Search

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren's syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren's syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.

Background/Aims The objective of this study was to elucidate the histological structure of the absent microsurface patterns (MSPs) that were visualized by magnifying endoscopy with narrow-band imaging (M-NBI). Methods The study included consecutive gastric epithelial neoplasias for which M-NBI findings and histological findings could be compared on a one-to-one basis. The lesions were classified as absent MSPs and present MSPs based on the findings obtained using M-NBI. Of the histopathological findings for each lesion that corresponded to M-NBI findings, crypt opening densities, crypt lengths, crypt opening diameters, intercrypt distances, and crypt angles were measured and compared. Results Thirty-six lesions were included in the analysis; of these, 17 lesions exhibited absent MSP and 19 lesions exhibited present MSP. Comparing the histological measurements for absent MSPs vs. present MSPs, median crypt opening density was 0.9 crypt openings/mm vs. 4.8 crypt openings/mm (p

This study aims to investigate the clinicopathological features of in situ follicular neoplasm (ISFN) in Japan. ISFN is a rare condition formerly considered as an early precursor of follicular lymphoma (FL). This is a first original report of ISFN from Asian country. We reviewed 19 biopsy samples of ISFN. ISFNs were categorized into two groups: (1) ISFN, consisting of ISFN with strong positivity for BCL-2 immunohistochemical staining (IHC), and obvious translocation of BCL-2; and (2) ISFN-like FL, featuring cases without obvious translocation but having morphological features and characteristic IHC findings of ISFN. As control, we adopted obvious FL. For some cases showing coexisting ISFN and FL lesions in the same lymph node, we could conduct further clonality analysis for each lesion. Nine of the 19 cases of ISFN coexisted with FL or had a history of overt B- or T-cell lymphoma including FL. Statistical comparison among ISFN-like FL and FL showed no significant differences in pathological features. Molecular analysis suggested that ISFN lesion and FL lesion in the same lymph node each have a different clonality. ISFN coexists or associates with other overt lymphomas frequently.

Laryngeal sarcoidosis is rare, and reports of recurrences after treatment are even rarer. A 49-year-old Japanese female patient visited our clinic with a 7-day history of dysphagia. Her medical history included sarcoidosis involving the cervical lymph nodes. Endoscopic examination revealed a diffusely swollen epiglottis and bilateral edema of the arytenoids. Corticosteroids and antibiotics were administered, but the laryngeal edema did not subside. Contrast-enhanced computed tomography imaging revealed bilateral low-density areas in the arytenoids. Therefore, we performed bilateral arytenoid biopsy under direct laryngoscopic guidance. The histopathology showed noncaseating epithelioid cell granulomas with moderate to severe chronic inflammatory infiltration; based on these findings, we diagnosed the patient as having laryngeal sarcoidosis. While the laryngeal edema did not recur for several months, arytenoid edema recurred 9 months after systemic steroid therapy. The patient's medical history of sarcoidosis of other organs as well as the presentation of edema of a supraglottic lesion and histopathological findings of noncaseating epithelioid cell granulomas led us to make the diagnosis of laryngeal sarcoidosis again. Laryngeal sarcoidosis is an important consideration in the differential diagnosis of laryngeal edema, as its therapy and clinical course are different from those for other diseases that present with laryngeal edema. A cautious follow-up of patients diagnosed as having laryngeal sarcoidosis is needed, because in rare cases, the laryngeal edema can become worse over time even after therapy.

An 83-year-old woman underwent laparoscopic distal gastrectomy and Billroth Ⅱ reconstruction for gastric cancer. Since histopathological examination revealed that the lesion was Stage ⅢA, she had started taking S-1 as an adjuvant chemotherapy 7 weeks after gastrectomy. Seventeen days later after taking S-1 administration, she felt nauseous and self-interrupted. Nineteen days later, she was urgently hospitalized. Esophagogastroduodenoscopy(EGD)showed anastomotic lumen was open, but reconstructed small intestine mucosal damage was found, and reconstructed small intestine muscle layer was fused to anastomotic region. On 50th day of hospitalization, mucosa was regenerated and endoscopic balloon dilatation (EBD)was performed from 78th day. She was discharged on 151th day of hospitalization after 7 times of EBD. One year later, she does not need EBD and can be taken orally and has no recurrence.

