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A combined approach to cytoreduction in a patient with a neuroendocrine tumor G1 the ileum with multiple bilobar metastases G2 in the liver, originally considered as a candidate only for drug therapy, has been demonstrated. The first stage was laparoscopic resection of the ileocecal segment of the intestine, followed by interventional radiological intervention — a two-stage selective transarterial oil chemoembolization of the liver with bleomycin. As a result, 90 % cytoreduction, suppression of clinical manifestations, normalization of chromogranin A and serotonin were achieved. The patient continues to be observed without signs of progression for more than 4 years from the moment of diagnosis. It is shown that multidisciplinary treatment of patient with timely inclusion of interventional-radiological interventions allows achieving long-term favorable results in patients with advanced stage of disease.

To determine the rate of missed CT findings of ileal carcinoid tumor prior to pathologic diagnosis and the resultant diagnostic delay. Initially, 74 patients with abdominal and pelvic CT prior to pathologically-proven diagnosis of ileal carcinoid were identified. Patients were excluded when the original CT study (n = 6) or report (n = 4) was not available, resulting in a final cohort of 64 patients (mean age, 58.3 years; 29 M/35F); 27 (42%) patients had more than one abdominal CT prior to diagnosis. All available CT studies prior to diagnosis were retrospectively reviewed for the presence of the primary ileal tumor and metastatic disease (mesenteric and hepatic). Primary ileal tumors were prospectively missed on at least one CT scan in 64% (32/50) of patients with retrospectively identifiable disease. CT findings of mesenteric spread were missed at least once in 46% (25/54) of cases where present in retrospect. By the final pre-operative CT, hepatic metastases and bowel wall thickening were present in 55% (35/64) and 52% (33/64) of cases, respectively. In patients with missed ileal and/or mesenteric findings resulting in diagnostic delay, mean delay was 40 months (range 4–98 months). Initial presentation of ileal carcinoid tumor, even with mesenteric involvement, is often missed prospectively at abdominal CT, leading to delay in diagnosis until bowel or mesenteric findings become more obvious, or hepatic metastatic disease manifests. Radiologists should make a concerted effort to evaluate the bowel and mesentery in patients with long-standing vague abdominal symptoms.

Cardiac metastases are uncommon in neuroendocrine tumors (NET). Features of patients with metastatic carcinoid tumor involving the heart have not been well described. Although carcinoid tumors have been described in almost every organ, few reported cases of confirmed cardiac involvement. The overall incidence of myocardial carcinoid metastases among patients with metastatic carcinoid disease is about 4%. There is limited knowledge on the prevalence, clinical presentation, and management of heart metastasis. The availability and increasing use of modern imaging techniques leads to more frequent discovery of rare metastatic sites. Our aim is to further clarify the clinical manifestations of heart metastasis, to increase the knowledge of rare localizations of NET metastasis and insist on the systematic screening of metastatic carcinoid heart disease as an integral part of the echocardiographic evaluation in patients with carcinoid syndrome. We report the case of a 67-year-old male with history of a low differentiated neuroendocrine tumor, presenting with palpitations and dyspnea and which investigations have led to diagnose myocardial metastasis signing the progression of the NETs.

