Schwannomas of the gastrointestinal tract: clinicopathological features of 12 cases including a case of esophageal tumor compared with those of gastrointestinal stromal tumors and leiomyomas of the gastrointestinal tract

We analyzed the clinicopathological features of 12 gastrointestinal (GI) schwannomas and compared them with those of 37 GI stromal tumors (GISTs) and 15 leiomyomas. Grossly, the schwannomas showed rubbery to firm, yellow-white to tan, glistening, and often trabeculated cut surfaces, resembling soft tissue schwannomas. The GISTs were firm to soft or fish-flesh tan, gray-pink, or variegated tumors with a degenerative change, and the leimyomas resembled typical uterine leiomyomas. Histologically, GI schwannomas were moderately cellular tumors with focal significant nuclear pleomorphism and rare mitotic figures. A characteristic peripheral lymphoid cuff was observed in all cases, but was indistinct in two cases. The GISTs were highly cellular spindle cell, epithelioid or, occasionally, pleomorphic tumors with basophilic appearance. Leiomyomas were paucicellular tumors with eosinophilic appearance. Immunohistochemically, schwannomas were S-100 protein- and glial fibrillary acidic protein (GFAP)-positive, but were negative for c-kit, CD34, and smooth muscle actin (SMA). GISTs were all c-kit- and/or CD34-positive, but GFAP-negative. Leiomyomas were SMA-positive and were negative for c-kit, CD34, S-100 protein, and GFAP. The mean Ki-67 index of schwannoma was 0.7, and those of GIST and leiomyoma were 5.9 and 0.3, respectively. The patients with schwannomas and leiomyomas had a favorable outcome, whereas 12 patients with GISTs showed progression and died of disease. The separation of GISTs from schwannomas is clinically important because the former group has a high risk of malignant behavior. GI schwannomas differed from the conventional soft tissue schwannomas in that they had peripheral lymphoid cuffs, lacked fibrous capsule and vascular hyalinization, and rarely showed degenerative changes. GI schwannomas, however, resembled soft tissue schwannomas in many aspects, and the clinical, gross, histological, and immunohistochemical features were different from those of GISTs and leiomyomas.     

胃肠分化极好腺癌的概念及临床病理学特征

胃肠分化极好的腺癌病例罕见,细胞学和组织结构异型性低,早期尤其是活检标本诊断比较困难。另外这类病变诊断名词较多,部分定义模糊,临床应用也较混乱。针对上述现状,本文对这类病变的临床病理特征进行系统的总结,希望提高大家对此病的认识,达到规范化诊断的目的。     

胃肠道上皮样血管肉瘤四例临床病理学观察

目的探讨胃肠道血管肉瘤的临床病理特征、免疫表型、诊断及鉴别诊断。方法分析4例胃肠道血管肉瘤的临床病理资料,荧光原位杂交(FISH)检测C-MYC基因是否扩增。结果2例发生在胃,1例在十二指肠与空肠交汇处,1例位于回肠。4例均为中老年人,女1例,男3例。年龄51~62岁。镜下,肿瘤在黏膜层与肌层浸润性生长;见大片血湖及含铁血黄素沉积,形成假裂隙样或腺样结构;瘤细胞呈梭形或上皮样。免疫组织化学4例均弥漫强阳性表达CD31、ERG、Fli-1;3例弥漫强阳性表达CD34;3例不同程度的表达细胞角蛋白和上皮细胞膜抗原;3例FISH检测C-MYC均为阴性。结论胃肠道血管肉瘤是一种罕见的起源于血管内皮细胞的恶性肉瘤,活检标本极易被误诊为低分化腺癌。     

Adenocarcinomas of the gastrointestinal tract with prominent rhabdoid features

Rhabdoid tumor, first described in kidneys of infants and children, is an aggressive tumor that has been reported in several extrarenal locations. Gastrointestinal tumors with rhabdoid features are extremely rare. The effect of the rhabdoid phenotype on the aggressiveness of gastrointestinal tumors remains unclear. We present four cases of rhabdoid tumors of the gastrointestinal tract involving the esophagus, stomach, and small intestine and discuss the clinicopathologic, immunohistochemical, and ultrastructural features. In the four cases reported herein, the patients' ages ranged from 52 to 73 years, and tumor size ranged from 3.8 to 13 cm in greatest dimension. The noncohesive rhabdoid cells exhibited an eccentric nucleus with a paranuclear inclusion, which was shown by electron microscopic examination to be composed of intermediate filaments. On immunohistochemical staining, the tumor cells were positive for vimentin and cytokeratin. Three patients developed distant metastasis shortly after diagnosis and died of disease within 2 to 10 months after initial presentation. A retrospective review of outcomes of the current cases and previously published literature showed that 12 (75%) of the 16 patients died within 6 months of presentation. Recognition of the rhabdoid phenotype in gastrointestinal tract neoplasms is important because this feature is associated with poor prognosis and unresponsiveness to conventional therapy.     