Introduction: Kawasaki disease (KD), which is the most common multisystem vasculitis with unknown causes in childhood, causes coronary artery (CA) aneurysms. High-dose intravenous immunoglobulin (IVIG) is the most standard treatment. However, the problem with current high-dose IVIG therapy is that it is inefficient for both patients and cost because it contains ineffective parts and requires a high total dose. It has recently been reported that sialic acid in the fragment crystallizable (Fc) region has an anti-inflammatory effect. Hypothesis: In the KD model, a small amount of sialic acid-rich (SR-)Fc has an anti-inflammatory effect equivalent to that of high-dose IVIG. Methods: A Candida albicans cell wall extract (CAWE)-induced KD-like vasculitis model was established in DBA/2 mice. IgG, antigen-binding fragment (Fab), and Fc with 2g/kg/0.5ml, and IgG, SR-IgG, and SR-Fc with 0.2g/kg/0.5ml, and placebo 0.5ml were administered intraperitoneally 3 times every other day (n=5, respectively). Four weeks after treatment, we compared the incidence of CA inflammation between each group. Histological analysis was performed by using a quantified scoring system: grade 1; 100cels/HPF, grade 4; distraction of elastic lamina, grade 5; neointimal proliferation. Results: All five mice in placebo group had the infiltration grade 3 or higher (100%), whereas none of the mice in IgG group had the infiltration (0%). In Fab group, three out of four had the infiltration (75%, one mouse died), whereas in Fc group, only one out of five had the infiltration (20%). In one-tenth dose IgG group, two out of four had the infiltration (50%, one mouse died), whereas in one-tenth dose SR-IgG and SR-Fc group, 20% or less of them had the infiltration (one out of five, zero out of five, respectively). Treatment with one-tenth dose SR-IgG and SR-Fc significantly suppressed CAWE-induced vasculitis ( p Conclusions: It was shown that the immunoglobulin effect was mainly in the Fc region and one-tenth dose SR-Fc had an anti-inflammatory effect equivalent to that of high-dose IgG in a Candida -induced KD mouse model. One-tenth doses SR-Fc may be an alternative treatment to high-dose IVIG in KD patients.

Background and study aims A white opaque substance (WOS) has been observed in the epithelia of gastric, duodenal, and colorectal epithelial adenomas and carcinomas, using magnifying endoscopy (ME). The WOS has been reported to be derived from a dense accumulation of minute lipid droplets in the epithelium. This study aimed to investigate whether the WOS in colorectal hyperplastic polyps was derived from lipid droplets accumulated in the epithelium, as observed in the case of gastric, duodenal, and colorectal epithelial neoplasms. Patients and methods We analyzed 30 consecutive patients who were positive for the WOS, as visualized in colorectal hyperplastic polyps by ME with narrow-band imaging and 30 consecutive patients who were negative for the WOS. Biopsy specimens obtained from the polyps were immunostained with anti-adipophilin antibody to determine the correlation between the presence of the WOS and that of lipid droplets in the epithelium. Results In all patients, the epithelial cells were histologically positive for adipophilin. However, the area of adipophilin-positive epithelial cells in the WOS-positive group was significantly larger than that in the WOS-negative group (P Conclusions This study reveals that the WOS visualized in the superficial layers of colorectal hyperplastic polyps is produced by a dense accumulation of minute lipid droplets in the epithelia of the polyps.

Primary central nervous system lymphoma (PCNSL) is a rare malignant tumor of the central nervous system. It is associated with poor prognosis. Early diagnosis and subsequent planning of adequate treatment strategy are relevant to improve survival and reduce neurological deficit. Specifically, there are no reports of successful endoscopic biopsy for PCNSL of the corpus callosum in the splenium with bilateral visuomotor ataxia. An 74-year-old woman presented to our hospital with anorexia, depression and ataxia beginning six months earlier. Head computed tomography and magnetic resonance imaging showed malignant tumor suspected in the corpus callosum. Endoscopic biopsy was performed via the low parieatal approach, suspecting GBM or PCNSL. She had no new postoperative neurological deficits and was pathologically diagnosed with diffuse large B-cell lymphoma (DLBCL). She is currently undergoing radiation chemotherapy with a modified Rankin Scale score of 2. Regarding preoperative symptoms, ataxia was considered to be bilateral visuomotor ataxia caused by damage to the corpus callosum in the splenium, and anorexia and depression were considered symptoms of the surrounding limbic system. Delay in the diagnosis of PCNSL can lead to a poor prognosis. Visuomotor ataxia should also consider the potential for the corpus callosum in the splenium lesion, including PCNSL, and appropriate imaging and pathological diagnosis with endoscopic biopsy can contribute to a good clinical outcome.

There are few reports of pituitary adenomas (PA) mimicking dementia. Delay in disease diagnosis and treatment may result in poor clinical outcome. We experienced a rare case where endoscopic transsphenoidal surgery (eTSS) effectively treated a gonadotroph adenoma mimicking dementia and report on literature considerations. We report the case of a 72-year-old man with chief complaints of cognitive decline, bradykinesia, anorexia, dressing apraxia, and vigor decline over several months. He was admitted to our hospital for scrutiny in a disoriented state. Blood tests showed hyponatremia and thyroid hormone depression. Magnetic resonance imaging showed a pituitary tumor, and preoperative endocrine stress tests showed reduced reactivities of growth hormone, adrenocorticotropic hormone/cortisol, and luteinizing hormone/follicle-stimulating hormone. Symptomatic pituitary adenoma was suspected, and eTSS was performed. The permanent pathological diagnosis was of gonadotroph adenoma. Postoperatively, the hyponatremia, cognitive decline, movement retardation, loss of appetite, dressing apraxia, and limb edema markedly improved. The patient was discharged under hydrocortisone 15 mg/day administration without complications. The endocrine stress test performed 2 months postoperatively showed secondary hypoadrenocorticism, while the other endocrine functions had normalized. No recurrence had occurred by 30 months postoperatively; the medication of hydrocortisone was gradually discontinued and the patient at the time was still being followed as an outpatient with modified Rankin Scale score 0. Secondary hypothyroidism and secondary hypoadrenocorticism due to the pituitary tumor primarily caused the condition. It is important to consider PA in the differential diagnosis of dementia, and early diagnosis and treatment can contribute to a patient's good clinical outcome.

