Managing end-stage carcinoid heart disease:A case report and literature review

BACKGROUND Gastroenteropancreatic neuroendocrine neoplasms(GEP-NENs)are rare tumors,often diagnosed in an advanced stage when curative treatment is impossible and grueling symptoms related to vasoactive substance release by tumor cells affect patients’quality of life.Cardiovascular complications of GEP-NENs,primarily tricuspid and pulmonary valve disease,and right-sided heart failure,are the leading cause of death,even compared to metastatic disease.CASE SUMMARY We present a case of a 35-year-old patient with progressive dyspnea,back pain,polyneuropathic leg pain,and nocturnal diarrhea lasting for a decade before the diagnosis of neuroendocrine carcinoma of unknown primary with extensive liver metastases.During the initial presentation,serum biomarkers were not evaluated,and the patient received five cycles of doxorubicin,which he did not tolerate well,so he refused further therapy and was lost to follow-up.After 10 years,he presented to the emergency room with signs and symptoms of right-sided heart failure.Panneuroendocrine markers,serum chromogranin A,and urinary 5-hydroxyindoleacetic acid were extremely elevated(900 ng/mL and 2178µmol/L),and transabdominal ultrasound confirmed hepatic metastases.Computed tomo-graphy(CT)showed liver metastases up to 6 cm in diameter and metastases in mesenteric lymph nodes and pelvis.Furthermore,an Octreoscan showed lesions in the heart,thoracic spine,duodenum,and ascendent colon.A standard trans-thoracic echocardiogram confirmed findings of carcinoid heart disease.The patient was not a candidate for valve replacement.He started octreotide acetate treatment,and the dose escalated to 80 mg IM monthly.Although biochemical CONCLUSION Carcinoid heart disease occurs with carcinoid syndrome related to advanced neuroendocrine tumors,usually with liver metastases,which manifests as right-sided heart valve dysfunction leading to right-sided heart failure.Carcinoid heart disease and tumor burden are major prognostic factors of poor survival.Therefore,they must be actively sought by available biochemical markers and imaging techniques.Moreover,imaging techniques aiding tumor detection and staging,somatostatin receptor positron emission tomography/CT,and CT or magnetic resonance imaging,should be performed at the time of diagnosis and in 3-to 6-mo intervals to determine tumor growth rate and assess the possibility of locoregional therapy and/or palliative surgery.Valve replacement at the onset of symptoms or right ventricular dysfunction may be considered,while any delay can worsen right-sided ventricular failure.

Goblet cell carcinoid of the appendix:Six case reports

BACKGROUND Goblet cell carcinoid(GCC)of the appendix is a rare tumor characterized by neuroendocrine and adenocarcinoma features.Accurate preoperative diagnosis is very difficult,with most patients complaining mainly of abdominal pain.Computed tomography shows swelling of the appendix,so diagnosis is usually made incidentally after appendectomy based on a preoperative diagnosis of appendicitis.Even if a patient undergoes preoperative colonoscopy,accurate endoscopic diagnosis is very difficult because GCC shows a submucosal growth pattern with invasion of the appendiceal wall.CASE SUMMARY Between 2017 and 2022,6 patients with GCC were treated in our hospital.The presenting complaint for 5 of these 6 patients was abdominal pain.All 5 patients underwent appendectomy,including 4 for a preoperative diagnosis of appendicitis and the other for diagnosis and treatment of an appendiceal tumor.The sixth patient presented with vomiting and underwent ileocecal resection for GCC diagnosed from preoperative biopsy.Although 2 patients with GCC underwent colonoscopy,no neoplastic changes were identified.Two of the six patients showed lymph node metastasis on pathological examination.As of the last followup(median:15 mo),all cases remained alive without recurrence.CONCLUSION As preoperative diagnosis of GCC is difficult,this possibility must be considered during surgical treatments for presumptive appendicitis.