Compliance changes of the gastrointestinal tract in streptozotocin-induced diabetic rats

In order to elucidate diabetic gastrointestinal disorders, we measured the length, diameter, volume, and intraluminal pressure of the isolated segments of the duodenum, jejunum, ileum, and proximal colon during injection of Tyrode's solution into them, as well as wet weight. Wet weight of the stomach and of each intestinal segment of 3 cm length were similar between normal and diabetic preparations, except the duodenum. Wet weight of the diabetic duodenum was significantly heavier than that of normal. However, capacity of all diabetic gastrointestinal segments was significantly larger than that of normal ones, even after corrected for wet weight (ml/g wet weight). During saline injection, normal intestinal segments were more easily distended longitudinally than circumferentially. Contrarily, diabetic ones were distended more circumferentially than normal, as well as longitudinally. Pressure-volume relationships showed that pressure inside of the diabetic gastrointestinal tract increased much more moderately than that of normal one according to volume increase during saline injection. Similarly, tension inside of diabetic intestinal segments increased much more moderately than that of normal ones. Chord and slope compliance of diabetic gastrointestinal tract was generally larger than those of normal one. Histologically, there are no remarkable differences in cross-sectional area between normal and diabetic intestinal segments after usual fixation without intraluminal fixative injection. However, diabetic segments were much more remarkably dilated than normals were, when fixed after fixative injection. Greater compliance or distensibility of the diabetic gastrointestinal tract seemed to be one basic ground for dilatation, atony, larger appearance, transit delay, and motile disorders of the diabetic gastrointestinal tract.     

Stromal tumours of the gastrointestinal tract: a clinicopathological and ploidy analysis of 33 cases

The clinicopathological and DNA flow cytometric data of 33 patients with stromal tumours of the gastrointestinal tract (STGIT) were analysed to select pathological features of prognostic value. Tumours had been previously classified as benign (21 cases) or malignant (12 cases). Data relating to poor prognosis statistically were local invasion, pathological grade, size greater than 10 cm, mitotic index (MI) and necrosis. Pathological grade was related to local invasion. Aneuploidy did not correlate with poor survival although a common trend was detected between both. DNA content may help to predict prognosis of STGIT, but its real value has not yet been clearly established. Currently, stage (invasion), size, MI and pathological grade remain the most useful prognostic factors.     

胃肠道钙化性纤维性肿瘤临床病理分析

目的探讨胃肠道钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)的临床病理学特征、免疫表型、诊断及鉴别诊断。方法回顾性分析7例胃肠道CFT的临床病理学及免疫表型特征,并复习相关文献。结果 7例患者中,男性2例,女性5例。发病部位:胃4例、小肠2例、直肠1例。临床表现:发生于胃的患者主要为上腹痛,发生于小肠的1例患者出现肠套叠,其余为体检或手术偶然发现,大体为黏膜下直径0.7~4 cm包块。镜下见肿瘤边界清晰,主要由丰富的透明变性的胶原纤维构成,呈漩涡状、束状及席纹状排列,肿瘤细胞呈梭形,无细胞异型性及核分裂象;7例均可见散在分布的砂砾体及多少不一的炎细胞浸润,2例可见营养不良性钙化。免疫表型:瘤细胞vimentin阳性,1例CD34局部阳性,1例可见CD117散在阳性细胞,desmin、DOG1、S-100、SMA、H-caldesmon和ALK均阴性,β-catenin胞核阴性,Ki-67增殖指数1%~3%。7例患者6例无复发,1例失访。结论 CFT是一种少见的间叶源性良性肿瘤,发生于胃肠道者临床罕见,临床表现及检查无特异性,特征性的组织学形态较易诊断,但需与其他间叶源性疾病鉴别。     

Plasma blood group changes in gastrointestinal tract carcinoma.

The levels of A, H, I, and i plasma antigens and of anti-B, anti-I, and anti-T antibodies were measured in 70 subjects with colonic or gastric carcinoma. These studies showed a significant increase in A plasma activity of the A subjects, and in H plasma activity of the O subjects, while 25% of the tested subjects showed increased I plasma activity. There was no difference in i plasma activity between cancer patients and healthy subjects. These results take into account the marked polymorphism acquired by neoplastic tissue, which is capable also of producing a greater quantity of antigens than that of healthy subjects. Nevertheless this heterogeneity forms a barrier to the clinical measurement of these plasma antigens for screening neoplasms. The significant fall in the amount of anti-T antibodies seemed to be secondary to the absorption of these antibodies on the surface of the tumour cells.     