Funding Acknowledgements none Background Right side heart valve involvement is a well known phenomenon in metastatic carcinoid disease. In recent times bioprosthetic valves have been widely used for replacement of stenotic and regurgitant native heart valves. Melody Transcatheter Pulmonary Valve (MTPV, Medtronic, Minneapolis, MN, USA) has been approved for treatment of native pulmonary valves as well as failing biological prostheses and conduits. We present the first case to our knowledge of severe native pulmonary valve steno-insufficiency due to metastatic carcinoid syndrome treated with MTPV implantation. Case report A 57-year-old woman is known since 2007 for metastatic carcinoid syndrome treated with surgical resection followed by chemotherapy (tamoxifene, everolimus) and radiotherapy. For 2 years she has been known for tricuspid and pulmonary involvement, which aggravated progressively, leading to symptomatic right heart failure. She was therefore submitted for multimodality imaging evaluation (see picture) and right heart catheterization. Transthoracic echocardiogram, cardiac MRI and right heart catheterization confirmed severe pulmonary valve steno-insufficiency. Cardiopulmonary exercise testing revealed a severe limitation of aerobic exercise capacity (VO2 max 14.4 ml/min/kg). After heart team discussion it was decided to treat the patient in a 2 step approach, initially with implantation of a Melody valve in pulmonary position and subsequently with surgical repair of the tricuspid valve. Pulmonary valve was treated by placement of a 39 x 25 mm ANDRA stent and subsequent implantation of a 22 mm Melody valve. Before stenting, coronary angiography with simultaneous balloon inflation in RVOT was performed to exclude coronary artery compression. During the procedure the patient developed hypotension, which was treated effectively with Octeotride. Post-operative echocardiogram showed a significant reduction in pulmonary valve gradient and no significant residual regurgitation. Conclusions We treated effectively severe pulmonary steno-insufficiency due to metastatic carcinoid tumor with MTPV implantation. No complications occurred. Multimodality imaging is fundamental for patients selection and preoperative planning of prosthetic valve dimensions. Abstract P641 Figure. Multimodality imaging of PV

This is the first report of laparoscopic-endoscopic cooperative surgery (LECS) for an ileal tumor. A 50-year-old man was admitted to our hospital with a positive fecal occult blood test. Colonoscopy detected a submucosal tumor with intussusception located in the ileum, 10 cm oral from the Bauhin valve. On further examination, he was diagnosed with an ileal lipoma. There were no signs of malignancy. LECS was performed for the ileal tumor. After submucosal elevation by injecting saline solution, a mucosal incision was made circumferentially along the tumor. A full-thickness incision was created endoscopically and laparoscopically on the circumferential mucosal incisional line. The tumor was withdrawn intraluminally by endoscopy. The defect of the ileal wall was closed laparoscopically in an axial direction with linear staplers. Histologically, the tumor was a 25-mm ileal lipoma with negative resection margins and no malignancy.

BACKGROUND/PURPOSE To report a unique case of a pulmonary carcinoid tumor unilaterally metastatic to the iris and ciliary body and bilaterally to the choroid that was conservatively followed. METHODS A 46-year-old woman presented with bilateral choroidal lesions and a left iris tumor. Ultrasound biomicroscopy disclosed a ciliary body component. A diagnosis of metastatic carcinoid tumor was made based on the clinical features. Rather than an excision, photodynamic therapy, or radiation treatment, as has been reported in all previous cases of carcinoid tumor metastatic to the iris, the patient was observed. RESULTS Excellent vision was maintained for 8 years. The iris tumor gradually enlarged, but the choroidal lesions remained unchanged. The iris with the carcinoid tumor gradually acquired a brown pigmentation; this is the first reported case of acquired iris heterochromia in the setting of carcinoid tumor. CONCLUSION We conclude, in cases of metastatic carcinoid in which visual acuity is excellent and the patient is asymptomatic, that observation of the ocular lesions is an acceptable course of action. The iris heterochromia is believed to have been caused by secretory factors produced by the tumor.

The breast is an unusual site for carcinoid metastasis. Due to increasing survival rates for carcinoid tumors, however, awareness of their rare complications is important. Carcinoid metastasis to the breast typically presents as a palpable breast mass or a mass on screening mammogram. Because imaging findings are nonspecific, the diagnosis is established through histological findings of neuroendocrine features corresponding with the known primary carcinoid pathology. Correctly distinguishing metastatic carcinoid from primary breast carcinoma is crucial to avoid more invasive procedures required for the latter. Two cases of metastatic carcinoid to the breast are presented with review of the literature.

