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Gastrointestinal stromal tumor (GIST) account for 1% of all gastrointestinal neoplasms and are the most common mesenchymal tumor of gastrointestinal tract. There are considered to originate fom the intestinal cell of Cajal, an intestinal pacemaker cell, characterized usually express the KIT protein on immunohistochemistry. The stomach (40-60%) and small intestine (30-40%) are the most common locations. Diagnosis of these tumors is difficult to establish, because symptoms are vague and traditional diagnostic tests are not specific. GISTs shows a wide variety of clinical behaviours ranging fom benign to frankly malignant, making the outcome totally unpredictable. Surgery is the standard treatment of local GIST while Imatinib (tyrosine kinasi inhibitor) is considered as the standard treatment of metastatic disease. Resistence to Imatinib is also becoming a major clinical problem but new tirosyne kinase inibitor are being studied to improve the treatment and survival. The present paper is a review of the salient features of epidemiology, pathophysiology, diagnosis, therapy and prognostic factors of GIST

Malignant neoplasms of the small bowel are among the rarest types of cancer, accounting for only 2% of all gastrointestinal neoplasms. Owing both to the intrinsic difficulty of common radiographic and endoscopic methods in visualising the entire small bowel and the lack of typical physical findings, a delay in diagnosis is common. Recently, magnetic resonance (MR) imaging has become a widely accepted imaging modality in the study of suspected small-bowel neoplasms due to its ability to depict, without exposure to ionising radiation and with excellent soft-tissue contrast, intraluminal disorders in conjunction with mural, extraparietal and regional abnormalities. The aim of this pictorial review is to illustrate the MR appearance of malignant small-bowel neoplasms.

The role of imaging in functioning endocrine tumours (FETs) is primarily to detect the tumour, that is, to verify lesion number and location. Radiological detection of carcinoid tumours is limited by typical tumour location throughout the gastrointestinal tract or appendix and is therefore dependent on the tumour being large enough to make it recognisable in that site. The most common FET is insulinoma, which is commonly characterised by the typical appearance of a hypervascular lesion at multidetector-row computed tomography and magnetic resonance imaging. A particularly important role is played by intraoperative ultrasound in defining the exact number of lesions, their relationship with adjacent vascular structures and the pancreatic duct for the purposes of correct surgical planning (enucleation or resection). In the setting of nonfunctioning endocrine tumours (NFETs), which manifest late as large masses causing compression symptoms or as incidental findings, imaging is not primarily aimed at tumour detection, as this is relatively easy given the large size of the lesions. Rather, its role is to characterise the tumour and, in particular, to differentiate pancreatic NFET from ductal adenocarcinoma, as in comparison, malignant NFETs have a more favourable prognosis (5-year survival rate 40% compared with 3%-5% for adenocarcinoma) and therefore require different treatment approaches. As NFET are often malignant, they also require accurate staging and appropriate follow-up. In 80% of cases, NFETs have a "typical" imaging appearance: location in the pancreatic head, large dimensions (diameter between 5 and 15 cm, >10 cm in 30% of cases), capsule, sharp and regular margins owing to the expansile and noninfiltrative growth pattern, solid density and arterial hypervascularity. Some 20% of NFETs display different imaging characteristics ("atypical" appearance) as a result of arterial hypovascularity due to the presence of abundant fibrous stroma. Lastly, a small percentage of NFETs has yet a different appearance ("unusual") due to the cystic nature and/or diffuse location throughout the pancreatic parenchyma.

The observation of a number of cases of intestinal carcinoid tumours prompted the authors to review the literature in order to define the principal biological and anatomopathological aspects and the current therapeutic choices. The diagnosis is often obtained on the basis of anatomopathological examination. The kind of surgical treatment is still a matter of controversy: minimal or extended resection? A number of criteria may orient the surgeon towards major surgery, such as tumour size, node involvement, infiltration of the serous membrane, and liver metastases.

We report our experience over the period 2000-2004 with the diagnosis and treatment of primary small bowel neoplasms in the emergency setting, stressing the importance of a correct preoperative diagnosis for the implementation of specific treatment. From January 2000 to September 2004 we treated 5674 patients, 8 of whom (0.8%) presented a primary small bowel neoplasm emergency. The diagnosis was made using small bowel ultrasound. We detected 8 cases of primary small bowel neoplasms using ultrasound. This examination was specific for the small bowel and enabled us to obtain an accurate preoperative diagnosis. A correct diagnosis of primitive small bowel neoplasm is necessary in order to institute the right treatment. The main treatment is surgery in our experience, the gold standard being laparoscopy but only when performed by expert surgeons.