Schwannoma of the gastrointestinal tract: a clinicopathological, immunohistochemical and ultrastructural study of 33 cases

AIMS: Thirty-three cases of gastrointestinal schwannomas were analysed to elucidate their peculiar clinicopathological, immunohistochemical and ultrastructural features. METHODS AND RESULTS: The patients were 16 men and 17 women, whose ages ranged from 27 to 81 years (median 52.6 years). Tumour size ranged from 10 to 120 mm in diameter. Follow-up in 23 cases from 6 months to 13 years showed no recurrences or metastases. Microscopically, all tumours were composed of spindle cells with focal epithelioid cells in four cases. In all cases except one, there were peripheral cuff-like lymphoid aggregates. Immunohistochemically, tumours were strongly positive for S100 protein and vimentin, the tumours were variably positive for nestin (78.8%, 26/33) and glial fibrillary acidic protein (63.6%, 21/33), three tumours had CD34+ cells, but all were negative for CD117, alpha-smooth muscle actin and desmin. Ultrastructurally, the tumours were composed of elongated spindle shaped cells with prominent parallel membranous structures. CONCLUSIONS: Gastrointestinal schwannomas have characteristic histological features, especially the presence of a lymphoid cuff, that are different from their soft tissue and central nervous system counterparts. Gastrointestinal tract schwannomas behave in a benign fashion.     

Granular cell tumours of the gastrointestinal tract: expression of nestin and clinicopathological evaluation of 11 patients

AIMS: Granular cell tumours (GCTs) in the gastrointestinal (GI) tract are rare, with few series reported in the literature. Nestin is a recently identified intermediate filament protein that is expressed in neuroectodermal stem cells and skeletal muscle progenitor cells and has been shown to be expressed in gastrointestinal stromal tumours (GISTs) and GI schwannomas. Herein, we describe the clinicopathological and immunohistochemical features of 11 GI GCTs, introducing nestin as an additional marker that identifies these tumours. METHODS AND RESULTS: The archives of the departments of pathology at London Health Sciences Centre (London, Ontario) and St Michael's Hospital (Toronto, Ontario) were searched for GCTs occurring in the GI tract, yielding 11 cases. Histological features were assessed and immunohistochemistry was performed with S100 protein, nestin, glial fibrillary acidic protein (GFAP), CD34, desmin, CD117, and inhibin-alpha. Charts were reviewed for clinical information. Ages at diagnosis ranged from 31 to 73 years; there were six males and four females. All GCTs were solitary, six in the oesophagus, three in the caecum, one in the rectum and one perianal. Most lesions were discovered incidentally. The size of the GCTs ranged from 4 mm to 30 mm. All were submucosal, typically firm, with a white-yellow appearance. Histologically, the GCTs showed moderate cellularity, predominantly solid growth with areas of nesting. While lesional cells were mainly plump and polygonal, areas of spindling were present in several tumours, more frequently in the colorectum. Margins were circumscribed. Nuclei were round to oval, with even chromatin and small nucleoli. Mitoses were rare to absent and necrosis was absent in all cases. Staining with periodic acid-Schiff, with diastase predigestion, showed globular and diffuse positivity within the cytoplasm. Moderate to strong expression of S100 protein and nestin was observed in 11 of 11 and seven of seven tumours, respectively. GFAP, CD34, desmin, CD117 and inhibin-alpha were negative. While patients were variably managed with resection or observation, all remain clinically well, without disease progression. CONCLUSIONS: Although rare, GI GCTs have characteristic clinicopathological features. Nestin may be a useful immunohistochemical marker for identifying these tumours; the presence of this persistent stem cell cytoskeletal filament within GI GCTs suggests that these lesions may arise from a multipotential stem cell in the GI tract.     

Microsatellite instability in adenocarcinomas of the upper gastrointestinal tract. Relation to clinicopathological data and family history.

We analyzed 66 adenocarcinomas arising in the upper gastrointestinal tract for microsatellite instability at eight microsatellite loci to investigate the role of these genetic alterations in the etiology of these tumors. We identified alterations in at least one locus in 11/46 adenocarcinomas of the stomach, in 2/15 adenocarcinomas arising in Barrett's esophagus, and in 1/5 adenocarcinomas of the duodenum and jejunum. Microsatellite instability in gastric tumors was found in 5/22 of intestinal, 1/3 of mixed, and 5/21 of diffuse type tumors. No relationship to the tumor stage (TNM), age, and survival time of the patients was observed. One patient had two synchronous gastric tumors both exhibiting microsatellite instability at multiple loci. His family history revealed four individuals in the maternal line afflicted with gastric carcinoma in three generations. Our data show that microsatellite instability is a genetic event in 11 to 24% of tumors of the upper gastrointestinal tract. The observation of microsatellite instability and a familial clustering of gastric tumors may suggest a genetic predisposition for a subset of gastric tumors, which may be identified by microsatellite analysis.     