Endoscopic ultrasonography with fine needle infusion (EUS-FNI) of alcohol is the most reported method for EUS-guided tumor ablation. Several studies have reported successful EUS-guided ablation of pancreatic neuroendocrine tumors. However, these tumors have been relatively small (

Consensus guidelines for the management of patients with digestive neuroendocrine tumors : well-differentiated jejunal-ileal tumor/carcinoma

Carcinoid tumors are indolent neoplasms derived from the enterochromaffin cells which have a wide anatomic distribution. Most common primary sites include gastrointestinal tract and bronchopulmonary system. Despite their slow growing nature, carcinoid tumors possess metastatic potential, and breast is a rare but known site of metastasis. We report a case of breast metastasis from carcinoid tumor of the terminal ileum in a 53 year old woman who initially presented with a breast mass detected by screening mammography. A review of literature was performed for this rare presentation of breast as the initial site of detection of metastatic carcinoid tumor.

Introduction:: A neuroendocrine tumor has known as a neuroendocrine system tumor. Rarely, neuroendocrines have found in other areas, like the liver, gallbladder, bile ducts, kidneys, ovaries or testicles. Case Presentation:: We have a 41-year-old woman has referred to our medical center, complaining of fullness and vague pain on her right upper quadrant. The liver scan, sonography, MRI demonstrated multi lobular cysts in 6th and 7 th seg-ments of her liver and chest imaging was normal, oc-terotid scan has not shwon metastatic neuroendocrine tour of liver. Conclusions:: Liver could be the location of metastatic neuroendo-crine tumors, for example metastatic carcinoid tumor. Therefore, it was so important to diffrentatiate pri-mary neuroendocrine tumor from metastatic neuro-endocrine tumors.

Octreotide is one of the somatostatin analogue used for the treatment of endocrine tumors principally to suppress hormone secretion and to inhibit tumor growth. We experienced a case with multiple endocrine neoplasia type 1 who small amount of octreotide dramatically relieved the lumber pain caused by metastatic bone tumor. He had recurrent bronchial carcinoid tumors that metastasized to liver and bones. The spontaneous and radiated pain by bone tumors subsided within a few minutes after the initial injection of octreotide and the effect persisted for several hours. Combination therapy of octreotide and interferon alpha-2b significantly reduced the size of metastatic liver tumors and inhibited further growth of metastatic bone tumors for the last 27 months. The use of octreotide may be a good option for controlling pain by metastatic bone disease and combination therapy of octreotide and interferon alpha-2b is worth to try for patients with inoperable metastatic carcinoid tumor.

OBJECTIVE To describe severe hypocalcemia and acute renal failure associated with zoledronic acid and interferon alfa in a patient with metastatic carcinoid tumors. CASE SUMMARY A 39-year-old white man with metastatic carcinoid tumor tolerated treatment with subcutaneous long-acting octreotide monthly and interferon alfa 6 million units 3 times weekly for 6 months. Due to multiple bony metastases, zoledronic acid was prescribed as a monthly 4-mg intravenous infusion over 30 minutes to prevent skeletal-related events. Although the first infusion went well, the patient developed severe hypocalcemia and acute renal failure after the second zoledronic infusion. DISCUSSION Bisphosphonates may infrequently cause symptomatic hypocalcemia, especially among patients who have vitamin D deficiency or hypoparathyroidism or receive treatment with an aminoglycoside. Our literature review suggests that zoledronic acid and interferon alfa may exert additive effects on the inhibition of osteoclasts, thus potentially precipitating hypocalcemia. Renal dysfunction may not be a direct consequence of interferon alfa. However, altered mental function due to hypocalcemia may lead to dehydration and further exacerbate renal dysfunction, a known adverse effect of zoledronic acid. Since therapeutic indications of both interferon alfa and zoledronic acid continue to expand, clinicians should be aware of these serious adverse reactions and potential interaction. Supportive treatment with hydration, calcium supplement, and oral calcitriol resulted in resolution of hypocalcemia, but only partial improvement of azotemia. CONCLUSIONS In our patient with metastatic carcinoid tumor, treatment with zoledronic acid and interferon alfa was associated with symptomatic hypocalcemia and acute renal failure.