Thymic carcinoid with multiple bone metastases:A case report

BACKGROUND Thymic carcinoid(TC)is a rare entity among anterior mediastinal malignancies.TCs are neuroendocrine carcinomas that constitute approximately 2%–5%of all thymic epithelial tumors.CASE SUMMARY The study reported a rare TC with multiple bone metastases.A 77-year-old man presented with a 2-month history of lower back pain and weight loss of 5 kg.Magnetic resonance imaging scans revealed damage to the lumbar spine,sacrocaudal vertebrae and iliac crest,suggesting bone metastasis;computed tomography(CT)scan of the thorax showed a calcified anterior mediastinal mass;positron emission tomography-CT demonstrated multiple abnormal bone signals;and laboratory work-up showed no endocrine abnormalities.Fine-needle aspiration biopsy revealed predominantly single small,round to oval cells with scant cytoplasm and some loose clusters,suggesting endocrine manifestations.The pathological diagnosis was atypical carcinoid,which tend to originate from the thymus and was classified as intermediate-highly invasive.The patient underwent anlotinib-targeted therapy.Anlotinib(12 mg)was administered daily for 2 wk,after which the patient was allowed to rest for 21 d.Follow-up CT after one year demonstrated that the tumor had shrunk by approximately 29%after therapy.Treatment has a long stable disease benefit of more than 2.5 years.CONCLUSION These findings demonstrated that anlotinib is a promising treatment regimen for patients with TC and multiple bone metastases.

Carcinoid syndrome caused by a pulmonary carcinoid mimics intestinal manifestations of ulcerative colitis:A case report

BACKGROUND Pulmonary carcinoids are rare,low-grade malignant tumors characterized by neuroendocrine differentiation and relatively indolent clinical behavior.Most cases present as a slow-growing polypoidal mass in the major bronchi leading to hemoptysis and pulmonary infection due to blockage of the distal bronchi.Carcinoid syndrome is a paraneoplastic syndrome caused by the systemic release of vasoactive substances that presents in 5%of patients with neuroendocrine tumors.Due to such nonspecific presentation,most patients are misdiagnosed or diagnosed late and may receive several courses of antibiotics to treat recurrent pneumonia before the tumor is diagnosed.CASE SUMMARY We report the case of a 48-year-old male who presented with cough,dyspnea,a history of recurrent pneumonitis,and therapy-refractory ulcerative colitis that completely subsided after the resection of a pulmonary carcinoid.CONCLUSION We report and emphasize pulmonary carcinoid as a differential diagnosis in patients with nonresponding inflammatory bowel diseases and recurrent pneumonia.

Iridociliary Body Metastasis of Atypical Carcinoid: Case Management with Intravitreal Anti-Vascular Endothelial Growth Factor Injections

We report a rare case involving a 52-year-old female diagnosed with an atypical bronchial carcinoid tumor with metastases to the mediastinum, hilar lymph nodes, breast, and pancreas. In additional, the patient had metastases to the iris and ciliary body, resulting in progressive vision loss in her left eye. Treatment was successful by intravitreal injections of anti-vascular endothelial growth factor.

A case of primary ovarian carcinoid and the review of the literature

Objective:To investigate the clinicopathologic features of primary ovarian carcinoid.Methods:A case of primary ovarian carcinoid of the ovary were reported.Clinicopathological analysis was performed.Results:The case was just an island-like carcinoid.The tumor cells were round or polygonal,arranged into island-like or pseudo chrysanthemum clusters,and got bilateral tubal and oophorectomy.No evidence of recurrence was consulted in 28 months of follow-up.Conclusion:Primary ovarian carcinoid is rare in clinic.The diagnosis should be differentiated from granulosa cell tumor and sertolithoid cell tumor,and metastatic carcinoid should be excluded.As a low-grade malignant tumor,most ovarian carcinoids have a real good prognosis.

