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Solitary fibrous tumors (SFTs) are rare mesenchymal tumors that can occur at any location. Since the identification of specific NAB2-STAT6 fusion in SFTs, the fusion gene variants, NAB2 exon 4-STAT6 exon 2/3 and NAB2 exon 5/6/7-STAT6 exon 16/17/18, have been reported to be associated with clinicopathological features, and the latter variant is predominant in meningeal SFTs. SFTs developing in the salivary glands are rare, and more rarely, those involving ectopic salivary glands (ESGs) have been reported in the cerebellopontine angle (CPA); however, their characteristics remain not well understood. In this study, we performed a clinicopathological and molecular analysis of 3 cases of meningeal SFT with ESGs. All cases presented with an extra-axial mass in the CPA, which is a rarer location for intracranial ESGs compared to the sellar region. Histologically, except for the presence of ESGs, there was no significant difference between current cases and ordinary SFTs. The ESGs demonstrated no cellular atypia, and although the spindle tumor cells were immunopositive for STAT6, the ESGs were negative in all cases, supporting that the ESGs are non-neoplastic components. In 1 case, ESGs were found only in the primary tumor and disappeared in recurrence/dissemination. Of note, molecular analysis identified NAB2 exon 4-STAT6 exon 2 in all cases. In conclusion, our results suggest that ESGs particularly in the CPA may be associated with SFTs and that meningeal SFTs with ESGs may be associated with the minor fusion variant of NAB2-STAT6 in the intracranial lesions.

Background. The occurrence of idiopathic (primary) membranous glomerulonephritis (MGN) is relatively uncommon in childhood. Although a proportion of children with idiopathic MGN may show isolated antinuclear antibody (ANA) positivity, its clinical impact remains unclear. Methods. During the past 12 years, we have treated six children with idiopathic MGN at our institution. Of these, three children with isolated ANA positivity were retrospectively evaluated. Results. The patients consisted of two boys and a girl, aged 5, 12, and 13 years at presentation, respectively. They showed proteinuria of around 1 g/day with hematuria, and isolated ANA positivity without anti-DNA antibody or hypocomplementemia. A percutaneous renal biopsy revealed MGN stage II in two of the children and stage III in one, without endothelial tubuloreticular inclusion. Two children received a 6-month course of prednisolone therapy. At the latest observation (mean interval of 42 months), all showed negative for proteinuria, and no patients had progressed to systemic lupus erythematosus (SLE). A decrease in the ANA titer with subsidence of urine abnormalities was observed in two patients. Conclusion. Although the long-term prognosis of ANA-positive idiopathic MGN in childhood remains unclear, isolated ANA postivity itself may be nonspecific, and may not indicate a subsequent progression to SLE.

Two Japanese sisters with persistent uveitis showed significant increased levels of urinary beta-2 microglobulin. A percutaneous renal biopsy performed in the younger sister revealed tubulointerstitial nephritis (TIN) with helper/inducer T cell infiltrates. Also, abnormal 67-gallium accumulation in the kidneys, suggesting TIN, was observed in the other one at the same time. Although patients with the syndrome of tubulointerstitial nephritis and uveitis (TINU) have been reported to date, its occurrence in siblings has rarely been seen. Both of them shared same human leukocyte antigen (HLA) DR6, suggesting the potential association between HLA-DR6 and TINU.

A 9-year-old boy was referred to our hospital because of significant hematuria and proteinuria associated with hypocomplementemia. Although he had had a 3-year history of asymptomatic persistent proteinuria detected by urine screening of school children, he did not visit a physician. Finally, he visited a regional hospital, and hypocomplementemia was noted there. A percutaneous renal biopsy performed hospital day 4 revealed diffuse endocapillary proliferative glomerulonephritis with severe tubulointerstitial changes. Although his hematuria and hypocomplementemia spontaneously subsided within a month, proteinuria remained. A renal biopsy performed 4 months after the first renal biopsy revealed the lesion suggesting advanced focal segmental glomerulosclerosis (FSGS). Despite corticosteroid treatment, his proteinuria persisted, and he developed end stage renal failure. These clinical observation indicated that he had acute glomerulonephritis (AGN) superimposed on non-nephrotic FSGS, and that the episode of AGN might cause exacerbation of the FSGS.