While carcinoid tumor is a relatively common neoplasm in surgical pathology, fine-needle aspiration (FNA) cytology as a method of primary diagnosis has only been reported in the literature a few times. We report on the case of a 42-yr-old female with multiple large metastatic tumor deposits in her liver, pelvic adnexae, bones, and lungs, with an unknown primary. FNA was performed on one of the liver masses, and was diagnosed using routine histochemical and immunohistochemical stains as carcinoid tumor. No follow-up tissue diagnosis has been obtained. However, the patient is still alive with her tumor 1 yr later. Fine-needle aspiration cytology can be a useful and safe tool in the diagnosis of metastatic carcinoid tumors, avoiding the need for surgery which would not otherwise be indicated for treatment.

A 59-year-old man was admitted to the hospital because of melena and dizziness. Barium X-ray study of the small intestine revealed a filling defect in the ileum. Partial resection of the ileum was performed with a diagnosis of ileal tumor. The tumor was 1.8×1.5×1.8cm in size, and located on a site, 60cm oral to the Bauhin's valve. Histopathological diagnosis was leiomyoblastoma. Thereafter the patient had liver metastasis and peritoneal dissemination which were resected respectively, however, he died of metastases to the whole body 4 years and 2 months after the initial operation. Leiomyoblastoma of the small intestine is relatively rare and presents difficulty in diagnosis of the degree of malignancy of the tumor. Surgical resection of the tumor is said to carry a good prognosis. Nevertheless, some patients have a poor prognosis like this case and careful observation of clinical course would be mandatary.

BACKGROUND Pellagra is a rare clinical syndrome caused by niacin deficiency, and although most commonly nutritional in origin, may be secondary to carcinoid syndrome. CASE A premenopausal nulliparous woman with metastatic carcinoid tumor developed isolated vulvovaginal manifestations characterized by well-demarcated symmetrical vulvar erythema, mild scaling, ulcerations, and dyspareunia. Tryptophan levels were low. Treatment with nicotinamide resulted in dramatic improvement; however, after 2 years of effective treatment, she developed lichen sclerosus. CONCLUSION Patients with carcinoid tumor presenting chronic vulvar lesions should be evaluated for pellagra. Subsequent development of lichen sclerosus at the site of vulvar dermatosis may be a feature of the "Koebner phenomenon," which describes appearance of lichen sclerosus in scarred areas or sites prone to friction.

A 77-year-old woman was admitted to our hospital with anemia and anorexia. Neither gastrointestinal endoscopy nor computed tomography showed by abnormal findings; however, a capsule endoscopy, performed to detect obscure gastrointestinal bleeding, revealed a tumor in the ileum. When we tried to take biopsies of the ileal tumor by push enteroscopy via the anus, we found another tumor in the transverse colon. On exploration, tumors were identified in the ileum and the transverse colon. Thus, we resected the ileum and transverse colon with regional lymph node dissection. Histologic sections from both the ileum and colon revealed moderately differentiated adenocarcinomas with no lymph node metastasis.

Carcinoid tumors are neoplasms that are seen in all portions of the gastrointestinal tract. In some instances, primary tumors can go undiagnosed or misdiagnosed owing to nonspecific presenting symptoms. In this case report we discuss a patient with vague abdominal symptoms and radiographic findings previously attributed to Crohn's disease, who at operative exploration was found to have a metastatic carcinoid tumor. This report demonstrates the nonspecific nature of metastatic carcinoid tumor and how its presentation can masquerade as more common gastrointestinal system abnormalities.

Radiation recall is a rare side effect observed in patients treated with certain medications after radiation therapy. This effect mimics a radiation-induced tissue reaction in expression; however, it occurs outside of the traditional time course and only in the presence of a catalyzing agent. The authors report a case of radiation recall resulting from an interaction between radiation delivered during a fluoroscopically guided hepatic chemoembolization for treatment of metastatic carcinoid tumor and the oral chemotherapeutic agents capecitabine and temozolomide administered 7 weeks later.