A 74-year-old man was initially diagnosed as having cT4N0M0 and Gleason score 5+4 prostate adenocarcinoma in 2012. Systemic therapy was initiated with luteinizing hormone-releasing hormone (LH-RH) agonist and bicalutamide, and serum prostate-specific antigen (PSA) levels fell to a nadir of 0.02 ng/ml from 25.55 ng/ml.After 3 years of initial treatment, the patient presented with metastatic castrate-resistant prostate cancer (mCRPC) with extensive bulky lymphadenopathy and a serum PSA of 4.81 ng/ml. Open biopsy of the left supraclavicular lymph node revealed metastasis by poorly-differentiated adenocarcinoma of prostatic origin. He continued to receive LH-RH agonist and bicalutamide and underwent seven courses of docetaxel (DOC) chemotherapy plus prednisolone. Computed tomography showed partial response in all but one metastatic pelvic lymph node, which gradually increased in size. The mCRPC response to DOC was heterogeneous, and DOC chemotherapy was stopped because of toxicity and progressive disease.Second-line hormonal therapy with enzalutamide and LH-RH agonist was started and after 6 months, computed tomography revealed complete response in the metastatic lymph nodes based on response evaluation criteria in solid tumors (RECIST); PSA levels decreased to 0.01 ng/ml. The patient has been in complete remission for 28 months.

Background It has been reported that it is sometimes difficult to make a diagnosis of gastric cancer detected after Helicobacter pylori (H. pylori) eradication. Therefore, we conducted a study to determine the usefulness of magnifying endoscopy using vessel plus surface classification system for making a diagnosis of early gastric cancer after H. pylori eradication. Method Usefulness of the markers of the vessel plus surface classification system for diagnosing gastric cancer using magnifying endoscopy with narrow-band imaging was investigated for gastric cancer detected after H. pylori eradication (H. pylori-eradicated group) vs. H. pylori-positive gastric cancer (H. pylori-positive group). Results 85 lesions were included in this study. The H. pylori-eradicated group comprised 27 lesions, whereas the H. pylori-positive group comprised 58 lesions. As for magnified endoscopic findings based on the vessel plus surface classification system, the respective positivity rates in the H. pylori-eradicated group vs. those in the H. pylori-positive group were as follows: demarcation line, 100% (27/27) vs. 100% (58/58); irregular microvascular pattern, 100% (27/27) vs. 100% (58/58); and irregular microsurface pattern, 78% (21/27) vs. 95% (55/58). There was no significant difference in the positivity rates of the demarcation line and irregular microvascular pattern between the groups. The positivity rate of the irregular microsurface pattern was significantly lower in the H. pylori-eradicated group than in the H. pylori-positive group (P=0.03). Conclusion We presume that the microvascular pattern is more useful than the microsurface pattern as a diagnostic marker in cases of gastric cancer detected after H. pylori eradication.

Background and study aims Magnifying endoscopy with narrow-band imaging (M-NBI) is reported to be useful in diagnosing invasion depth of superficial esophageal squamous cell carcinoma (SCC), but accurate diagnosis of deep submucosal invasion (SM2) has remained difficult. However, we discovered that irregularly branched microvessels observed with M-NBI are detected in SM2 cancers with high prevalence. Thus, this retrospective study aimed to investigate the diagnostic performance of irregularly branched microvessels as visualized by M-NBI for predicting SM2 cancers. Patients and methods Patients with superficial esophageal SCC lesions that were endoscopically or surgically resected at our hospital between September 2005 and December 2014 were included. Endoscopic findings by M-NBI of these lesions were presented to an experienced endoscopist who was unaware of the histopathological diagnosis and who then judged whether irregularly branched microvessels were present. Using the invasion depth according to postoperative histopathological diagnosis as the gold standard, we determined the diagnostic performance of the presence of irregularly branched microvessels as an indicator for SM2 cancers. Results A total of 302 superficial esophageal SCC lesions (228 patients) were included in the analysis. When irregularly branched microvessels were used as an indicator of SM2 cancers, the diagnostic accuracy was 94.0 % (95 % confidence interval [CI]: 91.1–96.1 %), sensitivity was 79.4 % (95 % CI: 66.6–88.4 %), specificity was 95.9 % (95 % CI: 94.3–97.0 %), positive predictive value was 71.1 % (95 % CI: 59.6–79.1 %), and negative predictive value was 97.3 % (95% CI: 95.7–98.5 %). Conclusions Irregularly branched microvessels may be a reliable M-NBI indicator for the diagnosis of cancers with deep submucosal invasion.

