PRIMARY TUMORS OF THE SMALL INTESTINE (ANALYSIS OF 102 PATIENTS)

Primary tumors of the small intestine are uncommon and a correct preoperative diagnosis is extremely difficult. From 1964 to 1983, 102 cases of this disease were detected surgically at our hospital. In order to investigate the problems involved in diagnosis and treatment, a comprehensive analysis of small intestinal tumors is made in this paper.

Clinical analysis of primary anaplastic carcinoma of the small intestine

Primary anaplastic carcinoma is a rare variant of small intestinal cancer. Most reports of primary anaplastic carcinoma of the small intestine are isolated case reports, therefore the clinicopathological features, therapeutic management, and surgical outcome of this tumor type remain unclear. This review analyzes the available clinical characteristics of primary anaplastic carcinoma of the small intestine and investigates key differences from differentiated adenocarcinoma of the small intestine. A Medline search was performed using the keywords ＇small intestine＇ and ＇anaplastic carcinoma＇ or ＇undifferentiated carcinoma＇. Additional articles were obtained from references within the papers identified by the Medline search. The literature revealed a poor prognosis for patients who underwent surgical resection for anaplastic carcinoma of the small intestine, which gave a 3-year overall survival rate of 10.8% and a median survival time of 5.0 mo. The literature suggests that anaplastic carcinoma~ is markedly more aggressive than differentiated adenocarcinoma of the small intestine. Surgical resection with the aim of complete tumor removal provides the only beneficial therapeutic option for patients with anaplastic carcinoma of the small intestine, because chemotherapy and radiation therapy have no significant effect on the rate of survival. However, despite complete tumor resection, most patients with anaplastic carcinoma of the small intestine are at great risk of disease recurrence. Multicenter clinical trials are expected to provide additional therapeutic strategies and establish the efficacy of multimodality adjuvant therapy. This report also highlights the importance of a systematic diagnostic approach for anaplastic carcinoma of the small intestine.

Diagnosis and management of small bowel neuroendocrine tumors:A state-of-the-art

This review provides an update on the epidemiology,pathophysiology,symptoms,diagnosis and treatment of neuroendocrine neoplasms(NENs)of the small bowel(SB).These NENs are defined as a group of neoplasms deriving from neuroendocrine cells.NENs are currently the most common primary tumors of the SB,mainly involving the ileum,making the SB the most frequently affected part of the gastrointestinal tract.SB NENs by definition are located between the ligament of Treitz and the ileocecal valve.They are characterized by small size and induce an extensive fibrotic reaction in the small intestine including the mesentery,resulting in narrowing or twisting of the intestine.Clinical manifestations of bowel functionality are related to the precise location of the primary tumor.The majority of them are non-functional NENs and generally asymptomatic;in an advanced stage,NENs present symptoms of mass effect by non-specific abdominal pain or carcinoid syndrome which appears in patients with liver metastasis(around 10%).The main manifestations of the carcinoid syndrome are facial flushing(94%),diarrhea(78%),abdominal cramps(50%),heart valve disease(50%),telangiectasia(25%),wheezing(15%)and edema(19%).Diagnosis is made by imaging or biochemical tests,and the order of request will depend on the initial diagnostic hypothesis,while confirmation will always be histological.All patients with a localized SB NEN with or without near metastasis in the mesentery are recommended for curative resection.Locoregional and distant spread may be susceptible to several therapeutic strategies,such as chemotherapy,somatostatin analogs and palliative resection.

New progress in CT and MRI examination and diagnosis of small intestinal tumors

Precise examination and diagnosis of small intestinal tumors is difficult because of the curved course and overlapping canal of the small intestine. Traditional technology for intestinal canal examination and endoscopy cannot exhibit the intestinal wall and extra-luminal structure well. With the development and advancement of multi-slice spiral computed tomography and magnetic resonance imaging (MRI), computed tomography enteroclysis (CTE) and magnetic resonance enteroclysis (MRE) are widely used in the examination and diagnosis of small intestinal tumors. CTE and MRE, with three-dimensional imaging capabilities and excellent soft-tissue contrast, can analyze the abnormalities of peripheral intestinal structure as well as the tunica mucosa. In addition, these two technologies can clearly reveal the localization, appearance, degree of mesenteric infiltration and remote tumor metastasis, which increases our cognition of the imaging diagnosis for intestinal tumors. Here we review recent progress in imaging (CT and MRI) examination and diagnosis of small intestinal tumors.

