Optimal Lymphadenectomy in Small Bowel Neuroendocrine Tumors: Analysis of the NCDB.

BACKGROUND: Current National Comprehensive Cancer Network guidelines for resectable small bowel neuroendocrine tumors (NETs) recommend regional lymphadenectomy. However, no consensus exists on the optimal nodal harvest. METHODS: The National Cancer Database was queried for patients with resectable small bowel NETs (1998-2013). Patients with metastatic disease and missing lymph node harvest data were excluded. We performed logistic regression of factors determining nodal positivity and multivariable survival analyses. RESULTS: Of 11,852 patients, 81.8% underwent lymphadenectomy. 79.3% were node positive (N+) and 46.9% of patients had tumors < 1 cm. Independent predictors of N+ were large tumor size, ileal location, and neuroendocrine carcinoma histology. Logistic regression found no difference between observed and expected proportions of N+ patients with lymphadenectomy greater than or equal to eight nodes. Lower metastatic node ratio predicted improved survival on multivariable analysis and is associated with high-volume institutions. CONCLUSION: Small bowel NETs have high rates of nodal metastasis, even in patients with small tumors, and many patients do not undergo lymphadenectomy despite the clear benefit. Lymphadenectomy of eight nodes is optimal to identify N+ patients. Additionally, minimizing metastatic node ratio with complete regional lymphadenectomy is associated with improved survival in these patients.

Iron deficiency anaemia in a coeliac: a cause for concern?

A 70-year-old woman with a 15-year history of coeliac disease was admitted for treatment and investigation of symptomatic anaemia. Of note, she was recently commenced on aspirin therapy for retinal artery occlusion. This followed a normal gastro-duodenoscopy, colonoscopy and CT abdomen as workup for iron deficiency anaemia. On this occasion, the patient was further investigated with small bowel capsule endoscopy. This revealed an ulcerated lesion in her proximal jejunum suspicious for malignancy, namely lymphoma. A biopsy of the lesion confirmed adenomatous changes and high-grade dysplasia without malignant changes. Given the endoscopic appearances of the lesion, she was further evaluated with a small bowel MRI. This revealed a 3 cm lesion with associated lymphadenopathy but no distant metastases. She proceeded to resection of her small bowel which confirmed an invasive adenocarcinoma of her proximal jejunum. She is currently undergoing adjuvant chemotherapy on an outpatient basis.

Small bowel adenocarcinoma of the jejunum: a case report and literature review.

BACKGROUND: In practice, small bowel cancer is a rare entity. The most common histologic subtype is adenocarcinoma. Adenocarcinoma of the small bowel (SBA) is challenging to diagnose, often presents at a late stage and has a poor prognosis. The treatment of early-stage SBA is surgical resection. No standard protocol has been established for unresectable or metastatic disease. CASE PRESENTATION: We report here on a 26-year-old man with SBA in the jejunum, lacking specific symptoms and with a delay of 6 months in diagnosis. The diagnosis was finally achieved with a combination of balloon-assisted enteroscopy, computed tomography scans, positron emission computed tomography scans and the values of carcino-embryonic antigen and carbohydrate antigen 19-9. The patient underwent segmental intestine with lymph node resection, followed by eight cycles of FOLFOX palliative chemotherapy with good tolerance. As of the 11-month postoperative follow-up, there has been no evidence of recurrent disease. CONCLUSIONS: This case is reported to arouse a clinical suspicion of SBA in patients with abdominal pain of unknown cause. We also provided evidence in this case of a response to palliative chemotherapy with FOLFOX. Because the incidence of SBA is very low, there is a need for further studies to evaluate the possible application of newer investigative agents and strategies to obtain a better outcome within the framework of international collaborations.

[Recurrence of Jejunal Carcinoma in the Ileum and Ovary - A Case Report].

