Clinico-pathologic Features of Esophageal Poorly Differentiated Neuroendocrine Carcinomas, a Case Series and Review of Literature

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S64-S64
Author(s):  
I Perveze ◽  
D Rao
Keyword(s):  
Metastatic Disease ◽  
Correct Diagnosis ◽  
Case Series ◽  
Disease Process ◽  
Malignant Neoplasm ◽  
Distal Esophagus ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Pathologic Features ◽  
Poorly Differentiated ◽  
Neuroendocrine Carcinomas

Abstract Introduction/Objective Esophageal neuroendocrine carcinomas (NECs) constitute an extremely rare and distinct group of neoplasms. The clinico-pathologic characteristics are poorly understood and differ starkly from high grade well differentiated neuroendocrine tumors. Only one case series describing clinico-pathologic features of NECs has been described in recent literature. Methods We describe a case series of three patients highlighting the typical presentation, diagnostic techniques and pathologic attributes for this rare disease process. Results All three patients were middle age to elderly with two males (ages 58 and 83) and one female (age 72). The typical presentation was dysphagia (2/3, 66%) followed by severe GERD (1/3, 33%). Typical locations were distal esophagus (2/3, 66%) and gastro-esophageal junction (1/3, 33%). Computed tomography showed bulky masses in all patients (3/3, 100%) with median size of 5.6 cm. PET imaging showed metastatic disease (mediastinal or epigastric lymphadenopathy and lung metastasis) in 2/3, 66% of the patients. All the patients were found to have necrotic masses on EGD with biopsy showing poorly differentiated neuroendocrine carcinoma in all (3/3, 100%). Immuno- histochemical stains revealed Synaptophysin positivity in 3/3, 100%, Chromogranin in 1/3, 33% and CD56 in 1/3, 33% of the patients. Patients with metastatic disease at presentation (2/3, 66%) opted for palliative care while the patient without any evidence of metastasis (1/3, 33%) underwent surgical resection followed by chemotherapy. Conclusion ENCs are an exceedingly rare group of malignant neoplasm with variable presentations posing a diagnostic challenge for the clinician and pathologist alike. Most patients tend to be elderly with advanced disease at presentation and guarded prognosis. This case series underscores the importance of including this entity among the differential diagnosis for poorly differentiated carcinomas of the distal esophagus. IHC with positive neuroendocrine markers particularly Synaptophysin helps make the correct diagnosis when combined with typical morphology.

CDX2 Is a Useful Marker of Intestinal-Type Differentiation: A Tissue Microarray–Based Study of 629 Tumors From Various Sites

2005 ◽  
Vol 129 (9) ◽  
pp. 1100-1105 ◽  
Author(s):  
Lindsey B. De Lott ◽  
Carl Morrison ◽  
Saul Suster ◽  
David E. Cohn ◽  
Wendy L. Frankel
Keyword(s):  
Squamous Cell ◽  
Signet Ring Cell ◽  
Squamous Cell Carcinomas ◽  
Intestinal Type ◽  
High Grade ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Lung Adenocarcinomas ◽  
Colorectal Adenocarcinomas ◽  
Neuroendocrine Carcinomas

Abstract Context.—CDX2, a critical nuclear transcription factor for intestinal development, is expressed in intestinal epithelium and adenocarcinomas. Objectives.—To determine if CDX2 is a useful marker for intestinal-type differentiation and to correlate tumor histology with CDX2 staining in colorectal adenocarcinomas. Design.—Tissue microarrays from 71 colorectal adenocarcinomas, 31 hepatocellular carcinomas, 47 lung adenocarcinomas, 55 squamous cell carcinomas of the lung, 69 neuroendocrine carcinomas of the lung and 43 of the pancreas, 57 pancreatic adenocarcinomas, and 256 endometrial adenocarcinomas were stained with antibody against CDX2. Results.—CDX2 staining was positive in 51 (71.8%) of 71 colorectal cancers, including 38 (74.5%) of 51 well- or moderately differentiated tumors and 13 (65.0%) of 20 high-grade tumors. Of the high-grade tumors, 5 (71.4%) of 7 mucinous, 3 (100%) of 3 signet ring cell, and 5 (50.0%) of 10 poorly differentiated tumors were positive. Other tumors showing occasional CDX2 staining included 1 of 30 well- or moderately differentiated neuroendocrine carcinomas of the lung and 2 of 43 from the pancreas, 1 of 47 lung adenocarcinomas, 3 of 57 pancreatic adenocarcinomas, and 15 of 256 endometrial carcinomas. Hepatocellular, poorly differentiated neuroendocrine carcinoma of the lung and squamous cell carcinomas of the lung were not immunoreactive for CDX2. Conclusions.—CDX2 is a useful marker for intestinal-type differentiation, is rarely seen in tumors from the other sites evaluated, and may be useful in determining the site of origin for some metastatic tumors. However, CDX2 is not a sensitive marker for poorly differentiated colorectal carcinoma.