Lung cancer is usually diagnosed at an advanced stage and metastases are present in 50% of patients. Small bowel metastases from lung cancer are rare, being more frequent in patients with melanoma, uterine, ovarian, kidney or gastrointestinal cancer, or osteosarcoma. From November 1998 to August 2003, 740 cases of lung cancer (641 non-small-cell lung cancer and 99 neuroendocrine tumours) were diagnosed. We also observed 64 patients with malignant pleural mesothelioma and performed 23 pleuropneumonectomies. Over the same period we admitted 4 patients (one recurrent) with small bowel metastases, three from lung cancer and one from malignant mesothelioma. The clinical symptoms were bowel occlusion and intestinal bleeding. Radiological techniques such as small bowel enema and CT enteroclysis were used with positive results. In one patient with intestinal bleeding capsular endoscopy revealed a bleeding metastasis. All patients were operated on. Neither mortality nor morbidity were observed. All patients were discharged after a median stay of 10 days. One patient is still alive and disease-free 39 months after the first intestinal surgery for metastases. Intestinal metastases from lung cancer are rare and the diagnosis is often late. In some cases the clinical manifestations of the metastases are observed before those of the primitive tumour. However, in the presence of small bowel occlusion and intestinal bleeding of uncertain origin, clinical history-taking is very important and diagnostic procedures must be performed to exclude a secondary pathology.

Lung carcinoma is the first cause of death for tumors; in Italy there are from 35,000 to 40,000 new cases a year, with a global survival to 5 years from the diagnosis in 13% of cases. The gastric and small bowel metastases are rare, respectively 0.4% and 1.1% and the cases reported in the literature are rare. There is often a poor symptomatology and diagnosis is usually based on post-mortem examination. Yet, sometimes, the first and only demonstration of the secondary illness is a surgical complication, whether it be a haemorrhage, a perforation or an occlusion. The authors report their experience, from April 1999 to March 2003, of 3 cases of small bowel metastases, presented in 1 case with a perforation and in 2 cases with an occlusion and of 1 case of gastric metastasis, which presented with a haemorrhage. These patients were treated by emergency surgery, with 1 case only of postoperative mortality. The 1st patient died 6 months after surgery, and the 2nd patient 6 days after, the 3rd patient 4 months after the 1st operation and the 4th patient is still in follow-up after 6 months. The authors present a review of the literature and some considerations of diagnosis and surgical treatment.

We report the case of a patient with von Recklinghausen's disease, who was admitted with a diagnosis of acute abdomen due to small bowel neurofibromatosis. The patient was submitted to an abdominal CT scan that showed a homogeneous round lesion, with a regular margin, probably belonging to the small bowel and with the appearance of a benign lesion that probably caused an intestinal intussusception. The patient was submitted to a surgical procedure that mainly consisted in multiple small bowel resections. The histopathological examination confirmed the benign nature of the lesions. About one third of patients affected by von Recklinghausen's disease present involvement of the bowel, but only 5% of them are symptomatic. The intestinal tumours are usually neurofibromas and are mainly localized in the jejunum. However, there have also been reports of stromal, nervous and endocrine tumours and even other tumours not belonging to these categories, including adenocarcinoma. The overall incidence of intestinal malignancy in patients with von Reckilnghausen's disease is about 10%. The surgical operation, as well as the histopathological and immunochemical examination of the intestinal lesions are of crucial importance for the treatment of the complications of intestinal neurofibromatosis and for the treatment and diagnosis of malignancy.

The authors describe the first ever reported case of a malignant pecoma of the uterus infiltrating the ovary, the tube and two bowel loops. This extremely rare tumour usually shows a benign behaviour and seems to arise from the perivascular epithelioid cells (PEC).

Intestinal malignant neoplasms are extremely rare (1% of all solid tumours) and leiomyosarcomas represent 20% of them. The authors report the experience of 5 cases (M:F ratio = 0.6), aged 30-69 yrs old, treated in the period 1985-95. The best results have been obtained in 2 cases, characterized by low grading and submitted to curative resections. The others presented local and distant (mostly hepatic) extensions with a poorer prognosis (1-3 yrs. survival). Leiomyosarcomas are particularly binding because of their rarity and aspecific symptomatology, determining late diagnosis in most cases. The clinical course, the surgical and complementary management, the istology and the prognosis have been analysed. Nowadays 5 yrs-survival is very low and the prognosis remains severe because of local and distant metastases, already present at laparotomy. New chances may come out from better diagnostic techniques and from new complementary chemotherapeutical associations.