Small bowel carcinoma has poor prognosis. The basis of treatment is surgical resection. There are no established guidelines for chemotherapy. We report a case in which we performed surgical resection of recurrent jejunal carcinoma. A 62-year-old woman underwent laparoscopic partial resection of the small intestine for primary jejunal carcinoma. The final diagnosis was T3N0M0, fStage II A. After 16 months of follow-up, she developed abdominal pain and vomiting. We diagnosed recurrence of jejunal carcinoma in the ileum and right ovary. Single-port laparoscopic small intestinal resection and right ovariectomy were performed. The patient underwent curative resection for recurrent lesions. The type of tumor in the ileum and right ovary was consistent with primary jejunal carcinoma by histopathological examination, and was diagnosed as recurrence of jejunal carcinoma. She is now on adjuvant chemotherapy with XELOX.

[A Case of Small Bowel Adenocarcinoma That Was Diagnosed and Treated using Laparoscopic Surgery].

A 40-year-old man presented to our department with chief complaints of nausea and abdominal pain, and was diagnosed with small intestine ileus. After hospitalization, he underwent intestinal tract decompression using an ileus tube. A small bowel tumor was suspected as the cause of the intestinal obstruction. We then performed laparoscopic surgery for diagnosis and resection. In the intraoperative findings, stenosis near the small intestine tumor could be confirmed. The patient therefore underwent laparoscopic resection of a segment of the small intestine. Following rapid intraoperative pathological examination, the tumor was identified as well-differentiated adenocarcinoma with metastasis of the intermediate mesenteric lymph nodes. We then performed dissection of the main lymph nodes using small laparotomy incisions. Adjuvant chemotherapy with XELOX(130mg/m2 L-OHP on day 1 and 2,000 mg/m2 capecitabine on days 1-14)was administered for 6 months. Currently the patient is in relapse-free survival.

Risk factors for surgery in pediatric intussusception in the era of pneumatic reduction.

INTRODUCTION: Surgical treatment is still necessary for intussusception management in a subgroup of patients, despite advances in enema reduction techniques. Early identification of these patients should improve outcomes. METHODS: The medical records of patients treated for intussusception at our institution from 2006 to 2011 were reviewed. Univariate and multivariate analyses, including stepwise logistic regression, were performed. RESULTS: Overall, 379 patients were treated for intussusception, and 101 (26%) patients required operative management, with 34 undergoing intestinal resection. The post-operative complication rate was 8%. On multivariate analysis, failure of initial reduction (OR 9.9,p=0.001 95% CI, 4.6-21.2), a lead point (OR 18.5,p=0.001 95% CI, 6.6-51.8) or free/interloop fluid (OR 3.3,p=0.001 95% CI, 1.6-6.7) or bowel wall thickening on ultrasound (OR 3.3,p=0.001 95% CI, 1.1-10.1), age <1 year at reduction (OR 2.7,p=0.004, 95% CI, 1.4-5.9), and abdominal symptoms>2 days (OR 2.9,p=0.003, 95% CI, 1.4-5.9) were significantly associated with a requirement for surgery. Similarly, a lead point (OR 14.5, p=0.005 95% CI, 2.3-90.9) or free/interloop fluid on ultrasound (OR 19.8, p=0.001 95% CI, 3.4-117) and fever (OR 7.2, p=0.023 95% CI, 1.1-46) were significantly associated with the need for intestinal resection. CONCLUSION: Abdominal symptoms>2 days, age<1 year, multiple ultrasound findings, and failure of initial enema reduction are significant predictors of operative treatment for intussusception. Patients with these findings should be considered for early surgical consultation or transfer to a hospital with pediatric surgical capabilities.

[Exceptional bilocular inflammatory myofibroblastic pseudotumour (IMT) characterized by recurrent thoracic tumour growth and a rare (adult) second manifestation within the mesenteric tissue of the small intestine]. / Ungewöhnlicher bilokulärer inflammatorischer myofibroblastärer Pseudotumor (IMT), charakterisiert durch thorakales Rezidiv und seltene, da adulte, Zweitmanifestation im Dünndarmmesenterium.

