大城 久

Extraskeletal osteosarcoma is an uncommon high-grade malignant soft tissue sarcoma. Well-differentiated extraskeletal osteosarcoma is thought to have a better prognosis than classical extraskeletal osteosarcoma, but dedi fferentiation after recurrence has also been reported. We present a case of a primary retroperitoneal extraskeletal osteosarcoma in a 62-year-old Japanese woman. Abdominal CT revealed a large mass with diffuse calcification in the right retroperitoneal space and tumor resection was performed. The histopathological diagnosis was well-di fferentiated retroperitoneal extraskeletal osteosarcoma. She was followed up by CT every 6 months without adjuvant radiotherapy and chemotherapy for 31 months until anaplastic highgrade spindle cell sarcoma recurred in the retroperitoneum. Our case is the seventh reported description of well-differentiated extraskeletal sarcoma, and the first to arise in the retroperitoneum and recur as an entirely dedifferentiated spindle cell sarcoma. Copyright © 2010 Hiromasa Arai et al.

A 66-year-old woman, who developed pulmonary tuberculosis at 17 years old, had a high fever in December, 2006. Computed tomographic (CT) scan showed a tumor in the left chalked kidney, which measured 7 cm in diameter with very low enhancement. Laboratory data showed the rise of acute phase reactants (erythrocyte sedimentation rate and c-reactive protein) and severe anemia. The cultures of sputum and urine revealed no Mycobacterium tuberculosis. With the diagnosis of left renal cell carcinoma in the chalked kidney, we performed left radical nephrectomy. Histopathological diagnosis was sarcomatoid renal cell carcinoma. Although sarcomatoid renal cell carcinoma is highly malignant and its prognosis is poor, her post-operative condition has been good without any adjuvant treatments and there have been no recurrent or metastatic lesions for 9 months. The supervention of renal cell carcinoma on renal tuberculosis is rare. The possible effects of tuberculous lesions on the development and progression of renal cell carcinoma are discussed.

Sinonasal malignant tumors constitute less than 1% of all neoplasms and approximately 3% of those of the upper aerodigestive tract.(1) Sinonasal malignant neoplasms most commonly affect the maxillary sinuses (approximately 60%), followed by the nasal cavity (approximately 22%), ethmoidal sinus (approximately 15%), and frontal and sphenoid sinuses (<3%). 1 Sinonasal neoplasms are diverse, with most being squamous cell carcinoma or variants (55%), followed by nonepithelial neoplasms (20%), glandular tumors (15%), undifferentiated carcinoma (7%), and miscellaneous tumors (3%).(1) We describe an exceedingly unusual case of ethmoidal lipid-rich carcinoma with focal glandular structures. To our knowledge, such a case has not been documented in the literature to date.

There are several reports describing [¹⁸F] fluorodeoxyglucose positron emission tomography (FDG-PET) findings in patients with lymphomatoid granulomatosis (LYG). We report a case of grade I LYG that showed increased uptake of FDG. The patient was a 63-year-old Japanese male who underwent an FDG-PET/computed tomography (CT) scan in screening for a malignant lesion. Increased uptake of FDG [maximum standard uptake value (SUV_max), 3.7] was observed in the right hilar region in FDG-PET and enhanced CT revealed a round, abnormal mass that also showed increased FDG uptake. The patient had no previous symptoms. A tumor biopsy was performed and the histological diagnosis was grade I LYG. Therefore, increased SUV_max in FDG-PET might be useful for diagnosing of LYG. [J Clin Exp Hematopathol 49(1) : 39-44, 2009]

