Laparoscopic Management of Obstructing Small Bowel GIST Tumor

Background:

Gastrointestinal stromal (GIST) tumors make < 1% of all gastrointestinal neoplasms and 20% of small bowel neoplasms. The most common acute presenting symptom of these tumors is gastrointestinal hemorrhage with obstruction being rare. We discuss our laparoscopic approach to 2 patients with small bowel GIST tumors that presented to our institution with obstruction of the small bowel.

Case Presentation:

Two patients presented to the emergency department with signs and symptoms of small bowel obstruction. On workup, each was found to have a solid lesion either within or adjacent to the small bowel at the point of obstruction and both were emergently taken to the operating room. The pathologic diagnosis of small bowel GIST tumor was the same in both cases, but the pathophysiologies of the obstructing tumors were different.

Results:

Both patients underwent laparoscopic surgery with successful resection of the lesions. The details and crucial points of the laparoscopic approach to these tumors are described with specific attention to its appropriateness and safety in treating GIST tumors. Attention to particular details of the manipulation and management of the bowel in the face of obstruction and removal of the lesions is described.

Conclusion:

The laparoscopic approach to GIST tumors of the small bowel, even in the face of emergent surgery, is a safe method.     

Gastrointestinale Stromatumoren (GIST)

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the gastrointestinal tract. GIST occur predominantly in the stomach and less frequently in the extraduodenal small bowel, the colon, and the rectum; rarely, GIST can be found in the esophagus and the duodenum. Due to their biological behavior, the primary treatment goal for localized primary GIST is complete resection, without the need for lymphadenectomy or wide resection margins. Thus, gastric wedge resections and segmental resections of the small bowel are the most common surgical procedures for treating primary GIST. Surgical therapy of extensive primary tumors or of metastatic or recurrent GIST should be integrated into a multimodal therapeutic concept that includes targeted therapy with tyrosine kinase inhibitors, such as imatinib.     

Concomitant jejunal sarcomatoid carcinoma and gastric GIST in patient with polymyalgia rheumatica: A case report

INTRODUCTIONSarcomatoid carcinoma (SCA) of the small bowel is an extremely rare tumor with only 21 cases reported in literature and GISTs are relatively rare gastrointestinal neoplasms.PRESENTATION OF CASEWe report a case of an 85 year-old female admitted with intestinal obstruction in June 2010. She suffered from polymyalgia rheumatica and was under surveillance for a presumed gastric GIST. A laparotomy was performed with resection of the jejunal obstruction and complete excision of the gastric mass. Histology confirmed a gastric GIST and sarcomatoid carcinoma of the small bowel. The patient was discharged 21 days after the operation and died on the 88th post-operative day.DISCUSSIONSynchronous GISTs and other malignancies have been reported over the last years with increasing frequency.Sarcomatoid carcinoma of the small bowel is an aggressive neoplasm with poor survival rates and surgery is the cornerstones of treatment. Given its unpredictable clinical behaviour and concomitant association with other malignancies, GISTs require adequate surgical resection with careful, long-term follow-up.CONCLUSIONThis is the first case of concomitant gastric GIST with Sarcomatoid carcinoma of the small bowel, and the first report of sarcomatoid small bowel carcinoma in association with polymyalgia rheumatica.     

Stromal tumors of the small intestine: personal experience and review of the literature

Malignant gastointestinal stromal tumors (M-GIST) are rare mesenchymal tumors that arise in the wall of the gastrointestinal (GI) tract. Small intestinal GIST account for approximately 35% of all GIST the diagnosis of these tumors is difficult to establish, because the symptoms are vague and non-specific and traditional endoscopy is commonly unsatisfactory. Because of the infrequent nature of malignant small bowel tumors, the natural history and factors affecting outcome remain poorly defined; stage at presentation and complete surgical resection seem to be the main prognostic factors. For these rare tumors, surgery remains the treatment of choice, with little efficacy reported for irradiation, chemiotherapy, or both. Two cases of GIST of the jejunum, complicated by intestinal bleeding and intestinal obstruction respectively, are presented and a review of the literature is made.     

