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We report a case of immunotactoid glomerulonephritis (ITG) in a patient with cold agglutinins. An 86-year-old Japanese male with a history of hypertension, dyslipidemia, and gastric malignancy presented to our hospital for the evaluation of proteinuria and hematuria. He had an elevated blood pressure of 200/77 mmHg and edema of the lower extremities. Initial blood test results revealed an impaired renal function (creatinine, 1.37 mg/dL) and hypoalbuminemia (albumin, 2.6 g/dL). His estimated daily urinary protein was 5.89 g/g creatinine, meeting the diagnostic criteria for nephrotic syndrome. The selectivity index for proteinuria indicated low selectivity (0.329). We conducted a renal biopsy to identify the cause of nephrotic syndrome. Immunofluorescence microscopy demonstrated positive staining of IgM, C4, and C1q. Electron microscopy exhibited mesangial expansion with inflammatory cells and a lobular structure, suggesting membranoproliferative glomerulonephritis. Subendothelial deposits containing microtubular structures with a diameter of approximately 30–200 nm were found, concurrent with the criteria for the diagnosis of ITG. Screening for lymphoproliferative diseases and immunological abnormalities revealed a positive direct Coombs test result and the presence of cold agglutinins. Paraproteinemia was absent. The similarities between cold agglutinin disease and ITG, including the production of autoantibodies and involvement of complement pathways, raise the possibility that cold agglutinins played a role in the development of ITG; however, we were unable to prove it due to difficulties in detecting cold agglutinins on renal histology. We discuss the possible implications for pathogenesis considering prior reports on nephrotic syndrome being potentially associated with cold agglutinins.

Although the diagnosis of malignant pleural mesothelioma at an in situ stage was traditionally challenging, it is now possible owing to advances in molecular biological methods such as P16 fluorescence in situ hybridization or BRCA1-associated protein 1 immunohistochemistry. Here, we report the first case, to our knowledge, of total parietal pleurectomy for mesothelioma in situ. Future follow-up and accumulation of cases are necessary to determine whether total parietal pleurectomy could be applied as a treatment for mesothelioma in situ or not.

We report a case of nail-patella syndrome (NPS) with unusual thinning of the glomerular basement membrane (GBM) associated with a novel heterozygous variant in the LMX1B gene. A 43-year-old female patient with a previous diagnosis of NPS, referred to our hospital for persistent proteinuria, underwent a renal biopsy, which revealed minor glomerular abnormalities. She underwent a second renal biopsy at the age of 56 owing to the presence of persistent proteinuria and decline in serum albumin, meeting the diagnostic criteria for nephrotic syndrome. Light microscopy demonstrated glomerulosclerosis and cystic dilatation of the renal tubules. Notably, electron microscopy revealed unusual thinning of the GBM, which is quite different from typical biopsy findings observed in patients with NPS, characterized by thick GBM with fibrillary material and electron-lucent structures. Comprehensive genetic screening for 168 known genes responsible for inherited kidney diseases using a next-generation sequencing panel identified a novel heterozygous in-frame deletion-insertion (c.723_729delinsCAAC: p.[Ser242_Lys243delinsAsn]) in exon 4 of the LMX1B gene, which may account for the disrupted GBM structure. Further studies are warranted to elucidate the complex genotype-phenotype relationship between LMX1B and proper GBM morphogenesis.

A 75-year-old man was showed wall thickening just below esophagogastric junction(EGJ)by gastroscopy(GS). Biopsy indicated mucinous carcinoma. He was referred to our hospital. Computed tomography(CT), PET-CT showed EGJ cancer and splenic tumor. EGJ cancer was diagnosed GE, Siewert Type Ⅱ, GrePostAnt, Type 1, cT2, cN0, cM0, cStage Ⅰ. The patient underwent total gastrectomy, lower esophagectomy, D2+ #19, 20, 110, 111, 112 lymph nodes dissection, Rou-en- Y reconstruction, distal pancreatectomy, splenectomy, cholecystectomy, and enterostomy. Postoperative complication was pancreatic fistula(Grade Ⅱ). Pathological diagnosis was esophagogastric junction cancer, neuroendocrine carcinoma(NEC), GE, Siewert Type Ⅱ, GrePostAnt, Type 1, pT2(MP), pN1, pM0, pStage ⅡA. Splenic tumor was diagnosed splenic malignant lymphoma, large B-cell, diffuse(DLBCL), NOS, low-immediate risk. Patient was discharged 15 days after the operation and underwent adjuvant chemotherapy with S-1. In this case, he started taking S-1 because the prognosis of NEC is poorer than PSML. There was no evidence of recurrence after 5 months from gastrectomy. As a result of searching for"neuroendocrine tumor"and"malignant lymphoma"in the JAMAS, there was no report of NEC associated with malignant lymphoma. We experienced the rare case of primary splenic malignant lymphoma associated with EGJ NEC. In the case of gastric cancer with splenic tumor, malignant lymphoma of spleen should be concerned.