Epithelioid angiomyolipoma (eAMLoma) is an uncommon renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis (TSC). It is composed of polygonal large-sized tumor cells arranged in an epithelioid manner. Differential diagnosis from renal cell carcinoma (RCC) is often challenging because of its epithelioid morphology. Herein is reported three cases of eAMLoma, involving one in a 28-year-old man with TSC and two in women without TSC (34 and 62 years of age, respectively). The male TSC patient had microscopic conventional AMLomas in the same kidney. All patients were positive for melanoma (reactive with HMB45 antibody, and positive for melan A, tyrosinase and microphthalmia transcription factor) and smooth muscle markers (positive for alpha-smooth muscle-specific actin), but not for epithelial markers (cytokeratin, epithelial membrane antigen). In particular, the translocation RCC is an important differential diagnostic candidate, in terms of the positive reaction with HMB45 and morphological similarity. The present tumor samples did not show any reactivity for transcription factor binding to IGHM enhancer 3 or transcription factor EB, which excluded the possibility of translocation RCC. The possibility of eAMLoma should be evaluated as a diagnostic candidate, especially in cases of renal tumors (i) in young patients; (ii) associated with TSC; or (iii) with an epithelioid morphology and a high nuclear grade.

Background: Differentiating primary sclerosing cholangitis (PSC) and sclerosing cholangids caused by autoimmune pancreatitis (SC-AIP) is often challenging. Recently, endoscopic findings of the duodenal papilla in cases with AIP or PSC were reported by Unno and Parlak, although the endoscopic differentiation of these 2 conditions has not yet been fully clarified. Objective: Our purpose was to clarify, the endoscopic findings of the duodenal papilla in patients with SC-AIP and those with PSC and to determine criteria for the differentiation of these conditions. Design: Case series. Setting: Restrospective. Patients: Twenty-seven,en patients With SC-AIP and 12 patients with PSC who had undergone ERCP were identified from out database. We reviewed these records to determine whether the duodenal papillary findings (swollen papilla/normal papilla/small papilla) might be potentially useful for differentiating SC-AIP and PSC. Immunohistopathological findings for the duodenal papilla were also examined by using immunoglobulin G4 (IgG4) among the infiltrating plasma cells. Interventions: ERCP, biopsy specimen taken from duodenal papilla. Main Outcome Measurements: The presence of a swollen duodenal papilla with IgG4-positive plasma cells was useful for discriminating SC-AIP from with PSC. Results: A swollen duodenal papilla was observed in 63% (17/27) of the patients with SC-AIP, whereas there was no swelling of the duodenal papilla of the patients with PSC. A small papilla was recognized in 50% (6/12) of the patients with PSC. IgG4-positive plasma cells in the duodenal papilla were significantly detected in the patients with SC-AIP but not in the patients with PSC. Limitation: Single-center study. Conclusions: Characteristic duodenal endoscopic papillary features in patients with SC-AIP, such as a swollen duodenal papilla and positive immunostaining for IgG4, might be helpful for discriminating this condition from PSC.

Herein is reported a case of mucin-producing carcinoma of the gallbladder in a 55-year-old Japanese woman. Although the patient's status and laboratory data initially suggested biliary pancreatitis due to gallstone, radiography and endoscopy confirmed the presence of pancreaticobiliary maljunction and a gallbladder tumor with excessive mucin, in which the duodenal papilla and the common bile duct were impacted. Following surgery, the gallbladder tumor was histopathologically diagnosed as a mixed endocrine-exocrine carcinoma. The carcinoma predominantly consisted of papillary, but also contained some tubular adenocarcinomatous components. Additionally, small foci of small cell and large cell neuroendocrine carcinomatous components were observed. There was no evidence of lymph node metastasis, distant metastasis, or direct invasion outside the gallbladder. Thus, the final classification of pT2N0M0 stage II was given to this lesion, according to the Union Internationale Contre le Cancer guidelines. The postoperative course was uneventful, and the carcinoma had not recurred in the absence of chemoradiotherapy for a period of 20 months. Mucin-producing gallbladder carcinoma is a rare clinical condition that can occur in patients with pancreaticobiliary maljunction. Detailed investigation of this condition is important to develop and refine effective therapeutic strategies.

