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Aims Xp11 rearrangement in renal cell carcinoma (RCC) typically involves gene fusion to the gene encoding transcription factor E3 (TFE3), a member of the microphthalmia-associated transcription factor family on chromosome Xp11.2. Dual-colour break-apart fluorescence in-situ hybridisation (FISH) is recommended to confirm histological diagnoses. Recently, RNA-binding motif protein 10 (RBM10), encoded by a gene on chromosome Xp11.3, was identified as a chimeric partner of TFE3; thus, RBM10-TFE3 fusion results from paracentric inversion. RBM10-TFE3 RCC may yield a false-negative result in FISH analysis of TFE3 expression. The aim of the present study was to investigate the clinicopathological features of RBM10-TFE3 RCC. Methods and results Ten patients with RBM10-TFE3 RCC aged 31-71 years were investigated. Histological analysis, immunostaining, dual-colour break-apart FISH for TFE3, reverse transcription polymerase chain reaction and sequencing analysis were performed. No patient had a history of exposure to chemotherapy. Two of these patients died of RCC, and three were alive but developed metastases. Microscopically, the tumours were composed of a mixed architecture of tubulocystic and papillary patterns with scattered psammoma bodies. The tumours showed strong nuclear immunoreactivity for TFE3. FISH showed consistent closely spaced split signals in the RCCs of four patients, and polysomic signals with occasional closely spaced split signals in the RCCs of six patients. Of the latter six patients, five had renal failure, and four developed tumours in kidneys subjected to haemodialysis. Conclusions The present study suggests that the carcinogenesis of RBM10-TFE3 RCC in some, but not all, patients may be associated with chronic kidney disease. The aggressive nature of RBM10-TFE3 RCC should be considered, as five patients experienced metastases.

Cotyledonoid dissecting leiomyoma (Sternberg tumor) is a rare variant of the uterine smooth muscle tumor. Although this tumor is a benign tumor clinically and pathologically, the appearance and growth pattern is unusual, so it may be misdiagnosed as malignancy. We report two cases of cotyledonoid dissecting leiomyoma of the uterus that occurred in two 44- and 31-year-old women, respectively. Total hysterectomy and bilateral salpingo-oophorectomy were performed in one of the patients, and myomectomy was done in the other one. Macroscopically, both tumors were grape-like exophytic masses resembling placental tissue. The patients were well after surgery, and one patient gave birth. To our knowledge, this is the first case report of a successful delivery after myomectomy of this tumor. To prevent aggressive surgery it is important to recognize that this tumor is a benign and unusual appearing variant of leiomyoma. A fertility-sparing surgical procedure should be considered if the patient wishes to preserve her fertility.

症例は49歳，男性．1カ月前から心窩部痛，半月前から尿の黄染に気づき来院した．腹部CT検査で膵頭部に不均一な造影効果を持つ腫瘤を認め，腫瘤の頭側に嚢胞性病変を認めた．MRCP検査では，総胆管と主膵管が嚢胞と腫瘍により圧排され拡張していた．血管造影検査では明らかな血管侵襲を認めず，膵頭部の腫瘤は不均一な造影効果を認めた．膵頭部癌と術前診断し，膵頭十二指腸切除術を施行した．術後，病理組織検査で4.3cm×4.2cm大の高分化型非機能性膵内分泌癌と診断された．主膵管は腫瘍によって破壊され，腫瘍の頭側に貯留嚢胞を認めた．総胆管は腫瘍により圧排され十二指腸乳頭近くで閉塞していた．術後経過は良好で退院した．

A case of endometrioid adenocarcinoma supposedly arising from endometriosis of the rectum is reported. Malignant transformation is uncommon but a well-known complication of endometriosis. In the present case, it was proved by histopathological findings and immunophenotype such as cytokeratin7+/cytokeratin20-/estrogen receptor+. The cause of rectal endometriosis in this case might have been related with previously received hormone replacement therapy for ovarian endometriosis. Following surgical removal of the lesion, this patient underwent adjuvant chemotherapy with paclitaxel and carboplatin, although this kind of therapy is still controversial as to its effectiveness.