The authors present a case of small bowel primitive lymphoma found in a 66-years-old female. After having briefly reviewed the literature they direct their attention to the clinical-instrumental diagnostic difficulties of this neoplasm which is often discovered only when it has already invaded various organs. Therefore surgical therapy is not always radical. This fact in addition to the fatally rapid postoperative course led the authors to discuss the problems involved in a correct treatment of this pathology.

The authors underline that, although rarely found, leiomyomas of the gastrointestinal tract are the most frequent benign tumours of the small intestine. On occasions, from being a simple autoptic finding or diagnosed during the course of explorative laparotomy, they may be responsible for emergency abdominal surgery, hemorrhage, occlusion or perforation. The authors discuss the experience acquired in two cases-treated during the last two years, both of which were symptomatic. In one case there was a palpable abdominal mass, and in both the key symptom was the appearance of melena a few days before being admitted to hospital. The most striking problem of this rare pathology is its diagnosis owing to both the absence of a specific clinical symptoms, and the difficulties shown in radiological diagnostics of the small intestine. In the authors' experience CAT using a contrast medium is an important instrument, whereas clyster of the small intestine is of scarce value. Explorative laparotomy was essential for removing any doubts regarding diagnosis. Both patients underwent extensive intestinal resection, including the mass and relative mesenteric wedge, with TT single-layer anastomosis. The postoperative period was normal.

Four cases of intestinal carcinoids are described. Authors remake considerations about diagnosis and treatment in this field.

The clinical consequences of intestinal malabsorption are extremely variable and a dissociation between malabsorption, malabsorption syndrome and enteropathy is often noted. Enteropathy does not always results in malabsorption and in an alteration of the tests exploring the absorptive function. The following have particular relevance in clinical practice: coeliac disease, malabsorption induced by microbiologic agent (including Whipple's disease), post-surgical malabsorption and selective carbohydrate malabsorption. In particular, coeliac disease has been analyzed in its various aspects, from studies with organ cultures to immunological hypotheses, from the classical variety to subclinical forms and to serious complications, such as enteropathy-associated T cell lymphoma. Malabsorption syndromes are dramatically underdiagnosed: in the typical case of coeliac disease, enteropathy represents a clinical iceberg, and the discovery of the submerged portion, represented by the polymorphous subclinical varieties, has just started. As far as intestinal malabsorption is concerned, the main clinical problem regards diagnosis.

The authors report their experience in the management of five patients affected by small bowel neoplasms (2 adenocarcinomas, 1 carcinoid tumor, 1 leiomyosarcoma and 1 non-Hodgkin's lymphoma) operated from June '92 to September '93. Diagnostic and therapeutical aspects of this uncommon pathology are discussed.

Lymphomas of the small bowel are rare tumors with few characteristics. Clinical and radiological diagnosis is difficult, because they may be confused by inflammatory or neoplastic forms, especially in primary involvement of the small intestine. Observation of three cases of primary extranodal lymphomas of the small bowel prompted a review of problems relating to the histology, clinical manifestations and the staging classification. This system is an important aid in the evaluation of the treatment and prognosis of these forms.

The Authors report their experience in cases of severe lower intestinal bleeding. On the basis of personal data and previously reported results, endoscopy is shown to be the primary and sometimes essential method, especially in the case of colo-rectal bleeding, both in terms of its diagnostic precision and possible therapeutic uses. This technique was used to treat all emergency cases, obtaining a diagnostic resolution in 85.6% and hemostasis and/or the concomitant removal of the lesion in 52.3% of cases.

Tumours of the small intestine are extremely rare and have a particularly severe prognosis. The impossibility of making an early diagnosis due to the scarcity of precise symptoms during the early stages of the disease and the lack of safe instrumental tests limits the results of treatment which is essentially surgical. In order to contribute to the study of the disease and improve its prognosis, the authors report the cases which they have observed and review the literature.

Mortality from colon-rectal cancer is rising steadily in Italy; of malignant tumours, those of the nitestine come second after those of the lung. Hence the ned for early diagnosis based on the search for occult blood in the stools. This test takes on important significance for screening purposes in subjects at risk. Alongside diagnosis it is important to effect primary prevention diet, food and environmental factors). A negative result of the identification of occult blood in the stools absolutely does not exclude tumour of the intestinal tract. A positive finding, on the other hand, does not necessarily confirm the presence of a tumour. Such an investigation was carried out at the Analysis Laboratory of the Sant'Antonio di Ovada Hospital, Health Unit 74, in the period between October '87 and June '88. The samples were taken from hospitalised patients and from out-patients. The samples from hospitalised patients totalled 324 and 20 proved positive for occult blood; of the out-patient samples (176), positivity was observed in 12. Hospitalised patients were recontrolled and results were confirmed positive. The out-patients did not present for further controls.