Adrenocortical carcinoma (ACC) in childhood is rare: in 2002, only three new cases were reported in Japan. Although there is no established therapeutic management system, margin-free complete surgical excision of the tumor is very important to a satisfactory outcome. We report a case of ACC with bilateral pulmonary metastasis in a 10-year-old boy. Preoperative chest computed tomography (CT) showed pulmonary metastasis with near-water density. The patient was treated successfully with complete surgical resection of the primary tumor and adjuvant chemotherapy, including mitotane. After bilateral lung surgery for the metastatic lesions, the plasma dehydroepiandrosterone sulfate level became negative.

Reported herein is a case of endometrial adenocarcinoma without myometrial invasion that metastasized to the pancreas in a 69-year-old Japanese woman who had a history of hysterectomy. Although systemic radiography could not detect any metastasis in the whole body before hysterectomy, imaging performed 2 months after the hysterectomy consisting of CT, magnetic resonance imaging, gallium scintigraphy and positron emission tomography identified a solitary pancreatic tumor. Imaging demonstrated an intracystic papillary growth in the pancreas, suggesting intraductal papillary mucinous neoplasm or mucinous cystic neoplasm. Excised in distal pancreatectomy, the tumor was diagnosed as a pancreatic primary, an invasive papillary adenocarcinoma at first, but both the endometrial tumor and the pancreatic tumor demonstrated similar morphology and immunohistochemistry. Furthermore, the identical nucleotide mutation of TP53 gene was observed from both the endometrial and pancreatic tumors. The pancreatic tumor was therefore confirmed to be a metastasis from the primary endometrial adenocarcinoma. Metastasis to the pancreas from endometrial carcinoma is extremely rare but must be considered even if the previous cancer was treated at an early stage. Histopathological comparison study and genetic analysis are important for the correct diagnosis of metastasis.

Lung cancer associated with Sweet's syndrome is extremely rare. There are only seven reports of such cases. As far as could be determined from a comprehensive search, there is no reported operative case of lung cancer with this syndrome in the world literature. A 75-year-old Japanese man was diagnosed as having Sweet's syndrome. A chest computed tomography (CT) scan to screen for malignant lesions associated with this syndrome revealed an abnormal shadow in the lung. Although [F-18]2-fluoro-2-deoxy-D-glucose positron emission tomography showed no abnormal uptake, lung cancer was most strongly suspected by chest CT. His erythema improved rapidly with steroid therapy and he underwent a segmentectomy (S-6) of the right lower lobe. A pathological examination revealed lung adenocarcinoma (pT1N0M0: Stage Ia). The patient was discharged from the hospital without any worsening of Sweet's syndrome. We herein report a first operative case of an early stage lung adenocarcinoma with this syndrome.

Clear cell sarcoma (CCS) of soft tissue is a rare sarcoma with morphologic similarities to malignant melanoma but a distinct genetic background including a chromosomal translocation, t(12;22)(q13;q12), or a resultant EWSR1-ATF1 fusion gene. In addition, the tumors occurring in the gastrointestinal tract may have a variant fusion gene EWSR1-CREB1. This study analyzed the clinicopathologic and molecular genetic features of 33 CCSs of soft tissue. The patients' ages ranged from 13 to 73 years (median, 30 y), and there was a male predominance (20 males, 13 females). The tumors were located in the deep soft tissues of the extremities (N = 25) or in the trunk or limb girdles (N = 8). The median tumor size was 4cm (range, 1 to 15cm). The tumor cells were either spindle or epithelioid, and they were arranged predominantly in a short fascicular (N = 19) or a solid sheetlike growth pattern (N = 14). Minor histologic variations included the existence of rhabdoid cells (N = 8), bizarre pleomorphic cells (N = 6), alveolar structures due to loss of cellular cohesion (N = 3), and a seminomalike pattern (N 2). Tumor necrosis was evident in 14 tumors, and the mitotic activity ranged from 0 to 43 mitotic figures (MF)/10 high-power fields (HPF) (mean: 4MF/10 HPF). Immunohistochemically, the tumors were consistently positive for S-100 protein (33/33) and variably or focally for HMB45 (32/33), microphthalmia transcription factor (26/32), Melan A (23/32), CD57 (25/33), bcl-2 (30/32), synaptophysin (14/32), CD56 (7/32), epithelial membrane antigen (12/33), cytokeratin (AE1/AE3) (1/32), CD34 (3/32), c-erbB-2 (10/32), c-kit (5/32), and c-met (5/32). a-Smooth muscle actin, desmin, and cytokeratin (CAM5.2) were negative. Reverse transcription-polymerase chain reaction using RNA extracted from formalin-fixed, paraffin-embedded tissues demonstrated transcripts of the EWSR1-ATF1 (31/33) or EWSR1-CREB1 fusion gene (2/33). In 26 cases with available clinical information, local recurrences and metastases developed in 2 and 15 patients, respectively. Ten patients were dead of the disease, and the overall survival rate was 63% at 5 years. However, no clinicopathologic or molecular variables associated with the patients' prognosis were identified. This study confirms that CCS is an aggressive soft tissue tumor with a melanocytic phenotype and wider morphologic variations than had been generally considered. In cases with unusual histologic findings, molecular detection of the EWSR1-ATF1/CREB1 fusion genes provides critical information regarding the diagnosis of the tumor.