scholarly journals Ki67 proliferative index of the neuroendocrine component drives MANEC prognosis

2018 ◽  
Vol 25 (5) ◽  
pp. 583-593 ◽  
Author(s):  
Massimo Milione ◽  
Patrick Maisonneuve ◽  
Alessio Pellegrinelli ◽  
Federica Grillo ◽  
Luca Albarello ◽  
...  
Keyword(s):  
Digestive System ◽  
Multivariable Analysis ◽  
Prognostic Index ◽  
Primary Site ◽  
Beta Catenin ◽  
Ki67 Index ◽  
Risk Of Death ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Pathologic Features ◽  
Poorly Differentiated

Mixed adenoneuroendocrine carcinomas (MANECs) are composed of a poorly differentiated neuroendocrine carcinoma (NEC) and a non-neuroendocrine (non-NEC) neoplastic epithelial component, each representing at least 30% of the tumor. At present, prognostic factors for MANECs remain largely unexplored. We investigated the clinical-pathologic features of a large multicenter series of digestive system MANECs. Surgical specimens of 200 MANEC candidates were centrally reviewed; diagnosis was confirmed in 160 cases. While morphology, proliferation (mitotic count (MC), Ki67 index) and immunophenotype (p53, SSTR2a, beta-Catenin, Bcl-2, p16, Rb1, ALDH, mismatch repair proteins and CD117) were investigated separately in both components, genomic (TP53,KRAS,BRAF) alterations were searched for on the entire tumor. Data were correlated with overall survival (OS). MANEC sites were: 92 colorectal, 44 gastroesophageal and 24 pancreatobiliary. Median OS was 13.2 months. After adjustment for primary site, Ki67 index of the NEC component (but not of the non-NEC component) was the most powerful prognostic marker. At multivariable analysis, patients with Ki67 ≥ 55% had an 8-fold risk of death (hazard ratio (HR) 7.83; 95% confidence interval (CI) 4.17–14.7;P < 0.0001) and a median OS of 12.2 months compared to those with Ki67 < 55% (median OS 40.5 months). MC (HR 1.51; 95% CI 1.03–2.20,P = 0.04) was a weaker prognostic index. Colorectal primary site (HR 1.60; 95% CI 1.11–2.32;P = 0.01) was significantly associated with poorer survival. No single immunomarker, in either component, was statistically significant. This retrospective analysis of a large series of digestive system MANECs, showed that the NEC component, particularly its Ki67 index, was the main prognostic driver.

scholarly journals The Lung Mass and nodule: a case series

2021 ◽  
Author(s):  
Azam Jan ◽  
Naseer Ahmed ◽  
Nabil Iftikhar Awan ◽  
Bahauddin Khan ◽  
Mujahid-ul Islam ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Correct Diagnosis ◽  
Case Series ◽  
Female Child ◽  
Lung Mass ◽  
Radiological Findings ◽  
X Ray ◽  
Smoking Exposure ◽  
Poorly Differentiated ◽  
Timely Treatment

Lung mass is an abnormal region of 3 cm or more in size present in the lungs mainly due to underlying pulmonary caner. It is usually round, opaque and poorly differentiated on X-ray. Common etiological key players are smoking, exposure to asbestos, radon, however, familial history may also play a role. We presented retrospectively 7 cases of lung mass and nodule encountered during our clinical practice. We have discussed their clinical presentation, manifestation, medical history, radiological findings and differential diagnosis. In this case series, most of the patients were young, only 2 cases were older patients. There was one infant one month old, one female child 12 years old, one female 25 years, 2 males, 22 and 21 years, one male of 50 years and another male of 60 years age. Correct diagnosis on the basis of clinical profile, radiological findings and histology may help in proper management and hence, timely treatment of the patient.

Pulmonary Neuroendocrine Carcinomas

2002 ◽  
Vol 126 (5) ◽  
pp. 545-553 ◽  
Author(s):  
Qin Huang ◽  
Alona Muzitansky ◽  
Eugene J. Mark
Keyword(s):  
Neuroendocrine Tumors ◽  
Neuroendocrine Carcinoma ◽  
Large Cell ◽  
Small Cell ◽  
The Body ◽  
Low Grade ◽  
Typical Carcinoid ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Neuroendocrine Carcinomas