Background and ObjectivesParaneoplastic cerebellar degeneration (PCD) is characterized by a widespread loss of Purkinje cells (PCs) and may be associated with autoantibodies against intracellular antigens such as Yo or cell surface neuronal antigens such as the P/Q-type voltage-gated calcium channel (P/Q-VGCC). Although the intracellular location of the target antigen in anti–Yo-PCD supports a T cell–mediated pathology, the immune mechanisms in anti–P/Q-VGCC-PCD remain unclear. In this study, we compare neuropathologic characteristics of PCD with anti–P/Q-VGCC and anti-Yo autoantibodies in an archival autopsy cohort.MethodsWe performed neuropathology, immunohistochemistry, and multiplex immunofluorescence on formalin-fixed and paraffin-embedded brain tissue of 1 anti–P/Q-VGCC, 2 anti–Yo-PCD autopsy cases and controls.ResultsAnti–Yo-PCD revealed a diffuse and widespread PC loss together with microglial nodules with pSTAT1+ and CD8+granzymeB+ T cells and neuronal upregulation of major histocompatibility complex (MHC) Class I molecules. Some neurons showed a cytoplasmic immunoglobulin G (IgG) staining. In contrast, PC loss in anti–P/Q-VGCC-PCD was focal and predominantly affected the upper vermis, whereas caudal regions and lateral hemispheres were spared. Inflammation was characterized by scattered CD8+ T cells, single CD20+/CD79a+ B/plasma cells, and an IgG staining of the neuropil in the molecular layer of the cerebellar cortex and neuronal cytoplasms. No complement deposition or MHC-I upregulation was detected. Moreover, synaptophysin was reduced, and neuronal P/Q-VGCC was downregulated. In affected areas, axonal spheroids and the accumulation of amyloid precursor protein and glucose-regulated protein 78 in PCs indicate endoplasmatic reticulum stress and impairment of axonal transport. In both PCD types, calbindin expression was reduced or lost in the remaining PCs.DiscussionAnti–Yo-PCD showed characteristic features of a T cell–mediated pathology, whereas this was not observed in 1 case of anti–P/Q-VGCC-PCD. Our findings support a pathogenic role of anti–P/Q-VGCC autoantibodies in causing neuronal dysfunction, probably due to altered synaptic transmission resulting in calcium dysregulation and subsequent PC death. Because disease progression may lead to irreversible PC loss, anti–P/Q-VGCC-PCD patients could benefit from early oncologic and immunologic therapies.

A 73‒year‒old man, living in the United States since 1985, visited the University of Minnesota Medical Center with a complaint of upper abdominal pain in December 2019. Gastroendoscopy revealed a depressed lesion in the U area of the stomach, and the biopsy results indicated a diagnosis of por/sig. On further examination, the condition was diagnosed as gastric cancer of cStage II/III. A gastrectomy procedure was planned after treatment with neoadjuvant chemotherapy(NAC). After 1 course of mFOLFOX6, the patient decided to undergo treatment in Japan. He travelled to Japan in February and visited our hospital. In March, he underwent total gastrectomy plus D2 dissection plus Roux‒en‒Y reconstruction. The final diagnosis was gastric cancer, U, Less, ypType 2, ypT3(ss), ypN1, sM0, yfStage IIB, and the therapeutic effect was Grade 2. DS therapy was started as adjuvant chemotherapy in April. After 8 courses, he returned to the United States. After moving to the United States, he personally imported S‒1 and continued adjuvant chemotherapy at the University of Minnesota Hospital. The guidelines in Japan do not recommend NAC. Given that Grade 2 therapeutic effect was obtained even with 1 course of the treatment, FOLFOX therapy could be effective as preoperative chemotherapy for advanced gastric cancer cases.

A 70-year-old man visited his doctor because of hip pain and gross hematuria. Digital rectal examination and computed tomography revealed a prostatic abscess. Although antibiotics were prescribed, his symptoms did not improve; he was transferred to our hospital, where transurethral fenestration of the prostatic abscess was performed. During prostatic fenestration, a papillary mucous membrane was confirmed inside the cavity of the prostatic abscess. Pathological examination revealed a mucinous adenocarcinoma of the prostate. Although hormonal therapy was administered, magnetic resonance imaging revealed tumor extension necessitating robot-assisted radical prostatectomy. Mucinous adenocarcinoma of the prostate is rare, and to our knowledge this is the first report of mucinous adenocarcinoma of the prostate diagnosed after transurethral treatment of a prostatic abscess.