Background: The rates of spontaneous remission and relapse of autoimmune pancreatitis (AIP) are not known. Objective: To study the clinicopathologic factors predictive of remission and relapse in cases of AIP. Design: Retrospective study. Patients: Of the 20 patients with AIP, complete response to steroid therapy was recognized in 12 patients, and the remaining 8 patients improved without steroid therapy. Seven patients experienced a relapse. Results: Patients who were seronegative for immunoglobulin (Ig) G4, had no obstructive jaundice, no diabetes mellitus, no swelling of the duodenal papilla, negative staining of the duodenal papilla for IgG4, and focal pancreatic swelling showed a greater tendency toward spontaneous remission (P <.05). The results of multivariate analysis revealed that negative staining of the duodenal papilla for IgG4 was the only independent predictor of spontaneous remission of AIP (odds ratio [OR] 1.395, P =.0304). Seropositivity for IgG4, diffuse swelling of the pancreas, and the presence of stricture in the lower part of the bile duct were significantly associated with a relapse of AIP (P <.05) according to the results of univariate analysis, whereas the results of multivariate analysis revealed only diffuse pancreatic swelling as an independent predictor of a relapse of AIP (OR 26.197, P =.0331). Conclusions: Endoscopic findings are of useful prognostic value, because patients with AIP and with negative staining of the duodenal papilla for IgG4 appeared to have a higher frequency of remission without steroid therapy. Patients with AIP and with diffuse pancreatic swelling were found to be at an increased risk of relapse after the initial steroid administration.

虫垂粘液嚢胞腺腫は稀な疾患である. 今回, われわれは高CEA血症を呈し術後正常化した虫垂粘液嚢胞腺腫の2例を経験したので報告する.<br>症例1 : 58歳, 女性. 2005年3月近医にて右卵巣腫大を指摘され, 婦人科を受診しCTにて右卵巣腫瘍が疑われ12月当院婦人科を受診し入院. CEA=9.3と高値を示し, CT, MRI, USで右下腹部に嚢胞状腫瘤を認めた. 2006年2月当科で回盲部切除術施行. 術後CEA=1.8と正常化した.<br>症例2 : 50歳, 女性. 2005年4月悪性リンパ腫の経過観察中のCTで回盲部に腫瘤を指摘された. 11月のCTで増大が認められ2006年3月入院となった. CEA=5.9と高値を示した. CT, USで右下腹部に嚢胞状腫瘤を認めた. 2006年3月盲腸部分切除術施行. 術後CEA=1.6と正常化した.<br>いずれも病理検査で虫垂粘液嚢胞腺腫と診断され, 免疫染色ではCEA陽性であった.<br>88年以降本邦で原著として報告されCEA高値を示す虫垂粘液嚢胞腺腫は自験例を含め40例であった.

