Carcinoid Tumors in the Middle Ear:a Case Report and Literature Review

Middle ear carcinoid tumor (MEC T) is rare. Only 46 cases of MECT have been reported in the literature since the first case of MECT was described in 1980. We present here a case of primary MECT initially diagnosed as inflammatory aural polyp. The case was a 43-year-old women complaining of right ear chronic otorrhea and hearing loss over a period of five years, with a blockage sensation in the right ear for two years. Audiometry showed conductive hearing loss in the right ear. Physical examination and CT scans showed a mass in the right external auditory canal and middle ear, surrounding the ossicular chain. Pathologic study of surgically removed specimen revealed features of carcinoid tumor with positive staining to chromogranin A and synaptophysin in tumor cells. Local radiation of 60 Gy was applied. The patient has been followed up for more than one year. Postoperative histopathological examination showed no evidence of MECT recurrence one year after surgery, but inflammatory changes in the middle ear. Relevant literatures were reviewed. Clinical, histopathological, immunohistochemical and ultrastructural features of MECT, and strategies in MECT diagnosis and management are discussed.

Endoscopic ultrasonography and submucosal resection in the diagnosis and treatment of rectal carcinoid tumors

Rectal carcinoid tumors are often diagnosed as .submucosal tumors on radiological or endoscopic examinations. Usually, its definite size and character, the infiltration of muscularis propria and vessels can not be correctly diagnosed preoperatively. In recent years, endoscopic ultrasonography （EUS） has been used in the diagnosis of submucosal tumors of the gastrointestinal tracts , Conventional endoscopic polypectomy allows local excision of local tumors, but is often associated with tumor involvement of the resection margin necessitates further interventions.

Clinicopathologic analysis of eight cases of pancreatic carcinoid tumors

Carcinoids of the pancreas are exceedingly rare tumors that originate from the enterochromaffin cells of the gastroenteropancreatic neuroendocrine system. According to a recent report, pancreatic carcinoids are found in only 0.58% （79/13 715 cases） of the entire carcinoid group. To date, very limited information regarding the detection and diagnosis of this entity has been reported in the available literature. Although pancreatic carcinoid tumors grow slowly and show an indolent clinical behavior, they are potentially malignant. Due to the late onset of clinical symptoms and delayed diagnosis of the tumor, there was a high incidence of distant metastases that hampered long-term survival for many patients, leading to an unfavorable overall prognosis.

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.

Goblet cell carcinoid of the appendix and mixed adenoneuroendocrine carcinoma: Report of three cases

Neuroendocrine neoplasms are the most common epithelial tumors among appendix tumors. Appendix tumors that are completely or partially composed of neuroendocrine cells are divided into two categories: Classic carcinoid tumors and goblet cell carcinoid tumors(GCCT). They are known to progress more aggressively than classic(neuro) endocrine tumors. In this study, three cases with acute appendicitis symptoms are presented, including their clinical and histopathological findings. Microscopic examination detected GCCT in two cases and mixed adenoneuroendocrine carcinoma in one case, in addition to acute appendicitis.

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.

Management of incidentally discovered appendiceal neuroendocrine tumors after an appendicectomy

Appendiceal neuroendocrine tumors(aNETs)are an uncommon neoplasm that is relatively indolent in most cases.They are typically diagnosed in younger patients than other neuroendocrine tumors and are often an incidental finding after an appendectomy.Although there are numerous clinical practice guidelines on management of a NETs,there is continues to be a dearth of evidence on optimal treatment.Management of these tumors is stratified according to risk of locoregional and distant metastasis.However,there is a lack of consensus regarding tumors that measure 1-2 cm.In these cases,some histopathological features such as size,tumor grade,presence of lymphovascular invasion,or mesoappendix infiltration must also be considered.Computed tomography or magnetic resonance imaging scans are recommended for evaluating the presence of additional disease,except in the case of tumors smaller than 1 cm without additional risk factors.Somatostatin receptor scintigraphy or positron emission tomography with computed tomography should be considered in cases with suspected residual or distant disease.The main point of controversy is the indication for performing a completion right hemicolectomy after an initial appendectomy,based on the risk of lymph node metastases.The main factor considered is tumor size and 2 cm is the most common threshold for indicating a colectomy.Other factors such as mesoappendix infiltration,lymphovascular invasion,or tumor grade may also be considered.On the other hand,potential complications,and decreased quality of life after a hemicolectomy as well as the lack of evidence on benefits in terms of survival must be taken into consideration.In this review,we present data regarding the current indications,outcomes,and benefits of a colectomy.