Primary hepatic carcinoid:A case report and literature review

Carcinoids are tumors derived from neuroendocrine cells and often produce functional peptide hormones.Approximately 54.5% arise in the gastrointestinal tract and frequently metastasize to the liver.Primary hepatic carcinoid tumors(PHCT) are extremely rare;only 95 cases have been reported.A 65-year-old man came to our attention due to occasional ultrasound findings in absence of clinical manifestations.His previous medical history,since 2003,included an echotomography of the dishomogeneous parenchymal area but no focal lesions.A computed tomography scan performed in 2005 showed an enhanced pseudonodular-like lesion of about 2 cm.Cholangio-magnetic resonance imaging identified the lesion as a possible cholangiocarcinoma.No positive findings were obtained with positron emission tomography.Histology suggested a secondary localization in the liver caused by a low-grade malignant neuroendocrine tumor.Immunohistochemistry was positive for anti chromogranin antibodies,Ki67 antibodies and synaptophysin.Octreoscan scintigraphy indicated intense activity in the lesion.Endoscopic investigations were performed to exclude the presence of extrahepatic neoplasms.Diagnosis of PHCT was established.The patient underwent left hepatectomy,followed by hormone therapy with sandostatine LAR.Two months after surgery he had a lymph nodal relapse along the celiac trunk and caudate lobe,which was histologically confirmed.The postoperative clinical course was uneventful,with a negative follow-up for hematochemical,clinical and radiological investigations at 18 mo post-surgery.Diagnosis of PHCT is based principally on the histopathological confi rmation of a carcinoid tumor and the exclusion of a non-hepatic primary tumor.Surgical resection is the recommended primary treatment for PHCT.Recurrence rate and survival rate in patients treated with resection were 18% and 74%,respectively.

SURGICAL TREATMENT AND PROGNOSIS OF BRONCHIAL CARCINOID TUMOR

In order to understand the effect of surgery in the treatment of the bronchial carcinoid tumor and the factors affecting prognosis, 18 cases of bronchial carcinoid tumor are presented, including 5 cases with Cushing's syndrome. There were lobectomy in 10, lung wedge resection in 3, excision of intraluminal tumor of bronchus in 3, exploratory thoracotomy in 2 cases. No operation death. Pathological examination revealed 14 cases were typical carcinoid tumor and 4 cases were atypical carcinoid tumor. By 2-13 years follow-up . 3 , 5 and 10 years survival rate were 82% , 78%and 70% respectively. Bronchial carcinoid tumor is often confused with small cell carcinoma of lung, the correct diagnosis can be obtained by light microscopic, electron microscopic and immunohistochemistry studies. Those patients accompanied with ectopic-ACTH secretion always have Cushing,s syndrome , resection of tumor can produce gho result. Proper operation method depends on the location of the tumor and patient's extent of cardiac and pulmonary reserve.Atypical carcinoid tumor had high malignancy and poor prognosis. The size of tumor, lymph node involvement and adjuvant therapy seem no definite effect on the patients' survival rate.

Direct costs of carcinoid syndrome diarrhea among adults in the United States

BACKGROUND The burden of carcinoid syndrome(CS)among patients with neuroendocrine tumors is substantial and has been shown to result in increased healthcare resource use and costs.The incremental burden of CS diarrhea(CSD)is less well understood,particularly among working age adults who make up a large proportion of the population of patients with CS.AIM To estimate the direct medical costs of CSD to a self-insured employer in the United States.METHODS CS patients with and without CSD were identified in the IBM®MarketScan®Database,including the Medicare Supplemental Coordination of Benefits database.Eligible patients had≥1 medical claim for CS with continuous health plan enrollment for≥12 mo prior to their first CS diagnosis and for≥30 d after,no claims for acromegaly,and no clinical trial participation during the study period(2014-2016).Baseline demographic and clinical characteristics,including comorbidities and treatment,were analyzed using descriptive statistics.Measures of healthcare resource use and costs were compared between patients with and without CSD,including Emergency Department(ED)visits,hospital admissions and length of stay,physician office visits,outpatient services,and prescription claims,using univariate and multivariate analyses to evaluate associations of CSD with healthcare resource use and costs,controlling for baseline characteristics.RESULTS Overall,6855 patients with CS were identified of which 4,043 were eligible for the analysis(1352 with CSD,2691 with CS only).Baseline demographic and clinical characteristics were similar between groups with the exception of age,underlying tumor type,and health insurance plan.Patients with CSD were older,had more comorbidities,and received more somatostatin analog therapy at baseline.Patients with CSD required greater use of healthcare resources and incurred higher costs than their peers without CSD,including hospitalizations(44%vs 25%)and ED visits(55%vs 31%).The total adjusted annual healthcare costs per patient were 50%higher(+$23865)among those with CSD,driven by outpatient services(+56%),prescriptions(+48%),ED visits(+26%),physician office visits(+21%),and hospital admissions(+11%).CONCLUSION The economic burden of CSD is greater than that of CS alone among insured working age adults in the United States,which may benefit from timely diagnosis and management.

Metastatic type 1 gastric carcinoid:A real threat or just a myth?