小児科一般診療での微小変化型ネフローゼ症候群 (MCNS) に対する免疫抑制剤mizoribine (MZB)，ciclosporine (CsA) を用いた免疫抑制療法の現状を明らかにするために，当科で治療管理中のMCNS例で経過中にMZB，CsAを使用した7例を臨床的に検討した。対象は男児5例，女児2例でMCNS発症年齢は2歳3カ月から8歳5カ月 (平均5歳2カ月)，MZB，CsAが開始された年齢は3歳8カ月から11歳9カ月 (平均7歳2カ月) であった。病型は6例がステロイド依存性，1例が抵抗性であり，4例では既にcyclophosphamideが使用されていた。依存性の6例では先ずMZBが使用され，2例 (33.3%) で再発回数，ステロイド剤の減量効果を認めた。4例では無効と判断され，2例は azathioprine，2例はCsAで治療された。抵抗性の1例はCsAが使用され，CsA投与の3例ではいずれも臨床的に有効であった。MZB，CsAの臨床応用は一般診療でのMCNSの病勢管理を行い易くしつつある。

We report a case of primary low-grade lymphoma of mucosa-associated lymphoid tissue (MALT) arising from the kidney in a 30-year-old man with an abdominal mass in the right flank detected by ultrasonography. Radical nephrectomy was performed under a preliminary diagnosis of renal cell carcinoma. The final histological diagnosis was MALT-type lymphoma. To the best of our knowledge, only five reports of primary MALT-type lymphoma in the kidney have been published in the literature so far. All cases were surgically treated and only one case went on to chemotherapy postoperatively.

Fibrolamellar carcinoma (FLC) is a variant of hepatocellular carcinoma characterized by distinct pathological features. The presence of intracellular lumina resembling bile canaliculi was previously reported in tumor cells of FLC on electron microscopy. Using light microscopy, we describe the presence of intracellular lumina in FLC, which was resected from a 15-year-old Japanese girl, as round structures lined with a brush-like border. These lumina occasionally contained bile. Light microscopic examination of 1 micron thick serial sections of Epon-embedded tissue samples showed that the lumina were located in the intracellular space without any connection to the intercellular space. However, we also detected a small number of lumina that were lined by microvilli, which were present between adjacent tumor cells. Results suggest that the presence of the intracellular lumina in tumor cells probably represents a common histopathologic feature of FLC.

Adenomyoepithelioma (myoepithelioma) of the breast in a 47-year-old man is reported. The tumor consisted of a prominent proliferation of spindle cells surrounding mammary ducts. Immunohistochemical and electron microscopic observations confirmed the myoepithelial origin of these spindle cells. This is the first report of an adenomyoepithelioma of the breast that developed in a male.

An 11-month-old girl was referred to our hospital because of nausea and poor physical activity. She had a 5-month history of infantile spasms, which were successfully treated with valproic acid (VPA) and vitamin B6. Laboratory studies revealed hyperchloremic metabolic acidosis, mainly due to distal type renal tubular acidosis (RTA). Although a renal biopsy, performed 2 months after the onset of RTA, did not demonstrate tubulointerstitial lesions, her clinical course, in which administration of VPA led to an episode of RTA, with gradual subsidence on VPA removal, suggested that the probable causative agent of her distal type RTA was VPA. Although proximal type RTA associated with VPA administration has been reported, distal type is rarely seen. To our knowledge, a similar case has not been reported previously.

