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.

Upper gastrointestinal carcinoid tumors incidentally found by endoscopic examinations

AIM: This study shares Asian clinical experiences of carcinoid tumors that originated in the upper gastrointestinal tract.METHODS: From May 1987 to June 2002, we had found only 13 cases of histologically confirmed carcinoid tumors in the upper gastrointestinal tract by endoscopic examinations. There were eight males and five females.The mean age was 53.16±20.51 years that ranged from 26 to 82 years. Each of their clinical presentations,locations, tumor morphology, and size and the treatment outcome were analyzed and discussed.RESULTS: One patient had a polypoid lesion at the lower esophagus, nine were stomach lesions and three located at the duodenum. All patients with polypoid and submucosal tumor types were of small size (＜1.7 cm) and all patients survived after simple excision or polypectomy.Four of the five patients in tumor mass forms died and the tumors were more than 2.0 cm in size.CONCLUSION: Carcinoid tumors rarely originated from the upper gastrointestinal tract and are usually found accidentally after endoscopic study. Bigger size (more than 2 cm) tumor masses may indicate a more severe disease and poor prognosis.

Gastric carcinoid tumor in a patient with a past history of gastrointestinal stromal tumor of the stomach

Gastrointestinal stromal tumor is the most common mesenchymal tumor in the gastrointestinal tract. It may coexist with other type of cancers,and if so,the tumors usually involve the stomach. The most common associated cancers are gastrointestinal carcinomas. We report a 65-year-old woman with a history of gastric gastrointestinal stromal tumor who had undergone subtotal segmental gastrectomy. New polypoid lesions were detected on a follow-up gastroscopy one year later. The lesions were biopsied and found to be carcinoid tumors. There was serum hypergastrinemia,and type 1 gastric carcinoid tumor was diagnosed. A total gastrectomy was performed. Pathologic examination revealed both carcinoid tumors and a recurrent gastrointestinal stromal tumor.

Treatment of solitary gastric carcinoid tumor by endoscopic polypectomy in a patient with pernicious anemia

Type I gastric cardnoid tumors result from hypergastrinemia in 1%-7% of patients with pernicious anemia. We diagnosed pernicious anemia in a 48-year-old female patient with complaint of fatigue for three months. She had no gastrointestinal symptoms. Endoscopic examination ot the upper gastrointestinal tract revealed atrophic gastritis and a polypoid lesion in the corpus of 3-4 mm in size. Endoscopic polypectomy was performed. Histopathological examination of the specimen revealed positive chromogranin A and synaptophysin stainings compatible with the diagnosis of a carcinoid tumor. Serum gastrin level was increased, urinary 5-hydroxyindoleacetic acid was within the normal range. There was no other symptom, sign, or laboratory finding of a carcinoid syndrome in the patient. No metastasis was found with indium-ill octreotide scan, computed tomographies of abdomen and thorax. Type I gastric carcinoid tumors are only rarely solitary and patients with tumors 〈 1 cm in size may benefit from endoscopic polypectomy.

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.

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.

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%,respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.

Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome

Von HippeI-Lindau syndrome （VHL） is a rare autosomal-dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intraluminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

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.

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.

Ligation-assisted endoscopic submucosal resection with circumferential mucosal incision for duodenal carcinoid tumor

Here we present the case of a 64-year-old female with a duodenal carcinoid tumor treated by ligation-assisted endoscopic submucosal resection(ESMR-L) with circumferential mucosal incision(CMI). Band ligation was effective in resecting the duodenal carcinoid tumor after CMI,with an uneventful post-procedural course. Histopathological examination showed clear tumor margins at deeper tissue levels. Thus,in the present case,ESMR-L with CMI was useful for the treatment of duodenal carcinoid tumor.

Primary renal carcinoid tumor: A rare cystic renal neoplasm

We present the case of a 21-year-old man with an incidentally detected cystic renal mass.A well-defined,solid mass measuring approximately 8 cm x 6 cm with a cystic component was identified in the left kidney by abdominal multidetector computed tomography(CT) and ultrasonography.The mass was well-enhanced on the corticomedullary CT phase and washout of enhancement occurred on the nephrographic phase.The mass contained peripheral wall and septal calcifications in the cystic component.The lesion was resected and diagnosed as a primary renal carcinoid tumor.Primary carcinoid tumors of the kidney are extremely rare.This case is notable because of the rarity of this neoplasm and its unique radiologic and pathologic findings.A review of previously reported cases in the literature is also presented.