We report a case of a splenic inflammatory pseudotumor (myofibroblastic tumor) in a 43-year-old man with a 5-year history of chronic bronchitis and sleep apnea syndrome. The patient was hospitalized because of a screen-detected splenic mass lesion. His sputum cultures revealed Mycobacterium avium complexes on only one occasion. Imaging studies revealed a 7 cm solitary tumorous lesion, and differential diagnoses of splenic hamartoma, hemangioma, lymphoma, and angiosarcoma were obtained from the radiologist. A splenectomy followed by pathological investigations was performed. By histology, the lesion contained fibroblastic or myofibroblastic spindle cell proliferations, accompanied by variable degrees of inflammatory cell infiltration. Ziehl-Neelsen staining did not reveal acid-fast bacteria. Immunohistochemically, the fibroblastic or myofibroblastic spindle cells were positive for vimentin, human smooth muscle actin, and muscle actin, but negative for desmin, CD8, CD21, CD23, CD35, p80, Epstein-Barr virus LMP, and human herpesvirus type 8. The infiltrating lymphoid cells demonstrated a nonneoplastic pattern. The results of in situ hybridization for Epstein-Barr virus encoded RNA were negative. The postoperative course was uneventful and he has had no recurrence in 22 months. His sleep apnea syndrome and chronic bronchitis have resolved spontaneously since the splenectomy. [J Clin Exp Hematopathol 47(2) : 83-88, 2007]

Objectives: To evaluate the clinical significance of a swollen main duodenal papilla and the associated immunohistopathologic findings in patients with autoimmune pancreatitis (AIP). Methods: Seventeen consecutive patients with AIP registered between April 2001 and October 2005 who underwent both endoscopic retrograde cholangiopancreatography and endoscopic biopsy were enrolled in this study. The endoscopic features, stromal inflammatory cell infiltrate (SICI), and results of immunohistochemical examination of the duodenal papilla using IgG4, CD3, and CD79a antibodies were retrospectively reviewed. These findings in the AIP patients were compared with those in 12 patients with chronic alcoholic tumor-forming pancreatitis (CAP). The numbers of cells in the SICI and of IgG4-positive plasma cells per high-power field were counted in all the histopathologic specimens. Results: A swollen main duodenal papilla was observed in 11 (11 [64.7%]/17) patients with AIP and 4 (4 [33.3%]/12) patients with CAP (P &lt 0.05). Resolution of the swollen main duodenal papilla was observed in all of these 11 patients with AIP (11 [100%]/11) in response to treatment with corticosteroids. On the other hand, the 6 patients without elevated serum IgG4 or a swollen duodenal papilla, but with a swollen pancreas, improved even without corticosteroid treatment. The number of cells in the SICI in the AIP patients was significantly higher than that in the CAP patients. Although in 13 of 17 AIP patients, infiltration by IgG4-positive plasma cells was detected in the duodenal papilla, no such significant infiltration of the duodenal papilla by IgG4-positive plasma cells was observed in the patients with CAP (P &lt 0.05). More predominant T-cell infiltration of the duodenal papilla was recognized in the AIP patients than in the CAP patients (P &lt 0.05). Conclusions: These results suggest that a swollen main duodenal papilla with IgG4-positive plasma cell and T-cell-dominant infiltration and an abundant stromal cell infiltrate are characteristic findings in AIP. We suggest that these findings may be valuable adjuncts to the diagnosis of AIP as well as for selecting suitable candidates for corticosteroid therapy. © 2007 Lippincott Williams and Wilkins, Inc.

Brain metastasis Is a critical complication of small-cell lung cancer (SCLC), resulting In rare long-time survival. We report a case of a 72-year-old man who displayed a very unique clinical appearance, with a large metastatic brain tumor that grew much faster than primary SCLC. The brain tumor expressed high levels of vascular endothellal growth factor (VEGF) that was negative In primary lung tumor. The patient, who underwent brain surgery and chemotherapy against SCLC, has survived for &gt; 2 years with a good performance status since Initial brain symptoms occurred. Weak expression of VEGF In primary tumor might be associated with good prognosis. However, VEGF upregulation could occur after metastasis, resulting In aggressive tumor growth.