The patient was a 74-year-old man suffering from tuberculotic chronic pyothorax. He had hematemesis in January 2006. Hb was 6.1 g/dl. A type 2 tumor 3 cm in diameter was found in the vaulted region on the greater curvature side. It was diagnosed as a malignant lymphoma. WBC and differential count were normal, and the patient tested negative for HTVL-1 antibody. sIL2-R was elevated to 1,500 U/ml. The superficial lymph nodes were not palpable. CT examination was not remarkable for the liver and spleen. There was no generalized lymph node enlargement. Based on these findings, a diagnosis of malignant lymphoma of gastric origin was made. As the patient had respiratory disorders, too, wedge-shaped gastrectomy was performed to inhibit invasion. Pathological examination revealed CD3 positive large atypical lymphocytes diffusely, EBV positive, HP negative. As a result, a diagnosis of non-Hodgkin T-cell lymphoma was made. The tumor did not return for 1 year and 8 months after surgery, but the patient died of sudden aggravation of respiratory disorders in September 2007. Pathological anatomy was performed. The gastric remnant was left with lymphoma, and the bone marrow and systemic lymph nodes were negative for a malignant lymphoma. The possibility of stomach metastasis from the preoperative pyothorax-related malignant lymphoma was considered, but was ruled out because the lungs were devoid of a malignant lymphoma. We report a case of an extremely rare malignant T-cell lymphoma of gastric origin.

症例は72歳，男性．約2カ月続く全身倦怠感，食欲不振を主訴に近医を受診し，肝腫瘍の診断で当科紹介され精査加療目的に入院．腹部CT検査および腹部超音波検査にて肝S6に径約4cm腫瘤像を認め，転移性肝癌もしくは胆管細胞癌が疑われた．また，腹部血管造影検査では肝細胞癌は否定的であった．肝悪性腫瘍を疑うも画像的検査で確定診断を得られず，超音波ガイド下肝針生検の結果，炎症性細胞浸潤と結合織の増生からなる病変を認め，炎症性偽腫瘍と診断した．その後，発熱および右季肋部痛が出現．保存的治療で軽快しないため，肝部分切除術を施行した．手術検体の病理結果も生検と同様であった．肝の炎症性偽腫瘍は比較的稀で，しばしば診断に苦慮する疾患である．今回われわれは悪性疾患との鑑別に苦慮し，生検で診断しえた肝炎症性偽腫瘍の1手術例を経験したので，若干の文献的考察を加え報告する．

症例は31歳，男性．突然の左側腹部痛を主訴に当院を受診した．腹部CT検査で下腹部正中に約10cm大の腫瘤を認め，腹腔内腫瘍の診断にて緊急入院となった．血液検査では炎症反応の上昇と共に，AFP 13,000ng/mlと著明な上昇を認めた．その後の検査でも確定診断には至らず，腹部症状も増悪してきたため診断，治療を目的に緊急手術を施行した．腫瘤は左後腹膜から立ち上がる茎を有し，反時計方向に720度回転し茎捻転を起こしていた．摘出標本は10×8×8cm大，腫瘤の割面は暗赤色モザイク状で，大部分は出血壊死に陥っていた．病理組織学的には精巣上体と精巣網を認める精巣由来の混合型胚細胞腫瘍であった．陰嚢内に正常の精巣が2個認められたことから自験例は，多精巣症に発症した混合型胚細胞腫瘍と診断した．多精巣症は検索した限り自験例を含め本邦報告例29例と極めて稀な疾患であり，若干の文献的考察を加え報告する．

We attempted to discriminate between carcinoma in situ (CIS) and the intraductal invasion/cancerization of invasive ductal carcinoma (IDC) of the pancreas, by comparing the histological patterns of the intraductal components and those of venous invasion. Specimens from 30 patients with IDC were examined histopathologically. Intraductal components and blood vessel invasion in IDC were assessed in specimens stained with hematoxylin & eosin and elastica van Gieson (EVG). Intraductal components of IDC were found in 28 of the 30 cases of IDC, in 261 ducts, and in 2.3 ducts per one section of one case, on average. The intraductal components of IDC were classified into three histological patterns, as follows: low papillary (including flat), tubular (including solid and cribriform), and mixed (low papillary plus tubular). The incidences of the low papillary, tubular, and mixed patterns in the 261 ducts, were 39% (102 ducts), 56% (145 ducts), and 5% (14 ducts), respectively. The histological pattern of venous invasion was tubular in all but 1 of the 26 cases, and this 1 case showed low papillary patterns as well as a tubular pattern. A tubular pattern of intraductal components in IDC of the pancreas indicates intraductal invasion, while a low papillary pattern indicates CIS or carcinoma in another location to which it has spread.