Giant GIST of the mesocolon: report of a case

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal cells of the gastrointestinal tract, that strongly express a class III receptor tyrosine kinase, called KIT, due to some mutations in the KIT proto-oncogene. Two thirds of GISTs are found in the stomach, 20% to 50% in the small bowel (one third in the duodenum), and 5% to 15% in colon and rectum; GISTs, however, may rarely be found also in the oesophagus, omentum, mesentery or the retroperitoneum. Their treatment is strictly surgical, and only R0 resection can achieve good RESULTS: Treatment with Imatinib seems to be promising in case of unresectable or metastatic GIST, even if some trials are studying its effects after curative resection. GIST of the mesocolon are rare, and as in the other locations, require extensive surgery. The Autohrs report a case of giant malignant GIST arising from transverse mesocolon, treated by en-bloc resection of the tumor with a segment of transverse colon and great omentum.     

Gastrointestinal stromal tumor with diffuse mesenteric metastases

Although malignant gastrointestinal stromal tumors (GISTs) sometimes show peritoneal dissemination, diffuse metastasis to only the mesentery is rare. We describe the unusual case of GIST in a 69-year-old woman who showed multiple nodules restricted only to the mesentery except the surface of the small and large bowel, omentum, and abdominal wall. These small nodules were similar to those seen in leiomyomatosis peritonealis disseminata. Histological and immunohistochemical findings were consistent with GIST. This case of GIST shows an apparently unique and rare spreading pattern.     

Phytobezoars as a cause of small bowel obstruction associated with a carcinoid tumor of the ileocecal area

Carcinoid tumors are slowly growing malignant neoplasms associated with an indolent clinical course. About 60% of such tumors are located within the gastrointestinal tract. We describe an unusual case of small bowel obstruction associated with a carcinoid tumor of the ileum. A 70-year-old woman was presented with abdominal pain, vomiting, and clinical signs of mechanical bowel obstruction. X-ray and CT-scan of the abdomen showed hydroaeric levels and the presence of intraluminal hyperdense "stones", presumably of gallbladder origin. A diagnostic laparotomy revealed that a large part of the terminal ileus was edematous, with prominent evidence of intestinal loop adhesions. The edematous part of the ileum was resected. Incision of the intestinal wall revealed a 2-cm soft mass at 8 cm from the ileocecal valve, where the presence of ten fruit pits obstructed the intestinal cavity. Histopathological examination confirmed the diagnosis of a carcinoid tumor. An interesting case of small-bowel obstruction with a double cause is presented: an ileal carcinoid and fruit pit bezoars. The pathophysiology of the obstruction is discussed.     

Gastrointestinal stromal tumors: case reports and review of the literature

The Authors describe four cases of gastrointestinal stromal tumours (GIST) two of them were localized in the stomach, the others in the ileum. GIST are neoplasms of mesenchymal origin which develop inside the wall of the digestive tract. The most frequent site is the stomach, followed by the small bowel; less commonly these tumors can affect the oesophagus, the colon and the rectum. GIST originate from precursors of the interstitial cells of Cajal, which are localized in the gastro-intestinal wall and are involved in the regulation of the peristalsis. The treatment is surgical resection. For advanced disease there is a new interesting treatment based on the imatinib, a tyrosine kinase inhibitor.     