Comparative study of intestinal tuberculosis and primary small intestinal lymphoma

AIM: To characterize the clinical, radiological, endoscopic and pathological features of intestinal tuberculosis (ITB) and primary small intestinal lymphoma (PSIL).

Endoscopic characteristics of small intestinal malignant tumors observed by balloon-assisted enteroscopy

BACKGROUND Capsule endoscopy and balloon-assisted enteroscopy(BAE) enable visualization of rare small bowel conditions such as small intestinal malignant tumors.However,details of the endoscopic characteristics of small intestinal malignant tumors are still unknown.AIM To elucidate the endoscopic characteristics of small intestinal malignant tumors.METHODS From March 2005 to February 2017,1329 BAE procedures were performed at Keio University Hospital. Of these procedures,malignant tumors were classified into three groups,Group 1: epithelial tumors including primary small intestinal cancer,metastatic small intestinal cancer,and direct small intestinal invasion by an adjacent organ cancer; Group 2: small intestinal malignant lymphoma; and Group 3,small intestinal gastrointestinal stromal tumors. We systematically collected clinical and endoscopic data from patients' medical records to determine the endoscopic characteristics for each group.RESULTS The number of patients in each group was 16(Group 1),23(Group 2),and 6(Group 3),and the percentage of solitary tumors was 100%,43.5%,and 100%,respectively(P < 0.001). Patients' clinical background parameters including age,symptoms,and laboratory data were not significantly different between the groups. Seventy-five percent of epithelial tumors(Group 1) were located in the upper small intestine(duodenum and ileum),and approximately 70% of gastrointestinal stromal tumors(Group 3) were located in the jejunum. Solitary protruding or mass-type tumors were not seen in malignant lymphoma(Group2)(P < 0.001). Stenosis was seen more often in Group 1,(68.8%,27.3%,and 0%;Group 1,2,and 3,respectively; P = 0.004). Enlarged white villi inside and/or surrounding the tumor were seen in 12.5%,54.5%,and 0% in Group 1,2,and 3,respectively(P = 0.001).CONCLUSION The differential diagnosis of small intestinal malignant tumors could be tentatively made based on BAE findings.

Small intestinal hemolymphangioma treated with enteroscopic injection sclerotherapy: A case report and review of literature

BACKGROUND Hemolymphangiomas are rare malformations composed of both lymphatic and vascular vessels and are located in the pancreas,spleen,mediastinum,etc.Small intestinal hemolymphangioma is extremely rare and often presents as obscure gastrointestinal bleeding.It is rarely diagnosed correctly before the operation.Endoscopic injection sclerotherapy is usually used as a management of bleeding in esophageal varices and was occasionally reported as a treatment of vascular malformation.The treatment of small intestinal hemolymphangioma with enteroscopic injection sclerotherapy has not been reported.CASE SUMMARY A 42-year-old male complained of recurrent episodes of melena and dizziness,fatigue and reduced exercise capacity for more than 2 mo.Gastroduodenoscopy and blood test revealed a gastric ulcer and anemia.Treatment with oral protonpump inhibitors and iron did not improve symptoms.We then performed a capsule endoscopy and anterograde balloon-assisted enteroscopy and revealed a hemolymphangioma.Considering it is a benign tumor without malignant potential,we performed enteroscopic injection sclerotherapy.He was discharged 4 days later.At follow-up 3 mo later,the melena disappeared.Balloon-assisted enteroscopy revealed an atrophied tumor atrophied and no bleeding.Argon plasma coagulation was applied to the surface of the hemolymphangioma to accelerated healing.When he returned for follow-up 1 year later,anemia was resolved and the tumor had been cured.CONCLUSION Balloon-assisted enteroscopy and capsule endoscopy are effective methods for diagnosis of hemolymphangioma.Enteroscopic injection sclerotherapy is an effective treatment.

Perforation of metastatic melanoma to the small bowel with simultaneous gastrointestinal stromal tumor

The gastrointestinal tract (GIT) is a common site of metastases for malignant melanoma. These metastatic tumors are often asymptomatic. We describe a case of a 58-year-old male who presented with a sudden onset of generalized abdominal pain. The patient's past medical history was significant for lentigo melanoma of the right cheek. Laparotomy was performed and two segments ofsmall bowel, one with a perforated tumor, the other with a non-perforated tumor, were removed. Histology and immunohistochemical staining revealed the perforated tumor to be a metastatic malignant melanoma and the non-perforated tumor was found to be a gastrointestinal stromal tumor (GIST). The patient was discharged 7 d postoperatively. To the best of our knowledge, this is the first reported case in the literature of a simultaneous metastatic malignant melanoma and a GIST. Surgical intervention is warranted in patients with symptomatic GIT metastases to improve the quality of life or in those patients with surgical emergencies.