AIM:To describe disease characteristics and treatment modalities in a group of rare patients with metastatic gastric carcinoid type 1(GCA1).METHODS:Information on clinical,biochemical,radiological,histopathological findings,the extent of the disease,as well as the use of different therapeutic modalities and the long-term outcome were recorded.Patients’data were assessed at presentation,and thereafter at 6 to 12 monthly intervals both clinically and biochemically,but also endoscopically and histopathologically.Patients were evaluated for the presence of specific symptoms;the presence of autoimmune disorders and the presence of other gastrointestinal malignancies in other family members were also recorded.The evaluation of response to treatment was defined using established WHO criteria.RESULTS:We studied twenty consecutive patients with a mean age of 55.1 years.The mean follow-up period was 83 mo.Twelve patients had regional lymph node metastases and 8 patients had liver metastases.The primary tumor mean diameter was 20.13±10.83mm(mean±SD).The mean Ki-67 index was 6.8%±11.2%.All but one patient underwent endoscopic or surgical excision of the tumor.The disease was stable in all but 3 patients who had progressive liver disease.All patients remained alive during the follow-up period.CONCLUSION:Metastatic GCA1 carries a good overall prognosis,being related to a tumor size of≥1 cm,an elevated Ki-67 index and high serum gastrin levels.

Endoloop ligation after endoscopic mucosal resection using a transparent cap: A novel method to treat small rectal carcinoid tumors

BACKGROUND Local endoscopic resection is an effective method for the treatment of small rectal carcinoid tumors, but remnant tumor at the margin after resection remains to be an issue.AIM To evaluate the efficacy and safety of resection of small rectal carcinoid tumors by endoloop ligation after cap-endoscopic mucosal resection(LC-EMR) using a transparent cap.METHODS Thirty-four patients with rectal carcinoid tumors of less than 10 mm in diameter were treated by LC-EMR(n = 22) or endoscopic submucosal dissection(ESD)(n =12) between January 2016 and December 2017. Demographic data, complete resection rates, pathologically complete resection rates, operation duration, and postoperative complications were collected. All cases were followed for 6 to 30 mo.RESULTS A total of 22 LC-EMR cases and 12 ESD cases were enrolled. The average age was48.18 ± 12.31 and 46.17 ± 12.57 years old, and the tumor size was 7.23 ± 1.63 mm and 7.50 ± 1.38 mm, respectively, for the LC-EMR and ESD groups. Resection time in the ESD group was longer than that in the LC-EMR group(15.67 ± 2.15 min vs 5.91 ± 0.87 min; P < 0.001). All lesions were completely resected at one time. No perforation or delayed bleeding was observed in either group.Pathologically complete resection(P-CR) rate was 86.36%(19/22) and 91.67%(11/12) in the LC-EMR and ESD groups(P = 0.646), respectively. Two of the three cases with a positive margin in the LC-EMR group received transanal endoscopic microsurgery(TEM) and tumor cells were not identified in the postoperative specimens. The other case with a positive margin chose follow-up without further operation. One case with remnant tumor after ESD received further local ligation treatment. Neither local recurrence nor lymph node metastasis was found during the follow-up period.CONCLUSION LC-EMR appears to be an efficient and simple method for the treatment of small rectal carcinoid tumors, which can effectively avoid margin remnant tumors.

Goblet cell carcinoid tumors of the appendix: An overview

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.

Goblet cell carcinoids of the appendix:Tumor biology,mutations and management strategies

Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers.Goblet cell carcinoids(GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features.This review focuses on the current diagnostic procedures,pathogenesis,possible signaling mechanisms and treatment options for GCC.Perspectives for future research are discussed.The tumor likely arises from pluripotent intestinal epithelial crypt base stem cells.Previous findings of Notch signaling as a tumor suppressor in Neuroendocrine tumors may have a similar role in this tumor too.Loss of Notch signaling may be the driver mutation with other successive downstream mutations likely favors them into progressing and behavior similar to poorly differentiated adenocarcinoma with minimal neuroendocrine differentiation.A multidisciplinary approach is suggested for optimal outcomes.Surgery remains the main treatment modality.Simple appendectomy may be sufficient in early stages while right hemicolectomy is recommended for advanced tumors.Cytoreductive surgery with heated intraperitoneal chemotherapy may improve survival in a select few with metastatic peritoneal disease.These tumors have an unpredictable behavior even in early stages and local recurrence and delayed metastases may be seen.Lifelong surveillance is warranted.