We report a case of membranous nephropathy (MN) in a patient with tuberculosis infection and lung adenocarcinoma. A 50-year-old Filipino woman underwent a renal biopsy for the evaluation of proteinuria and hematuria. Immunofluorescence analysis revealed positive staining of IgG in the glomerular basement membrane and mesangial matrices, while electron microscopy demonstrated the presence of sub-epithelial deposits, suggesting MN. To screen for secondary causes of MN, we conducted a computed tomography (CT) scan of the chest and abdomen, which revealed a ground-glass opacity in the middle lobe of the right lung and an enlarged paraaortic lymph node. A T-SPOT test was positive, suggesting the possibility of a latent tuberculosis infection, as she was asymptomatic. A follow-up chest CT scan showed persistent presence of the ground-glass opacities, suggesting a non-infectious cause. Video-assisted thoracoscopic resection of the middle right lobe and partial resection of the lower right lobe were performed because the possibility of lung cancer could not be excluded. Notably, pathological analysis of the lung revealed adenocarcinoma in the middle lobe and epithelioid granuloma in the lower lobe, suggesting an active tuberculosis infection. One month after surgery, anti-tuberculosis treatment was initiated. Thereafter, her proteinuria, which had increased to 6 g/gCre preoperatively, began to decrease. Five months after surgery, the patient achieved complete remission. The speed of remission suggests that tuberculosis likely played a primary role in the etiology of MN. Our case underscores the importance of screening tests for infections and malignancies in patients with MN, even if suggestive symptoms are absent.

We report a case of nail-patella syndrome (NPS) with unusual thinning of the glomerular basement membrane (GBM) associated with a novel heterozygous variant in the LMX1B gene. A 43-year-old female patient with a previous diagnosis of NPS, referred to our hospital for persistent proteinuria, underwent a renal biopsy, which revealed minor glomerular abnormalities. She underwent a second renal biopsy at the age of 56 owing to the presence of persistent proteinuria and decline in serum albumin, meeting the diagnostic criteria for nephrotic syndrome. Light microscopy demonstrated glomerulosclerosis and cystic dilatation of the renal tubules. Notably, electron microscopy revealed unusual thinning of the GBM, which is quite different from typical biopsy findings observed in patients with NPS, characterized by thick GBM with fibrillary material and electron-lucent structures. Comprehensive genetic screening for 168 known genes responsible for inherited kidney diseases using a next-generation sequencing panel identified a novel heterozygous in-frame deletion-insertion (c.723_729delinsCAAC: p.[Ser242_Lys243delinsAsn]) in exon 4 of the LMX1B gene, which may account for the disrupted GBM structure. Further studies are warranted to elucidate the complex genotype-phenotype relationship between LMX1B and proper GBM morphogenesis.

We estimated the telomere lengths of neoplastic and non-neoplastic mesothelial cells and examined their correlation with asbestos exposure and the expression of markers of mesothelial malignancy. Cell blocks of pleural effusion obtained from 35 cases of non-neoplastic disease (NN), 12 cases of malignant mesothelioma (MM) and 12 cases of carcinomatous effusion due to lung adenocarcinoma (LA) were examined. Fifteen of the 35 NN cases had pleural plaques (NNpp+) suggestive of asbestos exposure, and the other 20 cases had no pleural plaques (NNpp-). Telomere length was measured using the tissue quantitative fluorescence in situ hybridization method, and expressed as normalized telomere-to-centromere ratio. NN cases had significantly longer telomeres than MM (P < 0.001) and LA (P < 0.001) cases. Telomeres in NNpp+ cases were slightly shorter than those of NNpp- cases (P = 0.047). MM and LA showed almost the same telomere length. NN cases with shorter telomeres tended to show aberrant expression of epithelial membrane antigen (EMA), CD146, glucose transporter 1 (GLUT1) and IGF-II messenger RNA-binding protein 3 (IMP3). These results suggest that telomere shortening and subsequent genetic instability play an important role in the development of MM. Measurement of telomere length of cells in pleural effusion might be helpful for earlier detection of MM.

A 73-year-old woman was referred to our department with a complaint of asymptomatic gross hematuria. Dynamic computed tomography revealed a complicated (Bosniak type IIF) cyst in the upper pole of her right kidney, which was diagnosed as a calyceal diverticulum. The diagnosis was confirmed by ureteroscopy. The diverticulum was filled with a soft protein matrix that was difficult to completely remove from the inner surface of the calyceal diverticulum. Endoscopy combined with intrarenal surgery (ECIRS) was performed to completely remove the matrix. Percutaneous nephroscopy further revealed papillary lesions on the surface of the diverticulum, confirmed as squamous cell carcinoma on pathological assessment. A laparoscopic right radical nephroureterectomy was performed, with curative intent. Pathological assessment confirmed a high-grade squamous cell carcinoma with renal parenchymal invasion (pT3). Although carcinomas in a calyceal diverticulum are highly uncommon, when present, these tend to be high-grade neoplasms that deeply invade the parenchymal wall. As the effective management of these lesions is difficult, early-stage diagnosis is required for curative treatment. We report the case of squamous cell carcinoma in a calyceal diverticulum that was difficult to diagnose on preoperative computed tomography, urinal cytology examination, and ureteroscopy but was found during ECIRS.

Recent case reports have shown that hepatitis E virus (HEV) infection can cause chronic hepatitis in immunosuppressed or immunocompromised patients. A 37-year-old woman suffered from prolonged elevation of aminotransferases after chemotherapy for Burkitt's lymphoma and was diagnosed with chronic hepatitis E due to a transfusion during chemotherapy. After an 8-month administration of ribavirin, complete HEV clearance was not achieved, likely due to prolonged hypogammaglobulinemia. This case indicates that HEV infection should be ruled out during liver dysfunction in immunosuppressed or immunocompromised patients and suggests that an alternative therapeutic strategy for such patients will be needed.