Prognostic impact of number of examined lymph nodes on survival of patients with appendiceal neuroendocrine tumors

BACKGROUND The prognosis of patients with appendiceal neuroendocrine tumors(ANETs) is related to lymph node(LN) metastasis and other factors.However,it is unclear how the number of examined LNs(ELNs) impact on survival.AIM To determine the factors affecting the cancer-specific survival(CSS) of patients with ANET and to evaluate the impact of the number of ELNs on survival.METHODS A total of 4583 ANET patients were analyzed in the Surveillance,Epidemiology,and End Results database.Univariate survival analysis was used to identify factors related to survival and the optimal number of ELNs and lymph node ratio(LNR) were determined by the Kaplan–Meier method.The survival difference was determined by CSS.RESULTS Except for sex,the other factors,such as age,year,race,grade,histological type,stage,tumor size,ELNs,LNR,and surgery type,were associated with prognosis.The 3-,5-,and 10-year CSS rates of ANET patients were 91.2%,87.5,and 81.7%,respectively(median follow-up period of 31 mo and range of 0-499 mo).There was no survival difference between the two surgery types,namely,local resection and colectomy or greater,in both stratifications of tumor size ≥ 2 cm(P = 0.523)and < 2 cm(P = 0.068).In contrast to patients with a tumor size < 2 cm,those with a tumor size ≥ 2 cm were more likely to have LN metastasis(χ~2 = 378.16,P < 0.001).The optimal number of ELNs was more than 11,7,and 18 for all patients,nodenegative patients,and node-positive patients,respectively.CSS rates of patients with a larger number of ELNs were significantly improved(≤ 10 vs ≥ 11,χ~2 =20.303,P < 0.001;≤ 6 vs ≥ 7,χ~2 = 11.569,P < 0.001;≤ 17 vs ≥ 18,χ~2 = 21.990,P < 0.001;respectively).ANET patients with an LNR value ≤ 0.16 were more likely to have better survival than those with values of 0.17-0.48(χ~2 = 48.243,P < 0.001) and 0.49-1(χ~2 = 168.485,P < 0.001).CONCLUSION ANET ≥ 2 cm are more likely to develop LN metastasis.At least 11 ELNs are required to better evaluate the prognosis.For patients with positive LN metastasis,18 or more LNs need to be detected and lower LNR values(LNR ≤ 0.16) indicate a better survival prognosis.

Meckel Diverticulum Carcinoid Tumor Complicated by Ileal Invagination. About One Case at Vichy Hospital

Meckel’s diverticulum is a common pathology in children and rare in adults. We present a case of a 79-year-old patient in which a significant gastrointestinal bleeding, whose paraclinical explorations confirmed a fatty tumor of the terminal ileum. The exploratory coelioscopy revealed a tumor of the Meckel diverticulum complicated of intestinal invagination, resected at the same time with resection and extracorporeal anastomosis by mini coelio-guided laparotomy. Histology confirmed the presence of heterotopical tissue of the duodenal mucosa with Brunner cells and a carcinoid tumor. In conclusion, this clinical case shows that coelioscopy can be considered a very important diagnostic and therapeutic tool in this pathology especially in the elderly.

Neuroendocrine Tumor of Small Intestine, a Diagnostic Challenge

Incidence of neuroendocrine tumors (NET) has significantly increased in the past three decades. In the small intestine, NET are the most frequent tumors, even more frequent than adenocarcinomas. Due to atypical presentations and late symptoms, NET in the small intestine frequently represent a diagnostic challenge. It is important to take these tumors into consideration in differential diagnosis of gastrointestinal neoplasms. Surgeons, oncologists, endocrinologists, and gastroenterologists should understand the disease characteristics and management alternatives. This document aims to review the key points of NET and main diagnostic tools. We present the case of a 50-year-old male who presented lower gastrointestinal bleeding. Imaging and endoscopic studies showed no conclusive findings. A capsule endoscopy showed multiple ulcered lesions with neoplastic aspect in the distal jejune. Due to the multifocal nature of the lesions, clinicians suspected NET-associated digestive bleeding. The patient underwent exploratory laparoscopy with ileectomy and radical abdominal lymphadenectomy. Histopathologic examination confirmed the suspected diagnosis of NET. This case reflects the complexity of diagnostic approach and differential diagnoses for these tumors.

