scholarly journals Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Nagesh T. Sirsath ◽  
K. Govind Babu ◽  
Umesh Das ◽  
C. S. Premlatha
Keyword(s):  
Paranasal Sinus ◽  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Differentiation ◽  
Complete Response ◽  
The Body ◽  
Intracranial Extension ◽  
Neuroendocrine Neoplasms ◽  
Rare Site ◽  
Poorly Differentiated

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

scholarly journals INSM1 Is a Highly Specific Marker of Neuroendocrine Differentiation in Primary Neoplasms of the Gastrointestinal Tract, Appendix, and Pancreas

2020 ◽  
Vol 153 (6) ◽  
pp. 811-820 ◽  
Author(s):  
Kelsey E McHugh ◽  
Sanjay Mukhopadhyay ◽  
Erika E Doxtader ◽  
Christopher Lanigan ◽  
Daniela S Allende
Keyword(s):  
Goblet Cell ◽  
Neuroendocrine Differentiation ◽  
Neuroendocrine Neoplasms ◽  
Specific Marker ◽  
Low Grade ◽  
Ki 67 ◽  
Neuroendocrine Markers ◽  
Primary Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated

Abstract Objectives INSM1 has been described as a sensitive and specific neuroendocrine marker. This study aims to compare INSM1 with traditional neuroendocrine markers in gastrointestinal neuroendocrine neoplasms. Methods Retrospective review (2008-2018) was used to retrieve paraffin-embedded tissue from 110 gastrointestinal neuroendocrine neoplasms and controls that was subsequently stained with INSM1, synaptophysin, chromogranin, CD56, and Ki-67. Results INSM1 was positive in 16 of 17 (94.1%) gastric, 17 of 18 (94.4%) pancreatic, 13 of 18 (72.2%) small bowel, 17 of 21 (81.0%) colonic, and 26 of 36 (72.2%) appendiceal tumors. INSM1 was positive in 58 of 70 (82.9%) well-differentiated neuroendocrine tumors, 17 of 20 (85.0%) poorly differentiated neuroendocrine carcinomas, 8 of 11 (72.7%) low-grade goblet cell adenocarcinomas (grade 1), and 6 of 9 (66.7%) high-grade goblet cell adenocarcinomas (grade 2/3). INSM1 sensitivity for neuroendocrine neoplasms (80.9%) was less than that of synaptophysin (99.1%), chromogranin (88%), and CD56 (95.3%); specificity was higher (95.7% vs 86.0%, 87.3%, and 86.0%, respectively). Conclusions INSM1 is a useful marker of neuroendocrine differentiation in gastrointestinal neuroendocrine and mixed neuroendocrine neoplasms. Compared with traditional neuroendocrine markers, INSM1 is less sensitive but more specific.

First-Line Irinotecan Combined with 5-Fluorouracil and Leucovorin for High-Grade Metastatic Gastrointestinal Neuroendocrine Carcinoma

2013 ◽  
Vol 99 (1) ◽  
pp. 57-60 ◽  
Author(s):  
Zedong Du ◽  
Yi Wang ◽  
Yi Zhou ◽  
Feng Wen ◽  
Qiu Li
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Progression Free Survival ◽  
Disease Stage ◽  
Neuroendocrine Neoplasms ◽  
High Grade ◽  
First Line ◽  
Laboratory Findings ◽  
Poorly Differentiated ◽  
Prospective Trials ◽  
Line Setting

Aim and background High-grade gastrointestinal neuroendocrine neoplasms, ie, poorly differentiated neuroendocrine carcinomas, with no effective therapeutic approaches, have a high ability to metastasize. Methods A review of the hospital information system was performed. Patients with histologically proven gastrointestinal neuroendocrine carcinoma who were treated with irinotecan combined with 5-fluorouracil and leucovorin in a first-line setting were eligible for analysis. We extracted information on age, sex, disease stage, laboratory findings, radiological findings, pathological findings, chemotherapy, effectiveness and adverse events of therapy, and outcomes. Results Eleven patients were included in the study. Partial response was observed in 7 patients. Median progression-free survival and overall survival were 6.5 (95% CI, 5.1–7.9) and 13.0 (95% CI, 9.8–16.2) months, respectively. No treatment-related deaths occurred. Conclusions The results demonstrated that irinotecan combined with 5-fluorouracil and leucovorin is an active regimen with acceptable toxicity for patients with metastatic high-grade gastointestinal neuroendocrine carcinoma that merits further investigation in prospective trials.

scholarly journals Poorly differentiated neuroendocrine rectal carcinoma with uncommon immune-histochemical features and clinical presentation with a subcutaneous metastasis, treated with first line intensive triplet chemotherapy plus bevacizumab FIr-B/FOx regimen: an experience of multidisciplinary management in clinical practice