Kawasaki disease is the most common acute systemic vasculitis of unknown etiology in children [1] and can cause inflammation of the coronary arteries leading to aneurysms. Candida albicans extract is one of the materials commonly used to induce coronary arteritis in a mouse model of Kawasaki disease [2, 3]. Here we report an optimized method for preparing C. albicans cell wall extra (CAWE).

Background/Aims: While the occurrence of multiple whitish flat elevated lesions (MWFL) was first reported in 2007, no studies on MWFL have been published to date. The present retrospective observational study aimed to clarify the endoscopic findings and clinicopathological features of MWFL. Methods: Subjects were consecutive patients who underwent upper gastrointestinal endoscopy as part of routine screening between April 2014 and March 2015. The conventional white-light, non-magnifying and magnifying narrow-band images were reviewed. Clinical features were compared between patients with and without MWFL. Results: The conventional endoscopic findings of MWFL include multiple whitish, flat, and slightly elevated lesions of various sizes, mainly located in the gastric body and fundus. Narrow-band imaging enhanced the contrast of MWFL and background mucosa, and magnifying narrow-band imaging depicted a uniformly long, narrow, and elliptical marginal crypt epithelium with an unclear microvascular pattern. Histopathological findings revealed hyperplastic changes of the foveolar epithelium, and parietal cell protrusions and oxyntic gland dilatations were observed in the fundic glands, without any intestinal metaplasia. The rate of acid-reducing drug use was significantly higher in patients with MWFL than in those without (100% [13/13] vs. 53.7% [88/164], p

Before surgery for primary aldosteronism (PA), localization is evaluated with adrenal vein sampling (AVS). A 56-year-old Japanese woman had a right adrenal mass, hypokalemia, and a high aldosterone/renin ratio. Stress tests confirmed the diagnosis of PA. Subsequently, preoperative AVS was performed and right adrenal hemorrhage (AH) occurred unexpectedly. Because hypertension persisted, laparoscopic right adrenalectomy was performed. Postoperatively, the blood pressure was normalized. Pathological examination revealed an adrenal cortical adenoma largely unaffected by necrosis and hemorrhage. Previous reports have also indicated that AH may not ameliorate PA. We discussed the clinical progress of AH and the measures to prevent causing AH.

An 81-year-old man was admitted to our hospital with abdominal distension due to refractory ascites of unknown origin. He subsequently died of aspiration pneumonia. Autopsy revealed a diagnosis of biphasic malignant peritoneal mesothelioma (MPM) containing both epithelioid and sarcomatous components. The diagnosis of MPM is often difficult because serum tumor markers, imaging studies, and the cytology of ascites may not provide enough information. Accordingly, peritoneal biopsy is necessary in order to diagnose MPM based on the histological and immunohistochemical findings.

This study aims to investigate the clinicopathological features of in situ follicular neoplasm (ISFN) in Japan. ISFN is a rare condition formerly considered as an early precursor of follicular lymphoma (FL). This is a first original report of ISFN from Asian country. We reviewed 19 biopsy samples of ISFN. ISFNs were categorized into two groups: (1) ISFN, consisting of ISFN with strong positivity for BCL-2 immunohistochemical staining (IHC), and obvious translocation of BCL-2; and (2) ISFN-like FL, featuring cases without obvious translocation but having morphological features and characteristic IHC findings of ISFN. As control, we adopted obvious FL. For some cases showing coexisting ISFN and FL lesions in the same lymph node, we could conduct further clonality analysis for each lesion. Nine of the 19 cases of ISFN coexisted with FL or had a history of overt B- or T-cell lymphoma including FL. Statistical comparison among ISFN-like FL and FL showed no significant differences in pathological features. Molecular analysis suggested that ISFN lesion and FL lesion in the same lymph node each have a different clonality. ISFN coexists or associates with other overt lymphomas frequently.

This paper provides an overview of the principles of a vessel plus surface (VS) classification system to explain the diagnostic system of early gastric cancer using image-enhanced magnifying endoscopy. Furthermore, this paper introduces the magnifying endoscopy simple diagnostic algorithm for gastric cancer (MEADA-G) developed according to the VS classification system, with a description of the procedures performed for diagnosis. In addition to the diagnostic system, white opaque substance (WOS), light blue crest (LBC), white globe appearance (WGA), and vessels within epithelial circle (VEC) patterns, which are representative findings that can be observed in the gastric mucosa by image-enhanced magnifying endoscopy, are also described. Image-enhanced magnifying endoscopy is particularly useful in the diagnosis of differentiated-type early gastric cancer. It is important to use the appropriate clinical strategies based on a comprehensive understanding of the usefulness and limitations of the diagnostic system described in this paper.