Abstract Context.—Primary pulmonary neuroendocrine tumors are traditionally classified into 3 major types: typical carcinoid (TC), atypical carcinoid (AC), and large cell neuroendocrine carcinoma (LC) or small cell neuroendocrine carcinoma (SC). Confusion arises frequently regarding the malignant nature of TC and the morphologic differentiation between AC and LC or SC. Objective.—To provide clinicopathologic evidence to streamline and clarify the histomorphologic criteria for this group of tumors, emphasizing the prognostic implications. Patients.—To minimize variability in diagnostic criteria and treatment plans, we analyzed a group of patients whose diagnosis and treatment occurred at a single institution. We reviewed 234 cases of primary pulmonary neuroendocrine tumors and thoroughly studied 50 cases of resected tumors from 1986 to 1995. Results.—On the basis of morphologic characteristics and biologic behaviors of the tumors, we agree with many previous investigators that these tumors are all malignant and potentially aggressive. Based on our accumulated data, we have modified Gould criteria and reclassified these tumors into 5 types: (1) well-differentiated neuroendocrine carcinoma (otherwise called TC) (14 cases, with less than 1 mitosis per 10 high-power fields [HPF] with or without minimal necrosis); (2) moderately differentiated neuroendocrine carcinoma (otherwise called low-grade AC) (6 cases, with less than 10 mitoses per 10 HPF and necrosis evident at high magnification); (3) poorly differentiated neuroendocrine carcinoma (otherwise called high-grade AC) (10 cases, with more than 10 mitoses per 10 HPF and necrosis evident at low-power magnification); (4) undifferentiated LC (5 cases, with more than 30 mitoses per 10 HPF and marked necrosis); and (5) undifferentiated SC (15 cases, with more than 30 mitoses per 10 HPF and marked necrosis). The 5-year survival rates were 93%, 83%, 70%, 60%, and 40% for well, moderately, and poorly differentiated, and undifferentiated large cell and small cell neuroendocrine carcinomas, respectively. We found nodal metastasis in 28% of TC in this retrospective review, a figure higher than previously recorded. Conclusion.—Using a grading system and terms comparable to those used for many years and used for neuroendocrine tumors elsewhere in the body, we found that classification of pulmonary neuroendocrine carcinomas as well, moderately, poorly differentiated, or undifferentiated provides prognostic information and avoids misleading terms and concepts. This facilitates communication between pathologists and clinicians and thereby improves diagnosis and management of the patient.

scholarly journals Comprehensive analysis of mutational and clinicopathologic characteristics of poorly differentiated colorectal neuroendocrine carcinomas

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sun Mi Lee ◽  
Chang Ohk Sung
Keyword(s):  
Overall Survival ◽  
Survival Rates ◽  
Driver Genes ◽  
Clinicopathologic Characteristics ◽  
Targeted Next Generation Sequencing ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Oncogenic Driver ◽  
Generation Sequencing ◽  
Neuroendocrine Carcinomas

AbstractPoorly differentiated neuroendocrine carcinoma (NEC) is a rare subtype of colorectal cancer (CRC). This study aimed to investigate clinicopathologic characteristics of colorectal NECs and elucidate genomic differences and similarities between colorectal NECs and colorectal adenocarcinomas (ACs). A total of 30 colorectal NECs were screened for frequently identified CRC oncogenic driver genes by targeted next-generation sequencing of 382 genes. The median age of the patients was 67 years (range, 44 to 88 years). NECs occurred predominantly in the rectum (47%) and exhibited multiple adverse prognostic pathologic factors, including frequent lymphatic and vascular invasions, high rates of lymph node metastasis and distant metastasis and advanced TNM stage. The 1-, 3-, and 5-year overall survival rates of NEC patients were 46.7%, 36.4%, and 32.7%, respectively, with a median overall survival period of 11.5 months. In a molecular analysis, NECs showed high rates of BRAF mutation (23%), predominantly p.V600E (71%), and alterations in RB1 (47%), particularly deletion (57%). The frequencies and distributions of other genes, such as KRAS, APC, SMAD4, and PIK3CA, and microsatellite instability status were similar to those of ACs. These findings provide beneficial information for selecting therapeutic options, including targeted therapy, and a better understanding of the histogenesis of this tumour.

scholarly journals Perioperative Chemotherapy in Poorly Differentiated Neuroendocrine Neoplasia of the Bladder: A Multicenter Analysis

2020 ◽  
Vol 9 (5) ◽  
pp. 1351 ◽  
Author(s):  
Giuseppe Lamberti ◽  
Maria Pia Brizzi ◽  
Sara Pusceddu ◽  
Fabio Gelsomino ◽  
Giovanni Di Meglio ◽  
...  
Keyword(s):  
Metastatic Disease ◽  
Performance Status ◽  
Tumor Resection ◽  
Stage Iv ◽  
Progression Free Survival ◽  
Free Survival ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Multicenter Analysis ◽  
Poorly Differentiated ◽  
Carcinoma Of The Bladder