BACKGROUND: Despite its rare occurrence, inflammatory myofibroblastic pseudotumour (IMT) is relevant in the differential diagnosis of intestinal lesions. By the mean of an extraordinary case report, tumour site and specific characteristics, finding of the correct diagnosis, therapeutic management, and outcome of extrapulmonary IMT is decribed based also on relevant references from the literature. CASE REPORT: A 39-year old man experienced a multifocal thoracic recurrence and abdominal metastasis of IMT 12 years after successful primary resection of pulmonary IMT. The intra-abdominal lesion localised in the jejunal mesenteric tissue was removed surgically (resection status, R 0) by segmental resection of the mid-jejunum (length: 80 cm) followed by jejunojejunostomy. Histology evaluation confirmed IMT. Thoracic surgeons advised against a surgical approach to the pulmonary and thoracic lesions because of their number and proximity to the superior vena cava as well as mediastinal infiltration. Despite receiving repeated advice from his physicians, the patient has not agreed to combined immunosuppressive treatment with cyclophosphamide and steroids, because of his desire for children. He underwent 5 months of systemic steroid treatment, starting in the third postoperative month, which he then chose to stop because of Cushing symptomatology. He agreed to a computed tomography (CT) scan follow-up 12 months after surgery, which revealed slight local progression of the remaining pulmonary lesion. Administration of a second steroid medication was initiated at a lower dose. No further CT scans were obtained. At present, he is consulting with an alternative medicine practitioner. CONCLUSION: This report documents a rarely described case of IMT at a jejunal mesenteric tumour site, interpreted as an uncommon late and extraordinary, metastatic, multifocal recurrence found 12 years (!) after surgical resection of the primary pulmonary tumour.

Oxaliplatin/5-fluorouracil/leucovorin (FOLFOX4) regimen as an adjuvant chemotherapy in the treatment of advanced jejunal adenocarcinoma: a report of 2 cases.

OBJECTIVE: To present our clinical experience of 5-fluorouracil/leucovorin/oxaliplatin (FOLFOX4) regimen administered as an adjuvant chemotherapy to 2 patients with advanced jejunal adenocarcinoma. CASE PRESENTATION AND INTERVENTION: A 55-year-old woman presented with recurrent upper abdominal pain, nausea and vomiting. A small bowel series as well as the abdominal computed tomography scan revealed an irregular narrowing lesion at the proximal jejunum. The patient then underwent an exploratory laparotomy and the jejunal adenocarcinoma with localized peritoneal metastasis was found (R0 resection, T3N1M1, stage IV). Chemotherapy with FOLFOX4 regimen of 12 cycles was initiated after the curative resection. No adverse event was observed during the period of chemotherapy. She has been well without evidence of recurrence for over 20 months postoperatively. The second case was a 77-year-old female presenting with mechanical ileus. Surgical exploration revealed a proximal jejunal adenocarcinoma with regional lymph node involvement (R0 resection, T3N1M0, stage III). She also received the FOLFOX4 chemotherapy of 12 cycles with an uneventful course. No obvious toxicity developed except for temporary grade I peripheral neuropathy and skin eruption. This patient has survived well and has been free of this disease for over 12 months since the operation. CONCLUSION: This report showed that adjuvant chemotherapy with FOLFOX4 regimen seems effective and well tolerated in these 2 patients with advanced jejunal adenocarcinoma. Further investigation of a large number of patients with long-term follow-up is needed to confirm these findings.

Imaging of a small bowel cavernous hemangioma: report of a case with emphasis on the use of computed tomography and enteroclysis.

Hemangiomas of the small bowel are rare benign tumors, that are dangerous since they may cause massive or occult gastrointestinal bleeding. We describe a case of a jejunum cavernous hemangioma detected by computed tomography (CT) and barium studies. An abdominal CT scan (with intravenous contrast agent) depicted a pronounced contrast enhanced lesion arising from the front wall of a loop of the proximal ileum. Enteroclysis revealed a small intramural nodular defect.

Cytoreductive surgery and intraperitoneal hyperthermic chemotherapy for peritoneal carcinomatosis from small bowel adenocarcinoma.

