scholarly journals Mixed Adenoneuroendocrine Carcinoma Is a Rare but Important Tumour Found in the Oesophagus

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Mohammad Murad Kasim Kadhim ◽  
Marie Louise Jespersen ◽  
Hans Kristian Pilegaard ◽  
Marianne Nordsmark ◽  
Gerda Elisabeth Villadsen

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract that consists of a dual adenocarcinomatous and neuroendocrine differentiation, each component representing at least 30% of the tumour. We report a case of a 68-year-old man who presented with two-month history of postprandial pain and vomiting. Gastric endoscopy revealed a polypoid mass in the lower part of the oesophagus. In contrast to the majority of these tumours, this biopsy was immunohistochemically positive for chromogranin A, and synaptophysin and Ki-67 index was 50% and the tumour was diagnosed as poorly differentiated neuroendocrine carcinoma of the oesophagus. The patient underwent surgery and lower oesophagus resection was performed. Based on the histopathology and immunohistochemistry of the tumour in the oesophagogastrectomy specimen, a mixed adenoneuroendocrine carcinoma (MANEC) was diagnosed. The objective of this case report is to advocate for the focus on the MANEC diagnosis as such patients need to be referred to a centre of excellence with expertise in NET tumours, to have the correct diagnostic work-up, treatment, and secondary diagnostic procedures performed at progression, as this will have paramount influence of the choice of treatment.

2020 ◽  
Vol 48 (8) ◽  
pp. 030006052094791
Author(s):  
Xu Li ◽  
Dezhao Li ◽  
Xiaodong Sun ◽  
Guoyue Lv

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract with both epithelial and neuroendocrine components. We describe a case of a 57-year-old man who presented with yellow sclera and dark urine. Contrast-enhanced computed tomography (CT) showed enlargement of the intra and extrahepatic bile ducts and a neoplastic lesion at the end of the common bile duct which was indistinguishable from the adjacent head of the pancreas and duodenum. A pancreaticoduodenectomy was performed and histopathological examination of resected samples showed that tumour was a complex lesion with adenocarcinoma and neuroendocrine components positive for neuroendocrine markers (chromogranin A and synaptophysin) with a Ki-67 labelling index of 40%. The patient was diagnosed with MANEC in the ampulla of Vater with a neuroendocrine carcinoma component of approximately 70%. Ampullary MANECs are highly aggressive tumours with a high risk for distant metastases and a poor prognosis. Therefore, establishing a standard therapeutic strategy is crucial.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2094432
Author(s):  
Sudha Shahi ◽  
Tika Ram Bhandari ◽  
Prakash Bahadur Thapa ◽  
Deependra Shrestha ◽  
Kiran Shrestha

Foreign body esophagus remains one of the common medical emergencies which may lead to significant morbidity and mortality. Sharp objects, batteries, and elderly with foreign body esophagus should be treated with emergent removal owing to the complications that might ensue. Endoscopic removal is the preferred choice of treatment but for large foreign body, sharp foreign body, and so on, rigid esophagoscopic removal might be more preferable. Foreign body esophagus though an obvious situation might at times be missed. It is important to make an early definitive diagnosis. We report a unique case of missed foreign body (denture) esophagus despite the obvious signs and symptoms. Definitive diagnosis was made only after 6 years due to the lack of definitive diagnostic procedures and expertise. The foreign body was impacted in the mucosal wall of the esophagus requiring Gastric resection and anastomosis (with McKeown procedure). With this we have tried to highlight the pitfalls in the diagnosis and management of foreign body esophagus. We report a case of a 55-year-old female who presented to the Emergency Room with history of progressive dysphagia and odynophagia for 6 years which was aggravated for the past 6 months. A radiological diagnosis was made. It was followed by a failed attempt of endoscopic removal which warranted the surgical removal of the foreign body.


2021 ◽  
Vol 108 (Supplement_2) ◽  
Author(s):  
M Cuthbert ◽  
R Cuthbert ◽  
S Karamsadkar ◽  
A Minicozzi ◽  
C Chan

