Small-cell carcinoma of the gastrointestinal tract: a retrospective study of 64 cases

Small-cell carcinoma (SmCC) of the gastrointestinal tract is a very rare and aggressive malignancy. To better define its clinicopathological features, the records of all patients with this disease seen at Memorial Sloan Kettering Cancer Center between 1980 and 2002 (n=64) were reviewed. The most common primary tumour locations were in the large bowel and oesophagus. Predisposing medical conditions for non-small-cell cancers, positive family cancer history, and metachronous tumours were common. In all, 37% had mixed tumour histology and 48% presented with extensive disease, according to the Veterans' Administration Lung Study group (VALSG) staging system used for small-cell lung cancer. Treatment outcome in limited disease (LD) suggested a role for surgery and chemotherapy. Platinum-based regimens resulted in a 50% response rate. The 2-year survival was 23% and two prognostic factors were identified, the extent of disease according to the VALSG system (P<0.01) and TNM stage (P=0.03). Anatomic location had no clinical impact. In conclusion, SmCC from various gastrointestinal sites can be viewed as one clinical entity. Mixed tumour histology is common and may affect therapy. Surgery, combined with chemotherapy, should be considered for LD. The value of the VALSG system was implied and possible differences from small-cell lung cancer were noted.     

Primary follicular lymphoma of the gastrointestinal tract: a study of 25 cases and a literature review.

BACKGROUND: To describe better the clinical, biological, endoscopic and pathological presentations, as well as the outcome, of primary follicular lymphoma (FL) of the gastrointestinal (GI) tract. PATIENTS AND METHODS: From November 1983 to February 2001, 25 eligible patients with primary FL of the GI tract were retrieved from several French Departments of Pathology departments based on histological diagnosis and immunophenotype. Median age was 56 years (range 44-71) with a sex ratio female/male of 2 (17/8). RESULTS: Abdominal pain was the main presenting symptom followed by intestinal obstruction. The small intestine was the most common site of involvement. Lesions were unifocal in the majority of patients (15/25). A pattern similar to lymphomatous polyposis was observed in 50% (7/14) of patients. Twelve patients had stage I, 10 patients stage II and three patients stage IV disease, and there was minimal extra intestinal involvement. Lymphoma tissues were composed of neoplastic follicles, most of which were grade 1 according to the World Health Organization (WHO) classification. The immunophenotype of the lymphoma cells was CD20+, CD10+, bcl2+ and CD5-. In tissue samples, IgH/bcl2 rearrangement at the MBR locus was present in 11 of 14 patients tested. Seven patients did not receive any treatment; four of them progressed after a median follow-up of 37.5 months. Treatment was otherwise heterogeneous, and complete remission was obtained in 15 patients which lasted for a median of 31 months. Relapses were either in the GI tract (n = 3) or outside the GI tract (n = 3). After a median follow-up of 34 months (range 5-203), 22 patients were still alive (complete remission, 11; partial remission, three; stable disease, six; progressive disease, two). CONCLUSIONS: Primary FL of the GI tract is a predominantly female lymphoma that most frequently involves the small intestine. Since the endoscopic and clinical presentation may not be different from lymphomatous polyposis, which is often associated with mantle cell origin of tumor cells, it is mandatory to perform an immunohistological and, if possible, a molecular analysis of GI lymphoma. The course of the disease is indolent and does not differ from nodal FL. Thus, therapy may not be required unless significant clinical symptoms are present or until disease progression.     

CD117弱表达及阴性表达的胃肠道间质瘤13例分析

  目的 分析 CD117弱表达及阴性表达的胃肠道间质瘤的特点。方法 回顾性分析 13 例经手术病理证实的 CD117弱表达及阴性表达胃肠道间质瘤的特点。结果　肿瘤位于胃部 6 例，小肠 4 例，结直肠 2 例，大网膜 1 例。腹部疼痛不适 6 例，呕血或黑便 3 例。病理学检查肿瘤为良性者 5 例，恶性者8 例。CT 检查表现为软组织密度，5例病变含有囊变及出血，所有病变均未见钙化。良性肿瘤大小（最长径×最短径）平均为3.4 cm×2.8 cm；恶性肿瘤大小平均为7.5 cm×6.2 cm。13 例患者均经手术治疗，手术切除率100 ％。10 例术后获随访6个月~ 4 年，有1 例死于肿瘤复发转移，其余至今仍生存。结论　CD117弱表达及阴性表达的胃肠道间质瘤的好发部位为胃体。CT 扫描有助于 CD117弱表达及阴性表达胃肠道间质瘤的定位和良恶性的判断。手术是其治疗的主要方法。     