A 64-year-old woman who had been diagnosed with carcinoid tumor of the large intestine over a decade earlier presented with an acute right periorbital inflammatory reaction. On examination, 2 disc-shaped, gelatinous masses were noted on the right upper and lower tarsal conjunctivae. Excisional biopsies of these masses revealed metastatic carcinoid tumor. Her total burden of metastatic carcinoid tumor continued to increase during 2 years of follow-up, with new metastatic foci in the right choroid, in the left upper and lower eyelids, and subcutaneously in the neck and chest. Despite a comprehensive treatment approach including radionuclide therapy with the somatostatin peptide analog, 90Y-DOTATOC, the patient succumbed to the disease and died. The authors report an unusual case of a gastrointestinal carcinoid tumor metastatic to the tarsal conjunctivae, eyelids, choroid, and orbit.

The case of a 39-year-old patient with ectopic Cushing's syndrome due to a metastatic carcinoid tumor is presented. Palliative therapy consisting of 800 mg ketoconazole and 0.3 mg SMS 201-995/die resulted in clinical remission and correction of hypokalemia and hypercortisoluria. Combined therapy was clearly superior to monotherapy with ketoconazole or SMS 201-995, respectively. Side effects were not observed, the tumor masses remained unchanged throughout the observation period of now 19 months.

A case of leiomyoblastoma arising in the ileum is reported, together with a review of the literature.A 69-year-old woman was hospitalized with the chief compliant of general fatigue. Presence of anemia and melena suggested gastrointestinal hemorrhage, but routine examinations failed to identifity the bleeding source. Abdominal scintigraphy with Tc-99m-labeled red blood cell, small bowel enteroclysis and superior mesenteric angiography revealed a ileal tumor which may be originated from the smooth muscle. Segmental ileectomy was carried out to extirepate the tumor. Histologically it was a very rare leiomyoblastoma.

PURPOSE The purpose of this study was to assess the efficacy and safety of intraoperative enteroscopy (IOE) in patients undergoing minimally invasive surgery. METHODS Twelve patients underwent minimally invasive surgery and IOE at Miyazaki University Hospital. Patients included 11 men and 1 woman. After extraction of the intestine via minilaparotomy, enterotomy was performed, and a sterilized enteroscope was inserted. RESULTS Length of the skin incision was 5.7+/-0.2 cm (mean+/-standard error). Length of the small intestine observed enteroscopically was 334+/-19 cm. Distance from the ligament of Treitz to the orally observed jejunum was 11.8+/-3.6 cm. In 5 of 9 patients with Crohn disease, additional lesions were found by IOE that were not found by preoperative examination. One additional tumor was found in 1 patient with ileal tumor. Postoperative complications occurred in 2 patients. CONCLUSION IOE is efficacious in patients undergoing minimally invasive surgery.

Carcinoid tumor of the testis represents only 0.23% of all testicular malignancies. We report the first case of bilateral asynchronous carcinoid tumor of the testis with a primary site in the small bowel. A 49-year-old man presented with several months of painless enlargement of the left testis. Orchiectomy revealed carcinoid tumor. The patient presented to the emergency room 2 weeks later and was found to have small bowel obstruction due to an ileal mass. The resected mass was the primary source of the carcinoid tumor. Four years later, examination revealed carcinoid tumor in his remaining testis.