We present a 67-year-old male with a rare case of collecting duct carcinoma producing granulocyte colony-stimulating factor. He was referred to us with lumbago and fever. Laboratory testing showed leukocytosis and computed tomography revealed a left renal mass. After chemotherapy with gemcitabine and nedaplatin, computed tomography showed a partial response. A left nephroureterectomy was performed. The histopathological diagnosis was collecting duct carcinoma producing granulocyte-colony-stimulating factor. White blood cell levels were reduced to 9900/mm(3). No recurrence was detected after completion of adjuvant chemotherapy and the patient was discharged from the hospital. However, 3 months later, computed tomography revealed multiple lung metastasis. Nedaplatin- based chemotherapy was administered but computed tomography after chemotherapy identified a progressive disease. The patient died 9 months after the operation.

検診を契機に発見された淡明細胞腺癌の1例を経験したので報告する．症例は76歳，男性で，胸部CT上，左肺S³末梢に単発性の腫瘤性病変を認めた．経気管支肺生検等で確定診断に至らず，左肺部分切除術を施行した．術中迅速凍結組織診断で腺癌が疑われたため，左肺上葉切除術及び縦隔リンパ節郭清に術式を変更した．術前術後の全身の画像診断にて他臓器に腫瘍性病変を認めなかったため，臨床的に原発性肺腫瘍を考えた．術後の病理学的検索で，病変の90％以上を淡明細胞が占め，ごくわずかに乳頭状構造を呈することが分かった．免疫組織化学的にはケラチン陽性，EMA陽性，Thyroid transcription factor-1陽性，Surfactant apoprotein A陽性，HMB45陰性を示し，肺原発の淡明細胞腺癌の診断を得た．術後22ヵ月間経過しているが，再発はなく，健康である．

はじめに:内視鏡的切除された大腸粘膜下組織浸潤癌(以下,大腸sm癌と略記)につき,HE染色とD2-40免疫染色標本によるリンパ管侵襲検出の程度を比較し,リンパ管侵襲検出に関する同免疫染色の有用性を検討した.対象と方法:内視鏡的切除された大腸sm癌30例につき連続切片を作成し,HE染色,D2-40免疫染色を行った.結果:HE標本でリンパ管侵襲は2例2病変,D2-40免疫染色では5例6病変であった.両者で一致したのは1例1病変,乖離がみられたのは5例6病変で,4例5病変がHE標本で過小評価され,1例1病変がHE標本で過大評価された.前者4例は,組織との空隙が乏しい,腫瘍胞巣やリンパ管が小型である,などのため,HE標本ではリンパ管侵襲と認識困難であったが,D2-40免疫染色では認識容易であった.後者1例は,HE標本でリンパ管とみなしたが,D2-40免疫染色陰性であった.一方,同免疫染色では,腫瘍胞巣を一見取り囲むように非特異的に染まることがあり,リンパ管侵襲と誤認しないよう注意が必要であった.考察:D2-40免疫染色は,内視鏡的切除術を受けた大腸sm癌におけるリンパ管侵襲の正確な評価に有用であると考えられた.

We report three infants and one teenager with fatal virus-associated haemophagocytic syndrome (VAHS). Two infants were admitted to our hospital because of cardio-pulmonary arrest (CPA). The third infant was admitted to our department because of fever and liver dysfunction, and he was diagnosed as combined immunodeficiency with predominant T cell defect. The teenager was diagnosed as systemic lupus erythema (SLE) when she was 10 years old and admitted to our department because of fever and thrombocytopenia. The histological findings for the four patients' organs revealed many haemophagocytic cells. The patients were infected by Parainfluenza virus type 2 (PIV2), Enterovirus (EV), Cytomegalovirus (CMV), and Epstein-Barr virus (EBV), respectively. Their laboratory data revealed elevated levels of ferritin and IL-6, which also suggested virus-associated haemophagocytic syndrome (VAHS). Although aggressive therapies were performed in all cases, the outcome was fatal. Further investigation would be needed to clarify the mechanism of VAHS and an effective therapeutic regimen is needed.