Mucinous cystic neoplasms (MCN) of the pancreas are mucin-producing cystic tumors with an ovarian-like stroma (OLS). In the present study MCN were obtained from 27 patients. These MCN were derived from 22 pancreas, three livers, spleen, and mesentery. MCN in various organs have common clinicopathological profiles, being unilocular or multilocular cystic tumors, with a fibrous capsule and lined by mucin-secreting epithelium associated with an underlying subepithelial OLS. The OLS showed strong positivity for alpha-smooth muscle actin (alpha-SMA) and vimentin and weak, focal positivity for desmin. Both estrogen receptors and progesterone receptors were expressed in the nuclei of OLS cells. In addition, 20 ovarian MCN and 13 normal ovaries were studied with particular attention to the stroma. The stroma of ovarian MCN was strongly immunopositive for alpha-SMA and vimentin and focally positive for desmin, whereas normal ovarian stroma was immunonegative for both alpha-SMA and desmin. The OLS of MCN mentioned here was similar to the septa of ovarian MCN but not to ovarian stroma. In conclusion, MCN in various organs should be lumped together as 'extra ovarian' MCN. The OLS was identified on the basis of myofibroblastic proliferation both in response to neoplastic development and dependent on hormones.

Purpose: To investigate the prevalence of human papillomavirus (HPV) infection in patients attending a gynecologic outpatient department, and to correlate the infection status with the presence or absence of uterine cervical lesions and the grades assessed by cytological or histological examinations. Methods: Five hundred and seventy-two subjects were studied. In all subjects, HPV detection by the hybrid capture method and a cervical cytological examination were performed Results: The HPV-positive rate in subjects with normal cytology was 12.3%. The detection rate was high (21.7%) in subjects aged in the twenties and low in the forties, and HPV was not detected in subjects aged in the sixties and seventies. When HPV-positive rates were examined according to cytological or histological grades, the rates were higher in subjects with abnormal cytology (P

Carcinosarcoma (spindle cell carcinoma) of the esophagus is a rare neoplasm that shows squamous cell carcinoma (SCC) with a variable component of spindle cell sarcoma. Clinical and pathologic features of this neoplasm have been well documented, but the histogenesis has long been a matter of speculation and dispute. In an attempt to clarify the clonality and genetic relationships in the evolution of this neoplasm, we microdissected a total of 36 carcinomatous and sarcomatous foci from six esophageal carcinosarcoma (CS) and analyzed the allelic status with 25 microsatellite markers on chromosomal arms 3p, 5q, 6q, 8p, 9p, 11q, 13q, 17p, and 18q. In all cases, we found multiple and homogenous allelic losses in both the carcinomatous and sarcomatous components, strongly supporting the concept of monoclonal origin for this neoplasm. Homogeneous allelic losses were detected most frequently on 17p (5 cases), a chromosomal arm that included the p53 locus, followed by 3p, 11q, and 13q (3 cases); 9p (2 cases); and 8p and 18q (1 case). Moreover, five of the six cases showed additional or divergent allelic losses at more than one chromosomal locus at some of the microdissected foci, indicating genetic progression (2 cases) or genetic progression and divergence (3 cases). In four cases, the genetic changes indicated that an original clone of a pure SCC apparently acquired carcinosarcomatous or sarcomatous phenotype by successive genetic changes. On the other hand, we saw no evidence for tumors in which a sarcoma appeared to give rise to a carcinosarcomatous or carcinomatous subclone in the examined cases. In conclusion, our data support the concept that esophageal CS is derived from a single clone originating from a SCC. Furthermore, we showed genetic heterogeneity to accompany the phenotypic divergence, with patterns of genetic alterations that are consistent with both progression and divergence within individual tumors.