Background Undifferentiated high-grade pleomorphic sarcoma in gastrointestinal tract is extremely rare, and its prognosis is poor. Case presentation An 82-year-old man visited a previous hospital complaining of fever, general fatigue, and shaking chill, for which he received antibiotics therapy. As the fever continued, he was referred to our hospital, where computed tomography and upper gastrointestinal endoscopy showed a 6-cm gastric tumor. A preoperative biopsy was consistent with a malignant mesenchymal tumor, but could not provide a definitive pathological diagnosis nor prove a cause-and-effect relationship between the chief complaint and the gastric tumor. The gastric tumor had grown to 8 cm in diameter within a month so we performed a partial gastrectomy. The pathological postoperative diagnosis was undifferentiated high-grade pleomorphic sarcoma that produced granulocyte colony-stimulating factor. The patient’s fever quickly improved, and he showed a good postoperative course. Conclusions We herein report a case of rapidly growing, undifferentiated, high-grade pleomorphic gastric sarcoma, which presented as a chief complaint of fever.

Crystal-storing histiocytosis (CSH) localized to the stomach is very rare, with only two cases reported to date. The present patient was a 55-year-old woman with no noteworthy symptoms. An area of discoloration was seen in the greater curvature of the gastric body on upper endoscopy by a local physician, and CSH was strongly suspected on biopsy examination. On upper endoscopy performed at our hospital, a flat, whitish, finely granular membrane with a diameter of 10 mm was seen in the greater curvature of the gastric body on white light observation. On magnifying endoscopy of the focal site, white granular material could be seen in the subepithelium, and the surface layer microvascular architecture image seen below the mucosal subepithelium was similar to the background, suggesting localization of the white material in a deep mucosal layer. On endoscopic ultrasound, this was depicted as multiple hyperechoic spots within the second layer corresponding to the involved area. Endoscopic submucosal dissection (ESD) was performed for a definitive diagnosis. Histopathological assessment of resected specimens revealed infiltration of abundant histiocytes including acidophilic crystalline structured material and positive plasma cells on anti-κ chain immunostaining, and CSH was diagnosed. Since this patient could have had a concomitant background autoimmune or hematologic disease, a systemic search was done. During this search, comorbid Sjögren's syndrome was discovered. This case of asymptomatic Sjögren's syndrome discovered during investigation of very rare gastric CHS is reported.

Aim: Recent reports have focused on the development of secondary amyloidosis (AMY) as a complication of Crohn's disease (CD). The present study was carried out to investigate the frequency of AMY secondary to CD, its clinical and endoscopic features, and the importance of duodenal biopsy in detecting this disease. Methods: This study involved 408 patients diagnosed with CD who were endoscopically and histologically examined at our hospital. At follow up, we analyzed the incidence of AMY complications, the clinical features of AMY and the methods to diagnose AMY. Results: The incidence of AMY was 2.5% (10/408). The disease type at the time of CD diagnosis was small and large bowel type (SL) in eight patients, small bowel type in one and large bowel type in one. The incidence of AMY was significantly higher in patients with SL than in patients with other disease types. The length of time from onset of CD to diagnosis of AMY was 14.1 ± 8.0 years. The cumulative incidence of AMY was 1.0% at 10 years and 5.7% at 20 years after onset. In terms of the method used to diagnose AMY, the positive rate of AMY diagnosis was 100% with endoscopic duodenal biopsy. Conclusion: The incidence of AMY as a complication of CD was low (2.5%). However, because this complication adversely affects patients' prognoses, it is important to check for the presence of AMY, particularly in the duodenum, in patients for whom more than 10 years have elapsed since the development of CD.

We herein report a case of huge gastric carcinoma showing an exophytic growth pattern. The gastric carcinoma measured 160 × 130 mm in size. A radical resection was judged to be impossible preoperatively since the tumor invasion of the pancreas and liver was demonstrated on computed tomography. A pancreaticoduodenectomy combined with a resection of the transverse colon was performed. A pathological examination demonstrated the tumor to directly invade the pancreas and transverse colon; however, no metastasis was observed in the regional lymph nodes. The patient is alive and doing well without any recurrence at 5 years postoperatively. To obtain a better prognosis for huge gastric carcinoma showing an exophytic growth pattern, extended radical surgery is recommended since the size of the exophytic mass sometimes does not indicate the extent of the tumor.

Reported herein is a 25-year-old woman who was treated for a large and highly atypical prolactin-producing pituitary adenoma. On presentation, she exhibited right hemiparesis and left-sided visual loss, associated with amenorrhea. A massive (>5 cm) intra- and suprasellar lesion was seen on imaging, and her serum prolactin level was 4408 ng/ml. The patient received dopamine agonist treatment preoperatively for 4 weeks. To resect the tumor, a two-stage excision was required. Histologically, the specimen was composed of polygonal or spindle cells showing marked nuclear pleomorphism and/or multinucleation. Fibrosis was also focally conspicuous. Differential diagnoses included pituitary adenoma, pituitary carcinoma, pituicytoma, paraganglioma, spindle cell oncocytoma, and meningioma. Immunohistochemically, the tumor cells were positive for prolactin, chromogranin-A, and synaptophysin, but were negative for glial fibrillary acidic protein, S-100 protein, epithelial membrane antigen, and vimentin. No apparent cerebrospinal or systemic metastases are found. Ultimately, prolactin-producing pituitary adenoma was diagnosed. Our case highlights the difficulty in definitively diagnosing an unusual prolactin-producing adenoma based on histopathology alone and the importance of referring to clinical information and immunohistochemical findings when deriving the diagnosis.