INTRODUCTION: Peritoneal carcinomatosis (PC) is associated with a dismal prognosis. Small bowel adenocarcinoma is a rare etiology for PC. Due to the rarity, poor prognosis, and lack of standard treatment, we chose to review our experience with this disease process treated with cytoreductive surgery (CS) and intraperitoneal hyperthermic chemotherapy (IPHC). METHODS: From a prospective database of IPHC patients, six patients diagnosed with PC from adenocarcinoma of the small bowel were identified. Between 1995 and 2004 these patients underwent CS and IPHC with Mitomycin C. A retrospective review was performed on these patients with mortality as the primary outcome measure. RESULTS: Three of the six patients in this series are still alive, with a mean follow-up of 19.7 months after treatment with CS and IPHC. Three patients died of disease progression 29, 30, and 45 months after IPHC. Median survival after diagnosis of small bowel adenocarcinoma was 54 months, while median survival after CS and IPHC for PC was 30.1 months. CONCLUSIONS: Small bowel adenocarcinoma with PC remains an unusual therapeutic challenge. Treatment with CS and IPHC is an attractive option for patients in this setting.

[Small intestinal polyposis in Peutz-Jeghers syndrome: combined treatment with surgery and intra-operative endoscopy]. / La poliposi del tenue nella sindrome di Peutz-Jeghers: trattamento combinato chirurgia-endoscopia intraoperatoria.

Polyps occur throughout the gastrointestinal tract in Peutz-Jeghers syndrome, but the most serious problems are encountered in the management of small bowel polyposis. We report here on a case of Peutz-Jeghers syndrome admitted to hospital for intestinal obstruction and anaemia. The patient was submitted to colonoscopy, oesophagogastro-duodenoscopy and small bowel enema. At laparotomy, multiple intussusceptions were found and we conducted a combined surgical-endoscopic approach. Most of the polyps were identified and removed endoscopically (snare polypectomy). Five enterotomies were performed to remove 18 very large polyps (> 3 cm). Finally, a limited portion of the jejunal tract (20 cm) was resected owing to the presence of multiple, large, obstructive polyps. None of the polyps showed cancerous transformation. The shortcomings of the traditional surgical approach include repeated small bowel resections and often early reoperation to manage complications caused by polyps missed at the time of previous surgery. If surgical intervention is required, intraoperative endoscopy is always indicated. Conservative surgical management, the role of intraoperative endoscopy, planned medical follow-up and the need for a national registration system are stressed.

Laparoscopic-assisted resection of bleeding jejunal leiomyoma.

Laparoscopic-assisted resection has shown remarkable improvements in the treatment of small bowel diseases, notably the leiomyomas. This case report documents the successful removal of a bleeding jejunal leiomyoma with the aid of laparoscopy. A 51-year-old man was admitted to the hospital twice within 3 months with melena. On the first admission, upper and lower gastrointestinal endoscopy were negative, and small bowel enema was inconclusive. On the second admission, Technetium-99 Red Blood Cells (Tm-99 RBC) scan showed dye extravasation (interpreted as from the left colon). Subsequent colonoscopy was normal, as was a barium enema. An elective superior mesenteric angiography revealed a benign-looking jejunal leiomyoma. Subsequent CT (intravenous contrast scan) revealed a 4.6 x 3.5 cm mass with neither extraluminal infiltration nor enlarged lymph nodes. The patient underwent successful laparoscopic-assisted resection of the leiomyoma and enjoyed an uneventful postoperative recovery. Subsequent histopathology confirmed the diagnosis. The diagnosis of small bowel leiomyoma is generally difficult because the conventional radiographic methods are unhelpful. These tumors are therefore best detected preoperatively with superior mesenteric angiography. Repeat of the noninvasive tests is strongly indicated if they are initially negative. Once discovered, small bowel leiomyoma should be resected to avoid catastrophic complications. Thereafter, it has an excellent prognosis. In this context, minimal-access surgery is a safe and approachable method to deal with such a problem.

Jejunal adenocarcinoma presenting as iron deficiency in a young man.

A 30-year-old man presented with iron deficiency anemia and occult blood in his stools. Both upper and lower gastrointestinal tract endoscopy were initially unrevealing. This case illustrates the importance of small bowel follow through/enteroclysis supplemented by endoscopic examination and biopsy for adenocarcinoma of the proximal jejunum.

Metastatic large-cell lung carcinoma presenting as gastrointestinal hemorrhage.