We estimated the telomere lengths of neoplastic and non-neoplastic mesothelial cells and examined their correlation with asbestos exposure and the expression of markers of mesothelial malignancy. Cell blocks of pleural effusion obtained from 35 cases of non-neoplastic disease (NN), 12 cases of malignant mesothelioma (MM) and 12 cases of carcinomatous effusion due to lung adenocarcinoma (LA) were examined. Fifteen of the 35 NN cases had pleural plaques (NNpp+) suggestive of asbestos exposure, and the other 20 cases had no pleural plaques (NNpp-). Telomere length was measured using the tissue quantitative fluorescence in situ hybridization method, and expressed as normalized telomere-to-centromere ratio. NN cases had significantly longer telomeres than MM (P 0.001) and LA (P 0.001) cases. Telomeres in NNpp+ cases were slightly shorter than those of NNpp- cases (P = 0.047). MM and LA showed almost the same telomere length. NN cases with shorter telomeres tended to show aberrant expression of epithelial membrane antigen (EMA), CD146, glucose transporter 1 (GLUT1) and IGF-II messenger RNA-binding protein 3 (IMP3). These results suggest that telomere shortening and subsequent genetic instability play an important role in the development of MM. Measurement of telomere length of cells in pleural effusion might be helpful for earlier detection of MM.

Neuroendocrine tumors of the bladder are rare, and the rarest neuroendocrine tumor of the bladder is large cell neuroendocrine carcinoma (LCNEC). Here, we report a histologically confirmed case of mixed large and small cell high-grade neuroendocrine tumor of the bladder, which was positive for chromogranin A and synaptophysin on immunohistochemical staining. Treatment consisted of TURBT (transurethral resection of bladder tumor) and systemic chemotherapy with cisplatin and irinotecan. To guide selection of the optimal treatment for LCNEC of the bladder, we also review the literature regarding the treatment of LCNEC of the bladder and of other sites.

The absence of highly specific markers for malignant mesothelioma (MM) has served an obstacle for its diagnosis and development of molecular-targeting therapy against MM. Here, we show that a novel mucin-like membrane protein, sialylated protein HEG homolog 1 (HEG1), is a highly specific marker for MM. A monoclonal antibody against sialylated HEG1, SKM9-2, can detect even sarcomatoid and desmoplastic MM. The specificity and sensitivity of SKM9-2 to MM reached 99% and 92%, respectively; this antibody did not react with normal tissues. This accurate discrimination by SKM9-2 was due to the recognition of a sialylated O-linked glycan with HEG1 peptide. We also found that gene silencing of HEG1 significantly suppressed the survival and proliferation of mesothelioma cells; this result suggests that HEG1 may be a worthwhile target for function-inhibition drugs. Taken together, our results indicate that sialylated HEG1 may be useful as a diagnostic and therapeutic target for MM.

A 48-year-old woman with a history of voiding difficulty visited our hospital. Magnetic resonance imaging revealed a periurethral tumor, which was pathologically diagnosed as an adenocarcinoma via transperineal needle biopsy. Radical cystectomy and urethrectomy were performed, and the urinary tract was reconstructed using an ileal conduit. Pathological examination of a resected specimen confirmed adenocarcinoma of the urethral diverticulum. The patient received adjuvant gemcitabine and cisplatin chemotherapy. She is alive at 5 months since the operation.

Mixed adenoneuroendocrine carcinoma of the colon and rectum is a very rare type of tumor.The aim of the present study was to evaluate the clinicopathological characteristics and prognosis of mixed adenoneuroendocrine carcinomas of the colon and rectum.This was a retrospective case-matched analysis (from March 2007 to December 2013).This study was conducted at Yokosuka Kyosai Hospital.One thousand three hundred six consecutive patients with a preoperative diagnosis of colorectal cancer and who underwent tumor resection were enrolled in the present study. Each patient diagnosed with mixed adenoneuroendocrine carcinoma was 1:2 matched with 2 counterparts who had been diagnosed with adenocarcinoma.Immunohistochemical staining for neuroendocrine markers (chromogranin A, synaptophysin, and CD56) was performed. Cases in which the neuroendocrine component accounted for30% of the tumor were diagnosed as mixed adenoneuroendocrine carcinomas.Among 1306 patients, 42 patients (3.2%) were diagnosed with mixed adenoneuroendocrine carcinoma and were compared with 84 patients with adenocarcinoma who had been randomly case matched. The average Ki-67-labeling index value was 78.0% (range, 30.0%-99.0%). Chromogranin A, synaptophysin, and CD56 positivity were observed in 42.9% (18/42), 81.0% (34/42), and 33.3% (14/42) of the tumors. Both the disease-free survival and overall survival were significantly worse for mixed adenoneuroendocrine carcinoma than for adenocarcinoma. Ten patients underwent treatment with oxaliplatin-based chemotherapy. The response rate was 40.0%; the median progression-free survival and overall survival were 6.3 months and 18.1 months.This was a retrospective single-institution study that included a limited number of cases. The treatment regimens used included different types of oxaliplatin-based chemotherapy.Mixed adenoneuroendocrine carcinoma of the colon and rectum has a poor prognosis after curative resection and should be distinguished from adenocarcinoma.