Revision of gastrointestinal mesenchymal tumours with CD117.

BACKGROUND: Tyrosinekinase inhibitors improve the treatment of gastrointestinal stromal tumours (GISTs) and their diagnosis has been facilitated by recently developed immunohistochemical markers. It is hypothesised that in the past, the true incidence of GISTs has been underestimated. AIMS: To study the clinicopathological features of previously resected mesenchymal tumours of the gastrointestinal tract and determine the accuracy of previous diagnostic results. PATIENTS AND METHODS: Patients with mesenchymal tumours of the gastrointestinal tract operated on between 1987 and 2002 were identified using medical and pathologic files. Immunohistochemical staining for CD117, CD34, desmin and S100 was performed, and diagnosis reviewed. RESULTS: Thirty-six mesenchymal tumours were reanalysed. Before revision, diagnosis of GIST was correctly made in only six cases. Supportive use of immunohistochemical markers for accurate diagnosis of the remaining 30 previously undefined mesenchymal tumours yielded 17 additional GISTs. Therefore, 23 of 36 (63%) gastrointestinal mesenchymal tumours were shown to be GISTs. CONCLUSIONS: The true incidence of GISTs has been underestimated. There is merit in reviewing the clinical diagnoses of all mesenchymal tumours of the gastrointestinal tract with modern immunohistochemical markers. This may enhance clinical decision making.     

Angiosarcoma of the gastrointestinal tract. A report of three cases

Three cases of angiosarcoma in the stomach, small bowel, and colon, respectively, are reported. The gastric lesion occurred many years after historic evidence of cutaneous Kaposi's sarcoma. Two of the three lesions exhibited multifocality, and all demonstrated aggressive biologic courses. The varied histologic features of angiosarcoma make this diagnosis difficult to establish in an unusual location such as the gastrointestinal tract. The use of immunohistochemistry for a panel of antibodies, particularly the successful reaction with Factor VIII-related antigen, is important in evaluating light microscopically unusual tumors. A delicate framework of collagen type IV was elicited in each case, but ultrastructurally correlated with short segments of basal lamina in one case. This suggests that the absence of the traditional morphologic expression of basal lamina does not preclude its presence and emphasizes the sensitivity of immunohistochemistry. The lack of previously reported cases of gastrointestinal angiosarcoma may be because the tumor is underrecognized or because it is truly a rare occurrence.     

Small-cell carcinomas of the gastrointestinal tract: a review.

PURPOSE: To improve our understanding of the entity of small-cell carcinoma (SmCC) of the gastrointestinal (GI) tract. METHODS: A MEDLINE search was done, using the terms "small cell carcinoma" or "oat cell carcinoma" combined with "gastrointestinal" or with any of the GI sites, for the period 1970 to 2003. The 138 eligible reports identified in this way were reviewed for clinical data. RESULTS: To date, approximately 544 cases of GI SmCC have been reported. The disease represents 0.1% to 1% of all GI malignancies, with the esophagus being the most common primary site. A majority of patients present with overt distant metastases. Systemic symptoms are common; ectopic hormonal secretion may occur. By light microscopy, GI SmCCs are essentially indistinguishable from primary pulmonary SmCC. The presence of non-SmCC components is common. Data from molecular analysis of the disease has identified some similarities to pulmonary SmCC. Chemotherapy represents the main treatment option, with modest impact on survival. In locoregional disease, the literature suggests that treatment be initiated using chemoradiotherapy and then, if metastatic disease is still excluded, surgical resection be considered. The disease is highly aggressive, and survival is in the range of several weeks for untreated patients and of 6 to 12 months for those receiving therapy. CONCLUSION: SmCC of the GI tract is a rare and lethal disease. Although there are many similarities to pulmonary SmCC, some differences between the two entities are suggested. While chemotherapy can achieve significant palliation, surgery may have a potential impact on long-term survival of patients with locoregional disease.     