Laparoscopic resection of synchronous gastric cancer and primary small intestinal lymphoma: A case report

Synchronous gastric cancer and primary small intestinal lymphoma are extremely rare. A 49-year-old woman was referred to our hospital with a history of upper abdominal pain for two weeks and was diagnosed with synchronous cancer. During hospitalization, the patient underwent laparoscopic distal gastrectomy + resection of bilateral ovaries + partial resection of both small intestine and descending colon. Pathological examination revealed a synchronous cancer consisting of early gastric cancer with poorly differentiated adenocarcinoma located in mucosa, with lymph node metastasis (3+/29) (T1N1M0, stage IB); and diffuse large B cell lymphoma of small intestine involving descending colon and bilateral ovaries, with lymph node metastasis (2+/5) (Ann Arbor IIE). The patient recovered well, without any obvious complications and was discharged on post-operative day 7. The patient received six cycles of chemotherapy after operation. She has been doing well with no evidence of recurrence for 13 mo.

Surgical aspects of small intestinal neuroendocrine tumors

Small intestinal neuroendocrine tumors(NETs)are a heterogeneous group of epithelial tumors with a predominant neuroendocrine differentiation.Although NETs are usually considered rare neoplasms,small intestinal NETs are the most common primary malignancy of the small bowel,with an increasing prevalence worldwide during the course of the past few decades.The indolent nature of these tumors often leads to a delayed diagnosis,resulting in over one-third of patients presenting with synchronous metastases.Primary tumor resection remains the only curative option for this type of tumor.In this review article,the various surgical aspects for the excision of small intestinal NETs are discussed.

Upfront surgery of small intestinal neuroendocrine tumors. Time to reconsider?

Small intestinal neuroendocrine tumors(SI-NETs) may demonstrate a widely variable clinical behavior but usually it is indolent. In cases with localized disease, locoregional resective surgery(LRS) is generally indicated with a curative intent. LRS of SI-NETs is also the recommended treatment when symptoms are present, regardless of the disease stage. Concerning asymptomatic patients with distant metastases, prophylactic LRS has been traditionally suggested to avoid possible future complications. Even the current European Neuroendocrine Tumor Society guidelines emphasize a possible effect of LRS in Stage IV SINETs with unresectable liver metastases. On the contrary, the 2017 National Comprehensive Cancer Network Guidelines on carcinoid tumors do not support the resection of a small, asymptomatic, relatively stable primary tumor in the presence of unresectable metastatic disease. Furthermore, a recent study revealed no survival advantage for asymptomatic patients with distant-stage disease who underwent upfront LRS. At the aforementioned paper, it was suggested that delayed surgery as needed was comparable with the upfront surgical approach in terms of postoperative morbidity and mortality, the length of the hospital stay and the rate of incisional hernia repairs but was associated with fewer reoperations for bowel obstruction. On the other hand, it is also important to note that some patients might benefit from a prophylactic surgical approach and our attention should focus on identifying this patient population.

Treatment with Parenteral Nutrition Support and Chinese Herbs in One Case of Primary Small Intestinal Lymphangiectasia

TNTESTINAL lymphangiectasia （IL） is a rare disease first reported by Waldmanin in 1961.1 Since then, no more than two hundred cases have been reported. IL is characterized by dilated lymphatic vessles in the intestinal wall and small bowel mesentery which induceloss of protein and lymphocytes into bowel lumen. We here report a case of IL in a young male patient who was admitted for complaint of recurrent diarrhea for ten years and aggravation of the illness one year ago. He was diagnosed by endoscopy and confirmed by pathology as a primary IL and treated by parenteral nutrition support and Chinese herbs.