Primary hepatic neuroendocrine carcinoma:clinical analysis of 11 cases

BACKGROUND: Primary hepatic neurocridocrine carcinoma (PHNEC) is extremely rare, and fewer than 300 cases have been reported in the English/Chinese-language literature, therefore it is difficult to make a proper diagnosis and determine a therapeutic approach. METHODS: Eleven PHNEC patients were admitted to our hospital between January 1996 and May 2008. Laboratory examination, digestive endoscopy, B-ultrasonography, CT, MRI, or PET-CT were performed on the patients for preoperative diagnosis. All patients received liver resection. Some patients received transcatheter arterial chemoembolization (TACE), percutaneous ethanol injection treatment (PEIT), or octreotide injection when a recurrence was found. The patients' clinical data were recorded and all patients were followed up. RESULTS: The patients were confirmed pathologically as having PHNEC. Their median follow-up time was 33 months (12-107 months). All patients survived, and the longest postoperative survival time was 107 months, the longest disease-free survival time was 98 months, the 1-year survival rate was 100%, and the 1-year recurrence rate was 45.5% (5/11). CONCLUSIONS: Since PHNEC is easy to confuse with hepatocellular carcinoma, careful screening of symptoms is needed to avoid misdiagnosis. Resection is the first choice of treatment for PHNEC and provides the most favorable outcomes including long-term survival. Other treatment such as TACE and PEIT can be considered as well, especially when a tumor recurs.

Flushing as atypical initial presentation of functional gallbladder neuroendocrine carcinoma: A case report

BACKGROUND Neuroendocrine neoplasms are rarely located in the gallbladder(GB),and carcinoid syndrome is exceedingly rare in patients with GB neuroendocrine neoplasms.CASE SUMMARY We report a case of GB neuroendocrine carcinoma(GB-NEC)in a 65-year-old man,who presented with flushing for 2 mo.Pathological specimens of the flushed skin revealed that mucin was deposited between the collagen bundles in the dermis.Computed tomography and magnetic resonance imaging indicated neoplasm in the GB with liver invasion and enlarged lymph nodes in the portacaval space.High fluorodeoxyglucose uptake was detected in lymph nodes in the portacaval space,but distant metastasis was not seen by positron emission tomography.Ultrasound-guided needle biopsy of the GB neoplasm was suggestive of high-grade NEC.Because of the functional characteristics of poorly differentiated NEC,en bloc cholecystectomy,resection of hepatic segments IVb and V,pancreaticoduodenectomy,and regional lymphadenectomy were performed.A diagnosis of poorly differentiated NEC was made by pathological findings and immunohistochemical staining data.Ki-67 index was>80%.The patient refused adjuvant therapy and passed away in the 7th month.CONCLUSION Distinctive manifestation combined with imaging helps make correct preoperative diagnosis.Radical surgery and adjuvant chemotherapy might improve prognosis.

Neuroendocrine carcinoma of the ampulla of Vater:a clinicopathologic evaluation

BACKGROUND:Only 105 cases of neuroendocrine tumor(NET)of the ampulla of Vater have been described, mostly as single case reports.The incidence of NET is rising.The changes in incidence may result from changes in detection.This study was to determine the relative incidence and clinicopathological characteristics of high- grade neuroendocrine carcinoma(small cell carcinoma and large cell carcinoma)of the ampulla of Vater at a single institution. METHODS:Sections from paraffin blocks of tumors of the ampulla of Vater taken from 45 patients who underwent Whipple’s procedure and 6 patients who underwent palliative bypass between September 2003 and January 2007 were subjected to immunohistochemical analysis.The clinical and pathological data from 5 patients diagnosed with NET of the ampulla of Vater were analyzed. RESULTS:The patients were 3 men and 2 women,ranging in age from 39 to 47 years(mean 44 years).Operative procedures included Whipple’s procedure in 4 patients and palliative bypass in 1 patient.Histopathological examination revealed large-cell neuroendocrine carcinoma in 2 patients,small cell carcinoma in 2,and carcinoid in 1.Three patients with high-grade neuroendocrine carcinoma who had undergone Whipple’s procedure died at postoperatively 7,11,and 13 months.The patient who had undergone palliative triple bypass died 3 months after surgery.CONCLUSIONS:The relative incidence of high-grade neuroendocrine carcinomas of the ampulla of Vater is higher than that generally expected.The tumors behave aggressively and have a dismal prognosis despite aggressive treatment.