Temperature changes in the human gastrointestinal tract and its rhythmic activity when empty

Summary The author conducted a prolonged parallel registration of the periodic motor gastric function on an empty stomach and of the temperature in the esophagus, stomach, duodenum and the initial portion of jejunum in man. Periodic changes of the temperature (especially in the body and the antral portion of the stomach and in the descending portion of duodenum) associated with periodic activity of digestive organs in fasting persons were revealed.The above temperature variations are caused by a complex of phenomena, comprising the periodic activity of digestive organs. These phenomena should be considered when studying the correlations and dynamics of temperature in the human gastrointestinal tract, on an empty stomach.Presented by Active Member of the AMN SSSR V.N. Ivanov     

Vascular malformations and hemangiolymphangiomas of the gastrointestinal tract: morphological features and clinical impact

PURPOSE: The purpose of our study was to describe the morphological features of gastrointestinal vascular malformations (VM) and of hemangiolymphangiomas (HLA) and to establish correlations with clinical characteristics. Significant findings: Fifteen VMs and 12 HLAs that were encountered over a period of 22 years, were retrospectively analyzed. The VMs often involved the colon, small intestine, but also the stomach, whereas none of the HLAs arose in the stomach. VMs were more frequently associated with gastrointestinal bleeding, ulcer and were larger than HLAs (p<0.01 for all comparisons). Intralesional hemorrhage and thrombosis were associated with VM (p=0.02 and p=0.05). Surgical resection was performed for 1 HLA and 14 VMs. Vessel abnormalities such as shunt vessels, wall tufts (excrescences) and arterialized veins were more frequent in VMs (p=0.01, p=0.04 and <0.01, respectively) whereas aneurysm-like cavities were observed in both lesion types. Mucosal abnormal vessels were observed only in VMs, whereas HLAs were associated with mucosal lymphatic clusters (p<0.01). Most HLAs contained a D2-40 hetero-geneously positive lymphatic component, were Glut-1 negative and CD31 reactive. There was no statistical difference in occurrence of associated autoimmune, tumoral and cardiovascular conditions between the two patient groups. CONCLUSIONS: The results of our study suggest that morphological features such as increased size, ulcer, thrombosis, hemorrhage and presence of aberrant mucosal vessels favor the diagnosis of VM. Co-existence of other clinical conditions such as cardiovascular disease, encountered in association with both lesion types, might exacerbate a tendency towards hemorrhage.     

女性下生殖道癌临床病理特征与人乳头状瘤病毒型别间的相关性研究

目的：探讨女性下生殖道癌的临床病理与人乳头状瘤病毒（HPV）型别之间的关系。方法：回顾性研究100例下生殖道癌（宫颈癌63例,外阴癌37例）的临床病理特征,并应用PCR技术检测每份标本的HPV状态。结果：在模板内参照阳性的87份中,宫颈癌54份,外阴癌33份,HPV阳性率在宫颈癌中为88．3％,以HPV16型（55．6％）和HPV18型（24．4％）为主,在33例外阴鳞癌中,HPV阳性仅见于基底细胞癌和混疣样癌,阳性率均为83．3％,以HPV16型为主（70．0％）;6例基底细胞样癌中有3例合并宫颈鳞状上皮肿瘤,其中2例宫颈与外阴的肿瘤均为HPV16型阳性;21例角化鳞癌则未检测出HPV－DNA,但有发病年龄高（平均63．3岁）。形态学上角化明显和预后较差等特征。结论：HPV16型和18型在宫颈癌中性阳率较高,而在外阴癌中HPV阳性的意义则因组织学类型而不相同。     

Functional and neurochemical changes of the gastrointestinal tract in a rodent model of Parkinson's disease

Patients with Parkinson's disease develop motor disturbances often accompanied by peripheral autonomic dysfunctions, including gastrointestinal disorders, such as dysphagia, gastric stasis and constipation. While the mechanisms subserving enteric autonomic dysfunctions are not clearly understood, they may involve the enteric dopaminergic and/or nitrergic systems. In the present study, we demonstrate that rats with unilateral 6-hydroxydopamine lesion of nigrostriatal dopaminergic neurons develop a marked inhibition of propulsive activity compared to sham-operated controls, as indicated by a 60% reduction of daily fecal output at the 4th week of observation. Immunohistochemical data revealed that 6-hydroxydopamine treatment did not affect the total number of HuC/D-positive myenteric neurons in both the proximal and distal segments of ileum and colon. Conversely, in the distal ileum and proximal colon the number of nitrergic neurons was significantly reduced. These results suggest that a disturbed distal gut transit, reminiscent of constipation in the clinical setting, may occur as a consequence of a reduced propulsive motility, likely due to an impairment of a nitric oxide-mediated descending inhibition during peristalsis.