A rare case of severe small bowel hemorrhage due to jejunal metastases from a large-cell type carcinoma of the lung is reported. A 69-yr-old Japanese woman presented with complaints of mild abdominal pain and liquid tarry stools 6 months following surgery for lung carcinoma. Gastroduodenoscopy and barium enema yielded unremarkable findings, although a subsequent small bowel enema revealed a large, 15-cm ulcerated mass in the jejunum. This tumor was resected and histology confirmed to be consistent with a metastasis from the primary undifferentiated large-cell carcinoma of the lung. The patient had an uneventful postoperative course and survived for 9 months. There have been only two prior case reports of major intestinal hemorrhage secondary to pulmonary carcinoma metastases in the English literature. Previous reports of such metastases of the small bowel have bowel have documented a very poor prognosis and our patient demonstrated the longest survival period to date. The clinical course of this patient suggests that the early diagnosis and palliative surgery for this complication provide a more favourable outcome.

Jejunojejunal intussusception secondary to leiomyoma.

A case of jejunojejunal intussusception in a 59 year old male secondary to a leiomyoma is reported. The patient presented with abdominal pain and vomiting. He was admitted with a diagnosis of gastroenteritis. Antegrade barium study showed a jejunojejunal intussusception with a soft tissue mass as the lead point. Computed tomography scan demonstrated the soft tissue mass to have properties suggestive of a leiomyoma. The diagnosis was confirmed on examination of the excised specimen.

[Digestive hemorrhage caused by jejunoileal tumors]. / Hemorragia digestiva por tumoraciones yeyunoileales.

Between January-73 and February-92 twelve patients with digestive hemorrhage due to primary jejunoileal tumors were treated. Eight cases had rectal bleeding and four chronic gastrointestinal hemorrhage as first clinical manifestation. Patients underwent upper endoscopy, colonoscopy and barium enema. Three out of nine barium meals (33%), two out of four ultrasonographies (50%) and eight out of nine arteriographies (89%) were abnormal. The barium meals showed submucosal lesions on two occasions and a jejunal luminal mass. The ultrasonography detected two intrabdominal masses. All arteriographies, except one with extravased intestinal contrast and other which showed a hypovascular zone, depicted homogeneous hypervascular images. All patients were operated on. Seven segmental enterectomies, four limited resections and a polipectomy were performed. Seven tumours were found in the jejunum, three in the jejunoleum and two in the ileum. There were four leiomyosarcomas, three leiomyomas, two polyps, one leiomyoblastoma, one adenocarcinoma and one lymphoma. Rebleeding and mortality were absent.

Obstructive jejunal adenocarcinoma in the Muir-Torre syndrome.

A young male patient was referred for endoscopic resection of a recto-sigmoid polyp and abdominal complaints. His medical history revealed a sebaceous adenoma resection from his back and a tubular adenoma excision from his right upper eyelid. After disclosure of a jejunal tumour on a small bowel enema and given the remarkable family history characterized by the frequent occurrence of bowel cancer, the diagnosis of Muir-Torre syndrome was established. This syndrome pertains to the combination of sebaceous gland tumours/adenomas in combination with gastrointestinal or genitourinary tract tumours. Inheritance takes place in an autosomal dominant manner. It usually occurs in males at an early age. Given its low malignancy potential, early detection of the syndrome renders a favourable prognosis. Careful examination and follow-up also involving the small intestine are mandatory for patients and relatives.

[Primary carcinoma of the jejunum combined with colon polyp--report of a case].

A 47-year-old female patient with a primary carcinoma of the jejunum combined with colon polyp is presented. Having had an attack of vomiting, she visited our hospital. Oral small intestine radiography revealed a localized stricture with ulceration in the jejunum. Small intestinal endoscopy showed a tumor with an irregular surface and hemorrhagic ulceration. Selective superior mesenteric arteriography revealed a stenosis and deviation of arteries. These findings strongly indicated cancer of the jejunum, and a barium enema study and a colonoscopic examination revealed a polyp in the ascending colon. The resected tumor was histologically diagnosed as a well differentiated adenocarcinoma. CEA-positive cells also were observed. Further, a polypoid lesion in the colon was histologically diagnosed as an adenoma.