Primary tumors of the small bowel

Primary tumors of the small bowel are uncommon, representing less than 6 per cent of all gastrointestinal tumors and less than 2 per cent of all malignant gastrointestinal tumors. This report concerns a twenty-five year survey of our clinical records from 1946 to 1971 which revealed 140 primary small bowel tumors, excluding periampullary tumors. Fifty-two of the neoplasms (37 per cent) were benign; eighty-eight (63 per cent) were malignant and included twenty-eight adenocarcinomas (31.8 per cent), twenty-four lymphosarcomas (27.3 per cent), nineteen carcinoids (21.6 per cent), and ten leiomyosarcomas (11.4 per cent). The average age at the time of diagnosis was 56.9 years for patients with benign tumors and 55.9 years for those with malignant tumors. The illusive and obscure nature of small bowel tumors is illustrated by the fact that 63.3 per cent of patients with benign lesions and 47.6 per cent of those with malignant lesions had symptoms for more than six months before the diagnosis was made. Bleeding was the most common present complaint in patients with benign neoplasms (52.9 per cent) whereas patients with malignant lesions more often had symptoms of obstruction (50.6 per cent). Most of the benign lesions were located proximally in the small bowel (duodenum, 34.6 per cent; ileum, 11.5 per cent), and most of the malignant lesions were located distally (duodenum, 17.0 per cent; ileum, 61.4 per cent). Treatment of patients with malignant lesions was radical excision whenever possible. Adjunctive radiation therapy was used for those with lymphoma. A second benign or malignant tumor occurred in 42.9 per cent of the patients with primary small bowel tumors. The average period of survival after diagnosis of a malignant small bowel tumor was 5.03 years: for patients with adenocarcinoma, 3.6 years; lymphosarcoma, 1.3 years; carcinoid, 6.8 years; and leiomyosarcoma, 8.3 years.     

Surgical options in the treatment of GIST of the upper portion of the stomach. Report of two cases

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal precursor cells of the gastrointestinal tract that may differentiate towards the interstitial cells of Cajal, pacemaker cells regulating autonomous motility of G.I. tract. Grading of GIST has been proven to be as difficult as their classification. Two thirds of GISTs are located in the stomach, 20-50% in the small bowel (one third in the duodenum), and 5-15% in colon and rectum; GISTs, however, may rarely be found also in the oesophagus, omentum, mesentery or the retroperitoneum. The distribuition of these tumors in the stomach is: pars media, 40%; antrum, 25%; pylorus, 20%; in less than 15%, GISTs location is next to the EGJ, in the cardia and in the iundus. The upper gastric third location of GISTs is not common, so their surgical management has been not yet well investigated. Total gastrectomy is considered the therapy of choice for the GIST located next to the EGJ, but wedge resection could be considered a surgical option in selected cases. The Authors describe 2 cases of GIST located just under the upper portion of the stomach and discuss about the different surgical options for GISTs of this region.     

肠梗阻治疗策略选择的今日观

肠梗阻由肠道机械性或功能性阻碍了消化产物正常的通过。它可以发生于胃远端、小肠、大肠,通常急性发病。虽然许多病例不需要手术治疗,但仍然是一个外科问题。肠梗阻原因繁多,应根据不同的发病原因,采取治疗手段也多种多样。通常手术治疗和针对病因的治疗是必需的,非手术治疗也是必需的。如今,心理治疗在治疗肠梗阻中的价值受到越来越多的关注。     

Gastrointestinal stromal tumors. A case of small intestine stromal tumor (SIST) with an uncertain biological aspect

Tumors of the small intestine are relatively rare. The diagnosis is difficult to establish because the symptoms are vague and non-specific. Although the small intestine constitutes 75% of the length and over 90% of the mucosal surface area of the gastrointestinal tract, only 1 to 2% of gastrointestinal malignancies occur in this segment. Metastases are usually present at the time of diagnosis. The outcome of these patients can be improved if the possibility of a malignant small bowel tumor is considered in all cases of unexplained abdominal pain or gastrointestinal bleeding, especially in younger age. Malignant tumors occur with increasing frequency in distal small bowel with a preponderance of malignant lesions in the ileum compared with the jejunum and the duodenum. Adenocarcinoma is the most common tumor of the primary malignant small bowel tumors, followed by carcinoid, lymphoma and leiomyosarcoma. Mesenchymal tumors of the gastrointestinal tract, traditionally regarded as smooth muscle tumors, have demonstrated different cellular differentiations based on immunohistochemical and ultrastructural features. Therefore the terms leiomyoma and leiomyosarcoma have been replaced by a more encompassing term, gastrointestinal stromal tumor (GIST). The majority of GISTs occurs in the stomach; stromal tumors involving the small intestine (SISTs) are far less common but seem to have greater malignant potential. The clinical a case of a small intestinal stromal tumor (SIST), localised in the jejunum and characterised by an uncertain histological aspect, is presented and a review of the literature is made.     