Carcinoid tumour of the gastrointestinal tract: prognostic factors and disease outcome.

This study represents retrospective analysis of 87 patients with a carcinoid tumour of the gastrointestinal tract seen and followed in the British Columbia Cancer Agency (BCCA) from 1960 to 1986. In 49 cases, the primary site was the small bowel. The rest of the cases were distributed as follows: 11 appendix, 10 rectum, 5 stomach, and 7 undetermined. We extrapolated the Dukes' and modified Astler-Coller surgicopathological classifications used for colorectal cancer for use in our cases of carcinoid tumour of the gastrointestinal tract. A strong correlation was found, using this staging, with disease-specific survival. Other prognostic factors included histologic differentiation, the presence of macroscopic residual disease after initial surgery, and level of 5-hydroxyindoleacetic acid (5-HIAA) in urine. Among 51 patients with surgically grossly removed disease, there was a tendency for the development of distant and distant/locoregional recurrence more often than locoregional recurrence alone. The liver was the commonest site of distant recurrence. Analysis of the effect of radiotherapy or chemotherapy on carcinoid tumour of the gastrointestinal tract proved unsuccessful because only a small portion of the patients had this treatment, and it was used mainly for palliation.     

Smooth muscle tumors of the gastrointestinal tract. A study of 56 cases followed for a minimum of 10 years

Cases of gastrointestinal smooth muscle tumor seen at M. D. Anderson Hospital and followed for a minimum of 10 years are presented. The tumors were classified as high-grade leiomyosarcoma (41 cases), low-grade leiomyosarcoma (13 cases), and leiomyoma (2 cases). All of the leiomyosarcomas originated in the stomach (21 cases), small intestine (29 cases), or rectum (4 cases) and appeared to have arisen from the muscularis propria. Leiomyosarcomas were considered high-grade when the maximal mitotic rate in ten consecutive high-power fields was ten or more and low-grade when this rate was lower (actual maximal rates in the low-grade group varied from 1-5/10 high-power fields). All patients with high-grade leiomyosarcoma died of tumor after intervals ranging from 5 to 90 months (median, 25 months). All but two with low-grade leiomyosarcoma also died of tumor, but frequently after much longer intervals (range, 42-221 months; median, 98 months; survival difference P = 0.002). Intervals to recurrence and metastasis were correspondingly longer in the low-grade group (up to 188 months). The two leiomyomas were small (less than 2 cm), had no mitotic figures, and were less cellular than any of the leiomyosarcomas. However, they both occurred in locations in which no leiomyosarcomas were seen (muscularis propria of the esophagus and muscularis mucosae of the rectum); therefore, the problem of distinguishing leiomyomas from leiomyosarcomas in sites where the latter did arise could not be resolved, particularly in view of the fact that fatal low-grade leiomyosarcomas had diameters as small as 1 cm and maximal mitotic rates as low as one per ten high-power fields. "Leiomyoblastoma" was not found to be an entity; it is recommended that this term be dropped.     

Cancer of the alimentary tract in the Sudan: a study of 546 cases.

Five hundred and forty-six primary malignant alimentary tract tumors in Sudanese patients are analyzed for frequency, age, sex, site, geographic distribution, and clinicopathologic presentations and are compared with other series. The disease is much less frequent in Sudan than in Western countries; within the Sudan itself it is much less prevalent in the South than the North. Generally, it tends to occur in younger age groups than in Western countries. Esophageal cancer is relatively common, especially in the Northern Province and has a female preponderance. Possible explanations are offered. Rectal cancer is more frequent than colonic cancer per se and occurs more often in males; more cases are recorded in relatively young patients. Colonic cancer has a predilection for the caecum. Alimentary cancer does not seem to be related to intestinal bilharziasis or other granulomas though these conditions may clinically mimic malignancy. The shortcomings in the collection of data are noted.     