背景:中枢性神経細胞腫central neurocytoma (以下, CN) は, 比較的新しい概念の腫瘍で細胞診の報告例はいまだ少ない. 今回われわれは, 右側脳室に発生したCNを経験したので, 術中の捺印および圧挫処理標本の細胞像を中心に報告する.症例:患者は28歳男性で, 頭部打撲にて救急外来を受診した. 頭部CTで右側脳室に石灰化を伴う腫瘍を認め, MRIでは腫瘍内部に嚢胞を伴っていた. 術中捺印細胞診標本で, 腫瘍細胞はライトグリーン淡染性の線維性物質を背景に, 散在性あるいは結合性の緩い細胞集塊としてみられ, 一部ではロゼット様の細胞配列を示していた. また, 核周囲が明るく抜けた細胞質を有する細胞境界明瞭な細胞も一部にみられた. 術中の組織所見では, エオジン淡染性の細胞質と均一な円形～類円形核を有する腫瘍細胞を認め, 細胞境界は不明瞭であった. 術中の細胞・組織所見と臨床所見からCNが疑われ, 術後の組織および免疫染色所見と電子顕微鏡的検索からCNと診断した.結論:CNの術中迅速診断において, 細胞診を併用することで, 凍結切片標本では捉えることのできない所見を得ることができた. これらの細胞学的所見に加え, 組織所見と臨床所見を考慮することによって, CNの術中迅速診断が可能になると思われた.

Rectovaginal (RV) endometriosis presents with a nodular lesion composed of fibromuscular and endometriotic tissue, and the fibromuscular tissue is the major component in the severe stage. The purpose of our study was to examine the extending process of fibromuscular tissue in RV endometriosis. Histological examinations using immunostains, were performed in 90 RV tissue specimens from 37 women. Fibrosis was present in 89 specimens. In each specimen, the intensity of the fibrosis differed from area to area: in mildly fibrotic areas, the collagen fibers were present around the endometriotic tissue, and in severely fibrotic areas, the fibrosis widely extended into fat and connective tissus as well as within the endometriotic tissue. In the 60 specimens containing endometriotic tissue, the increase in the amount of endometriotic tissue significantly correlated to the increase in degree of fibrosis in the entire tissue. The presence of aggregated smooth muscles, unassociated with blood vessels, was defined as smooth muscle metaplasia (SMM), which was always present within the fibrotic areas, and was observed in 80 specimens. The degree of SMM in the entire tissue was significantly correlated with the degree of fibrosis. From these findings, the following was hypothesized. Initially, endometriotic tissue was present sporadically and fibrosis was present around the endometriotic tissue. Thereafter, proliferation of endometriotic tissue and an increase in fibrosis occur consecutively. The SMM was present within the fibrotic areas, and it became more severe, correlating with the increase in fibrosis. In conclusion, this is the first report describing the extending process of the fibromuscular tissue of RV endometriosis from a histological viewpoint, and we think that recognization of this process is useful for histological diagnosis and clinical management of RV endometriosis.

Aims: To clarify the mechanism of origin of duodenal wall cysts in patients with chronic pancreatitis, developing into duodenal stenosis. Methods and results: Specimens from 12 pancreatoduodenectomized patients with chronic pancreatitis and 51 controls were studied histopathologically and immunohistochemically. Variously shaped cystic lesions, averaging about 15 mm in diameter, were found in the duodenum in six of the 12 patients with chronic pancreatitis, but were not observed in the controls. Each case had an average of two cysts, which were located mainly in the muscularis propria of the duodenum with or without submucosal or extraduodenal–peripancreatic extensions. The inner part of the cyst wall consisted of a moderate rim of granulation tissue, with both myofibroblasts and smooth muscle proliferation in the tissue surrounding the cyst and the submucosal layer of the duodenum, occasionally accompanied by an epithelial lining. A ductal structure in the muscularis propria of the duodenum, possibly a ductal component of ectopic pancreatic tissue, was found in five of the six cases. Some of these structures showed cystic changes. Three of the six patients had accompanying duodenal stenosis. Conclusions: Duodenal wall cysts occur mainly in the muscularis propria of the duodenum associated with both myofibroblasts and smooth muscle proliferation, and may result in duodenal stenosis. These cysts may be derived from a ductal component of ectopic pancreatic tissue.