Patient reportA 10-year-old, previously well girl was referred to ourhospital because of hematuria, proteinuria and peripheraledema after an episode of pneumonia. Since about 2 weekspreviously, she had been suffering from moderate fever,cough and fatigue. She visited a physician and wasdiagnosed as having pneumonia. A week-long course ofcefdinir (CFDN) therapy was not effective. Thus, mino-cycline (MINO) was started and resulted in improvement ofher clinical condition. After a 1-week course of MINO, shewas noted to have a puffy face and significant hematuria andproteinuria without oliguria. On admission, her bodytemperature was 36.8°C and blood pressure was140/90 mmHg. Her bodyweight was 30.5 kg, which wasretrospectively found to be 1.5 kg more than her disease-freeweight. No skin or mucosal lesions were observed. Physicalexamination was unremarkable except for peripheral edema.The chest x-ray still showed left lower lobe pneumonia.Urinalysis showed a specific gravity of 1.020, protein of154 mg/dL, 30 red blood cell sediments per high-poweredfield and a few granular casts with a sterlie culture.Laboratory studies showed the following values: white bloodcells, 5950 /µL; hemoglobin, 10.6 g/dL; hematocrit, 33%;platelets, 217 000 /µL; serum total protein, 6.7 g/dL;albumin, 3.6 g/dL; total cholesterol, 129 mg/dL; ureanitrogen, 10 mg/dL; creatinine, 0.62 mg/dL; sodium,143 mEq/L; potassium, 4.3 mEq/L; chloride, 110 mEq/L;calcium, 8.6 mg/dL; C-reactive protein, 0.64 mg/dL anderythrocyte sedimentation rate 45 mm/h. No pathogenicbacteria were isolated from her throat and sputa cultures.The results of immunologic examinations were as follows:IgG, 2436 mg/dL; IgA, 214 mg/dL; IgM, 310 mg/dL; anti-nuclear antibody (ANA), 1:80 with a homogenous pattern;anti-DNA antibody, less than 1.0 IU/mL (radioimmunoassay(RIA)); anti-streptolysin 0 (ASO), 1:160, anti-streptokinase(ASK), 1:1,280 and anti

We report a case of primary low-grade lymphoma of mucosa-associated lymphoid tissue (MALT) arising from the kidney in a 30-year-old man with an abdominal mass in the right flank detected by ultrasonography. Radical nephrectomy was performed under a preliminary diagnosis of renal cell carcinoma. The final histological diagnosis was MALT-type lymphoma. To the best of our knowledge, only five reports of primary MALT-type lymphoma in the kidney have been published in the literature so far. All cases were surgically treated and only one case went on to chemotherapy postoperatively.

A Japanese boy aged 9 years referred to our hospital because of steroid-resistant proteinuria. He had a 6-year history of unremitting proteinuria and was diagnosed as having minimal-change disease (MCD) by the repeated renal biopsies performed at the age of 3.5 years and 8.5 years, respectively. His proteinuria fluctuated ranging from 115 mg/100 ml to 645 mg/100 ml, and serum total protein ranged from 59 g/liter to 63 g/liter. The third renal biopsy at the presentation also revealed MCD. Thereafter he was treated with an anti-thrombocyte agent combined with an angiotensin converting enzyme inhibitor. Despite unremitting proteinuria of long duration, he did not have any complaints. At the age of 11.5 years, severe tubulointerstitial lesion was observed in the fourth renal biopsy. The fifth renal biopsy 6 months after the fourth finally revealed the lesion of focal segmental glomerulosclerosis (FSGS). Although the interpretation of his repeated renal biopsies were considered to be limited, these clinical observation suggested that his unremitting proteinuria of long duration might have been attributed to subsequent progression to FSGS.

Lipoblastoma in the prepatellar region was observed in a 7-month-old girl. The diffuse (lipoblastomatosis) and circumscribed forms have different radiological appearances. Lobular proliferation of fatty tissue with fibrous septae is characteristic of the circumscribed form.

A boy aged 3 years 8 months with Henoch-Schonlein purpura (HSP) developed significant proteinuria with hematuria 2 days after the appearance of purpura rash. Although thought to be purpura nephritis, a percutaneous renal biopsy revealed diffuse mesangial proliferative glomerulonephritis (MesPGN) without deposition of immunoglobulin A or complement. Since his urine screening test during a health check at the age of 3.5 years had been unremarkable, HSP might have played a role in the pathogenesis of his non-IgA MesPGN. To our knowledge, non-IgA MesPGN is an uncommon renal manifestation of HSP.

An 8-year-old girl with hypergammaglobulinemia showed an abnormal 67gallium accumulation in the orbits and parotid glands. Although she did not have any subjective siccant complaints, reported typical histopathological and sialographic changes suggesting Sjogren's syndrome (SjS) were observed in the salivary glands. Gallium scintigram might be a valuable and non-invasive diagnostic tool in the diagnosis of children with SjS without sicca symptoms.

An autopsy case of a 10-day-old newborn with generalized infection of Coxsackie virus B3 (CBV3) was reported. CBV3 was isolated from the blood before death. The patient died of cardiac failure. An immunofluorescent study was carried out on autopsy specimens fixed in formalin and embedded in paraffin. CBV3 antigen was detected in the heart, brain, kidney, lungs, spleen, thymus, and pancreas. In the pancreas CBV3 antigen was predominantly seen in the islet cells. No CBV3 antigen was found in the liver and adrenal glands. Electron microscopic examination revealed virion-like particles, 20 nm in diameter, in the endothelial cells of the myocardium.