Primary small bowel malignant tumors. Unrecognized until emergent laparotomy

Forty-five consecutive cases of primary small bowel malignant tumors treated at our institution from 1969-1983 were reviewed. Sixty-four per cent of the patients had surgical emergencies. Fourteen patients had intestinal obstruction, 11 had gastrointestinal (GI) hemorrhage, and 4 had perforation of the small bowel. The remaining 16 patients were explored for persistent symptoms, an abnormality on GI series, or a mass lesion on CT scan. Thirty-eight patients underwent resection of the tumor and seven had palliative bypass procedures. This study suggests that a high index of suspicion and early diagnostic evaluation including a small bowel series is necessary to prevent the large number of small bowel tumors presenting as surgical emergencies. In particular, small bowel tumors should be suspected in patients with abdominal pain of unknown etiology, unexplained weight loss, or occult GI bleeding.     

Villous tumors of the stomach and small bowel

Villous tumors of the stomach and small bowel are premalignant lesions with focal adenocarcinomatous change as high as 72%. They are frequently multiple and associated with other gastrointestinal neoplasms. Thirty percent of those in the stomach are associated with an independent gastric carcinoma. Villous tumors have morphological features that facilitate specific roentgenographic diagnosis. Endoscopic biopsy may confirm the diagnosis but may give false-negative results for malignant neoplasms and therefore should not delay treatment. After diagnosis, surgical resection is mandatory and subsequent radiological-endoscopic follow-up is essential.     

Sarcomatoid anaplastic carcinoma of the small bowel in cardiac transplant bearer

Sarcomatoid carcinoma is a rare form of primitive carcinoma of the small bowel; it is considered a variant of adenocarcinoma, histologically characterized by a typical biphasic pattern with epithelial- and mesenchymal-like cells. Twenty-one cases have been described in the literature, presenting as small bowel obstructions (twenty cases) or superior vena cava syndrome (one case). The authors report the case of a 56 year-old man on immunosuppressive therapy for a heart transplant, who underwent surgery after a history of repeated episodes of melena, anemization and bowel obstruction. The operation brought to light an intraluminal occlusive mass in the distal ileum, associated with other intraluminal neoplasms of different sizes throughout the small bowel. No evidence of mesenteric adenopathies or hepatic metastases were found. Histological examination and immunohistochemical stain showed an anaplastic sarcomatoid carcinoma. The tumor cells showed strong positivity for cytokeratin and vimentin, and negativity for CD117 and CD34, as well as for all other characteristic markers of mesenchymal tumors. Early diagnosis is usually very difficult, due to the lack of any stereotyped clinical expression and the difficult to study the small bowel. Small-bowel barium follow-through or video capsule endoscopy can be helpful. In most cases, an emergency surgical operation is performed without a clear preoperative diagnosis. The case report is completed by a review of the literature.     

Small Bowell Malignant Melanoma-Report of Three Cases and Review of Literature

Malignant melanomas have a predilection to metastasize to the small bowel. Three patients with malignant melanoma involving the small bowel are reported. Two patients were operated on for small bowel obstruction and the third for gastrointestinal bleeding with anemia. Two patients remained well 6 month and 2 years, respectively, after surgery. One patient died of metastatic cerebral melanoma 6 months postoperatively.

One should suspect small bowel metastasis in every patient with malignant melanoma in his past medical history, who presents with recent changes in bowel habits, intestinal obstruction or gastrointestinal bleeding. Preoperative assessment can only raise the suspicion, even with advanced imaging methods: capsule endoscopy, enteroscopy, CT or PET-CT. The only therapeutic procedure is surgical resection, offering both short term survival as well as an improvement in the quality of life. Although prognosis is dismal there are factors associated with prolonged survival: complete surgical resection with no residual primary or metastatic tumor, so-called primary small bowel tumors in patients aged more then 60 years, LDH < 200 U/L, lack of tumor spread in mesenteric lymph nodes.     

Cutaneous metastases from a gastrointestinal stromal tumor of the stomach: review of literature

Gastrointestinal stromal tumors (GIST) are neoplasms of mesenchymal origin which may or may not be malignant. Malignant GIST rarely metastasize to distal organs. A case of cutaneous metastasis from GIST has been presented. This case is particularly interesting because not only did the patient have multiple metastases from GIST, but eventually developed cutaneous metastasis. This phenomenon has not been reported in the literature. The most current literature on gastrointestinal stromal tumors has been reviewed.     