Abstract Introduction Mixed adenoneuroendocrine carcinoma (MANEC) is a rare neoplasm with dual adenocarcinomatous and neuroendocrine differentiation. Subgroup analysis demonstrates an increased frequency of both adenocarcinomas and neuroendocrine tumours in patients with Crohn’s disease (CD), though the incidence of MANEC is unknown. Method A 58-year-old male with a 31-year history of CD presented with small bowel obstruction. After failed conservative management, the patient underwent right hemicolectomy, with subsequent histology demonstrating MANEC. A literature search was performed to identify further cases of patients with concomitant MANEC and CD. Results 11 cases were identified. The mean duration of CD before presentation was 19.5 years, and 58% of cases involved the terminal ileum. 60% of cases demonstrated nodal spread and metastatic disease was evident in 25%. 42% of patients with MANEC were initially treated for an exacerbation of CD. Conclusions MANEC is a rare tumour of uncertain aetiology. The terminal ileum is commonly affected, with most cases exhibiting a longstanding CD history. Diagnosis is challenging, with symptoms of MANEC mirroring exacerbations of CD. Future research should strive to identify imaging modalities or biochemical markers which aid in distinguishing the two pathologies, preventing futile medical management of MANEC, and reducing the risk of metastatic spread due to delayed diagnosis.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Tatsuki Ishikawa ◽  
Katsunori Nakano ◽  
Masafumi Osaka ◽  
Kenichi Aratani ◽  
Kadotani Yayoi ◽  
...  

Abstract Background  Primary neuroendocrine tumors of the gallbladder (GB-NETs) are rare, accounting for 0.5% of all NETs and 2.1% of all gallbladder cancers. Among GB-NETs, mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder (GB-MiNENs) are extremely rare. Case presentation We present the case of a 66-year-old woman who was referred to us for the management of a gallbladder tumor (incidentally found during abdominal ultrasonography indicated for gallbladder stones). The patient had no history of abdominal pain or fever, and the findings on a physical examination were unremarkable. Blood tests showed normal levels of tumor markers. Imaging studies revealed a mass of approximately 10 mm in diameter (with no invasion of the gallbladder bed) located at the fundus of the gallbladder. A gallbladder cancer was suspected. Therefore, an open whole-layer cholecystectomy with regional lymph nodes dissection was performed. The postoperative course was uneventful, and she was discharged on postoperative day 6. Pathological findings showed GB-MiNENs with invasion of the subserosal layer and no lymph node invasion (classified T2aN0M0 pStage IIA according to the Union for International Cancer Control, 8th edition staging system). Analysis of the neuroendocrine markers revealed positive chromogranin A and synaptophysin, and a Ki-67 index above 95%. Fourteen months after the operation, a local recurrence was detected, and she was referred to another hospital for chemotherapy. Conclusions  GB-MiNENs are extremely aggressive tumors despite their tumor size. Optimal therapy should be chosen for each patient.


2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Sulai Liu ◽  
Zhendong Zhong ◽  
Meng Xiao ◽  
Yinghui Song ◽  
Youye Zhu ◽  
...  

Abstract Background The World Health Organization's updated classification of digestive system neuroendocrine tumors in 2010 first proposed the classification of mixed adenoneuroendocrine carcinoma (MANEC). The incidence of biliary malignant tumors with neuroendocrine tumors accounts for less than 1% of all neuroendocrine tumors. Moreover, the incidence of hilar bile duct with MANEC is very rare. Case presentation A 65-year-old female patient came to our hospital for repeated abdominal pain for more than 4 months and skin sclera yellow staining for 1 week. Contrast-enhanced computed tomography imaging and magnetic resonance results suggested a hilar tumor for Bismuth-Corlette Type II. The patient underwent radical surgery for hilar cholangiocarcinoma. Finally, the patient was diagnosed with hilar bile duct MANEC, staged 1 (pT1N0M0) based on the eighth edition of the AJCC. Histopathology showed that the tumor was a biliary tumor with both adenocarcinoma and neuroendocrine carcinoma. No evidence of recurrence and metastasis after 20 months of follow-up. Conclusions We first reported a MANEC that originated in the hilar bile duct. As far as we known, there were few reports of biliary MANEC, and the overall prognosis was poor. We also found that the higher the Ki-67 index, the worse the prognosis of this type of patient. Radical surgery is the most effective treatment.


2018 ◽  
Vol 10 (1) ◽  
pp. 2018034
Author(s):  
Roberto Antonucci ◽  
Nadia Vacca ◽  
Giulia Boz ◽  
Cristian Locci ◽  
Rosanna Mannazzu ◽  
...  

Severe hypereosinophilia (HE) in children is rare, and its etiological diagnosis is challenging. We describe a case of a 30-month-old boy, living in a rural area, who was admitted to our Clinic with a 7-day history of fever and severe hypereosinophilia. A comprehensive diagnostic work-up could not identify the cause of this condition. On day 6, the rapidly increasing eosinophil count (maximum value of 56,000/mm3), the risk of developing hypereosinophilic syndrome, and the patient’s history prompted us to undertake an empiric treatment with albendazole.The eosinophil count progressively decreased following treatment. On day 13, clinical condition and hematological data were satisfactory, therefore the treatment was discontinued and the patient was discharged. Three months later, anti-nematode IgG antibodies were detected in patient serum, thus establishing the etiological diagnosis. In conclusion, an empiric anthelmintic treatment seems to be justified when parasitic hypereosinophilia is strongly suspected, and other causes have been excluded.