Background: Malignant pleural mesothelioma (MPM) is a rare but fatal tumour. Although most MPM patients show pleural effusion at even the early stage, it is hard to diagnose as MPM at the early stage because a sensitive and reliable diagnostic marker for MPM has not been found in plasma or pleural effusion. Methods: In this study, we investigated whether intelectin-1 was specifically contained in MPM cells and the pleural effusion of MPM patient by immunohistochemistry, western blotting, and enzyme-linked immunosorbent assay. Results: Malignant pleural mesothelioma cell lines, but not lung adenocarcinoma cell lines, secreted intelectin-1. In immunohistochemistry, epithelioid-type MPMs, but neither pleura-invading lung adenocarcinomas nor reactive mesothelial cells near the lung adenocarcinomas, were stained with anti-intelectin antibodies. Pleural effusion of MPM patients contained a higher concentration of intelectin-1 than that of lung cancer patients. Conclusion: These results suggest that detection of intelectin-1 may be useful for a differential diagnosis of epithelioid-type MPM in immunohistochemistry and that a high concentration of intelectin-1 in pleural effusion can be used as a new marker for clinical diagnosis of MPM.

症例は67歳，男性．高血圧にて近医経過観察中に心窩部不快感出現．超音波検査，胸部レントゲンにて右中～下肺野に超手拳大の腫瘤を指摘され当院紹介となった．CTにて右胸腔～縦隔右側にかけて160×110mmの腫瘤を認めた．腫瘤は上大静脈，右房を圧排し，心膜，横隔膜への浸潤も疑われた．CTガイド下生検施行しspindle cell sarcomaの診断で手術が施行された．左側臥位にて審査胸腔鏡施行後，摘除可能と判断し，仰臥位，胸骨正中切開にて摘出術施行した．術後経過は良好で，第15病日に退院となった．術後病理検査にて脂肪肉腫と診断された．今回比較的まれな縦隔発生の脂肪肉腫の1手術症例を経験したので報告する．

症例は58歳女性．約1年前に右乳腺葉状腫瘍の手術歴がある．乳房手術時より右肺上葉に小結節影を認めていたが，その後増大傾向を示した．さらに新たな肺内結節影も出現し，多発結節影を呈した．これらの結節はいずれも境界明瞭で内部が均一な結節影であり，乳腺葉状腫瘍術後であることから転移性肺腫瘍が疑われた．手術にて肺腫瘍・肺門リンパ節を摘出したが，いずれも病理学的に非乾酪性類上皮細胞肉芽腫と診断され，両側肺門リンパ節腫脹も認めたことから，サルコイドーシスと診断した．サルコイドーシスにおいて肺野末梢に結節影を示す場合，辺縁に樹枝状影を伴うことが多く，本例のように境界明瞭な結節影を呈する症例は稀である．しかし，肺内リンパ節に病変がおよんだ場合には，本例のような腫瘤影を呈すことがある．そのため，末梢肺野の境界明瞭な結節影を示す症例で，特に悪性腫瘍の既往歴がある場合には，本疾患との鑑別を念頭に置く必要がある．

Follow-up colonoscopy of a 25-year-old Japanese man with ulcerative colitis (UC) who had undergone endoscopic mucosal resection twice for early colon cancers revealed the presence of a new 1.5-cm-diameter tumor in the sigmoid colon. It was diagnosed by preoperative biopsy as a poorly differentiated adenocarcinoma. Sigmoidectomy was performed, and the pathological findings revealed lymphoepithelioma-like carcinoma (LEC). In situ hybridization to detect Epstein-Barr virus (EBV)-encoded small RNAs showed positive signals in stromal lymphocytes, but weak signals in the tumor cells. The association between EBV and LEC was obscure in this case. Unlike typical UC-mediated colon cancers, the lesion was poorly differentiated, and negative for p53 signals immunohistochemically. These findings may hint at a novel mechanism of carcinogenesis in UC-mediated colorectal cancer.

症例は57歳, 女性. 上腹部痛を主訴に来院した. 腹部エコーで45mm大の腫瘤を指摘され, CTでは境界明瞭な腫瘍が肝左葉と胃の小彎に接していた. 術前は胃の壁外性Gastrointestinal stromal tumor (GIST) を疑った. 術中所見で腫瘍は径25×20mmで小網に主座を置き肝左葉の下面と胃の小彎壁に強固に癒着していた. 小網の一部を含めて腫瘍を切除した. 組織学的に腫瘍周囲を筋線維芽細胞で覆われ, 一部に短紡錘形で特定の配列を示さない細胞群を認め広汎な中心壊死を認めた. 腫瘍細胞は低異型度で分裂像は乏しかった. 免疫染色ではvimentin陽性, CD68 (Kp1) が部分的に陽性であったが, CD117 (c-kit), CD34は陰性で, 小網原発の間葉系腫瘍と診断した. 術後経過は順調で再発傾向を認めていない. 小網原発の腫瘍は稀であり, 特に本症例では起源を特定するのが困難であった.