Endoscopic mucosal resection for rectal carcinoids under micro-probe ultrasound guidance

AIM: To assess the therapeutic value of endoscopic mucosal resection (EMR) under micro-probe ultrasound guidance for rectal carcinoids less than 1 cm in diameter. METHODS: Twenty-one patients pathologically diagnosed with rectal carcinoids following colonoscopy in our hospital from January 2007 to November 2012 were included in this study. The patients consisted of 14 men and 7 women, with a mean age of 52.3 ± 12.2 years (range: 36-72 years). The patients with submucosal tumors less than 1 cm in diameter arising from the rectal and muscularis mucosa detected by micro-probe ultrasound were treated with EMR and followed up with conventional endoscopy and micro-probe ultrasound. RESULTS: All of the 21 tumors were confirmed by micro-probe ultrasound as uniform hypoechoic masses originating from the rectal and muscularis mucosa, without invasion of muscularis propria and vessels, and less than 1 cm in diameter. EMR was successfully completed without bleeding, perforation or other complications. The resected specimens were immunohistochemically confirmed to be carcinoids. Patients were followed up for one to two years, and no tumor recurrence was reported. CONCLUSION: EMR is a safe and effective treatment for rectal carcinoids less than 1 cm in diameter.

Somatostatin analogs for gastric carcinoids:for many, but not all

Gastric carcinoids(GCs) are classified as: type Ⅰ,related to hypergastrinemia due to chronic atrophic gastritis(CAG), type Ⅱ, associated with Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1, and type Ⅲ, which is normogastrinemic. The management of type-Ⅰ gastric carcinoids(GC1s) is still debated,because of their relatively benign course. According to the European Neuroendocrine Tumor Society guidelines endoscopic resection is indicated whenever possible;however, it is not often feasible because of the presence of a multifocal disease, large lesions, submucosal invasion or, rarely, lymph node involvement. Therefore,somatostatin analogs(SSAs) have been proposed as treatment for GC1 s in view of their antisecretive,antiproliferative and antiangiogenic effects. However,in view of the high cost of this therapy, its possible side effects and the relatively benign course of the disease,SSAs should be reserved to specific subsets of "high risk patients", i.e., those patients with multifocal or recurrent GCs. Indeed, it is reasonable that, after the development of a gastric neuroendocrine neoplasm in patients with a chronic predisposing condition(such as CAG), other enterochromaffin-like cells can undergo neoplastic proliferation, being chronically stimulated by hypergastrinemia. Therefore, definite indications to SSAs treatment should be established in order to avoid the undertreatment or overtreatment of GCs.

Which endoscopic treatment is the best for small rectal carcinoid tumors?

The incidence of rectal carcinoids is rising because of the widespread use of screening colonoscopy. Rectal carcinoids detected incidentally are usually in earlier stages at diagnosis. Rectal carcinoids estimated endoscopically as < 10 mm in diameter without atypical features and confined to the submucosal layer can be removed endoscopically. Here, we review the efficacy and safety of various endoscopic treatments for small rectal carcinoid tumors, including conventional polypectomy, endoscopic mucosal resection(EMR),cap-assisted EMR(or aspiration lumpectomy),endoscopic submucosal resection with ligating device,endoscopic submucosal dissection, and transanal endoscopic microsurgery. It is necessary to carefully choose an effective and safe primary resection method for complete histological resection.

Treatment of colorectal carcinoids:A new paradigm

It is often difficult to evaluate the grade of malignancy and choose an appropriate treatment for colorectal carcinoids in clinical settings. Although tumor size and depth of invasion are evidently not enough to stratify the risk of this rare tumor, the present guidelines or staging systems do not mention other clinicopathological variables. Recent studies, however, have shed light on the impact of lymphovascular invasion on the outcome of colorectal carcinoids. It has been revealed that the presence of lymphovascular invasion was among the strongest risk factors for metastasis along with tumor size and depth of invasion. Furthermore, tumors smaller than 1 cm, within submucosal invasion and without lymphovascular invasion, carry minimal risk for metastasis with 100% 5-year survival in the studies from Japan as well as from the USA. This would suggest that these tumors could be curatively treated by endoscopic resection or transanal local excision. On the other hand, colorectal carcinoids with either lymphovascular invasion or tumor size larger than 1 cm carry the risk for metastasis equivalent to adenocarcinomas. Therefore, it should be emphasized that histological examination of lymphovascular invasion is mandatory in the specimens obtained by endoscopic resection or transanal local excision, as this would provide useful information for determining the need for additional radical surgery with regional lymph node dissection. Although the present guidelines or TNM staging system do not mention the impact of lymphovascular invasion, this would be among the next promising targets in order to establish better guidelines and staging systems, particularly in early-stage colorectal carcinoids.