The origin of a ring chromosome in a monophasic synovial sarcoma of the diaphragmatic pleura of an 18-year-old man was investigated using spectral karyotyping (SKY) and fluorescence in situ hybridization (FISH). Conventional cytogenetic analysis revealed the following clonal karyotypic abnormalities: 47,Y,t(X;18)(p11.2;q11.2),t(11;19)(q12;q13.4),+12,-13,+r[6]. The SYT-SSX1 fusion transcript was detected with reverse transcriptase-polymerase chain reaction analysis. SKY analysis suggested that the ring chromosome was composed of material from chromosome 8. Subsequent FISH analysis with a whole-chromosome 8 paint probe confirmed the SKY results. This study demonstrates the usefulness of SKY as an adjunct for determining the chromosomal composition of ring chromosomes.

消化管出血で発症し, 非上皮性腫瘍との鑑別が困難であった巨大皺壁を伴う限局性肥厚性胃炎の1切除例を経験したので報告する. 症例は44歳の女性で, 全身倦怠感と黒色便が出現, 近医を受診し高度の貧血を指摘された. 精査・治療目的で当院に入院し, 胃内視鏡で, 体上部後壁に出血性潰瘍を認め止血術を行った. 翌日, 同病変は腫大した粘膜皺壁を伴う半球状の粘膜下腫瘍様の形態を呈していた. 潰瘍を合併したgastrointestinal stromal tumor (以下, GIST) を疑い生検組織診断・CT・MRI・超音波内視鏡 (endoscopic ultrasonography: 以下, EUS) を行ったが, いずれもGISTを示唆する所見はなかった. 後日行った胃内視鏡再検査では, 体上部小彎を中心とする一部で立ち上がりが急峻な結節腫瘤状または耳垂様の病変に変化しており, 悪性リンパ腫との鑑別が困難であった. 胃全摘術を施行, 病理組織学的には異所性胃腺管病変を伴った限局性肥厚性胃炎であった.

症例は18歳,女子. 2カ月前からの心窩部痛を主訴に来院し,上部消化管内視鏡検査で胃体下部小彎のIIc+IIIを認めた.生検で印環細胞癌と診断された.平成10年9月18日に幽門側胃切除術, D2リンパ節郭清を施行, Billroth I法で再建した.病変は2.7×2.3cmのIIc+III,病理組織学的には印環細胞癌で0 (IIc+III)m, Infγ, lyo, vo, ow (-) aw (-)であった.全胃癌中で若年者胃癌の頻度は1～3%と稀であるが, 20歳未満の早期胃癌は極めて稀である.本例を含め30例の本邦報告例について検討した.治癒切除された若年者早期胃癌の予後は良好なので,若年者においても胃癌の存在を念頭におき,上部消化管造影検査,上部消化管内視鏡検査を積極的に行うべきと考えられた.

Background/aims: The antigen-driven clonal proliferation of B cells within target tissue has been reported in some autoimmune diseases. The purpose of this study was to examine the clonal characteristics of B cells in the liver portal area of primary biliary cirrhosis (PBC). Methods: The liver portal area was microdissected from liver biopsy sections from two PBC patients. Genomic DNA was extracted and rearranged immunoglobulin heavy chain variable region (VH) genes were amplified and sequence analyzed. Results: Sixteen VH sequences from portal area 1A of patient 1 had three different rearrangements. Nineteen VH sequences from portal area 1B of this patient had three different rearrangements. In three sequences from the portal area 1B, a stepwise accumulation of somatic mutations was observed. Between the sequences from the two portal areas, no common VH sequence was observed. In patient 2, 15 VH sequences from portal area 2A had three different rearrangements. Fourteen VH sequences from portal area 2B had two different rearrangements. One rearrangement was present both in the portal area 2A and portal area 2B. Conclusion: The oligoclonal B cell proliferation and stepwise accumulation of somatic mutations suggested that an antigen-driven B cell response had occurred in the portal area of PBC.

The gastrointestinal (GI) tract is the most common site for the development of extranodal non-Hodgkin's lymphomas, with most cases having a diffuse architectural pattern. Follicular lymphoma (FL) of the stomach is a very rare disease, and little is known about the immuno-histochemical and molecular genetic characteristics of this type of lymphoma. Despite their rarity, FLs are an important consideration in the differential diagnosis of follicular hyperplasia of mucosa-associated lymphoid tissue (MALT), a relatively frequently encountered lesion with the potential to cause significant diagnostic difficulty, especially when assessment is based on scanty biopsy material. We report a 57-year-old woman with gastric FL, and describe the results of immunohistochemical and molecular genetic studies.

Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare pediatric liver malignancy that is extremely uncommon in middle-aged individuals. We studied 2 cases of UESL in middle-aged adults (1 case in a 49-year-old woman and the other in a 62-year-old man) by histology, immunohistochemistry, and electron microscopy to clarify the cellular characteristics of this peculiar tumor. One tumor showed a mixture of spindle cells, polygonal cells, and multinucleated giant cells within a myxoid matrix and also revealed focal areas of a storiform pattern in a metastatic lesion. The other tumor was composed mainly of anaplastic large cells admixed with few fibrous or spindle-shaped components and many multinucleated giant cells. In both cases, some tumor cells contained eosinophilic hyaline globules that were diastase resistant and periodic acid-Schiff positive. Immunohistochemically, the tumor cells showed positive staining for smooth muscle markers, such as desmin, alpha-smooth muscle actin, and muscle-specific actin, and also for histiocytic markers, such as alpha-1-antitrypsin, alpha-1-antichymotrypsin, and CD68. Electron microscope examination revealed thin myofilaments with focal densities and intermediate filaments in the cytoplasm of tumor cells. Our studies suggest that UESL exhibits at least a partial smooth muscle phenotype in middle-aged adults, and this specific differentiation may be more common in this age group than in children. Tumor cells of UESL with smooth muscle differentiation in middle-aged adults show phenotypic diversity comparable to those of malignant fibrous histiocytoma with myofibroblastic differentiation.

Aims: Most primary gastrointestinal lymphomas are of B-cell origin and T-cell origin is very rare. Recent studies have suggested that human T-cell lymphotrophic virus type 1 (HTLV-1) may be involved in the development of primary gastric T-cell lymphoma. We analysed 31 patients with primary gastric T-cell lymphoma in south-west Japan, an area endemic for HTLV-1, and determined their phenotypes, genotypes, and HTLV-1 status. Methods and results: Here we present 31 cases of primary gastric T-cell lymphoma in a HTLV-1-endemic area in Japan and analyse the clinical status, histology, phenotype and virus status. The median age at onset of primary gastric T-cell lymphoma was 57 years with a gender ratio of M:F = 1.58:1. Six of the 31 primary gastric T-cell lymphoma cases had HTLV-1 proviral DNA (five males, one female), nine of the 31 cases were positive for anti-adult T cell leukaemia antibody, without examination of HTLV-1 proviral DNA (five males, four females), eight were non-HTLV-1-associated primary gastric T-cell lymphoma (four males, four females) and the other eight cases were unknown. Primary gastric T-cell lymphoma usually presented as a large ulcerated tumour at the corpus to the antrum and histologically consisted of anaplastic large cell type (n = 2), pleomorphic large cell type (n = 3), pleomorphic medium and large cell type (n = 14), pleomorphic medium cell type (n = 11), and angioimmunoblastic T-cell lymphoma type (n = 1). There were no clear macroscopic and microscopic differences between HTLV-1-associated and non-HTLV-1-associated primary gastric T-cell lymphoma. Most patients died within 2 years of diagnosis, and both types of primary gastric T-cell lymphoma (with and without HTLV-1) were associated with poor prognosis. Cytotoxic marker analysis showed that HTLV-1-associated lymphomas were negative for TIA-1, while non-HTLV-1-associated lymphomas were positive for TIA-1. Conclusions: Our results suggest that in HTLV-1-endemic areas, patients with HTLV-1-associated primary gastric T-cell lymphoma should be managed carefully and that TIA-1 seems to be useful for identifying the aetiology of this lesion.

Hodgkin disease (HD) is characterized by the presence of Hodgkin and Reed-Sternberg cells (HRS) and a prominent lymphocytic infiltration. Various CXC and CC chemokines [e.g., interferon-gamma-inducible protein-10 (IP10), monokine induced by interferon-gamma (MIG) and TARC] are expressed on HRS cells. Our study was designed to investigate the expression of MIG, IP10 and TARC on HRS cells and their relations on lymphocyte infiltration. Immunohistochemical staining was performed using antibodies for CXCR3 (a specific receptor for IP10 and MIG), which is characteristic of Th1 helper phenotype and CCR3, CCR4 and ST2, which are features of Th2 cells. We studied 15 cases of HD [lymphocyte predominance (LP) type, 2; mixed cellularity (MC) type, 6; and nodular sclerosis (NS) type, 7]. All 6 MC cases contained TARC-, IP10- and MIG-expressing HRS cells, however only 2 of 5 NS cases contained TARC-expressing HRS cells, 3 of 7 NS contained MIG-expressing HRS cells and only 1 of 7 NS contained IP10-expressing HRS cells. Neither of the 2 LP cases contained HRS cells that expressed these chemokines. The chemokines were more frequently expressed by MC than by NS and LP types. IP10-, MIG- and TARC-positive HD cases contained higher numbers of Th2 lymphocytes (ST2- CCR3- or CCR4-positive lymphocytes), compared to IP10-, MIG- and TARC-negative HD cases (p0.05 or0.5). The number of CXCR3 (MIG and IP10 receptor)-positive lymphocytes (Th1 lymphocytes) was not different between MIG- and IP10-positive and -negative HD. Lymphocytes surrounding MIG- and IP10-positive HRS cells were more frequently CXCR3-positive, however, compared to MIG- and IP10-negative cases. The CCR4 (TARC receptor)-positive lymphocytes, surrounding HRS cells, were minority and the surrounding lymphocytes were not different between TARC-positive and negative cases. Our results indicate that MIG, IP10 and TARC chemokines influenced the response of Th2 cells in HD. It is possible, however, that IP10 and MIG may locally influence Th1 cells via cell migration.