症例は，39歳，女性．検診発見の胸部異常影につき精査したところ，胸部CTで両肺野に多発する結節影を認めた．特に左肺S6付近の径40mm大の腫瘤影は，胸壁腫瘍との鑑別が困難であった．診断を目的に胸腔鏡下生検を施行した．病理組織学的には，均一の紡錘形腫瘍細胞が錯走性の配列を示して増殖し，免疫染色，hormone receptor陽性所見，子宮筋腫の既往から，良性転移性平滑筋腫（benign metastasizing leiomyoma）と診断した．若干の文献的考察を交えて報告する．

Renal oncocytosis is a rare disorder in which numerous oncocytic nodules develop in the kidney. An additional case is reported here. The patient was a 51-year-old woman who had received hemodialysis for 27 years. Nineteen years previously she had developed a tumorous lesion in the right kidney, which had been diagnosed as oncocytoma with laparotomic biopsy. Recently the kidney was removed because of enlargement of the tumor. The renal parenchyma was entirely replaced with numerous brownish nodules. Histologically, the nodules were composed of nests of uniform oncocytic cells. Ultrastructurally, the oncocytic cells contained numerous mitochondria. Immunohistochemical features of the nodules were identical to those of sporadic oncocytomas, that is, immunophenotypes similar to the distal nephron and reactivity with antimitochondrial antigen. Based on these findings, the lesion was diagnosed as renal oncocytosis. It was not possible to determine whether the larger nodules should be diagnosed as oncocytoma or a part of oncocytosis. Additionally, the germ line mutation of the Birt-Hogg-Dubé (BHD) syndrome gene was examined using the genomic DNA obtained from the peripheral lymphocytes, which failed to show any gene alteration. Despite the rare occurrence pathologists and urologists should be aware of renal oncocytosis, as a precursor lesion of renal oncocytoma and chromophobe renal cell carcinoma.

Objective The standard operation for patients with stage IA lung adenocarcinoma is considered to be a lobectomy. Recently, some researchers have reported that patients with tumors showing greater proportions of ground-glass opacity (GGO) at computed tomography (CT) could be candidates for limited resection, because of its less aggressive nature. However, the lack of a precise definition or standard measuring method of GGO prevents its general use as an index for planning limited resection. Therefore, we attempted to define GGO based on CT number and measured it more objectively. Methods Between 1998 and 2001, 90 patients with clinical stage IA adenocarcinoma, who underwent standard or intentional limited resection and whose images of chest high-resolution CT were preserved in Digital Imaging and Communications in Medicine (DICOM) format, constituted the study population. The tumor shadow seen on the solid window (WL, -160HU; WW, 2HU) was regarded as the central solid area of the tumor seen on the lung window, and GGO was defined as the whole tumor area with the exception of the central solid area. Each area was measured using Scion Image (Scion Corp., Frederick, MD). We analyzed the relationship between the proportion of GGO and both of pathologic findings and recurrence. Results Among the 90 tumors, 31 (34.4%) were calculated to have a GGO area greater than or equal to 50%. Of these, 27 (87%) tumors were bronchioloalveolar carcinoma. Lymphatic and vascular invasions, or nodal involvement were found only in patients with a smaller proportion of GGO ( Conclusions Tumors with a greater proportion of GGO measured by our method are thought to have a less invasive nature. Our objective measuring method of GGO could be useful for future multicenter trials to elucidate the value of limited resection for clinical stage IA adenocarcinoma based on the proportion of GGO.

Background Instances of ectopic salivary gland tissue within the pituitary gland are rare, they are mostly asymptomatic, and the underlying pathophysiology of symptomatic cases is unclear. We report a case of intrasellar salivary gland rest that presented clinical symptoms and clearly related to inflammatory changes. Case Description In the present case, headache, bitemporal hemianopia, and hormone abnormality led to the detection of ectopic salivary gland tissue within the pituitary gland of a 24-year-old man. Imaging revealed a well-circumscribed intrasellar cystic lesion having a diameter of major axis of 16 mm, for which tumorectomy was performed using the nasal approach. The tumor was cystic with stringy content. Pathologic findings revealed that the lesion was composed principally of secretions lacking cell components, whereas the salivary gland tissue was found in the cyst wall. Dilated ducts due to the leakage of secretions were also observed. Acute and chronic inflammation was present around the salivary gland. Conclusions Not only are instances of symptomatic ectopic salivary glands rare, but this was also the first case detected to be caused by the pathophysiology involving the leakage of secretions from an ectopic salivary gland and associated inflammation. We report this case to help elucidate the pathophysiology of the condition.

