[Two cases of microscopic polyangitis with preceding interstitial pneumonia diagnosed by surgical lung biopsy].

We encountered 2 cases of microscopic polyangitis (MPA) with preceding interstitial pneumonia. The patients were elderly men, and radiological findings showed a UIP pattern in both cases. Furthermore, ANCA-associated vasculitis had been suspected since the first admission in case 1, because MPO-ANCA tests were positive, and the surgical lung biopsy revealed vasculitis in a honeycomb lesion. A diagnosis of idiopathic pulmonary fibrosis was made in case 2 because no findings of vasculitis were pathologically confirmed, and MPO-ANCA was not examined. Both patients were followed up without any treatment, but steroid therapy with immunosuppressants was initiated after a diagnosis of MPA was made from a renal failure complication. Remission continued in case 1, but patient 2 died due to a pulmonary hemorrhage complication, despite some temporary improvements. An autopsy revealed massive alveolar hemorrhage and neutrophil infiltration in the alveolar walls that suggested pulmonary capillaritis, in spite of the absence of pathological evidence, which was not detected in the honeycomb lesions. These 2 cases indicate that MPO-ANCA should be examined in the follow-up of chronic interstitial pneumonia. Furthermore, it should be assumed that the size of involved blood vessels, their distribution and the timing of occurrence may be different in individual case of ANCA-associated interstitial pneumonia.

Clinicopathological characteristics of primary gastric T-cell lymphoma.

AIMS: To investigate the clinicopathological characteristics of 20 primary gastric T-cell lymphoma (GTCL) cases without human T-lymphotropic virus type I infection in Japan, a non-endemic area for coeliac disease. METHODS AND RESULTS: Fifteen cases had no history of persistent diarrhoea or severe hypoproteinaemia. Histologically, 13 cases (65%) consisted of large cell lymphoma and seven (35%) were of medium-sized cells. Intraepithelial lymphoma cell invasion was found in three cases (15%). Two of 10 surgical cases (20%) showed intramucosal tumour cell spreading with enteropathy-like features. Helicobacter pylori CagA gene was detected in three of 10 cases (30%). The lymphoma cells of all 20 cases were positive for CD3 and/or TCRbetaF1 and negative for CD56. CD4- and CD8- lymphoma was found in 11 cases (55%), CD4+ lymphoma in seven (35%) and CD8+ lymphoma in two (10%). CD30+, CD5+ and CD25+ lymphomas were detected in nine (45%), 10 (50%) and 11 (55%) cases, respectively. Five-year survival of the 16 available cases was 54%. Early clinical stage and medium-sized cell lymphoma were significantly (P < 0.05) better prognostic factors. CONCLUSIONS: Patients with GTCL exhibit distinct clinicopathological findings and prognoses from those with enteropathy-associated T-cell lymphomas. GTCL may be mainly derived from lamina propria and parafollicular T cells.

Myeloperoxidase antineutrophil cytoplasmic antibody-associated vasculitis with diffuse tubulointerstitial nephritis.

Renal involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is characterized by focal segmental crescentic and/or necrotizing glomerulonephritis. Here, we report the case of a 66-year-old woman showing myeloperoxidase (MPO)-ANCA positivity and mononeuritis multiplex whose kidney biopsy revealed severe and diffuse tubulointerstitial nephritis despite the fact that crescentic necrotizing glomerulonephritis was focal. The mechanism of tubulointerstitial injury in ANCA-associated vasculitis remains unclear. Further studies are necessary to confirm the relationship between diffuse tubulointerstitial nephritis and ANCA-associated vasculitis.

Amiodarone-related pulmonary mass and unique membranous glomerulonephritis in a patient with valvular heart disease: Diagnostic pitfall and new findings.

Amiodarone is an anti-arrhythmic drug for life-threatening tachycardia, but various adverse effects have been reported. Reported herein is an autopsy case of valvular heart disease, in a patient who developed a lung mass (1.5 cm in diameter) and proteinuria (2.76 g/day) after treatment with amiodarone for a long time. The lung mass was highly suspected to be lung cancer on CT and positron emission tomography, but histologically the lesion was composed of lymphoplasmacytic infiltrates in alveolar walls and intra-alveolar accumulation of foamy macrophages containing characteristic myelinoid bodies, indicating that it was an amiodarone-related lesion. In addition, the lung tissue had unevenly distributed hemosiderin deposition, and abnormally tortuous capillaries were seen in the mass and in heavily hemosiderotic lung portions outside the mass. In the kidneys, glomeruli had membrane spikes, prominent swelling of podocytes and subepithelial deposits, which were sometimes large and hump-like. Autoimmune diseases, viral hepatitis, malignant neoplasms or other diseases with a known relationship to membranous glomerulonephritis were not found. The present case highlights the possibility that differential diagnosis between an amiodarone-related pulmonary lesion and a neoplasm can be very difficult radiologically, and suggests that membranous glomerulonephritis might be another possible complication of amiodarone treatment.