BMC Cancer ◽  
2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Gemma Bruera ◽  
◽  
Antonio Giuliani ◽  
Lucia Romano ◽  
Alessandro Chiominto ◽  
...  
Keyword(s):  
Rectal Carcinoma ◽  
Neuroendocrine Carcinoma ◽  
Locally Advanced ◽  
Complete Response ◽  
Neuroendocrine Cells ◽  
Perineal Pain ◽  
Pathological Examination ◽  
First Line ◽  
Triplet Chemotherapy ◽  
Poorly Differentiated

Abstract Background Neuroendocrine tumors (NETs) are heterogeneous, widely distributed tumors arising from neuroendocrine cells. Gastrointestinal (GI)-NETs are the most common and NETs of the rectum represent 15, 2% of gastrointestinal malignancies. Poorly differentiated neuroendocrine carcinomas of the GI tract are uncommon. We report a rare case of poorly differentiated locally advanced rectal neuroendocrine carcinoma with nodal and a subcutaneous metastasis, with a cytoplasmic staining positive for Synaptophysin and Thyroid Transcription Factor-1. Case presentation A 72-year-old male presented to hospital, due to lumbar, abdominal, perineal pain, and severe constipation. A whole-body computed tomography scan showed a mass of the right lateral wall of the rectum, determining significant reduction of lumen caliber. It also showed a subcutaneous metastasis of the posterior abdominal wall. Patient underwent a multidisciplinary evaluation, diagnostic and therapeutic plan was shared and defined. The pathological examination of rectal biopsy and subcutaneous nodule revealed features consistent with small-cell poorly differentiated neuroendocrine carcinoma. First line medical treatment with triplet chemotherapy and bevacizumab, according to FIr-B/FOx intensive regimen, administered for the first time in this young elderly patient affected by metastatic rectal NEC was highly active and tolerable, as previously reported in metastatic colo-rectal carcinoma (MCRC). A consistent rapid improvement in clinical conditions were observed during treatment. After 6 cycles of treatment, CT scan and endoscopic evaluation showed clinical complete response of rectal mass and lymph nodes; patient underwent curative surgery confirming the pathologic complete response at PFS 9 months. Discussion and conclusions This case report of a locally advanced rectal NEC with an unusual subcutaneous metastasis deserves further investigation of triplet chemotherapy-based intensive regimens in metastatic GEP NEC.

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

2011 ◽  
Vol 18 (S1) ◽  
pp. S1-S16 ◽  
Author(s):  
Günter Klöppel
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Stage ◽  
Neuroendocrine Neoplasms ◽  
Gastroenteropancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

Neuroendocrine neoplasms of the pancreas at dynamic enhanced CT: comparison between grade 3 neuroendocrine carcinoma and grade 1/2 neuroendocrine tumour

2014 ◽  
Vol 25 (5) ◽  
pp. 1375-1383 ◽  
Author(s):  
Dong Wook Kim ◽  
Hyoung Jung Kim ◽  
Kyung Won Kim ◽  
Jae Ho Byun ◽  
Ki Byung Song ◽  
...  
Keyword(s):  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Neoplasms ◽  
Enhanced Ct ◽  
Grade 3

scholarly journals Neuroendocrine neoplasms of lung, pancreas and gut: a morphology-based comparison

2020 ◽  
Vol 27 (11) ◽  
pp. R417-R432
Author(s):  
Atsuko Kasajima ◽  
Günter Klöppel
Keyword(s):  
Detailed Comparison ◽  
The Body ◽  
Neuroendocrine Neoplasms ◽  
Genetic Changes ◽  
Organ Systems ◽  
Hereditary Tumor Syndromes ◽  
Poorly Differentiated ◽  
Therapy Strategies ◽  
Genetic Profiles ◽  
Therapeutical Options

The bronchopulmonary (BP) and gastroenteropancreatic (GEP) organ systems harbor the majority of the neuroendocrine neoplasms (NENs) of the body, comprising 20 and 70% of all NENs, respectively. Common to both NEN groups is a classification distinguishing between well- and poorly differentiated NENs associated with distinct genetic profiles. Differences between the two groups concern the reciprocal prevalence of well and poorly differentiated neoplasms, the application of a Ki67-based grading, the variety of histological patterns, the diversity of hormone expression and associated syndromes, the variable involvement in hereditary tumor syndromes, and the peculiarities of genetic changes. This review focuses on a detailed comparison of BP-NENs with GEP-NENs with the aim of highlighting and discussing the most obvious differences. Despite obvious differences, the principle therapeutical options are still the same for both NEN groups, but with further progress in genetics, more targeted therapy strategies can be expected in future.