Recent trends in the treatment of well-differentiated endocrine carcinoma of the small bowel

Well-differentiated endocrine carcinomas of the small bowel are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that may cause carcinoid syndrome. However, many are clinically silent until late presentation with major effects. Initial treatment aims to control carcinoid syndrome with somatostatin analogs. Even if there is metastatic spread, surgical resection of the primitive tumor should be discussed in cases of retractile mesenteritis, small bowel ischemia or subocclusive syndrome in order to avoid any acute complication, in particular at the beginning of somatostatin analog treatment. The choice of treatment depends on the symptoms, general health of the patient, tumor burden, degree of uptake of radionuclide, histological features of the tumor, and tumor growth. Management strategies include surgery for cure (which is rarely achieved) or for cytoreduction, radiological interventions (transarterial embolization or radiofrequency ablation), and chemotherapy (interferon and somatostatin analogs). New biological agent and radionuclide targeted therapies are under investigation. Diffuse and non-evolving lesions should also be simplymonitored. Finally, it has to be emphasized that it is of the utmost importance to enroll these patients with a rare disease in prospective clinical trials assessing new therapeutic strategies.

Neuroendocrine carcinoma of gallbladder: report of 2 cases

BACKGROUND: Neuroendocrine carcinoma of the gall- bladder is rare. Its best treatment is not known. METHODS: Two patients underwent surgery earlier: one for suspected cholecystitis and the other for cholelithiasis. Magnetic resonance cholangiopancreatography ( MRCP ) showed residual lesions in the livers. The two patients un- derwent revision surgery followed by chemotherapy. RESULTS; Both patients tolerated the second stage surgery well, which was followed by chemotherapy with paclitaxel, ifosphamide and cisplatin for 6 cycles. They were treated this way for 8 months and 12 months post treatment, re- spectively. CONCLUSIONS: A proper diagnosis of neuroendocrine carcinoma is made often after surgery. As it is a slow grow- ing tumor and not very chemotherapeutically, sensitive sur- gery offers the best local control.

Surgical treatment of non-functioning pancreatic neuroendocrine tumors:current controversies and challenges

There has been a rising trend in the incidence and prevalence of non-functioning pancreatic neuroendocrine tumors(NFPanNETs).While a significant number of the newly diagnosed NFPanNETs are asymptomatic,a majority of patients will present with liver metastasis(LM)at the time of diagnosis.Surgical resection remains the only curative treatment,especially for localized NFPanNETs.While a majority of small NFPanNETs are indolent,some are not.This heterogeneity in tumor biology presents the surgeon with the unique challenge of determining which patient will benefit from surgery,given the morbidity of pancreatic surgery.There has been a recent push for a more aggressive approach to the care of these patients,given the emergence of data supporting such measures.However,the risk of over or under treatment has generated immense debate amongst experts in the field.The heterogeneity of current practice guidelines and institutional practices around the world is a reflection of the disparate opinion on the management of NFPanNET.In this review,we set out to examine the evidence regarding some of the most controversial and challenging aspects of the surgical treatment of NFPanNET.We evaluate the following questions;should patients with small NFPanNETs≤2 cm in size be resected;should patients with metastatic NFPanNETs undergo surgical debulking,and should there be resection of the primary tumor in the setting of non-resectable metastatic disease?Although there are currently no Level 1 data to answer these questions conclusively,we believe that the current literature supports a more aggressive approach to the management of NFPanNET.

Late recurrence and metastasis of an appendiceal goblet cell carcinoid 24 years after appendectomy

A 36-year-old woman presented with right iliac fossa pain in March 1988.Abdominal ultrasound showed nothing unusual and an appendectomy was performed for acute appendicitis.The resected appendix appeared inflamed without perforation.No enlarged lymph nodes were seen during surgery.Microscopic examination of the resected specimen showed acute appendicitis and a tumor in the lamina propria and submucosal layer,infiltrating to the deep muscular layer.Groups of four to ten tumor cells were arranged in small clusters which lacked central lumens and were widely separated by stroma.The tumor cells,which were distended with mucin and had crescentic nuclei,resembled signet ring cells （Figure 1A）.Keywords：recurrence; metastasis; appendiceal neoplasm; appendectomy; carcinoid tumor

Metastatic high-grade neuroendocrine tumor of mandible

Neuroendocrine tumors of the oral cavity and jaws are exceedingly rare.They include paragangliomas,a melanotic neuroectodermal tumor of infants,small cell carcinomas,and Merckel cell carcinomas.Most have been non-functional in nature.Breast,lung,liver,colon,and prostate are the most common reported primary malignancies which can metastasize to the oral cavity.In most cases,oral metastases involve maxilla and mandible rather than soft tissues.The premolar-molar region is the most common localization.The purpose of this article is to describe a rare case of a high grade neuroendocrine tumor of the mandible which metastasized from the cervix.