大腸は他臓器に比べ多重癌を合併する頻度が高く, また診断治療技術の向上に伴い他臓器癌との重複例も増加している。今回, われわれは喉頭癌, 肺癌, S状結腸癌 (異時性2病変) の四重重複癌症例を経験したので報告する。症例は75歳の男性。2000年2月喉頭癌に対して化学放射線療法が著効し消失した。2004年8月肺腺癌に対し左上葉切除施行。2004年11月大腸内視鏡 (CF) 施行し肛門縁 (AV) より30cmのS状結腸ポリープにポリペクトミー施行。腺腫内高分化腺癌であった。2005年7月CF施行しAVより45cmのS状結腸にIp型ポリープを認め, ポリペクトミー施行。病理検査にて中分化腺癌, sm2 ly1 v0で断端 (+) が疑われたため, 2005年9月腹腔鏡下S状結腸切除術D2郭清を行い病理検査ではn0で腫瘍の残存はなかった。術後経過は順調で現在外来通院中である。

Idiopathic retroperitoneal fibrosis is characterized by the deposition of dense fibrous connective tissue and varying degrees of lymphoid cell infiltration.(1) Some descriptions of this disease also refer to the presence of distorted plasma cells and to nuclear irregularities and clonal gene rearrangement in the lymphoid cells.(2,3) Reports of the incidence of malignant lymphoma, following an initial diagnosis of idiopathic retroperitoneal fibrosis and associated condition,(4-8) suggest that these sclerosing entities should perhaps be classified as lymphoid dyscrasia, which may be predisposed to forming low grade malignant lymphomas.(3) The aim of this study was to further investigate immunohematological abnormalities in cases of idiopathic retroperitoneal fibrosis.

Hemophagocytic syndrome (HPS) is caused by the hyperactivation of T-cells and macrophages. The clinical characteristics associated with this disease result from overproduction of cytokines including interferon-gamma (INF-gamma), tumor necrosis factor-alpha (TNF-alpha), interleukin-6 (IL-6). HPS presents with fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. We describe a 19-year-old female with fatal HPS. She had been given corticosteroid every other day for systemic lupus erythematosus (SLE) without flare Lip. The causative underlying disease was acute primary Epstein-Barr virus (EBV) infection. EBV genomes were detected by the polymerase chain reaction (PCR). To measure the virus load we use a real-time PCR assay to quantify the amount of EBV DNA in peripheral blood lymphocytes, lung, kidney, brain and liver at autopsy. Further in situ hybridisation (ISH) and immunohistochemical studies demonstrated that Epstein-Barr virus encoded small RNA (EBER) was detected in CD8(+) T-cells in bone marrow, lung, kidney, brain, liver and spleen. In each organ, mRNA levels of inflammatory cytokines (INF-gamma, TNF-alpha, IL-6) were highly detected compared with beta-Actin mRNA levels. These results Suggest that EBV-infected CD8(+) T-cells in each organ (peripheral blood lymphocytes, lung, kidney, brain and liver) may have an integral role in the pathophysiology of the HPS. (C) 2004 Elsevier B.V. All rights reserved.

A 72-year-old woman admitted for lower abdominal pain. She had a hard, mobile abdominal mass located from the subumbilical region to the pelvic cavity. Abdominal X-ray findings showed ileus of the small intestine. Computed tomography showed an abdominal mass containing air, necessitating surgery under a diagnosis of severe ileus due to the abdominal mass. We found in surgery that the mass originated from the mesentery of the small intestine near the promontrium, which involved part of the small intestine and sigmoid colon, i.e., 2 sites of the small intestine and part of the sigmoid colon. We excised 2 sites of the small intestine and part of the sigmoid colon with the tumor. The abdominal mass was pathologically diagnosed as CD56+ T-cell lymphoma of the small intestine. She refused chemotherapy and alimentary examination, and left hospital. But she caused perforated panperitonitis of the duodenum T-cell lymphoma considered to be simultaneity and multiple. She was died after the operation on the 83rd. The T-cell lymphoma of the small intestine are only 30 cases including our case. Especially, CD56+ T-cell Lymphoma has a high rate of perforation, the prognosis is so poor, and it was considered the disease which should be careful of.