The authors report four cases of benign tumors of the small bowel (2 leiomyomas and 2 lipomas). After a short review of the literature, they put in evidence the relatively infrequent occurrence and the difficulties involved in early preoperative diagnosis.

Two cases of diverticultis of the right colon are described which received surgical treatment. These lesions often simulate acute appendicitis and thus represent a intraoperative surprise Differential diagnosis using a neoblastic mass is often impossible. The paper stresses the importance of surgical treatment which in general terms is the same as the treatment of the diverticular mass of the left colon. In cases where it is difficult to decide between a phlogostic and neoblastic pathology, it is advised that a right hemicolectomy should be performed in line with criteria for oncological requirements.

The paper analyses the results of a large number of patients operated for large intestine carcinoma who then underwent a postoperative endoscopic follow-up to assess the number of metachronous tumours. The anatomicoclinical characteristics of the latter are discussed.

Two cases of carcinoma of the small intestine are described. In spite of its large epithelial surface, this organ is rarely a cancer site. For these reason, an account is given of cases observed over the previous year. In addition, mention is made of the pathological anatomy, symptomatology, preoperative investigation, and treatment of this form.

A rare case of primary lymphoma of the bowel in a 26 year old woman is reported and the complex problems of diagnosis and treatment are examined. The importance of establishing the primary or secondary nature of this tumour of the small bowel is emphasised. This is because primary intestinal tumours require the most radical treatment possible even in advanced stages of the disease in order to avoid complications and to identify the areas involved in the proliferative process. Finally the importance of continuing surgery with drug and radiation treatment in order to improve prognosis is emphasised.

An observed case of leiomyosarcoma of the small intestine triggered a review of the literature that highlights the difficulties encountered in the radical treatment of such lesions in view of both their extreme variety and their tardive and unclear symptoms.

Authors stress that multiple carcinomas of the large bowel are not exceptional at all and report 5 cases of metachronous cancers and 1 case of synchronous cancers. They are 4,4% of all the colo-rectal neoplasms operated by the "Istituto di Semeiotica Chirurgica dell'Università di Trieste". The Authors discuss the most important pathogenetic, pathological and diagnostic problems and emphasize the usefulness of a very careful intra-operative evaluation of the entire colon and of a post-operative follow-up for the early discovery and treatment of a second cancer in another tract of the large bowel.

The Authors present 10 cases of peripheral schwannomas observed in the Ancona University Surgical Clinic in the five-year period 1976-1980. An examination is made of the frequency of the various symptoms in relation to the different localisations, the diagnostic techniques, among which particular importance is attributed to echo tomography, and the principles underlying surgical treatment.

The presentation of a rare case of leiomyoma of Meckel's diverticulum in a boy of 5 yr forms the starting point for a discussion of such cancers. In adult age, the complication is not so rare as the sporadic publications would suggest, incidence of tumoral forms being around 1 : 50 M.D. with a high malignancy frequency. In paediatric age, on the other hand, the tumoral degeneration of a M.D. is an exceptional occurrence: a review of more than 1,200 reported cases and personal experience (48 cases of M.D. in 15 yrs) confirm the rarity of the observation. The need to seek M.D. on each occasion and remove it to prevent the risk of tumoral degeneration in adult age is stressed. M.D. surgical removal tecniques are commented on and emphasis laid on the desirability of ample intestinal resection in cases of cancer of the diverticulum.

Four cases of sarcoma of the small intestine (2 leiomyosarcoma, 1 fibrosarcoma, and 1 Hodgkin's disease) are described. In each case, diagnosis was the outcome of surgery: emergency operation for acute abdomen in three cases (2 mechanical ileus, 1 perforation); exploratory laparotomy for recurrent haemorrhage following negative X-ray examination per os and clyster of the digestive tract on a number of occasions. The relevant literature is surveyed. Attention is drawn to the different anatomopathological and clinical forms, and to the difficulty of diagnosis.

Hewitt's method for disinfection of the large intestine is described and a personal series of 84 total isoperistaltic washings carried out at the University of Milan 1st Surgical Pathology Department in the period July 1976 to May 1977 prior to surgery or diagnostic examination (manometry, pancolonscopy, barium enema etc.) is presented. The results confirmed the effectiveness and harmlessness of the method. Personal modifications permitting its wider application in large intestine diagnosis and surgery are mentioned. A personal method for selective radiological visualisation of the ileum and colon employing enterolusis is explained.