Primary non-Hodgkin lymphomas of the gastrointestinal tract: analysis of 41 cases

Forty-one patients suffering from primary non-Hodgkin lymphomas of the digestive tract have been observed over a period of 15 years. The primary sites were: the stomach in 27 cases, the small bowel in 8, the ileocecal region in 5, and the mesentery in 1. Patients were staged according to the modified Ann Arbor staging system proposed by Mushoff (20). Four kinds of management were employed: 1) surgery alone; 2) surgery and radiotherapy; 3) surgery, radiotherapy and chemotherapy; 4) surgery and chemotherapy. One patient was treated by chemotherapy alone. Radiotherapy was administered postoperatively and chemotherapy after or during radiotherapy. Generally, 2 opposed fields largely encompassing the tumor area and lomboaortic nodes if necessary, up to 25-30 Gy to the midline, were employed, with a booster dose up to 40-45 Gy to the involved area. Total abdominal irradiation was never employed. No clear difference emerged in survival rate nor in relapse-free survival among the four subgroups, but patients who underwent complete resection fared better than incompletely resected patients. However, these two subgroups were not homogeneous. A clear difference in survival rate did not emerge between patients treated or not with postoperative chemotherapy, whereas patients who relapsed after complete remission or those who never had complete remission had a poor prognosis.     

117例胃肠道间质瘤的预后影响因素分析

目的探讨胃肠道间质瘤(gastrointestinal stromal tumors,GIST)的预后影响因素及术后靶向治疗对预后的影响。方法回顾性分析中国医学科学院肿瘤医院2000年1月至2010年5月以手术为首次治疗方式的117例GIST病例的临床特点、影响预后的因素及术后靶向治疗对预后的影响。结果本组117例患者1,3,5年无病生存率分别为(96.1±1.9)%、(85.2±3.7)%、(80.1±4.2)%,中位生存期28个月。单纯手术组80例,复发21例,死亡20例,带瘤生存1例;手术联合靶向治疗37例,复发3例,死亡3例。结论发病部位、核分裂像和危险度分级是GIST患者预后的独立影响因素(P<0.05),术后辅助靶向治疗能够延长GIST患者无病生存期(P<0.05)。     

Paget's disease of the breast: a study of 43 cases.

Paget's Disease of the breast is caused by spread of duct carcinoma cells along the mammary ducts to the epidermis of the nipple and areola. This is a study of 43 cases of Paget's Disease of the breast. Though only few patients presented with a lump; a carcinoma, either DCIS or IDC or both were found in all cases. The presence of an underlying breast carcinoma in Paget's Disease of the breast suggests that radical mastectomy is the treatment of choice in this condition.     

Lymphoma of the gastrointestinal tract.

Non-Hodgkin's lymphoma (NHL) of the gastrointestinal (GI) tract accounts for 4% to 20% of all NHLs and is the most common extranodal site of presentation. The stomach is the major organ involved by GI lymphoma. Helicobacter pylori infection, immunosuppression after solid-organ transplantation, celiac disease, inflammatory bowel disease, and human immunodeficiency virus (HIV) infection may be risk factors for GI lymphoma. A significant proportion of gastric lymphomas are of low-grade histology and arise from mucosal-associated lymphoid tissue (MALT). Such MALT lymphomas may be associated with H. pylori infection and may undergo complete regression following eradication of H. pylori. Lymphoma of the small bowel, colon, and rectum may also occur, but are less common than gastric lymphoma. Distinct clinicopathologic entities, such as primary intestinal T-cell lymphoma, immunoproliferative small intestinal disease, and multiple lymphomatous polyposis have been described. Surgery, radiation therapy, and chemotherapy have been used in the treatment of GI lymphomas. However, the optimal management of these lymphomas has never been determined by prospective randomized clinical trials. Such trials by cooperative groups are needed to answer many of the vital unanswered questions concerning extranodal lymphomas of the GI tract.     

