CD34, CD117, and actin expression in phyllodes tumor of the breast

BACKGROUND: This study investigated the immunophenotypic patterns of CD34, CD117 (a product of the c-kit proto-oncogene), and actin (HHF35) in benign and malignant phyllodes tumors (PTs). We correlated the expression of CD34, CD117, and actin with histopathological grade. MATERIALS AND RESULTS: We analyzed 19 cases (7 benign and 12 malignant cases) of PTs using immunohistochemical analysis. Six of 7 benign PT stromal lesions stained positively for CD34, while only 3 of 12 cases of malignant PT were focally CD34 positive (P = 0.0106). Only 1 of the 7 benign PTs stromal lesions expressed CD117. Nine of the malignant PTs were composed CD117-positive fibroblasts. This result demonstrated that CD117 expression is associated with the malignant potential of PTs (P = 0. 0106). Actin (HHF-35) expression was found in 8 of 12 cases of malignant PTs (P = 0.027), but in only 1 of 7 cases of benign PTs. Actin expression was significantly (P = 0.04) correlated to frequent mitotic activity (>5 mitoses per 10 high-power fields). The immunophenotypic markers were not related to tumor size. Additionally, we sequenced part of the juxtamembrane region of the c-kit proto-oncogene and found point mutations in two malignant PTs. CONCLUSION: Our results demonstrated that expression of CD34 was associated with benign PTs, while CD117 and actin were preferentially expressed in malignant PTs. Our results implied that these immunohistological markers might be used for the histopathological grading of PTs.     

Does a water soluble contrast enema assist in the management of acute large bowel obstruction: a prospective study of 117 cases

We have performed a prospective study on 117 patients in order to assess the value of a single contrast water soluble enema in acute large bowel obstruction. In 99 cases where the plain films suggested a diagnosis of mechanical obstruction, the Diodone enema confirmed its presence in 52, and relocated its site in 11. There was free flow of contrast to the caecum in the remaining 35, 11 of whom were shown to have idiopathic pseudo-obstruction. The plain films suggested a diagnosis of pseudo-obstruction in 18 patients. The Diodone enema confirmed this diagnosis in 15, but revealed an unsuspected mechanical obstruction in two. The examination failed in two cases. We conclude that the water soluble single contrast enema is of value in the management of large bowel obstruction. It will confirm the diagnosis of mechanical obstruction and prevent unnecessary surgery in patients with pseudo-obstruction.     

Pseudo-pseudo-obstruction. A clinically relevant concept

Four patients with small bowel obstruction were treated nonoperatively after being misdiagnosed as chronic idiopathic intestinal pseudo-obstruction. Three patients with pseudo-pseudo-obstruction developed significant morbidity, and one patient died while being treated medically. Differentiation between pseudo-obstruction and mechanical obstruction can be difficult and may require laparotomy to establish the diagnosis. Intestinal manometry suggested a mechanical cause for obstruction in two patients and should be done in all patients that carry the diagnosis of intestinal pseudo-obstruction. Other clues to the diagnosis of intestinal pseudo-obstruction are discussed. Intestinal pseudo-obstruction is an uncommon disease that should not be a diagnosis of exclusion but should be diagnosed using historical, radiographic, manometric, pathologic, and operative data.     

Stromal tumor of the gallbladder with phenotype of interstitial cells of Cajal: a previously unrecognized neoplasm

We report a small, well-demarcated stromal tumor of the gallbladder in a 69-year-old woman. The tumor and associated cholelithiasis led to chronic cholecystitis symptoms. The wall of the gallbladder contained a 2.4-cm hypocellular nodule composed of bland spindle-shaped cells that were immunoreactive for vimentin, CD34, and CD117. With the latter antibody, which stains interstitial cells of Cajal (ICC), the neoplastic cells appear fusiform with elongated bipolar projections or dendritic-like cytoplasmic projections. The gallbladder wall adjacent to the tumor contained numerous CD117-positive cells in close contact with the normal smooth muscle cells, whereas two of 10 gallbladders with minimal chronic cholecystitis showed only a few CD117-positive cells. These findings provide evidence that this stromal tumor of the gallbladder shows ICC differentiation similar to some stromal tumors of the gut. The presence of numerous ICC in the uninvolved gallbladder wall suggests that this tumor might have evolved through hyperplasia of ICC.     