Background: Invasive ductal adenocarcinoma of the pancreas (IDAP) also spreads through the pancreatic ductal tree. The aim of this study was to clarify the clinicopathologic feature

Eleven cases with mucinous noncystic carcinoma (MC) of the pancreas were studied by histology and mucin immunohistochemistry, to elucidate the mechanism, or route of development, and pre-existing histological type of MC of the pancreas. These MCs were observed in close approximation to, or surrounding, intraductal papillary-mucinous carcinomas (IPMCs), and were centrally situated among ductal adenocarcinomas (DAs). Hence, the 11 cases originated from 8 IPMCs and 3 DAs. The mechanism and routes to MC were divided into four types as follows: IPMC directly invaded the stroma (4 cases), over-production of mucin in IPMC expanded the branches of the pancreatic duct possibly resulting in rupture (3 cases), DA underwent extreme mucinous degeneration (3 cases), and a recurrent form, as MC, at the surgical stump of IPMC (one case). The outcomes of MC cases with IPMC had variable survival rates, while those from DA had short durations. MUC immunoreactivity in MC was divided into three categories; anti-MUC1-positive only (2 IPMCs, 2 DAs), mixed anti-MUC1 and anti-MUC2-positive (3 IPMCs, one DA) and anti-MUC-positive only (3 IPMCs). Pre-existing MC histological types included both IPMC and DA. These two pre-existing types of MC involved mucin overproduction and mucinous degeneration. MUC immunoreactivity in MC revealed three patterns, which may be related to variable outcomes.

Two rare cases of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) arising in the thymus are reported. Both patients (a 61-year-old man and a 75-year-old woman) were suffering from Sjögren's syndrome and immunoglobulin (Ig)A kappa monoclonal gammopathy. Mixed IgA-IgG cryoglobulinemia was also present in the male case. Tumor cells expressed IgA and kappa antibody reactive proteins identical with serum IgA kappa M. Moreover, we could demonstrate rearrangements of the immunoglobulin heavy and light chain genes, which supported the monoclonal origin of tumor cells. Immunological abnormalities improved after thymectomy in one case in which the tumor cells were confined to the thymus, but not the other with regional lymph node involvement, suggesting a causal role for the tumor. MALT lymphomas of the thymus thus appear to be associated with immunological disorders such as Sjögren's syndrome or monoclonal gammopathy.

A 63-year-old woman was admitted to our hospital with complaints of abdominal distention and bilateral cervical masses. Her serum AFP was 11700 ng/dl. AFP was confirmed immunohistochemically by biopsy of a sigmoid colon tumor, yielding a diagnosis of AFP-producing carcinoma of the sigmoid colon, accompanied by multiple liver metastases, and systemic multiple lymph node metastases. Due to her poor general condition with mitral regurgitation, the patient began S-1 treatment. The patient's general condition rapidly worsened after 1 course of S-1 had no effect. She died on the 59th hospital day and a pathological autopsy was performed. We examined 67 cases of AFP-producing colon cancer in Japan, including our own, and report the findings with references.