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.

Contrast-Enhanced CT Colonography Features of Rectal Carcinoid Tumors

Purpose: The purpose of this study was to retrospectively assess the detectability and enhancement pattern of rectal carcinoid tumors on contrast-enhanced computed tomography colonography (CE-CTC). Methods: The study sample consisted of 12 lesions in nine patients of rectal carcinoid tumors. To evaluate the detectability, two radiologists reviewed axial and 3D images including air enema (AE), virtual endoscopy (VE), and virtual colon dissection (VCD). To determine the contrast enhancement pattern of the tumors, the CT attenuation value of each lesion was measured in three phases. Results: Four of the 12 lesions (33%) were detected on axial images in patients in both the prone and supine positions, the sizes of which were 6 mm or larger. Seven lesions (58%), including the four lesions mentioned above, were detected on 3D images including AE and VE in patients in the prone position, the sizes of which were 5 mm or larger. The ranges of CT attenuation values of the lesions at 40 s, 70 s and 240 s were 112 - 147, 116 - 140 and 82 - 97 HU, respectively. Conclusion: Rectal carcinoid tumors are challenging to detect on CE-CTC. They demonstrated enhancement at the early phase and washout at the delayed phase on CE-CTC.

Comparison of Patient Survival after Resection for Pulmonary Carcinoid Tumors Compared to Other Neuroendocrine Tumors: A United States Population Study

Background: We hypothesized: 1) resected pulmonary typical carcinoid (TC) will show a favorable clinical pattern compared to other bronchopulmonary neuroendocrine tumors (BPNETs);and 2) Atypical carcinoid (AC) and large-cell neuroendocrine carcinoma (LCNEC) patients will have similar outcomes. Methods: The Surveillance Epidemiology and End Result database was queried to compare demographics and tumor specific variables in patients undergoing resection for TC, AC, LCNEC and small cell lung cancer (SCLC) from 2001 to 2006. Results: Similar to LCNEC, AC patients had greater incidence of histologic positive lymph nodes compared to TC (P < 0.001). Survival analysis showed a mean survival of 40.0 ± 1.9 months for SCLC, 46.2 ± 2.2 months for LCNEC, 58.3 ± 2.3 months for AC, and 70.2 ± 0.2 months for TC tumors. TC patients demonstrated favorable survival, and SCLC patients poorer survival, compared to AC and LNEC patients (P < 0.01). Conclusion: AC tumors are a BPNET histology that exhibits clinical behavior distinct from TC. AC tumors should be staged, and treated with stage appropriate therapeutic strategies similar to other non-small cell lung cancers.

Sporadic gastric carcinoid tumor successfully treated by two-stage laparoscopic surgery:A case report

We report a case of sporadic gastric carcinoid tumor successfully treated by two-stage laparoscopic surgery.A 38-year old asymptomatic woman was referred to our hospital for evaluation of a submucosal tumor of the stomach.Endoscopic examination showed a solitary submucosal tumor without ulceration or central depression on the posterior wall of the antrum and biopsy specimens were not sufficient to determine the diagnosis.Endoscopic ultrasound revealed a tumor nearly 2 cm in diameter arising from the muscle layer and a computed tomography scan showed the tumor enhanced in the arterial phase.Laparoscopic wedge resection was performed for definitive diagnosis.Pathologically,the tumor was shown to be gastric carcinoid infiltrating the muscle layer which indicated the probability of lymph node metastasis.Serum gastrin levels were normal.As a radical treatment,laparoscopy-assisted distal gastrectomy with regional lymphadenectomy was performed 3 wk after the initial surgery.Finally,pathological examination revealed no lymph node metastasis.

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.

Concomitant gastric carcinoid and gastrointestinal stromal tumors:A case report

A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor （GIST） is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type 111 gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.

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.