Gastric cancer mimicking a submucosal tumor diagnosed by laparoscopic excision biopsy.

We report a case of advanced gastric cancer exhibiting the features of a submucosal tumor (SMT) of the gastric body. The patient was a 50-year-old male in whom a gastric SMT was detected during a mass screening examination. Upper gastrointestinal endoscopy revealed a protuberant tumor, 2 cm in diameter, covered with normal-appearing mucosa. Examination of an endoscopic biopsy specimen of the tumor revealed chronic gastritis with regenerative and erosive changes. An endoscopic ultrasound examination demonstrated a hypoechoic mass in the third layer of the gastric wall. The clinical diagnosis was gastric SMT, and the patient's course was monitored. Twenty-six months after the initial visit to our hospital, an endosonograph-guided biopsy revealed Group III (borderline lesion). Three months after the biopsy, the size of the SMT had increased slightly. Laparoscopic wedge resection of the tumor was performed to make a pathologic diagnosis and we were able to make a diagnosis of gastric lymphoepithelioma-like carcinoma intraoperatively. Gastrectomy with lymph node dissection was followed as curative surgery. Laparoscopic total excision biopsy is a useful technique in patients with a gastric SMT whose diagnosis has not been confirmed pathologically.

Renal complications in a patient with A-to-G mutation of mitochondrial DNA at the 3243 position of leucine tRNA.

A 27-year-old woman with short stature, sensorineural deafness, and renal dysfunction was hospitalized for evaluation. The serum lactate and pyruvate concentrations were elevated. The analysis of her mitochondrial DNA revealed an A-to-G mutation of the tRNA(Leu (UUR)) gene at the 3243 position. Renal biopsy revealed many sclerotic glomeruli, advanced tubulointerstitial changes, and numerous swollen mitochondria of the tubular cells. It was concluded that the patient's mitochondrial gene mutation was etiologically related to her nephropathy. The clinicopathologic features of this patient, as contrasted to the previous reports, suggested that renal involvement due to this mitochondrial gene mutation can be heterogeneous.

A subserosal uterus-like mass presenting after a sliding hernia of the ovary and endometriosis: a rare entity with a discussion of the histogenesis.

OBJECTIVE: To report the first case of a subserosal uterus-like mass. DESIGN: Case report. SETTING: A community-based hospital. PATIENT(S): A 44-year-old nulliparous woman who complained of a left inguinal mass had a medical history that was notable for two features. One was left oophorectomy for a sliding hernia at 10 months of age; the other was endometriosis at the oophorectomy site at 26 years of age. INTERVENTION(S): Tumorectomy. MAIN OUTCOME MEASURE(S): Not applicable. RESULT(S): Pathologic examination demonstrated that this subserosal mass mimicked a miniature uterus with a leiomyomatous lesion. CONCLUSION(S): As of September 2010, 23 cases of uterus-like mass had been reported. Three pathologic theories of uterus-like mass have been proposed: [1] congenital anomaly theory, [2] metaplasia theory, and [3] heterotopia. The pathogenesis of this rare entity is currently under debate. Most uterus-like masses have been connected to the genital organs (75.0%) and associated with endometriosis (50.0%). In the present case, the uterus-like mass developed at the surgical scar site of oophorectomy for a sliding hernia and a tumorectomy for endometriosis. We review the literature and discuss the theories regarding the histogenesis of uterus-like mass.

A case of age-related EBV-associated B-cell lymphoproliferative disorder metachronously showing two distinct morphologic appearances, one of a polymorphic disease resembling classical Hodgkin lymphoma, and the other of a large-cell lymphoma.