胃肠道间质瘤31例临床诊治分析

目的：探讨胃肠道间质瘤（GIST）的临床特点、治疗方法,以提高诊断和治疗效果。方法：回顾分析2001年12月—2009年12月31例胃肠道间质瘤患者的临床诊断、治疗、病理资料及随访。结果：GIST好发于胃和小肠,临床表现缺少特异性,16例（51.6%）GIST发生于胃,8例（25.8%）发生于小肠,3例（9.7%）发生于结直肠（其中2例伴有肝转移）,发生于肠系膜、网膜及腹膜后4例（12.9%）。CT检查呈现不规则软组织肿块,大部分边界完整。GIST表达CD117阳性者28例（90.3%）,CT34阳性者22例（71.0%）。18例恶性GIST中9例接受甲磺酸伊马替尼治疗,术后3年存活5例,9例未接受甲磺酸伊马替尼治疗者1例失访,术后3年存活0例。结论：在区别GIST与平滑肌瘤或神经源性肿瘤时,CD117、CD34具有重要价值。CT、内镜超声检查是目前诊断GIST常用和重要的手段,手术切除是首选的治疗方法,甲磺酸伊马替尼辅助治疗能够提高患者的生存期。     

Laparoscopic management of superior mesenteric artery syndrome.

OBJECTIVES: The differential diagnosis of intestinal obstruction includes mechanical obstruction, obstruction secondary to systemic disease, and idiopathic intestinal pseudo-obstruction. The causes of these are extensive; however, the majority of cases involve a mechanical cause. Superior mesenteric artery syndrome (SMAS) is a rare and controversial form of mechanical obstruction with just over 300 well-defined cases described in the literature. The diagnosis is often difficult to establish, even after surgery. In addition, this syndrome sometimes may be managed conservatively, leaving a definitive diagnosis unproven. We describe herein 2 patients with SMAS successfully treated with laparoscopic duodenojejunostomy. METHODS: Two cases of SMAS occurred in young men ages 23 and 34. The workup included a consultation with a gastroenterologist, an upper gastrointestinal (GI) endoscopy, upper GI series with small bowel follow-through, computed tomography scan, ultrasound of the abdomen, and abdominal aortogram. This diagnosis was established after consultation with the surgeon and the gastroenterologist in each case. RESULTS: Laparoscopic duodenojejunostomy was performed in each case, and both patients have had complete resolution of their preoperative symptoms. CONCLUSIONS: A laparoscopic approach to the management of superior mesenteric artery syndrome is a reasonable and successful way of treating these patients.     

Segmental ileal obstruction in neonates—a rare entity

Congenital segmental dilatation of a portion of the small intestine in neonates causing intestinal obstruction is quite uncommon. We hereby report 2 such cases. In both infants, there was localized dilatation of the ileum with collapse of the distal bowel and functional intestinal obstruction without loss of continuity of the bowel wall or lack of ganglion cells.     

Acute Colonic Pseudo-Obstruction in Vascular Patients (Ogilvie syndrome)

Two patients with acute colonic pseudo-obstruction are presented. Acute colonic pseudo-obstruction, first described by Ogilvie in 1948, is characterised by signs of colonic obstruction, but without mechanical obstruction to the intestinal flow. The current hypothesis states that it is caused by an imbalance between sacral parasympathetic excitatory nerves and sympathetic inhibitory nerves, thus inducing functional obstruction. It mostly develops in hospitalised patients with a variety of medical and surgical conditions.

Two such patients with extensive co-morbidities, developed progressive colon dilatation with signs of acute peritonitis, and were operated on urgently. In both patients, right hemicolectomy and postoperative medical treatment resulted in complete recovery.     