Clinical and ultrasound features of small intestinal stromal tumors in woman pelvic cavity

Objective： The purpose of this study was to search the ultrasound features of small intestinal stromal tumor （SIST） in women by reviewing their clinical data. Methods： The study group consisted of 18 female patients with pathologically proven SIST in our hospital from 2000 to 2009. Medical records were reviewed for clinical information such as manifestation, findings of ultrasonography and immunohistochemical phenotype. Results： The average age of 18 female patients with SIST was 55.2 years （ranged, 39-84 years）. Seventeen cases of them had abnormal hypogastric masses, no mass in one case. The shapes of masses were round or Iobulate beside tightly uterus or ovary. The echo of masses was low. It＇s borders were clear and sometimes irregular. The sizes of the masses varied from 6.89-193.05 cm3, the diameter ranged from 24 tom-110 ram, 85.3% of which 50 mm-110 mm. There were rare color flow signals under color Doppler velocity mode or color Doppler power mode. Exploratory laparotomy confirmed that 16 cases of the tumors originated from small intestine, and 2 cases from the ileocecat. The positive rate of the immunohistochemical phenotype of CDl17 was 88.2%, and CD34 was 76.5%. The examination of pathology confirmed that 16 cases were malignant and 2 cases borderline tumors. Eighteen cases underwent radical surgical resection. Conclusion： The characteristics of SISTs in women pelvic cavity are： age 〉 40 years, a mass （proven not from productive system by ultrasonography） in pelvic cavity, melena, positive findings in pathology and CD117/CD34, especially notice masses which diameter is more than 50 mm and possess potential malignant. Ultrasonography is a convenient and effective tool of screening SISTs.

Application of laparoscopy in diagnosis and treatment of massive small intestinal bleeding: Report of 22 cases

AIM: To investigate the diagnostic and therapeutic value of laparoscopy in patients with massive small intestinal bleeding. METHODS: Twenty-two patients with massive small in- testinal bleeding and hemodynamic alteration underwent laparoscopic laparotomy in our unit from December 2002 to April 2005. Post pathologic sites were found, laparos- copy- or laparoscopy-assisted part small intestinal resec- tion including pathologic intestinal site and enteroanas- tomosis was performed in all these patients. RESULTS: The bleeding sites were successfully detected by laparoscopy in all these 22 patients. Massive small intestinal bleeding was caused by jejunum benign stromal tumor in 8 cases, by jejunum potential malignant stromal tumor in 5 cases, by jejunum malignant stromal tumor in 1 case, by Mechel’s diverticulum in 5 cases, by small intestinal vascular deformity in 2 cases, and by ectopic pancreas in 1 case. A total of 16 patients underwent laparoscopy-assisted enterectomy and enteroanastomosis of small intestine covering the diseased segment and 6 patients received enterectomy of the diseased segment under laparoscope. No surgical complications occurred and the outcome was satisfactory. CONCLUSION: Laparoscopy in diagnosis and treatment of massive small intestinal bleeding is noninvasive with less pain, short recovery time and definite therapeutic efficacy.

Multiple metastases to the small bowel from large cell bronchial carcinomas

AIM: Metastases from lung cancer to gastrointestinal tract are not rare at postmortem studies but the development of clinically significant symptoms from the gastrointestinal metastases is very unusual. METHODS: Formalin-fixed, paraffin-embedded tissues were cut into 5 urn thick sections and routinely stained with hematoxylin and eosin. Some slides were also stained with Alcian-PAS. Antibodies used were primary antibodies to pancytokeratin, cytokeratin 7, cytokeratin 20, epithelial membrane antigen, vimentin, smooth muscle actin and CD-117. RESULTS: We observed three patients who presented with multiple metastases from large cell bronchial carcinoma to small intestine. Two of them had abdominal symptoms (sudden onset of abdominal pain, constipation and vomiting) and in one case the tumor was incidentally found during autopsy. Microscopically, all tumors showed a same histological pattern and consisted almost exclusively of strands and sheets of poorly cohesive, polymorphic giant cells with scanty, delicate stromas. Few smaller polygonal anaplastic cells dispersed between polymorphic giant cells, were also observed. Immunohistochemistry showed positive staining of the tumor cells with cytokeratin and vimentin. Microscopically and immunohistochemically all metastases had a similar pattern to primary anaplastic carcinoma of the small intestine. CONCLUSION: In patients with small intestine tumors showing anaplastic features, especially with multiple tumors, metastases from large cell bronchial carcinoma should be first excluded, because it seems that they are more common than expected.