Objective: In patients with clinical T1 N0 M0 lung adenocarcinoma, we investigated whether the proportion of ground-glass opacity area measured on high-resolution computed tomography was valuable for predicting the existence of lymph node metastasis, lymphatic invasion, or vascular invasion. Methods: Between 1994 and 1999, 111 patients with clinical stage IA adenocarcinoma underwent surgical resection of the lung at our hospital. Of these, 96 patients received high-resolution computed tomography of the chest, and they constituted the study population. The tumors were semiquantitatively classified into 5 groups on the basis of the proportion of ground-glass opacity area to whole tumor shadow on high-resolution computed tomography: group I, 0%; group II, 1% to 25%; group III, 26% to 50%; group IV, 51% to 75%; and group V, 76% to 100%. Correlations of computed tomographic findings, pathologic results of lymph node metastasis and lymphatic and vascular invasion, and the histologic subtype according to the new World Health Organization classification were examined. We also investigated the characteristics of the patients with ground-glass opacity areas on high-resolution computed tomography and their value for predicting lymph node metastasis. Results: Among the 96 patients, 15 (15.6%) had mediastinal lymph node metastases, and 3 (3.1%) had hilar node metastases. Regarding the proportion of the ground-glass opacity area of the tumors, 15 (15.6%) tumors were classified as group V, 11 (11.5%) as group IV, 9 (9.3%) as group III, 22 (22.9%) as group II, and 39 (40.6%) as group I, respectively. Of the 18 patients with lymph node metastases, no patients were found in groups IV and V, 2 (22.2%) were found in group III, 4 (18.2%) were found in group II, and 12 (30.8%) were found in group I (trend P =. 003), respectively. Twenty-six patients classified into groups IV and V also showed neither lymphatic invasion nor recurrence. All the smaller tumors (≤2.0 cm) in group IV or V were histologically proved to be bronchioloalveolar carcinoma. Adjusted for smoking status and other characteristics, patients without ground-glass opacity on high-resolution computed tomography had a significantly increased risk of concurrent lymph node metastasis compared with those with ground-glass opacity. Conclusion: In patients with clinical T1 N0 M0 adenocarcinoma, the proportion of ground-glass opacity area on thin-section computed tomography scans was a strong predictor for tumor aggressiveness and thus could be a useful index for planning limited surgical resection for these patients. J Thorac Cardiovasc Surg 2002;124:278-84

われわれは28年前に食道あからしあに対し胃弁移植術を施行し, 術後2次的に発生したと考えられるBarrett上皮由来の2型噴門部食道腺癌を経験した. 症例は55歳の男性で嚥下困難を訴え, 上部消化管内視鏡にて噴門部に2型進行癌を認めた. 生検では, 腫瘍からは中分化型管状腺癌, 腫瘍周囲のるごーる不染領域からは円柱上皮が得られた. 術前診断で噴門部進行Barrett食道腺癌と診断し, 下部食道・噴門側胃切除脾摘術を施行した. 病理組織学的には, 腫瘍はBarrett上皮に囲まれた乳頭腺癌であり, 胃壁には胃弁移植術による筋層断裂, 食道壁にはあからしあに相当する所見が認められた. 本例においては食道あからしあに対する胃弁移植術に起因する逆流性食道炎がBarrett食道を形成し, さらに腺癌が発症したものと考えられた.

中枢の気管支腔内にポリープ状に発育する定型的カルチノイドの2症例について, HRCT所見と病理所見を比較検討した. 1例は61歳男性で, 左主気管支内腔をほぼ閉塞する可動性のあるポリープ状の腫瘍を有し, 他の1例は39歳男性で, 右中間気管支幹内腔を占める可動性のあるポリープ状腫瘍を認め, 両者とも生検にて定型的カルチノイドと診断された. 術前に行われた造影HRCTではいずれも気管支内腔に軽度の造影効果を有する腫瘍としてとらえられ, 明らかな壁外進展の所見は認めなかった. 前者は残存肺の再膨張が得られず肺全摘術を, 後者は中間気管支幹管状切除術を施行した. 切除標本の病理検査では両者とも細い茎 (8mm, 9mm) を有しほぼ全体が気管支腔内に存在する腫瘍であったが (大きさ3.5×1.8×1.2cm, 2.0×1.3×1.0cm), いずれもわずかに気管支軟骨の外側にまで腫瘍浸潤を認めた. HRCTにて明らかな壁外成分や気管支壁の肥厚や不整所見を示さなかったが, 病理学的には気管支軟骨外まで浸潤していた中枢発生定型的気管支カルチノイド症例を2例経験したので報告した.

An 80-year-old man presented with abdominal fullness and vomiting. Laboratory data revealed severe anemia, an inflammatory response, and elevated white blood cell counts. Abdominal computed tomography indicated ileus caused by a jejunal tumor measuring 8cm in diameter. Although small-bowel endoscopy enabled visualization of the tumor, adequate biopsy specimens could not be obtained for accurate diagnosis. The patient's condition rapidly deteriorated, because of which surgical treatment could not be initiated. The patient died approximately 3 weeks after admission. High serum granulocyte colony-stimulating factor (G-CSF) levels were detected at autopsy. Immunohistochemical staining of the autopsy specimen indicated positive G-CSF levels in the jejunal tumor. On the basis of these findings, a final diagnosis of undifferentiated carcinoma of the jejunum producing G-CSF was made.