胃肠道间质瘤28例临床和病理分析

目的探讨胃肠道间质瘤(GISTs)的临床表现、组织学和免疫组化特点及治疗方法。方法对1998~2003年收治的28例GISTs病人的临床和组织学资料进行回顾性分析,应用免疫组织组化染色检测CD117、CD34、SMA、S100的表达。结果GIST主要发生在胃(60.7%)和小肠(25.0%),结直肠(10.7%)和肠系膜(1.25%)较少;本组中良性9例,交界性5例,恶性14例;免疫组织化学染色显示CD117、CD34、SMA、S100表达的阳性率分别是100%、71.4%、28.6%和10.7%。结论GISTs是胃肠道常见的间质性肿瘤,手术前诊断困难,CD117和CD34标记阳性是确诊GISTs最有价值的诊断依据,手术仍是主要治疗手段。     

CD30-positive atypical lymphoid cells in common non-neoplastic cutaneous infiltrates rich in neutrophils and eosinophils

CD30-positive cells characterize lymphomatoid papulosis and anaplastic large cell lymphoma but can also be found in nonneoplastic skin disorders. Purportedly, CD30 is useful in the differential diagnosis between insect bites and lymphomatoid papulosis. Recently, a subtype of neutrophil-rich CD30-positive anaplastic large cell lymphoma has been described, which may enter the differential diagnosis of cutaneous neutrophil-rich inflammatory infiltrates. We studied atypical CD30-positive lymphoid cells in five eosinophil-rich and 23 neutrophil-rich common nonneoplastic skin infiltrates. The eosinophil-rich cases included five insect bites. The neutrophil-rich cases included 9 inflammatory (hidradenitis suppurativa [n = 4], stasis ulcer [n = 2], ruptured cyst, rhynophyma, and Sweet syndrome); 12 infectious (bacterial [n = 8], viral [n = 2] and fungal [n = 2] etiologies); and 2 environmental (spider bites) cases. Atypical CD30-positive cells were found in 4 of 5 eosinophil-rich, 8 of 9 neutrophil-rich inflammatory, 6 of 12 neutrophil-rich infectious, and 2 of 2 neutrophil-rich environmental cases. Polymerase chain reaction analysis for B- and T-cell clonality and cell counts of neutrophils, eosinophils, plasma cells, B cells (using CD20), and T cells (using CD3) were performed in the cases that contained atypical CD30-positive lymphoid cells. CD30-positive cells averaged 4.8% of the cells counted in the areas where they were most concentrated. Of the 18 cases that amplified with polymerase chain reaction, all were polyclonal for T-cell receptor rearrangements; 10 were polyclonal and 8 oligoclonal for B-cell immunoglobulin rearrangements. There was no correlation between B-cell oligoclonality with CD30-positive cell counts, a particular disease, or a disease category. In conclusion, the presence of CD30-positive atypical lymphoid cells in 71.4% of the common nonneoplastic cases studied, even in the presence of clonal B-cell populations, warrants caution in the interpretation of these cells as malignant, particularly when dealing with the differential diagnosis of lymphomatoid papulosis or neutrophil-rich anaplastic large cell lymphoma.     

小肠折叠排列术治疗广泛粘连性肠梗阻

目的　总结小肠折叠排列术治疗广泛粘连性肠梗阻的技术经验。方法　回顾 1985年10月～ 2 0 0 1年 9月收治的 6 86例肠梗阻病例中 ,就手术中诊断为广泛粘连性肠梗阻并行小肠折叠排列术的 76例临床资料进行总结分析。结果　本组 76例中 ,74例痊愈出院 ,获随访 4 5例 ,随访时间 1个月～ 16年 ,平均 9年 ,无因粘连性肠梗阻而再次入院手术者 ,无肠瘘等并发症 ,死亡 2例分别于术后第 2、3d因感染中毒性休克死亡。结论　小肠折叠排列术是治疗广泛粘连性肠梗阻的有效术式 ,适用于反复发作、曾多次梗阻松解等病例 ,强调规范手术操作 ,注重整体治疗。     

Obstruction of the large and small intestine

This article provides an overview of mechanical small and large bowel obstruction with emphasis on newer diagnostic and therapeutic surgical techniques. Small and large intestinal pseudo-obstruction is discussed with reference to its diagnosis and appropriate treatment.     