A case of endometrioid adenocarcinoma supposedly arising from endometriosis of the rectum is reported. Malignant transformation is uncommon but a well-known complication of endometriosis. In the present case, it was proved by histopathological findings and immunophenotype such as cytokeratin7+/cytokeratin20-/estrogen receptor+. The cause of rectal endometriosis in this case might have been related with previously received hormone replacement therapy for ovarian endometriosis. Following surgical removal of the lesion, this patient underwent adjuvant chemotherapy with paclitaxel and carboplatin, although this kind of therapy is still controversial as to its effectiveness.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare inflammatory tumor-like lesion composed of vascular nodules and non-neoplastic stroma including spindle cells and inflammatory cells. The focus of our study was on the stromal proliferating process in SANT. Nine cases of SANT were examined. All cases showed alpha-smooth muscle actin (alpha-SMA) and vimentin on the spindle cells but not CD21, CD31, CD34, CD68, desmin, S100, human herpes virus-8, or anaplastic lymphoma kinase-1. In one case, 20-30% of the myofibroblasts in Epstein-Barr-virus (EBV)-positive spindle cells were detected using double-labeling immunohistochemistry for alpha-SMA and EBV-encoded small RNA in situ hybridization. A quantitative analysis of IgG and IgG4-positive plasma cells (pPCs) in SANT was performed. The median densities of IgG-pPCs and IgG4-pPCs in SANT were approximately four-fold and 13-fold higher than those in the normal spleens, respectively. In addition, there was a statistically significant increase of IgG4/IgG-pPCs ratio in SANT in comparison to the control specimens. In conclusion, the fibrogenesis in a subset of SANT may be associated with EBV-infected myofibroblasts in an overlapping immune reaction indicated by the presence of infiltrating IgG4-pPCs. Further investigation is needed to elucidate the association between SANT and IgG4-related sclerosing disease.

We report two cases of large gastrointestinal stromal tumor (GIST) of the stomach both of which were assessed as highly malignant, but took different clinical courses. Case 1: A 72-year-old male. Case 2: A 63-year-old female. The tumor size of Case 1 was suggestive of high malignancy, but only a partial gastrectomy was selected because it did not show any invasive findings. This patient has been followed up for 3 years post-operatively and no recurrence or metastasis has been noted. Case 2 had liver and lymph node metastases, which was consistent with high malignancy. We performed a total gastrectomy with distal pancreatosplenectomy and segmental liver resection. But after surgery, liver metastasis recurred therefore, imatinib mesylate was administered as adjuvant chemotherapy and since then, the tumor has been diminishing in size. No definitive evidence for adjuvant therapy has been established so far, but we suggest that post-operative adjuvant therapy is effective for high-risk GIST.

To examine aberrations and differences of cell cycle regulatory proteins between intraductal papillary-mucinous neoplasms (IPMNs) and pancreatic intraepithelial neoplasias (PanINs).In total, 47 IPMN lesions and 42 PanIN lesions were obtained from 26 patients with IPMN and 16 patients who underwent pancreatic surgery for invasive pancreatic ductal cancer or other diseases. They were subjected to conventional hematoxylin-eosin staining and immunostaining for p16INK4A and p53. The percentages of immunohistochemical positivity or negativity were compared between IPMN and PanIN, in accordance with the same histological grade of atypia. The Ki-67 labeling index was also counted in each lesion.Either the loss of p16INK4A expression or the overexpression of p53 was much more frequently observed among PanIN-3 than among carcinoma in situ in IPMN (P = 0.046 and 0.008, respectively). The Ki-67 labeling index was correlated with the histological grades of both PanINs and IPMNs (P = 0.0001 and P = 0.0001, respectively).There are different immunohistochemical expression patterns of p16INK4A and p53 between IPMNs and PanINs. These may substantiate their different genetic progressions to invasive carcinoma.

Invasive ductal carcinoma (IDC) of the pancreas arises in the ductal epithelium and extends into the pancreatic tissue via stromal invasion. Carcinoma in situ (CIS) is believed to be a precursor les

Accurate diagnosis of cystic lesions of the pancreas is important due to their varied clinical course and behavior. Pancreatic cystic neoplasms fall predominantly into two groups: mucinous cystic tu

The papilla of Vater is a cylindrical protuberance that houses a common channel or the terminations of the common bile duct and main pancreatic duct. In cases of pancreaticobiliary maljunction, the

We aimed to elucidate the origin/primary site of invasive ductal adenocarcinoma of the pancreas, based on the distribution of intraductal carcinoma components. These components were identified by a mural elastic fiber cuff.Thirteen specimens from patients with invasive ductal adenocarcinoma (microscopically, less than 2 cm in diameter) of the pancreas were studied histopathologically. Variants of invasive ductal adenocarcinoma and intraductal papillary-mucinous carcinoma were excluded.Intraductal carcinoma components of invasive ductal adenocarcinoma were found in 12 of the specimens 13 (92%), and were observed within the tumor mass and/or on its boundary, or outside the tumor mass. Intraductal components were characterized by low papillary projections lacking a fibrovascular core, with/without surrounding tubular structures, or by irregular stratification and pleomorphism of the epithelial cells. Invasive components mostly showed a tubular pattern with desmoplasia. The distribution of the intraductal components in the 12 specimens was as follows: in 9 (75%), they were in both the main pancreatic duct and large branch ducts; and in 3, they were in the smaller branch ducts only.Invasive ductal adenocarcinomas of the pancreas may originate most frequently from the main pancreatic duct or larger branch ducts, while the smaller ducts are less often the site of cancer origin.