Lymphomas of the upper GI tract: the role of radiotherapy

The most common site of extra-nodal non-Hodgkin's lymphoma (NHL) is the gastrointestinal tract, of which the stomach is the most common site. With the exception of extra-nodal marginal zone lymphoma of gastric mucosa-associated lymphoid tissue (MALT), where excellent long term results can be achieved by radiotherapy, the published literature lacks high quality studies evaluating the role, optimal dose and technique of such treatment in the management of gastro-intestinal lymphoma. Non-randomised studies support organ preservation and a role for local radiotherapy in gastric lymphoma.     

Early-stage carcinoids of the gastrointestinal tract: an analysis of 1914 reported cases

BACKGROUND: Gastrointestinal (GI) carcinoids, considered to be endocrine neoplasms with relatively low levels of malignancy, have never been examined in large, statistically reliable series to determine their true aggressive behavior in the early stage of both minute ( 10 mm. Although most patients (92.8%; 1777 of 1914) underwent an endoscopy and/or a wider resection of the lesions, nonresectable metastases were found in 22 patients (1.1%). Of these, 16 had undergone a laparotomy and 6 had not received surgery. Among 1001 patients with GI sm carcinoids, the 5YSRs after curative resection ranged from 98.3% in the rectum to 89.6% in the stomach (P < 0.05), representing an average of 96.7% for the entire series. CONCLUSIONS: Unexpectedly high aggressiveness in metastasis rates in both rectal and gastric sm carcinoids > 10 mm exhibiting values significantly higher than those of sm carcinomas were found in 1914 patients suffering from GI sm carcinoids. However, in sm carcinoids at either the minute or small tumor stage (tumors 相似文献   

Oral tegafur in the treatment of gastrointestinal tract cancers: a phase II study

Fifty patients affected by histologically confirmed gastrointestinal tract cancer (GTC) were treated with oral tegafur (TG) 1,000 mg m-2 p.o. on days 1-14 repeated after a 14 day interval. Out of 42 evaluable patients seven patients had a partial response (PR. 17%) with a median duration of 20.5 weeks, three had a minimal response (7%) with a median duration of 23.7 weeks, nine showed a stabilisation which lasted a median of 31.3 weeks, and 23 progressed (55%). No response was obtained in patients affected by carcinoma of the pancreas and the hepatobiliary system. All PRs were achieved in patients with metastatic disease to the liver. No response was seen in patients with bone, lung or nodal metastasis. Three PRs were obtained in patients resistant to 5-fluorouracil. The difference in survival between patients who achieved PR and those who had a stabilisation was not statistically significant. On the other hand the survival of patients with PR was significantly longer than that of patients who progressed. Oral TG was well tolerated by most patients. WHO grade 1-2 gastrointestinal and neurological toxicities were seen respectively in 36% and 25% of cases. Five patients had grade 3 nausea/vomiting and one had grade 3 diarrhoea. Our data suggest that oral TG is effective in the treatment of stomach and colorectal cancers.     

The role of endoscopic ultrasonography in the upper gastrointestinal tract: A review with illustrative cases

In this article the current applications of endoscopic ultrasound in the upper gastrointestinal tract and its adnexa, as well as the areas of likely development, are reviewed. Illustrative cases are shown from the author's experience. Pitfalls and limitations of the technique are also discussed.     

Diffuse large B-cell lymphoma presenting as multiple lymphomatous polyposis of the gastrointestinal tract

Primary gastrointestinal (GI) lymphomas constitute 5%-10% of all gastrointestinal tumors. Involvement of the entire GI tract as multiple polypoid lesions is very rare. Multiple lymphomatous polyposis is a distinctive and rare type of malignant GI lymphoma. Multiple lymphomatous polyposis is thought to represent mantle cell lymphoma of the GI tract. Approximately 60 cases of MLP have been reported so far. We report the first case of diffuse large B-cell lymphoma presenting as multiple lymphomatous polyposis of the GI tract in a 49-year-old patient with HIV and describe the clinicopathologic features. The patient was treated with a combination therapy of highly active antiretroviral therapy and CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) and exhibited complete remission.