Alterations of Cajal cells in patients with small bowel atresia

Purpose

Interstitial cells of Cajal (ICC) are regarded as the pacemaker cells of the gastrointestinal tract. There are some well-designed studies investigating the structure and function of ICC subsequent to experimentally induced intestinal obstructions. However, it remains unclear whether reduction of number of ICC primarily leads to mechanical obstruction of the bowel such as seen in intestinal atresia. We aimed to investigate the number of ICC in proximal and distal parts of the atresias of patients with small bowel atresia.

Patients and Methods

Twenty-one patients (13 male and 8 female; median age, 3 days; median gestation age, 38 weeks) with jejunal or ileal atresia underwent primary repair between 2001 and 2009. The demographic data were reviewed. The specimen of the distal and proximal parts of the atretic segments was investigated according to presence and number of ICC in the myenteric plexus using immunohistochemical methods. The jejunum segments of 14 newborns who died from causes other than bowel disease were examined as a control. Scoring and count systems were developed for the evaluation of ICC. A continuous layer of CD-117 immunoreactive Cajal cells around the myenteric plexus was scored as 3, whereas discontinuous and diminished Cajal cells were scored as 2. Few and sparse Cajal cells around the myenteric ganglia and in the muscle layer were scored as 1. If there was no Cajal cell at all, it was scored as zero. In addition, the number of ICC per field was counted. The scores and the numbers of ICC per field were compared in patients with small bowel atresia and control group.

Results

All patients but one survived. One patient was lost because of congenital cardiac anomalies. The median score of control subjects was 3 (range, 1-3). Both the proximal and distal segments of the atretic bowel had a median score of 1 in patients with atresia. Twenty patients' score of proximal (95%) and 19 patients' score of distal bowel segment (90%) had an ICC score of 2 or less. Only 1 control subject (7%) had an ICC score of less than 2. Results were statistically significant in controls and patients. The mean number of ICC in the control group was 5.36 ± 2.36; in distal segments of patients with atresia, it was 1.03 ± 1.4; and in proximal s\egments, it was 0.82 ± 1.56. The difference between the control group and the patients was statistically significant (P < .05).

Conclusion

We demonstrated a remarkable decrease of ICC in small bowel wall of patients with intestinal atresia; but we could not show whether the reduction of ICC is a primary event, which also participates in the pathogenesis of intestinal atresia, or whether the mechanical obstruction caused by any unknown etiology (eg, ischemia) leads to decrease in number of ICC.     

Coexistence of abdominal cocoon,intestinal perforation andincarcerated Meckel’s diverticulum in an inguinal hernia: Atroublesome condition

Sclerosing encapsulating peritonitis(SEP) is a rare dis-ease entity, in which the small intestine becomes en-cased and mechanically obstructed by a dense, fibrotic membrane. The disorder is characterized as either primary(idiopathic) or secondary to other causes. The idiopathic cases of SEP, which lack any identifiable eti-ology according to clinical, radiological and histopatho-logical findings, are also reported under the designation of abdominal cocoon syndrome. The most frequent presenting symptoms of all SEP cases are nausea, vom-iting, abdominal distention and inability to defecate, all of which are associated with the underlying intestinal obstruction. Persistent untreated SEP may advance to intestinal perforation, representing a life-threatening condition. However, preoperative diagnosis remains a particular clinical challenge, and most diagnoses are confirmed only when the typical fibrous membrane en-casing the small intestine is discovered by laparotomy. Here, we report the clinical presentation of an 87-year-old male with signs of intestinal obstruction and the ul-timate diagnosis of concurrent abdominal cocoon, rightincarcerated Meckel's diverticulum, and gastrointestinal perforation in laparotomy.     