scholarly journals P124 Gastroenteropancreatic Neuroendocrine Neoplasms in patients with Inflammatory Bowel Disease: An ECCO CONFER Multicentre Case Series

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S215-S216
Author(s):  
S Festa ◽  
G Zerboni ◽  
L Derikx ◽  
D G Ribaldone ◽  
G Dragoni ◽  
...  
Keyword(s):  
Neuroendocrine Tumour ◽  
Case Reports ◽  
Case Series ◽  
The Body ◽  
Neuroendocrine Neoplasms ◽  
Case Report Form ◽  
Related Factors ◽  
Common Location ◽  
Inflammatory Bowel

Abstract Background Neuroendocrine Neoplasms (NENs) are a heterogeneous group of tumours deriving from the diffuse endocrine system. NENs may occur almost everywhere in the body but are most common in the gastrointestinal tract, the pancreas, and the lungs, with gastroenteropancreatic (GEP) tumours representing 70% of all NENs. GEP-NENs have rarely been reported in association with inflammatory bowel diseases (IBDs) but no definitive relationship between these tumours and IBD has been established Methods This was an ECCO COllaborative Network For Exceptionally Rare case reports project (ECCO-CONFER). We included cases of GEP-NENs diagnosed in patients with IBD that met the diagnostic criteria for NEN according to the European Neuroendocrine Tumour Society. Data were retrospectively collected in a standardized case report form and analysed for event association with patient’s and IBD-related factors Results GEP-NEN was diagnosed in 100 patients with IBD [61% female, 55% Crohn’s disease, median age 48 years (IQR 37–59)]. Overall the most common location was the appendix (39/100) followed by the colon (22/100). Complete IBD-related data was available for 50 individuals with a median follow-up of 30.5 months (IQR 11.2–70) following NEN diagnosis. At the last follow-up data, 47/50 patients were alive. Three deaths occurred, of which 2 were related to NEN. Median duration of IBD at NEN diagnosis was 84 months (IQR 10–151), and in 18% of cases NEN and IBD were diagnosed concomitantly. 20/50 of NENs were at stage I (T1N0M0) and 28/50 graded G1 (ki 67 ≤2 %) at diagnosis. Incidental diagnosis of NEN either during follow-up or during surgery as well as receiving diagnosis of NEN concomitantly with IBD was significantly associated with an earlier NEN stage (p&lt; 0.01 and p&lt;0.02, respectively). Exposure to immunomodulatory and/or biologic therapy was not associated with advanced NEN stage or grade. Interestingly, primary GEP-NEN sites significantly correlated to the segment affected by IBD (62% vs 38% p = 0.02) Conclusion In the largest case series to date, prognosis of patients with concomitant GEP-NEN and IBD seems favorable. Incidental NEN diagnosis correlates with an earlier NEN stage and IBD-related therapies are independent of NEN stage and grade. The association of GEP-NEN location and the segment affected by IBD may suggest a possible role of inflammation in NEN tumorigenesis

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 NEUROENDOCRINE CARCINOMA AS SINONASAL MALIGNANCY

2019 ◽  
Vol VOLUME 7 (VOLUME 7 NUMBER 2 NOV 2018) ◽  
pp. 30-34
Author(s):  
Nitya Singh
Keyword(s):  
Nasal Cavity ◽  
Neuroendocrine Carcinoma ◽  
Histopathological Examination ◽  
Neuroendocrine Differentiation ◽  
Imaging Study ◽  
High Recurrence Rate ◽  
Neuroendocrine Neoplasms ◽  
Left Nasal Cavity ◽  
Intravenous Contrast ◽  
Rare Malignancy

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. Extremely rare site for neuroendocrine carcinoma are nasal cavity and paranasal sinuses and are aggressive neoplasm with a high recurrence rate and a tendency to metastasize. A 19-year male presented to the department of Otorhinolaryngology with left nasal obstruction, persistent headache for nine months and on and off bleeding from left nasal cavity for 2 months. On examination of the nasal cavity, a gross deviation of nasal septum with a red, friable, gelatinous, polypoidal mass with a tendency to bleed was seen in the left nasal cavity. The patient underwent endoscopic excision of mass under GA. Specimen was sent for histopathological examination which exhibited the properties of Neuro-endocrine carcinoma. Sinonasal NEC is an extremely rare malignancy. CT scan with intravenous contrast is the most effectual early imaging study, additionally MRI will further make a fine description of the tumor extension. Furthermore, Immunohisto-chemistry is an important tool that can be valuable in reaching a diagnosis. There is no clear recommendation regarding the treatment of the sinonasal neuroendocrine carcinoma, but the multimodal approach with proper counselling of patient is favoured and generally accepted.

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