髄膜癌症をきたした6例の剖検例を用いて,脳を病理組織学的に詳細に検索した.各々の症例から側脳室,第三脳室,そして第四脳室内の脈絡叢組織とその周囲の脳実質を切り出し,組織学的に検索した.6例とも顕微鏡的に脳実質内浸潤巣を伴っており,純粋な髄膜癌症ではなかった.血管周囲腔やクモ膜下腔における血液と腫瘍細胞の混在像はある特定の一部に限定する,選択的な分布であった.更に,脈絡叢基部における毛細血管の一部の壁は開放性となり,血液細胞成分と共に腫傷細胞のクモ膜下腔内への播種像が認められた.なお,脳表と異なり,脈絡までは腫瘍に対する組織反応は殆どなかった

Objective: This study was designed to understand the pathogenetic mechanism of pleural carcinomatosis. Patients and Methods: We collected 207 surgical cases of primary lung cancer and identified 43 cases with positive intraoperative pleural lavage cytology (PLC). We then histologically examined the pulmonary ligament (PL), visceral pleura (VP) and parietal pleura (PP) in these cases and found that 24 cases belonged to either the p0 or p1 categories of the Classification of Lung Cancer of the Japan Lung Cancer Society. Unfortunately the surgical cases were unsuitable for investigation of PL, because of severe operative manipulation involving the ligament. However, 15 autopsy cases of primary lung cancer and 63 of extrapulmonary primary origin were similarly investigated. Results: Surgical cases: Positive PLC cases showed a statistically significantly higher involvement in lymphatics and blood vessels of the lung parenchyma and hilar lymph nodes, than cases with negative PLC (p &lt 0.001). Autopsy cases: Varying degrees of pleural carcinomatosis were found in 8 cases (53.3%) of primary lung tumors, which were all adenocarcinoma in 2 out of the 8 cases, however, tumor cells were found, only in the PL. In addition, varying degrees of pleural carcinomatosis were observed in 21 cases (33.3%) of extrapulmonary origin, in which combined PL involvement was only observed in 5 cases. Furthermore, cancer cell clusters were found in the lymphatic cisterns of the PL in 29 autopsy cases (37.2%). Conclusion: There was also evidence of permeation of tumor cells in the cisterns into the pleural cavity via adjacent stomas in the PL. The PL was found to be a site with numerous lymphatic cisterns with stomas in the thoracic cavity. As a result, we consider that tumor invasion into the subpleural lymphatics might be a prerequisite for the development of pleural carcinomatosis.

気管支原発の顆粒細胞腫1例と本邦33例の文献報告について臨床細胞組織学的に検討した. 症例; 39歳, 男性. 健診時の胸部X線写真上, 左上肺野に異常陰影が指摘された. 気管支鏡検査で粘膜下腫瘍による左B¹⁺²cの閉塞所見が認められた. 擦過及び擦過後気管支洗浄細胞診検査においてシート状配列を示し, 豊富な顆粒状細胞質と小型類円形核からなる腫瘍細胞と, その細胞質内に封入体を認めたため, 顆粒細胞腫と診断した. 左上区区域切除術が施行された. 病巣部は長軸方向12mmにわたり, 白色乳頭状腫瘍であった. 組織学的には特徴的な大型好酸性顆粒状細胞質内にジアスターゼ耐性のPAS強陽性のangulate bodyが認められた. 免疫組織学的にS-100蛋白陽性で, 電顕的にはschwann cellの特徴を示していた. 術後4年間, 再発転移は認められていない. 結語; 本例は顆粒細胞腫の末梢神経細胞の起源説を支持する所見を有している.