Interstitial cells of Cajal as precursors of gastrointestinal stromal tumors

Interstitial cells of Cajal (ICC) are implicated in the regulation of gut peristalsis and are immunostained by antibodies against Kit (CD117), a tyrosine kinase receptor. Most gastrointestinal mesenchymal tumors (GIMTs) are of uncertain histogenesis, although many are CD34-positive. CD34 was found to colocalize with vimentin (Vim) and the Kit-positive networks of cells within and around neural plexi, indicating that ICC can be Vim- and CD34-positive. ICCs appear to be the only Kit+CD34+Vim+ cell in the gut. Formalin-fixed, paraffin-embedded tissues from 43 GIMTs were immunostained for Kit, CD34, Vim, PGP 9.5 (PGP, a neural marker), muscle-specific actin (MSA), and other markers including desmin (Des). Eight tumors were myoid (MSA+Des+Vim-Kit-CD34-), and one was a schwannoma (PGP+S100+Vim+Kit-CD34-), but 34 tumors were of uncertain histogenesis (gastrointestinal stromal tumors, GIST), exhibiting neither a complete myoid nor a schwannian immunophenotype. All 34 were Vim+, and 33/34 were either Kit (n = 30) or CD34 (n = 23) immunoreactive. Of these 34 GIST, 24 were negative for all myoid and neural markers, 6 were PGP+S100-, and 4 were MSA+Des-. The Kit+CD34+Vim+ immunophenotype of GIST suggests that they originate from, or have differentiated into, ICC-like cells; the term ICC tumor (ICCT) is suggested. Kit is a more sensitive marker than CD34 for ICCT, but both are required in tumor identification. All clinically malignant GISTs were pathologically malignant (size, mitoses) but also showed loss of either CD34 or Kit. "Blind" examination of electron micrographs in 10 tumors showed them to be heterogeneous. Some had features seen in normal ICC, but cells could not be positively identified as being adult ICC. GIMT may therefore be classifiable into those with pure myoid, schwannian (or neural) differentiation, but the majority are of ICC origin or show ICC differentiation immunophenotypically (ICCT).     

Chronic Intestinal Pseudo-Obstruction Related to Viral Infections

Chronic intestinal pseudo-obstruction (CIPO), one of the most severe gastrointestinal motility disorders, is a condition characterized by a clinical picture mimicking small bowel occlusion with related symptoms and signs in the absence of demonstrable mechanical obstruction. Analysis of full-thickness biopsy samples may unravel structural changes of the neuromuscular layer involving the whole gut, although the midgut is usually worst affected. Intestinal pseudo-obstruction can occur in association with systemic neurological, endocrine, and connective tissue diseases or malignancy but, when no recognizable etiology is found, CIPO is referred to as idiopathic (CIIPO). The latter form can be diagnosed early in life due to a genetic etiology or in adulthood when a viral origin may be considered. This review addresses the hypothesis that some systemic neurotrophic viral infections can affect the enteric nervous system thereby altering normal peristaltic activity. Available data are reviewed, focusing specifically on herpesviruses or polyomaviruses (JC virus). These suggest that in comparison to a proportion of CIIPO patients, healthy controls rarely harbor viral DNA in the myenteric plexus, leaving open the possibility that a viral infection might have an etiologic role in the development of CIIPO. The review thus provides some new perspectives in the pathophysiology and perhaps targeted treatment of CIIPO.     

Distinction of hepatocellular carcinoma from benign hepatic mimickers using Glypican-3 and CD34 immunohistochemistry.

Distinguishing a well-differentiated hepatocellular carcinoma (HCC) from normal and cirrhotic liver tissue or benign liver nodules, such as hepatic adenoma (HA) and focal nodular hyperplasia (FNH), may be very difficult in some cases, particularly in small needle core biopsies. We studied the expression of Glypican-3 (GPC3) and CD34 in 107 cases of HCC, 19 cases of HA, and 16 cases of focal nodular hyperplasia (FNH). In addition, we studied GPC3 expression in 225 cases of nonhepatic human tumors with epithelial differentiation. Ninety-four of 107 cases (88%) of HCC showed focal or diffuse cytoplasmic GPC3 staining, whereas all HA and FNH cases were GPC3-negative, and only 7 of 225 cases (3%) of nonhepatic tumors with epithelial differentiation expressed GPC3. The sensitivity and specificity of GPC3 for HCC was 88% and 97%, respectively. There were three CD34 staining patterns observed in hepatic tissue: negative, incomplete positive, and complete positive. In negative staining pattern, only blood vessels in portal triads or rare sinusoidal spaces immediately adjacent to portal tracts were positive. The negative staining pattern was seen in normal or cirrhotic liver tissue only. The complete CD34 staining pattern showed virtually all sinusoidal spaces with CD34-positive staining throughout the lesion. The complete CD34 staining pattern was seen in virtually all cases of HCC and in only some cases of HA and FNH. The incomplete CD34 staining pattern was characterized by either CD34 positivity in virtually all sinusoidal spaces in some but not all nodules or CD34 positivity in the peripheral sinusoidal spaces adjacent to portal triads. The incomplete CD34 staining pattern was seen in rare cases of HCC and in most cases of HA and FNH. We conclude that GPC3 is a very specific marker not only for differentiating HCC from nonhepatic tumors with epithelial differentiation, but also for differentiating HCC from HA and FNH. GPC3 immunoreactivity, in combination with a complete CD34 immunostaining pattern, greatly facilitates the accuracy of distinguishing between malignant hepatic lesions and benign mimickers.     