Context.—Carcinoid tumor metastatic to the breast is uncommon and can closely mimic a mammary carcinoma. The differentiation of metastatic carcinoid tumor from primary breast tumor is important, however, owing to different clinical management and prognosis. Objective.—The purpose of this study was to describe 2 patients with bilateral metastatic carcinoid tumors to the breast with different clinical manifestations. Design.—We examined the radiological, clinical, cytologic, histologic, immunohistochemical, and ultrastructural features of these 2 cases. Results.—In case 1, the tumor presented initially as a stellate mass on mammogram and was diagnosed as grade II infiltrating ductal carcinoma. It was only after the discovery of small intestinal, liver, ovarian, and contralateral breast masses, as well as careful morphologic and immunohistochemical evaluations, that the true nature of the tumor was realized. In case 2, the tumor initially presented as a small intestinal tumor with liver metastases and bilateral breast masses. The breast masses were diagnosed accurately as metastatic carcinoid tumor by morphologic and immunohistochemical evaluations. Conclusions.—Metastatic carcinoid tumor to the breast is uncommon, but poses a diagnostic challenge in that morphologically it can closely mimic a primary breast tumor. Careful attention to clinical features and the use of auxiliary immunohistochemical studies can help in arriving at the correct diagnosis.

Primary ovarian carcinoid tumors are uncommon, and carcinoid heart disease is a rare complication. Although carcinoid syndrome and carcinoid heart disease typically occur in the setting of metastatic carcinoid tumor, particularly involving the liver, this is not necessarily the case in patients with primary ovarian carcinoid tumors. After surgical resection of an ovarian carcinoid tumor, the prognosis is excellent; however, carcinoid heart disease can continue to progress. The following is a case report of a patient who, despite having complete resection of a primary ovarian carcinoid tumor, went on to develop progressive, debilitating carcinoid heart disease. This is an important scenario to recognize, because proper management and surgical intervention in carcinoid heart disease can be lifesaving.

This brief report describes an asymptomatic patient with a myocardial mass. Two-dimensional echocardiography, technetium Tc 99m cardiac nuclear scan, and transesophageal echocardiography were performed to define the mass. The mass, which involved the subvalvar right ventricular free wall, was resected and determined to be a metastatic carcinoid tumor by histologic and immunohistochemical analysis. Further investigation revealed the presence of a midgut carcinoid tumor located within the terminal ileum, which was also resected surgically. The patient recovered well after surgery and adjunctive chemotherapy. To our knowledge, this is the first report of comprehensive nuclear and echocardiographic imaging, supplemented by surgical and pathologic findings, in an asymptomatic patient with isolated myocardial metastasis of an ileal carcinoid tumor.

IMPLICATIONS We report a case of prolonged radiofrequency liver ablation for metastatic carcinoid tumor complicated by hemolysis, rhabdomyolysis, and transient acute renal failure. Brief radiofrequency liver ablation procedures or those for a small number of tumor sites are not associated with these complications.

Carcinoid tumors are rare, slow-growing neuroendocrine neoplasms that can cause a carcinoid syndrome. The majority of carcinoid syndromes are the result of multiple hepatic metastases and are usually unresectable. Medical therapy has not proven effective and often causes intolerable side effects. Cryoablation has emerged as a promising treatment for various hepatic lesions when resection is not an option and medical therapy has been exhausted. With the addition of laparoscopy, surgeons now possess a new technique to treat primary and various secondary liver lesions. We report for the first time laparoscopic cryoablation for the treatment of a carcinoid metastatic to the liver.

A 76-year-old patient with metastatic carcinoid tumor who had difficulty tolerating an oral diet and jejunostomy feedings became dehydrated and failed to thrive. His jejunostomy feeding regimen was manipulated to improve tolerance without success. Although his medical condition was untreatable, he was not considered terminally ill, and he was determined to be a reasonable candidate for home total parenteral nutrition (HTPN). However, he thought HTPN was an "excessive measure," and despite lengthy discussions with him by members of both the Hematology/Oncology and Nutrition Support services about the potential benefits of HTPN, he refused any further interventions involving nonvolitional nutrition support (jejunostomy feedings and HTPN). He was discharged to home receiving an oral diet as tolerated in the care of his family and hospice. This case illustrates the ethical considerations surrounding a patient's decision to forgo medical care that might prolong life.