Accessory cell tumour: a clinicopathological study of 16 aggressive tumours containing EBV-positive Hodgkin and Reed–Sternberg-like giant cells Aims: Lymph nodes contain non-lymphoid accessory cells including follicular dendritic cells and interdigitating dendritic cells. Functionally, these cells belong to the category of immune accessory cells involved in antigen presentation to B or T-lymphocytes. Neoplastic proliferation of these cells is very uncommon. We present here the clinicopathological features of 16 cases of accessory cell tumour. Methods and results: We performed electron microscopic and immunohistochemical examinations, and used in-situ hybridization for EBV-encoded RNA (ISH-EBV) to detect the EBV genome in 11 cases, and Southern blot analysis to assess EBV clonality in two cases. Tumour cells were composed of oval-to-spindle cells arranged in diffuse, vague storiform, fascicular and sometimes whorled patterns in a background of small lymphocytes. In all cases, binucleated or multinucleated Hodgkin and Reed–Sternberg-like giant cells were encountered. Staining for CD68 was positive in all cases. CD21, CD35, Ki-M4p, Ki-FDC1p, and S100 exhibited variable reactivity. ISH-EBV yielded positive labelling in seven of 11 cases, of which five exhibited EBV only in Hodgkin and Reed–Sternberg-like giant cells. Southern blot analysis showed clonality of EBV terminal repeats (EBV-TR) in the two cases examined. Electron microscopic examination showed that many of the tumour cells had numerous interwoven long villous cell processes connected by occasional desmosomes. Many tumours were very refractory to chemotherapy and radiation, with a few exceptions, and half of the cases classified initially as stage IV. A short survival time, of 10 months or less, was observed in seven of 16 patients. Conclusions: Our study identified more aggressive behaviour of accessory cell tumours. Our results suggest that EBV may potentially induce activation of accessory cells to form Hodgkin and Reed–Sternberg-like giant cells, which correspond with poor prognosis.

Apoptosis of histiocytes is a characteristic feature of necrotizing lymphadenitis (HNL). Recent studies have indicated that Fas and perforin-based pathways are involved in the apoptotic process of HNL. Elevated levels of serum interferon (IFN)-gamma are reported in HNL. The CXC chemokine interferon-gamma-inducible protein-10 (IP-10) and monokine induced by interferon-gamma (MIG) cause tissue necrosis, and interleukin (IL)-18 induces the expression of IFN-gamma and Fas ligand (FasL) by T and natural killer (NK) cells. This study was designed to determine the expression of IFN-gamma, IL-18, MIG and IP-10 in HNL. Ten cases of HNL were analyzed by using immunohistochemical staining and/or reverse transcriptase-polymerase chain reaction (RT-PCR). As a control, we included four cases of non-specific lymphadenitis. MIG and IP-10 proteins, which enhance the release of granzyme, showed a similar distribution pattern in viable tissues surrounding dead tissue, mostly within histiocytes, and lymphocytes in HNL. IL-18 was located within histiocytes, especially phagocytic histiocytes, but not within lymphocytes. In addition, IFN-gamma-positive lymphocytes were frequently detected in the surrounding dead tissue, and the lymphocytes in the same area were frequently positive for CXCR3, a specific receptor of MIG and IP-10. In non-specific lymphadenitis, MIG, IP-10 and IL-18 positive cells were detected, but their numbers were relatively small compared with HNL, while IFN-gamma positive cells were rarely encountered. Our findings suggest that the cytokine and chemokine pathways of IFN-gamma, IL-18, MIG and IP-10 play an important role in the pathogenesis of apoptosis associated with HNL.

Three women older than 75 years presented with spontaneous superficial temporal artery (STA) pseudoaneurysms manifesting as a pulsatile mass in the preauricular region. None of the patients had a history of trauma. Histological examination of the surgically removed masses identified pseudoaneurysms based on the presence of connective tissue and adventitia. Spontaneous STA pseudoaneurysms are extremely rare. We suggest that all 3 aneurysms were associated with latent dissection and external force exerted by the frames of glasses.