Laparoscopic resection of sporadic synchronous gastric and jejunal gastrointestinal stromal tumors: Report of a case

Multicentricity of gastrointestinal stromal tumors (GISTs) has been described only in patients with neurofibromatosis type 1 (NF1) or within the small intestine, and different pathogenetic mechanisms are involved. We report a case of synchronous sporadic gastric and jejunal GISTs, which were resected laparoscopically in a 67-year-old man. Immunohistochemical analysis revealed that both lesions were KIT (CD117)-positive, but that the gastric lesion was CD34-positive, whereas the jejunal one was Vimentin-, S-100-, and SMA-positive. Molecular analysis of mutations in KIT exons 9, 11, 13, and 17, and in PDGFRA exons 12 and 18 revealed the presence of a gastric sporadic GIST with a KIT mutation of the exon 11 and a jejunal sporadic GIST without KIT or PDGFRA mutations. To our knowledge, this is the first report of laparoscopically resected synchronous sporadic gastric and jejunal GISTs.     

The usefulness of small-bowel manometry in the diagnosis of gastrointestinal motility disorders

Motility disorders of the gastrointestinal (GI) tract have traditionally been diagnosed by excluding mechanical small-bowel obstruction. In order to diagnose GI motility disorders in a positive fashion, small-bowel manometry was performed on 15 patients who were referred to the authors with intestinal motility disorders. Intestinal manometry was performed after first positioning a 200-cm multilumen tube into the small intestine. Ports located at 10-cm intervals were perfused with sterile water and connected to pressure transducers to record intraluminal pressures with a multichannel chart recorder. This low compliance water perfusion manometry system allowed examination of both fasting and postprandial motility. Intestinal manometry was able to assist in the diagnosis of two patients that had true mechanical small-bowel obstruction. One patient had a stenosis of the gastrojejunostomy and three patients had a functional gastric outlet obstruction secondary to a motility disorder in the Roux limb. One patient had a functional obstruction from a reversed jejunal loop and eight patients were identified as having intestinal pseudo-obstruction. We found intestinal manometry was a helpful adjunct in the diagnosis of GI motility disorders.     

胃肠道间质瘤的诊治分析

目的 探讨胃肠道间质瘤的临床特征及诊治经验.方法 对2003年1月至2013年6月收治的共72例胃肠道间质瘤的临床病例进行回顾性分析.结果 72例胃肠道间质瘤患者中男性37例、女性35例,中位年龄61岁.发病部位：胃41例（56.94％）,小肠24例（33.33％）,结直肠6例（8.33％）,食管1例（1.39）.主要临床表现依次有腹痛、消化道出血、腹部肿块、肠梗阻、贫血.免疫组织化学检测CD117、CD34、SMA、DOC-1、S-100阳性率分别为98.61％（71/72）、70.83％（51/72）、34.72％（25/72）、100.00％（18/18）、5.56％（4/72）.所有患者均行手术治疗,其中2例行姑息性手术,其余70例均完整切除.21例行伊马替尼治疗,完全缓解（CR）3例,部分缓解（PR）16例,疾病稳定（SD）2例.1、3、5年生存率分别为93.79％、79.54％、53.69％.结论 胃肠道间质瘤临床表现不典型,确诊依靠组织学检查及免疫组织化学检查.手术完整切除联合靶向药物治疗可使胃肠道间质瘤患者获得满意疗效.