Gastrointestinal stromal tumors: actin expression, a new prognostic factor?

The aim of this study is to analyze the clinical outcome of gastrointestinal stromal tumors (GISTs) and to determine new prognostic factors. We perform a retrospective study of all the patients diagnosed with GIST in any location and operated on between 2000 and 2008 at our institution. We analyzed 35 patients, 16 males (45.7%) and 19 females (54.3%), with a mean age of 64 +/- 13.8 years. The tumors were located in the stomach in 22 patients (62.9%), in the small bowel in 10 (28.6%), and the retroperitoneum in three (8.6%). Referring to gastric GIST, endoscopy revealed an ulceration in the mucosa in five cases, suggesting an epithelial neoplasm. In all these cases, pathology of the biopsy specimen was nonconclusive. Survival rate at 1 and 5 years was 94.3 and 88.6 per cent, respectively. Disease-free survival at 1 and 2 years was 91.4 and 88.6 per cent, respectively. Analyzing prognostic factors, a lower disease-free survival was observed among patients with constitutional syndrome at diagnosis (P = 0.000), small bowel GIST (P = 0.037), and tumors not expressing actin (P = 0.015). A lower global survival was observed among men (P = 0,036), patients with an abdominal mass (P = 0.033) or with constitutional syndrome (P = 0.007) at diagnosis and tumors at a retroperitoneal location (P = 0.0002). Gastric GIST may be confused with epithelial neoplasms, modifying the surgery. In our patients, masculine gender, constitutional syndrome and abdominal mass at diagnosis, small bowel and retroperitoneal location, and actin negative tumors are bad prognostic factors.     

Small bowel intussusception caused by metastasis from anaplastic thyroid carcinoma: case report and literature review

Symptomatic involvement of the small bowel by metastasis from an extra-abdominal primary malignancy is rare, most commonly resulting from malignant melanoma and lung cancer; very rarely is small bowel involvement as first metastatic site. The Authors report a case of anaplastic thyroid carcinoma with lung metastasis, brain metastasis and an isolated metastasis to the small bowel leading intestinal obstruction due to small bowel intussusception. The Authors review the international literature about frequency, etiopathogenesis, clinical and diagnostic features and therapy of small bowel metastasis by extra-abdominal malignancies, especially by primary anaplastic thyroid carcinoma. Small bowel metastasis from extra-abdominal malignancies are very unusual, especially from anaplastic thyroid carcinoma, and the etiopathogenesis is still unknown. Clinical findings are typical for abdominal urgency, especially by small bowel obstruction from anaplastic thyroid carcinoma. Computed Tomography has an important role in detecting the type of intestinal obstruction despite it is often unable to diagnose an isolated metastasis. Best therapy is surgical resection, that allows the assessment of metastasis and the definitive staging. The prognosis is poor, despite long-term survival has been occasionally reported for isolated small bowel metastasis     

Anemia and jejunal intussusception: An unusual presentation for a metastatic phyllodes breast tumor

INTRODUCTIONPhyllodes tumor of the breast is a rare cause of breast cancer, accounting for less than 0.5% of breast cancers. These tumors are classified as benign, borderline, or malignant, with malignant tumors compromising nearly 25% of cases. Metastases occur in 20% of malignant tumors, lungs, bones, liver and brain being the frequent sites of metastases.PRESENTATION OF CASEWe present a case of a metastatic phyllodes tumor to the small bowel causing jejunal intussusception, symptomatic anemia, and small bowel obstruction.DISCUSSIONPatients with phyllodes tumor of the breast can develop disease recurrence even years after initial treatment. Phyllodes tumor metastasizing to the small bowel is extremely rare, with only three known previously described case reports in the literature.CONCLUSIONHigh risk patients, with a past medical history of phyllodes breast cancer, should be monitored closely. Even years after breast cancer treatment, these patients may present with gastrointestinal complaints such as obstruction or bleeding, and therefore metastatic disease to the small bowel should be considered on the differential with subsequent abdominal imaging obtained.