We report a case of age-related EBV-associated B-cell lymphoproliferative disorder (age-related EBV+ B-cell LPD) metachronously showing two distinct morphologic appearances: one of a polymorphic disease resembling classical Hodgkin lymphoma (CHL), and the other of a large-cell lymphoma. A 71-year-old man was admitted to the St. Marianna University Hospital because of fever and generalized lymphadenopathy. Right axillary lymph node biopsy revealed mixed cellularity classical Hodgkin lymphoma (MCHL). The patient was referred to the Tokyo Medical Center, where he was treated with chemotherapy and obtained CR. One year later, the patient again developed fever and generalized lymphadenopathy. Biopsy of the right cervical mass revealed a diagnosis of diffuse large B-cell lymphoma. The patient was treated with salvage chemotherapies and obtained the second CR. Two years later, the patient developed acute myeloid leukemia (AML). Although CR was achieved with chemotherapy, AML relapsed 5 months later and proved to be refractory. Two and a half years later, the patient developed right cervical lymph node enlargement. The biopsy again revealed diagnosis of MCHL. The patient died 2 months later. On reviewing all of the biopsy specimens, including the findings of immunohistochemistry and in situ hybridization, possibility of CHL was ruled out, because neoplastic giant cells resembling Hodgkin and Reed-Sternberg (HRS) cells were positive for both Oct2 and BOB.1, which has not been reported in CHL. Both HRS-like cells at the time of diagnosis of Hodgkin lymphoma and lymphoma cells at the time of diagnosis of non-Hodgkin lymphoma were positive for CD20 and EBV-encoded small RNAs. This case was finally diagnosed as having age-related EBV+ B-cell LPD. We report the case here as it underscores the difficulty in diagnosing age-related EBV+ B-cell LPDs and also suggests an important role of EBV in the pathogenesis of lymphoid neoplasms.

Glomerulonephritis with microtubular deposits associated with cryoglobulinemia and chronic active hepatitis.

A 65-year-old-woman presented with edema, ascites, proteinuria and abnormal liver function tests. A small amount of mixed cryoglobulin was detected in her serum. Liver biopsy revealed mild chronic active hepatitis, but tests for hepatotropic viral infection were negative. Electron microscopy of the renal biopsy revealed glomerular electron-dense deposits that contained numerous tubular structures. Renal amyloidosis and light chain deposition disease were ruled out by appropriate histological techniques. The ultrastructural findings of renal biopsy suggested either cryoglobulinemic glomerulonephritis or immunotactoid glomerulopathy. Although the exact interrelationship among the peculiar glomerulopathy, cryoglobulinemia and chronic active hepatitis in the present case remains undetermined, this report enlarges the spectrum of glomerulopathy characterized by extracellular deposition of microtubules.

Occult myopathy of the vastus intermedius muscles detected by magnetic resonance imaging in subclinical dermatomyositis: report of two cases.

Abstract The thigh muscles of two patients with dermatomyositis (DM) without muscle weakness or conspicuous creatine kinase elevations were studied by magnetic resonance imaging (MRI). Myositis limited to the vastus intermedius muscles (VIM) was detected in both patients, and in one, the diagnosis was confirmed by the findings of a biopsy specimen. Focal myositis of the VIM in early-stage DM, which otherwise would remain hidden by the relatively small muscle size and deep location, can be detected by MRI.

A case of postoperative recurrence of fibrolamellar hepatocellular carcinoma with increased vitamin B12 binding capacity in a young Japanese female.

A 17-year-old Japanese female underwent major hepatic resection for a huge fibrolamellar hepatocellular carcinoma that was compressing the inferior vena cava. The tumor was not exposed at the surgical margin but was very close to it. A recurrent lesion at the surgical margin of the liver and a lymph node metastasis were discovered 9 months postoperatively together with a marked elevation of vitamin B12 binding capacity. These lesions were resected, and vitamin B12 binding capacity decreased thereafter. Peritoneal dissemination was detected by CT 16 months postoperatively, together with recurrent elevation of vitamin B12 binding capacity. Several types of chemotherapy, including intraperitoneal injection of epirubicin, were applied and improved the patient's quality of life somewhat, but the patient died of recurrent disease 34 months after the initial hepatic resection. This is the first report in Japan of fibrolamellar hepatocellular carcinoma with increased vitamin B12 binding capacity as a useful marker. Fibrolamellar hepatocellular carcinomas, if resected, have a better prognosis than ordinary hepatocellular carcinoma in Japan, as well as in Western countries. An aggressive strategy should be chosen, which consists mainly of precise surgical resection and postoperative multimodality therapy, including chemotherapy.

Metanephric adenoma treated with laparoscopic nephrectomy.

A 31-year-old-female presented with a left renal mass detected incidentally during an abdominal ultrasound examination. Computed tomography and angiography revealed a hypovascular solid tumor 4.5 cm in diameter at the middle portion of the left kidney. Laparoscopic left nephrectomy was performed and macroscopic examination of the specimen revealed encapsulated tumor with grayish yellow cut surface. Histological examination demonstrated that the tumor cells were small scant cytoplasms, containing round and regular nuclei, forming a glomerular-like structure. There was no mitosis nor cellular atypia indicating a malignant structure. The histological diagnosis was metanephric adenoma.