Primary liposarcoma of bone is exceedingly rare. We report a case of primary pleomorphic liposarcoma in the humerus of a 38-year-old female. Radiographs demonstrated an expansile and osteolytic lesion extending from the head to the proximal part of the shaft in the right humerus. MR study showed a defined lesion in the humerus. The lesion had an iso-signal intensity and partial high-intensity lesion on T1-weighted images and a heterogeneous high signal intensity on T2-weighted images. Wide resection and proximal humeral endoprosthesis replacement were performed. Final pathological diagnosis of the lesion was pleomorphic liposarcoma. This is the second reported case of pleomorphic liposarcoma of the bone, and the first case presenting MRI findings for liposarcoma of bone.

Although primary Sjogren's syndrome (pSS) is an autoimmune exocrinopathy, the involvement of liver has been reported. Because no study focusing on autoimmune hepatitis (AIH) in pSS has been published, the purpose of the present study was to perform a clinical and histological examination of the liver, focusing on AIH, in 17 pSS patients. The patients had liver enzyme abnormalities without hepatitis virus infection. In all cases, biopsied livers were examined, and in 10 cases biopsied labial salivary glands were also examined histologically. Based on the authors' diagnostic criteria for AIH in pSS, the liver diseases consisted of AIH (eight cases, 47%), primary biliary cirrhosis (PBC; six cases, 35%), non-specified chronic hepatitis (two cases, 12%) and acute hepatitis (one case, 6%). Lymphoplasmacytic infiltrate, with predominancy of CD3(+) T cells, was noted in both the liver and salivary glands in the patients with AIH. The patients with AIH with severe interface hepatitis had a good response to immunosuppressive therapy. The comparison of liver histology between the PBC with pSS group and the PBC without pSS group showed that the incidence of lymphoid non-suppurative cholangitis was higher in PBC with pSS. In conclusion, the present study offers new information on the relatively common occurrence, diagnostic criteria and treatment effects of AIH in pSS.

The role of the ampullary mucosa, especially its distended glands at the papilla of Vater, has not been fully explored.Twenty-nine pancreatoduodenectomized specimens from pancreatobiliary diseases and 44 autopsied cases, as controls, were studied histopathologically and immunohistochemically.In 12 out of the 29 pancreatoduodenectomized cases the ampullary mucosa was in contact with the duodenal mucosa just at the outlet of the ampulla. In the remaining 17 cases, the ampullary mucosa overgrew beyond the ostium, replacing a portion of the surrounding duodenal mucosa, termed "distended glands," which measured an average of 1532 microm in length. The muscularis mucosae of the duodenum and the Oddi's sphincter muscle merged in an "end-to-end, sharp-angled" manner at the ostium in the former, whereas this occurred in an "end-to-side, less sharp, rather right-angled" manner in the latter. Immunohistochemically, the distended glands in some cases showed negative/weakly positive staining for anti-carbohydrate antigen (CA) 19-9 and a high proliferation index evaluated using Ki67. In the autopsied materials, distended glands were found in 24 out of the 44 cases.Distended glands of the ampullary mucosa were frequently found and only grew on the Oddi's sphincter muscle extension. They may represent not only malignant change but also an adaptive phenomenon for bile and pancreatic juice flow.