Sarcoidosis is a systemic granulomatous disease of unknown origin. We herein report a case of sarcoidosis in a chronic dialysis patient diagnosed by hypercalcemia without any common clinical manifestations. The onset of sarcoidosis in chronic dialysis patients is rare; to the best of our knowledge, only 23 cases have been reported. Evaluation of the 23 previously published cases revealed that a diagnosis of sarcoidosis was often achieved by the presence of sarcoidosis-related hypercalcemia without any common clinical presentations, as in the present case. This characteristic may arise from a specific immune deficiency and the unique physiology of 1,25-dihydroxyvitamin D3, a main cause of sarcoidosis-related hypercalcemia, in chronic dialysis patients. These clinical features may be useful to understand the pathogenesis of sarcoidosis.

A 37-year-old man developed Henoch--Schonlein purpura nephritis (HSPN) with nephrotic syndrome and rapidly progressive glomerulonephritis after otitis media and externa due to methicillin-resistant Staphylococcus aureus infection. Despite resolution of the infection and prednisolone therapy, his kidney disease worsened. However, the addition of cyclosporine A finally resulted in complete remission of the nephrotic syndrome. A review of similar cases with post-Staphylococcal infection HSPN revealed strong similarities between this entity and immunoglobulin A-dominant postinfectious glomerulonephritis (IgA-PIGN), an increasingly recognized form of PIGN typically associated with Staphylococcal infection, in both clinical and morphological features. Post-Staphylococcal infection HSPN may constitute a subgroup of IgA-PIGN.

The expression of the c-kit gene product has been examined in normal mast cells, mast cell neoplasms, and basophil/mast cell precursors obtained from patients with chronic myelogenous leukaemia (CML). Formalin-fixed, paraffin-embedded sections or smears fixed with formalin vapour were studied by immunohistochemical methods, using a polyclonal antibody against the c-kit gene product. Normal and neoplastic mast cells showed a positive immunoreaction for c-kit gene product, but neoplastic basophil/mast cell precursors from CML patients lacked c-kit gene product by immunohistochemical and flow cytometric methods, even in cells having mast cell granules, together with or without basophil granules. Mast cell tryptase was, however, expressed in normal and neoplastic mast cells and basophil/mast cell precursors containing mast cell granules. In addition, cells of monocyte/macrophage lineage lacked c-kit gene product. These findings indicate that the c-kit gene product may play an important role in the development and function of mast cell but not of cell of basophil and monocyte/macrophage lineage.

Sunitinib, a tyrosine kinase inhibitor targeting vascular endothelial growth factor receptors (VEGFRs), has become essential for treating imatinib-resistant malignant gastrointestinal stromal tumor. Recently, several cases have been reported that showed proteinuria and kidney dysfunction to be associated with anti-VEGF therapy. Although previous reports indicated that this side-effect is reversible, it is not well understood. We present here the case of a 72-year-old man who presented with nephrotic syndrome and renal dysfunction 6 months after administration of sunitinib. Sunitinib was discontinued, and nephrotic syndrome remitted spontaneously, but renal function recovery was limited. Nine months later, a renal biopsy was performed because sunitinib was again required and pathological examination was needed. The renal biopsy showed marked endothelial cell injury with focal segmental glomerulosclerosis and accelerated VEGF expression by podocytes. Sunitinib was then given at a reduced dose. Kidney dysfunction and nephrotic syndrome are rare but serious complications of sunitinib. The present case suggests that long-term treatment with a high dose of sunitinib can cause irreversible renal dysfunction, and that low-dose treatment makes these side-effects manageable.

We report a case of pancreatic metastasis from renal cell carcinoma detected 25 years after radical nephrectomy. A 74-year-old man, who had undergone radical nephrectomy for renal cell carcinoma at age 49, was found by computed tomography to have a strongly enhanced mass on the pancreatic head. The patient underwent pancreaticoduodenectomy and the pathological diagnosis was metastatic renal cell carcinoma. This was evidently a slow growing tumor because the metastatic pancreas tumor was well demarcated and the metastasis was found 25 years after the primary operation. Aggressive surgical treatment of isolated metastatic lesions offers a chance of long-term survival. Patients with a history of RCC should undergo a long-term follow-up to detect and evaluate metastasis to pancreas as well as other organs.

Paraganglioma is a rare neuroendocrine tumor which arises from extra adrenal paraganglionic cells of the autonomic nervous system. We report a case of a giant retroperitoneal paraganglioma. A 43-year-old man referred to our hospital for further examination of a retroperitoneal mass. The patient had neither familial nor past medical history. The blood and urine test, laboratory examinations including cathecolamines, were unremarkable. Abdominal computed tomography showed an enhancing solid mass 13 cm in diameter on the left kidney. The invasion to the left kidney was suspected. Angiography showed the left renal, splenic, middle suprarenal and left inferior diaphragmatic artery feeding the tumor. The splenic and left inferior diaphragmatic artery were embolized before surgical treatment. The tumor, left kidney, adrenal gland and spleen were surgically resected. Histological examination revealed extra-adrenal paraganglioma, and there was no invasion of the tumor to the left kidney, adrenal gland and spleen. The patient has now survived more than 10 months following the surgery without tumor recurrence.