Gastric carcinoids:Between underestimation and overtreatment

Gastric carcinoids(GCs),which originate from gastric enterochromaffin-like(ECL) mucosal cells and account for 2.4% of all carcinoids,are found increasingly in the course of upper gastrointestinal tract endoscopy.Current nosography includes those occurring in chronic conditions with hypergastrinemia,as the type 1 associated with chronic atrophic gastritis,and the type 2 associated with Zollinger-Ellison syndrome in multiple endocrine neoplasia type 1,and type 3,which is unrelated to hypergastrinemia and is frequently malignant,with distant metastases.The optimal clinical approach to GCs remains to be elucidated,depending upon type,size and number of carcinoids.While there is agreement concerning the treatment of type 3 carcinoids,for types 1 and 2,current possibilities include simple surveillance,endoscopic polypectomy,surgical excision,associated or not with surgical antrectomy,or total gastrectomy.Moreover,the recent introduction of somatostatin analogues represents a therapeutic option of possibly outstanding relevance.

Malignant carcinoid tumor of the cystic duct:a rare cause of bile duct obstruction

BACKGROUND:Carcinoid tumors of the extrahepatic biliary tree are extremely rare malignancies,accounting for 0.2%-2%of all gastrointestinal carcinoid tumors,while carcinoids of the cystic duct are an uncommon entity and an extremely unusual cause of bile duct obstruction.METHODS:After an extensive literature review,we retrospectively analysed 61 cases of carcinoid tumor of the biliary tree as well as one additional case of a 60-yearold female with symptoms and laboratory/imaging examination findings compatible with those of a malignant biliary tract obstruction.At laparotomy,resection of the gallbladder and common bile duct was performed.Histological study revealed a well-differentiated neuroendocrine carcinoma of the cystic duct.The patient remained disease-free at 16 months.RESULTS:Our presentation is the seventh case reported in the world literature.Compared to cholangiocarcinoma,analysis of the reviewed group indicates an increased incidence of extrahepatic carcinoid tumors in younger persons along with a slight female predominance.Statistically,the most common anatomic location is the common bile duct,followed by the perihilar region and the cystic duct.Jaundice is the most common finding.Curative surgery was realized in the majority of cases and longterm disease-free survival was achieved when surgery was curative.CONCLUSIONS:Carcinoid tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively,and nearly impossible to differentiate from non-neuroendocrine tumors.As surgery offers the only potential cure for both biliary carcinoids and cholangiocarcinoma,we recommend aggressive surgical therapy as the treatment of choice in every case of potentially resectable biliary tumor.

Regression of liver metastases of occult carcinoid tumor with slow release Lanreotide therapy

Few clinical studies have demonstrated an anti-proliferative activity of somatostatin (SST) analogs in carcinoids. We report the case of a woman with liver metastases of neuroendocrine tumor and no evidence of the primary tumor. The liver metastases were characterized by high proliferation index, immunoreactiviy for somatostatin receptor (SSTR)-l, 2, 3 and 5 and positive octreoscan. Urinary 5-hydroxyindolacetic acid, serum serotonin and chromogranin A were elevated. Slow release lanreotide (SR-LAN) therapy for 3 mo controlled clinical and biochemical signs of carcinoid tumor and caused a clear-cut reduction in the diameter of two liver metastases and disappearance of another lesion, with further reduction after 6 and 18 mo. We demonstrated a clear-cut long-lasting anti-proliferative effect of SR-LAN on liver metastases of occult carcinoid with high proliferation index and immunoreactivity for SSTR-1, 2, 3, and 5. Immunohistochemistry for SSTRs could be a suitable method for the selection of patients with metastatic carcinoid that may benefit from SST analog therapy.