Two Japanese brothers were diagnosed in their 20s with familial pulmonary fibrosis, the pathological findings of which were consistent with usual interstitial pneumonia (UIP). However, an atypical characteristic was observed in the lungs of these brothers; 2-mm areas of ‘honeycomb’ were identified throughout the lungs, which is smaller than the generally observed 5–10 mm honeycombing seen in UIP. Fibroblastic foci were demonstrated in the second eldest brother, but not in the eldest, which indicates that the lungs of the eldest brother was in a more advanced stage of fibrosis. Their youngest brother and parents have no clinical evidence of pulmonary fibrosis. All five family members had low values for the diffusion capacity of the lung for carbon monoxide (DLCO), suggesting the presence of an inheritable disease and the existence of different phenotypes. The genomic DNA of the affected brothers was sequenced for the reported surfactant protein C (SP-C) gene mutations in patients with familial pulmonary fibrosis, but none was documented. It is necessary to clarify the presence of novel gene mutations of SP-C or other genes to explain these particular pathological findings and the low DLCO observed in this family.

Purpose Esophageal carcinoma is a challenging target for radiotherapy. To improve treatment efficacy, an investigation of a predictive factor is desirable. In this study, we evaluated the significance of apoptosis and immunohistochemical staining for p53, Ki-67, c-erbB-2 (HER-2/neu), Ku (p70/p80), and DNA-PKcs for predictive markers of the responsiveness to chemoradiotherapy in esophageal squamous cell carcinoma. Methods and materials This retrospective analysis consisted of 34 patients with esophageal squamous cell carcinoma in whom tumor biopsy was performed before treatment. They were divided into chemoradiosensitive ( n = 13) and chemoradioresistant ( n = 21) groups according to the tumor response evaluated at a total radiation dose of 40 Gy. The biopsy samples were examined with immunohistochemical staining for various factors and with an in situ nick end labeling method for apoptosis. The examined data were compared between the two groups. Results The difference in the Ki-67, p53, Ku (p70/p80), DNA-PKcs labeling indexes and the apoptosis index in tumor cells between the chemoradiosensitive and chemoradioresistant groups was not statistically significant. The expression of c-erbB-2 oncoprotein was statistically significant in the chemoradioresistant group ( p = 0.02), although it did not correlate with survival. Conclusion c-erbB-2 immunostaining is useful for the prediction of chemoradioresistance in esophageal squamous cell carcinoma.

Many intraductal papillary-mucinous tumors (IPMTs) have been diagnosed with improvements in diagnostic imaging techniques. The histology of IPMT is various, including hyperplasia to invasive carcinoma. IPMTs are thought to occur multicentrically through the hyperplasia-adenoma-carcinoma sequence. IPMTs are characterized by genetic heterogeneity associated with histologic heterogeneity that may be due to slow growth and favorable prognosis. However, some IPMTs progress to invasive pancreatic cancer through malignant transformation with aggressive clonal progression. Thus some conventional pancreatic cancers may be derived from IPMTs. Although IPMTs and conventional pancreatic cancer initially occur in the ductal epithelium, they are thought to be totally different entities in terms of large or peripheral pancreatic duct origin, developmental style, genetic alterations, and prognosis. In future, differences and/or similarities will be discussed on the basis of molecular analyses of the carcinogensis of both tumors.

Invasive ductal adenocarcinoma of the pancreas (IDAP) also spreads through the pancreatic ductal tree. The aim of this study was to clarify the clinicopathologic features of IDAP with intraductal spread.We studied the intraductal spread of IDAP and its correlation with clinicopathologic parameters in a surgical series of 54 patients. The pancreatic ducts were analyzed by confirmation of mural elastic fibers with elastica van Gieson stain.Intraductal spread of carcinoma was identified in 37 patients (69%). Such spread was frequent in well-differentiated IDAP (93%), and the number of intraductal carcinoma foci was correlated with the grade of tumor differentiation (p0.001). The large branch ducts were the main route of intraductal spread (64.1%). The proliferation index, evaluated using Ki67, was lower in the intraductal carcinoma components than in the associated infiltrating carcinoma components (p0.001). The presence or absence of intraductal spread was not correlated with age, sex, tumor location, tumor size, or stage. IDAP with intraductal spread showed a tendency, although it was not significant (p = 0.092), to be associated with longer survival compared with IDAP without intraductal spread.IDAP, especially of the well-differentiated type, has a tendency to spread intraductally. The difference between the Ki67 labeling indexes in the intraductal and associated infiltrating carcinoma components suggests that these components show different biological behaviors.