotein Glomerulopathy

Lipoprotein glomerulopathy (LPG) is a newly recognized renal disease characterized by abnormal lipoprotein deposition in the glomeruli, dysbetalipoproteinemia, and a high level of plasma apolipoprotein (apo) E. We identified a novel apo E mutation in a 56-year-old Japanese male with LPG. Although the plasma cholesterol and triglyceride levels were normal, the levels of intermediate-density lipoprotein cholesterol and apo E were elevated to 13 mg/dl (0.336 mmol/l; 4.2±2.9 mg/dl, mean ± SD, in 12 normolipidemic controls) and 9.2 mg/dl, respectively. Biochemical analysis revealed an unusual apo E phenotype (E1/3). Apo E genotyping using DNA digested by a restriction enzyme (HhaI) identified a 66-bp fragment which was not seen with any of the common alleles. Sequence analysis of the amplified genomic DNA fragments showed a 9-bp deletion in exon 4 of the apo E gene resulting in a 3-amino acid deletion (residues 141–143). This novel mutation involves the region of the apo E molecule known to be critically involved in binding to its receptor, and this may well transform the apo E molecule, an inefficient ligand, to its receptor(s). How this mutations causes glomerular damage remains to be determined.

A hybrid carcinoma: adenoid cystic carcinoma and salivary duct carcinoma of the salivary gland. An immunohistochemical study.

Abstract Hybrid tumours of the salivary glands are very rare entities composed of two different tumours, each of which conforms with an exactly defined category. We describe an unusual hybrid carcinoma of the palate; it was comprised of an adenoid cystic carcinoma and a salivary duct carcinoma with a transitional region. These two different compartments showed different characteristics as regards cellular differentiation, proliferative activity, and expression of oncogene and tumour suppressor oncogene proteins, as revealed by using markers for muscle actin, keratin, vimentin, S-100 protein, GFAP, Ki-67, p53, and c-erbB-2 proteins. This case is the first reported with overexpression of p53 and c-erbB-2 proteins in the tumour entities. Salivary gland tumours consist of heterogeneous histological groups, and each has morphological diversity. This case indicates that some of the oncogene and tumour suppressor oncogene proteins may help to produce the histological heterogeneity of the salivary gland tumour.

Systemic Lupus Erythematosus Associated with Multiple Nodular Hyperplasia of the Liver

Three autospy cases of systemic lupus erythematosus with uinique association of multiple nodular hyperplasia (MNH) of the liver, portal hypertension, and hypertensive pulmonary vascular disease are reported. None of the patients had received oral contraceptive or androgenic steroid, but they were treated with glucocorticoids for 2 to 11 years. Raynauds phenomenon, sclerodactyly, and mild impairment of the kidney were the common clinical features. Macroscopically, MNH is characterized by many nodules scattered throughout the non‐cirrhotic liver, and histologically, each nodule is made up of normal‐appearing hepatocytes and not encapsulated. Portal tracts are scanty in the nodules. MNH seems to be a regenerative‐hyperplastic process, but its true nature still remains unclear. Relationships between MNH and portal hypertension, MNH and pulmonary hypertension, and collagen disease and pulmonary hypertension are discussed. A brief review of the literature concerning multiple benign hepatocellular tumors similar to MNH is also presented. ACTA PATHOL. JPN. 32: 547∼560, 1982.

ASSESSMENT OF HISTOLOGICAL FEATURES AND OUTCOME OF INTERFERON THERAPY IN CHRONIC HEPATITIS C

The correlation between the histological features of liver biopsy specimens before interferon (IFN) treatment and the clinical effect of IFN administration on chronic hepatitis C was investigated. A study of the relation between several histological features that were graded in 60 liver biopsy specimens from chronic hepatitis C patients before IFN treatment disclosed that the grade of portal fibrosis was positively correlated with the grade of other inflammatory features, including piecemeal necrosis and portal and lobular inflammation. The degree of portal fibrosis adversely affected the rate of normalization of ALT levels in chronic hepatitis C during and after IFN treatment. We reexamined 36 liver biopsy specimens that showed a moderate degree of portal fibrosis, and found that the degree of piecemeal necrosis was inversely correlated with the extent of lymphoid follicle formation in the portal tracts. During IFN therapy, the group of chronic hepatitis C patients who showed marked piecemeal necrosis and less lymphoid follicle formation in the liver specimens had a poor response to IFN treatment, whereas another group that showed marked lymphoid follicle formation and little piece-meal necrosis in the liver specimens had a good response to IFN. These relationships gradually disappeared after the completion of IFN treatment.