Immunofluorescent and electron-microscopic studies were performed to determine the distribution of viral antigens and particles and to clarify the relationship to myocardial lesions in two autopsy cases with generalized infection of Coxsackie virus B3 (CVB3) or cytomegalovirus (CMV). Case 1 was a full-term newborn female infant, without any congenital anomalies, who died of cardiac failure 10 days after birth. CVB3 was isolated from the blood before death. Necrosis of the muscle fibers was observed, frequently accompanying calcification. Numerous histiocytes and a few lymphocytes and neutrophils had infiltrated in and around the necrotic areas. Immunofluorescent study (IF) revealed CVB3 antigen in the muscle fibers and vascular endothelial cells. Case 2 was a female infant, born at 28 weeks of gestation, who died of fatal arrhythmia 50 days after birth. The infant had hemocephalus and a history of idiopathic respiratory distress and underwent an operation for patent ductus arteriosus. Cytomegalic cells were frequently found in the vascular endothelial cells in the myocardium and occasionally in muscle fibers. IF showed the presence of CMV antigen in both endothelial cells and muscle fibers. CVB3 and CMV antigens were detected predominantly in vascular endothelial cells rather than in the muscle fibers. Blood flow disturbance due to endothelial damage is a cause of the myocardial lesion in addition to the direct invasion of the muscle fibers by the virus.

Secretory carcinoma and mucinous carcinoma were found to coexist in the breast of a 67-year-old post-menopausal woman, although the tumors were separated by a thin fibrous septum. Histochemically, intra- and extracellular secretory materials in both carcinomas were strongly positive for alcian blue, PAS and mucicarmine staining, but immunohistochemically negative for alpha-lactalbumin and CEA. Membrane-bound intracytoplasmic vacuoles showing emiocytosis were observed in both the secretory and mucinous carcinomas by electron microscopy. No differences were observed between the tumor cells of secretory carcinoma and those of mucinous carcinoma by histochemistry, immunohistochemistry and electron microscopy. However, there were definite statistically significant differences in the results of morphometry of tumor cell nuclei. Secretory carcinoma is considered to be an anaplastic variant type of mucinous carcinoma.

Light and electron microscopic examinations were performed on two autopsy cases of ceroid-lipofuscinosis of the juvenile (Case 1) and late infantile (Case 2) types. Much ceroid-lipofuscin (CL) was found in nerve cells throughout the nervous system. In Case 1, CL had also accumulated in thyroid follicular cells, glomerular podocytes, and epithelial cells of the ductus epididymidis, and in the endothelium and smooth muscle of vessels. Electron microscopy showed CL in 5% of peripheral lymphocytes sampled when the patient was alive. In Case 2, an accumulation of CL was found in the vascular endothelial cells of the cerebrum, and Kupffer cells and sinusoidal endothelial cells of the liver. The CL was autofluorescent, and was seen to be composed of electron-dense granules, lipid droplets, lamellar structures, and curvilinear bodies by electron microscopy. Limiting membranes were often found surrounding CL granules. The dolichol level in the cerebral cortex was high in Case 1. Accumulation of CL was found in cells other than nerve cells, although the main signs and symptoms were caused by the involvement of nerve cells. The CL showed various ultrastructural features. ACTA PATHOL JPN 38: 1191∼1203, 1988.

Two sisters with juvenile metachromatic leukodystrophy are described. The patients were 17 and 20 years old. The younger sister died and an autopsy was perfomed. The elder sister keeps alive. A sural nerve biopsy of both cases revealed an accumulation of metachromatic lipid granules in the Schwann cells and macrophages. The autopsy also disclosed these granules especially in the brain, gallbladder, kidney and pancreas. A lipid analysis of the cerebral white matter showed sulfatide accumulation that was 1.5 times that of controls. Histochemically, the accumulated lipid was different in the brain from that in other organs. An electron microscopic examination of the accumulated metachromatic lipid granules showed various structures such as concentric lamellar, tuffstone, herringbone and hexagonal honeycomb appearances, and some ultrastructural differences between the nervous system and other organs. ACTA PATHOL JPN 38: 1041∼1051, 1988.