2021 ◽  
Vol 11 (1) ◽  
pp. 1891-1894
Author(s):  
Irene Thomas ◽  
Divya Surendran ◽  
Joy Augustine

Mixed adenoneuroendocrine carcinoma is a rare neoplasm with both epithelial and neuroendocrine components. To date, only a few cases of this neoplasm have been reported in the literature among which gastric mixed adenoneuroendocrine carcinoma is very rare. We are reporting a case of gastric mixed adenoneuroendocrine carcinoma with squamous cell differentiation. Histopathological features, biological behaviour and the treatment of this rare tumour type have been discussed briefly.


2017 ◽  
Vol 31 (1) ◽  
pp. 80-84 ◽  
Author(s):  
D. Adam ◽  
D. Iftimie ◽  
Gina Burduşa ◽  
Cristiana Moisescu

Abstract Background and importance: Butterfly vertebra is an uncommon congenital spinal anomaly, which can be easily mistaken for vertebral fracture, infection or tumor. Clinical presentation: We report the case of a 20-year-old male patient with a 3-year history of intermittent mid-thoracic spinal pain. Local examination showed a mild thoracic kyphoscoliosis. Neurological examination was normal. Computer tomography revealed the presence of a T6 butterfly vertebra associated with morphological anomalies in adjacent vertebral bodies and T6-T9 Schmorl’s nodes. MRI scan confirmed the diagnosis and additionally showed a mild spinal stenosis caused by apparent posterior epidural fat hypertrophy. The patient was treated with painkillers and physical therapy. Conclusion: Butterfly vertebra is a benign condition. Once diagnosed, additional diagnostic procedures are not necessary. Neurosurgeons must be aware of this congenital anomaly that should not be confused with a vertebral fracture.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e13029-e13029
Author(s):  
Sakina Sekkate ◽  
Laure Ladrat ◽  
Christelle Pouliquen ◽  
Celine Callens ◽  
Jean Francois Geay ◽  
...  

e13029 Background: First-line treatment with a CDK4/6 inhibitor in combination with an aromatase inhibitor for HR+/HER2- postmenopausal metastatic breast cancer (mBC) is considered as a standard of care based on the compelling data across all landmark trials. Methods: We report our experience about two cases with metastatic breast cancer treated by CDK4/6 inhibitor in combination with an aromatase inhibitor, which develop neuroendocrine differentiation. After observing an unusual massive progression, a biopsy of the metastasis was performed in both patients. Both histological analysis confirmed a lobular breast cancer with small cell neuroendocrine features and a high proliferation index (KI 67 90%). An NGS (Next generation sequencing) profile was realized on primary and metastatic tumors to detect new acquired genomic alterations. Results: The comparative analysis of the NGS results of primary and metastatic tumors revealed the occurrence of several events in both patients under treatment. For one patient, we identified new mutations in the genes CDH1, FANCG, PIK3CA, PTEN and TP53, which did not exist initially. For the second patient, we found an amplification of the segment containing FGFR1, CCND1, FGF4, FGF3, FADD genes and the deletion of the segment containing RB1 andTP53 genes. Such events were not observed on the primary tumor. Conclusions: Prostate adenocarcinoma may develop neuroendocrine features in later stages of castration-resistant prostate cancer but neuroendocrine differentiation is atypical in breast carcinoma. In case of unusual rapidly progressive disease after CDK4/6 inhibitor, biopsy of new metastases should be recommanded to search neuroendocrine differentiation, or druggable mutations to guide the treatment.


PEDIATRICS ◽  
1950 ◽  
Vol 6 (6) ◽  
pp. 897-907
Author(s):  
ARTHUR R. C. COLE

A brief review of the natural history of peptic ulcer in infancy and childhood is given. The symptomatology, differential diagnosis and diagnostic procedures are presented. The diagnosis of peptic ulcer should be considered more often in infancy and childhood and the proper diagnostic procedures instituted. A case of gastric ulcer simulating hypertrophic pyloric stenosis is presented. A summary of 31 cases with peptic ulcers found at autopsy at the Babies Hospital from January 1, 1924 to January 1, 1950 is given. From a summary of coexistent disease found in the autopsy cases at the Babies Hospital and reports from the recent literature, it would appear that diseases of the central nervous, gastrointestinal and respiratory systems are commonly present in cases which have shown peptic ulcer at autopsy.


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