Takayasu's aortitis with dissection in systemic lupus erythematosus

A forty-seven-year-old Japanese woman under treatment for systemic lupus erythematosus (SLE), complained of severe back pain. Chest X-ray and MRI showed an aneurysmal dilatation of the ascending aorta. Subsequently an aortic replacement was performed. Microscopically, the resected aorta showed Takayasus aortitis with chronic dissection. Both aortitis and dissection are rare events in SLE patients. To our knowledge, this is the first report of Takayasus aortitis with dissection in a patient with SLE.

Multiple pulmonary hyalinizing granulomas associated with systemic idiopathic fibrosis.

A 41 year‐old man with progressive nodular infiltration of the lung of about 2 years’duration died of cardiac and respiratory failure. Autopsy revealed bilateral multiple pulmonary hyalinizing granulomas (PHGs) diagnosed on the basis of the characteristic dense hyaline collagen bundles with nonspecific inflammatory infiltration. Constrictive pericarditis, retroperitoneal fibrosis, mediastinal fibrosis, fibrous thickening of the peritoneal and pleural surfaces, and fibrosis of soft tissue of the neck, flank, and hepatic hilar region were present, therefore, a diagnosis of systemic idiopathic fibrosis was made. The patient had anti thyroglobulin and anti‐thyroid microsomal antibodies and lymphocytic thyroiditis. The inflammatory process of PHG of the present case was active and the clinical course was progressive. PHG seems to be a lesion belonging to the systemic idiopathic fibrosis complex. Immunologic abnormalities may be related to PHG and systemic idiopathic fibrosis.

Secretory carcinoma of the breast in a 51-year-old male

Approximately 100 female secretory Carcinoma cases have been reported. Although thls tumor was Initially termed juvenile carcinoma as the patients were all children and adolescent females, subsequent reports demonstrated that it occurs in females of all ages. Moreover, to date, only five males with this tumor have been reported. As with the initial female secretory carcinoma cases, all five were children or young adutts. A very rare case of non‐invasive secretory carcinoma of the breast arising in a 51‐year‐old Japanese male Is described. He presented with a lump in his left breast. The surgically resected tumor was a typical secretory carcinoma histologically, except that there was no Infiltration of the surrounding stroma, and was composed of tumor cells with vacuolated cytoplasm that contained secretory materials. This case, the first recorded secretory carcinoma of the breast in a middle‐aged male, demonstrates that this tumor may also arise in mature males, as is the case in females. Physicians should not rule out the possibility of a secretory carcinoma of the breast regardless of patient age and gender.

Two cases of ectopic hamartomatous thymoma.

Abstract. Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm. Since it was named by Rosai et al. in 1984, 24 cases have been reported. We herein report two cases of EHT, one of which presented with massive myoid cells, and review the literature related to EHT. Both of our cases displayed the typical features of EHT: (1) nests of epithelial cells, including solid, cystic, or glandular epithelial islands; (2) spindle cells dominating the microscopic picture; and (3) adipose cells which intermingle haphazardly to impart a hamartomatous quality to the tumor. In this paper, we observed massive myoid cells and the transition from spindle epithelial cell to myoid cell in one of our cases. Immunohistochemical examinations showed that the main component of EHT, spindle cells, was positive for cytokeratin and epithelial membrane antigen (EMA). Intriguingly, the myoid cells simultaneously expressed cytokeratin, EMA, myoglobin, and creatine kinase-mm, suggesting that myoid cells may originate from epithelial cells and are an intermediate state between epithelial cells and muscular cells.

Vasculitis associated with septicemia: case report and review of the literature.

Abstract. We report an unusual case in which infectious endocarditis presented systemic vasculitis and glomerulonephritis as the initial manifestation of the disease. The patient was a 16-year-old girl with congenital cyanotic heart disease who presented with skin purpura, proteinuria, and hematuria. She had hypergammaglobulinemia, cryoglobulinemia, and positive circulating immune complexes. Renal biopsy revealed crescentic glomerulonephritis. Her serum C3 level, which was initially normal, became decreased, and prednisolone and azathioprine were administered with a tentative diagnosis of systemic lupus erythematosus (SLE). Soon after, she developed fever and renal failure. Blood culture grew Streptococcus pyogenes, and the diagnosis of infectious endocarditis was made. Eight cases of systemic vasculitis and glomerulonephritis associated with infectious endocarditis have been described in the literature. Infectious endocarditis should be included in the differential diagnosis of systemic vasculitis and glomerulonephritis.