Jejunal small ectopic pancreas developing into jejunojejunal intussusception:A rare cause of ileus

Intussusception is rare in adults. We describe a 62-year-old man with jejunal ectopic pancreas that led to jejunojejunal intussusception and ileus. The patient was admitted to our hospital because of intermittent abdominal pain. Plain abdominal radiography showed some intestinal gas and fluid levels. Abdominal CT scan demonstrated a target sign suggesting bowel intussusception. Jejunography using a naso-jejunal tube showed an oval-shaped mass about 15 mm in diameter with a smooth surface in the jejunum, which suggested a submucosal tumor (SMT), and edematous mucosa around the mass. Partial jejunal resection was carried out and the resected oval-shaped tumor, 14 mm×11 mm in size, was found to be covered with normal jejunal mucosa. The tumor was histologically diagnosed as type ectopic pancreas according to the classification proposed by Heinrich.Abdominal pain resolved postoperatively.This case reminds us that jejunal ectopic pancreas should be included in the differential diagnosis of intussusception caused by an SMT in the intestine.

Balloon-assisted endoscopic submucosal dissection for treating small intestinal lipomas:Report of two cases

BACKGROUND Most small intestinal lipomas are treated surgically,and some require repeated surgeries for multiple lipomas.However,application of endoscopic submucosal dissection(ESD)technology in the deep small intestine is rarely reported owing to the special anatomical structure of the small intestine,medical equipment limitations,and the lack of relevant experience among endoscopists.CASE SUMMARY Two patients with small intestinal lipomas treated at the Air Force Medical Center from November 2015 to September 2019 were selected to undergo balloonassisted ESD to treat the lipomas and explore the technical feasibility and safety of ESD for treating small intestinal lipomas.The two patients successfully underwent balloon-assisted ESD to treat four small intestinal lipomas,with a complete resection rate of 100%(4/4),without intraoperative or postoperative bleeding,perforation,or other complications.After 3-6 mo of postoperative follow-up,the clinical symptoms caused by the lipomas were significantly relieved or disappeared after treatment.CONCLUSION Balloon-assisted ESD is a safe and reliable new method for treating deep intestinal lipomas and shows good clinical feasibility.

Primary Squamous Cell Carcinoma of the Small Intestine： Pathogenesis and Clinical Features

Relative to the length and surface area of the gastrointestinal tract, malignant tumors of the small intestine are remarkably rare, with a global incidence of〈1 per 100,000 population. Among the forty different histological subtypes of small intestinal cancers, primary squamous cell carcinoma （SCC） is exceptionally rare with only occasional case reports in literature. The present study reported a case of primary SCC of the small intestine and reviewed all cases reported in English literature to provide a systematic overview of this rare disease.

Gastrointestinal neuroendocrine tumors in 2020

Gastrointestinal neuroendocrine tumors are rare slow-growing tumors with distinct histological,biological,and clinical characteristics that have increased in incidence and prevalence within the last few decades.They contain chromogranin A,synaptophysin and neuron-specific enolase which are necessary for making a diagnosis of neuroendocrine tumor.Ki-67 index and mitotic index correlate with cellular proliferation.Serum chromogranin A is the most commonly used biomarker to assess the bulk of disease and monitor treatment and is raised in both functioning and non-functioning neuroendocrine tumors.Most of the gastrointestinal neuroendocrine tumors are non-functional.World Health Organization updated the classification of neuroendocrine tumors in 2017 and renamed mixed adenoneuroendocrine carcinoma into mixed neuroendocrine neoplasm.Gastric neuroendocrine tumors arise from enterochromaffin like cells.They are classified into 4 types.Only type I and type II are gastrin dependent.Small intestinal neuroendocrine tumor is the most common small bowel malignancy.More than two-third of them occur in the terminal ileum within 60 cm of ileocecal valve.Patients with small intestinal neuroendrocrine tumors frequently show clinical symptoms and develop distant metastases more often than those with neuroendocrine tumors of other organs.Duodenal and jejunoileal neuroendocrine tumors are distinct biologically and clinically.Carcinoid syndrome generally occurs when jejuno-ileal neuroendocrine tumors metastasize to the liver.Appendiceal neuroendocrine tumors are generally detected after appendectomy.Colonic neuroendocrine tumors generally present as a large tumor with local or distant metastasis at the time of diagnosis.Rectal neuroendocrine tumors are increasingly being diagnosed since the implementation of screening colonoscopy in 2000.Gastrointestinal neuroendocrine tumors are diagnosed and staged by endoscopy with biopsy,endoscopic ultrasound,serology of biomarkers,imaging studies and functional somatostatin scans.Various treatment options are available for curative and palliative treatment of gastrointestinal neuroendocrine tumors.