There is scant evidence about optimal management of poorly differentiated neuroendocrine carcinoma of the bladder (BNEC). We performed a multicenter retrospective study on BNEC patients from 13 Italian neuroendocrine-dedicated centers to analyze strategies associated with better outcomes. Mixed adeno-neuroendocrine carcinomas (MANEC) were included. We analyzed overall survival (OS) in the overall cohort, relapse-free survival (RFS) in radically operated patients and progression-free survival (PFS) in patients who received chemotherapy for metastatic disease. Fifty-one BNEC patients were included (male: 46, median age: 70 years). Overall, median OS was 16.0 months, radical tumor resection was performed in 37 patients (72.5%) and 11 of these (29.7%) also received peri-operative platinum-etoposide chemotherapy. Median OS was longer in patients with better performance status (PS) and in those with stage I–III disease at diagnosis compared to stage IV. Among patients who underwent radical tumor resection (N = 37), RFS was longer in patients with better PS and showed a trend towards a longer RFS in those treated with peri-operative chemotherapy than with surgery alone (11 vs. 6 months; p = 0.078). Among 28 patients receiving chemotherapy for metastatic disease, PFS was 5.0 months and there was a trend towards improved PFS in patients receiving carboplatin-etoposide chemotherapy compared to other regimens. A multivariate model unmasked a significant association between carboplatin-etoposide chemotherapy and risk for disease progression or death (HR: 0.39 (95%CI: 0.16–0.96) p = 0.040). Performance status might be associated with improved RFS in radically operated patients, while type of chemotherapy might affect PFS in patients receiving chemotherapy for metastatic BNEC.

scholarly journals Large Cell Neuroendocrine Carcinoma of the Rectum Presenting with Extensive Metastatic Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Vinay Minocha ◽  
Sania Shuja ◽  
Robert Ali ◽  
Emely Eid
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Metastatic Disease ◽  
Lower Abdominal Pain ◽  
Large Cell ◽  
Large Cell Neuroendocrine Carcinoma ◽  
Pathological Examination ◽  
Resected Specimen ◽  
Rectal Mass ◽  
Poorly Differentiated ◽  
Neuroendocrine Carcinomas

Introduction. Rectal large cell neuroendocrine carcinoma (LCNEC) is a poorly differentiated neoplasm that is very rare and belongs within the poorest prognostic subgroup among primary colorectal neoplasms. Here, we describe a case of LCNEC of the rectum, which highlights the aggressive clinical course and poor prognosis associated with this disease.Case Presentation. We report a case of a 63-year-old male who presented to our hospital with a one-month history of lower abdominal pain, constipation, and weight loss. A computed tomography (CT) scan of the chest, abdomen, and pelvis revealed a rectal mass as well as metastatic disease of the liver and lung. Flexible sigmoidoscopy revealed a fungating, ulcerated and partially obstructing rectal mass located 6 cm from the anal verge. This mass was biopsied and pathological examination of the resected specimen revealed features consistent with a large cell neuroendocrine carcinoma.Conclusion. Rectal large cell neuroendocrine carcinomas are rare and have a significantly worse prognosis than adenocarcinomas. At diagnosis, a higher stage and metastatic disease are likely to be found. It is important to differentiate large cell, poorly differentiated neuroendocrine carcinomas from adenocarcinomas of the colon and rectum pathologically because patients may benefit from alternative cytotoxic chemotherapeutic regimens.

How endoscopic ultrasound (eus) was decisive to establish the correct diagnosis of patients – a case series

2019 ◽  
Author(s):  
M Razpotnik ◽  
S Bota ◽  
G Essler ◽  
J Weber-Eibel ◽  
M Peck-Radosavljevic
Keyword(s):  
Endoscopic Ultrasound ◽  
Correct Diagnosis ◽  
Case Series

Aumento de coroa estética sem elevação de retalho – abordagem minimamente invasiva

2020 ◽  
Vol 12 (45) ◽  
pp. 34-39
Author(s):  
Flavia Sukekava ◽  
Julia Helena Luiz ◽  
Paloma Palma ◽  
Jaques Luiz
Keyword(s):  
Minimally Invasive ◽  
Correct Diagnosis ◽  
Case Series ◽  
Minimally Invasive Techniques ◽  
Bone Removal ◽  
Use Of Technology ◽  
Treatment Plans ◽  
Invasive Techniques ◽  
Crown Lengthening ◽  
Gummy Smile

Gummy smile is a characteristic in which the patient exposes more than 2 mm of keratinized gingiva in forced smile. With a multifactorial cause, its correct planning depends directly on the correct diagnosis. Usually, the procedures that involve manipulation and the enlargement of the aesthetical crown lengthening are surgical. The objective of this case series was to show the advantages of surgical crown augmentation surgery in aesthetic areas with minimally invasive techniques. Three cases of gummy smile were presented, with different treatment plans and techniques for execution. In the 3 cases, bone removal was performed with piezoelectric ultrasound, which made the postoperative more comfortable for patients. These cases illustrate the use of technology to reduce morbidity in patients who need to undergo bone removal to treat gingival smile.

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