Management of Neuroendocrine Tumors of Unknown Origin

2011 ◽  
Vol 9 (12) ◽  
pp. 1397-1402 ◽  
Author(s):  
Ariel Polish ◽  
Maxwell T. Vergo ◽  
Mark Agulnik
Keyword(s):  
Family History ◽  
Neuroendocrine Tumors ◽  
Primary Tumor ◽  
Unknown Origin ◽  
Small Cell ◽  
Carcinoid Tumors ◽  
Primary Malignancy ◽  
Poorly Differentiated

Neuroendocrine tumors (NETs) of unknown origin account for more than 10% of all NETs. Most of these tumors are poorly differentiated and, thus, very aggressive. Establishing the location of the primary tumor can be challenging. Workup of these NETs of unknown origin includes a thorough family history, immunohistochemistry, imaging, and OctreoScan. If the location of the primary malignancy is not determined, treatment is often initiated based on the grade and level of differentiation of the tumor, with well- and moderately differentiated tumors treated as carcinoid tumors, whereas poorly differentiated tumors are treated similarly to small cell tumors. Therapy is chosen based on symptoms and with the goal of debulking tumor when feasible and safe.

scholarly journals Poorly Differentiated Small-Cell-Type Neuroendocrine Carcinoma of the Prostate: A Case Report and Literature Review

2018 ◽  
Vol 11 (3) ◽  
pp. 676-681 ◽  
Author(s):  
Kishore Kumar ◽  
Rafeeq Ahmed ◽  
Chime Chukwunonso ◽  
Hassan Tariq ◽  
Masooma Niazi ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Tumor Grade ◽  
Small Cell ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Cell Type ◽  
Variable Response ◽  
Ki 67 ◽  
Platinum Based Chemotherapy ◽  
Poorly Differentiated

Neuroendocrine cells are widespread throughout the body and can give rise of neuroendocrine tumors due to abnormal growth of the chromaffin cells. Neuroendocrine tumors divide into many subtypes based on tumor grade (Ki-67 index and mitotic count) and differentiation. These tumors can be further divided into secretory and nonsecretory types based on the production of peptide hormone by tumor cells. Poorly differentiated small-cell-type neuroendocrine tumors are one of the subtypes of neuroendocrine tumors. These tumors are less common; however, they tend to be locally invasive and aggressive in behavior with poor overall median survival. Treatment of the nonsecretory small-cell type is modeled to small-cell lung cancer with a regimen consisting of platinum-based chemotherapy and etoposide with variable response. Here, we present a case of poorly differentiated small-cell neuroendocrine tumor originating from the prostate.

scholarly journals Metastatic Extrapulmonary Small Cell Carcinoma to the Cerebellopontine Angle: A Case Report and Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-6
Author(s):  
Debebe Theodros ◽  
C. Rory Goodwin ◽  
Genevieve M. Crane ◽  
Jason Liauw ◽  
Lawrence Kleinberg ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Surgical Resection ◽  
Small Cell Carcinoma ◽  
Vestibular Schwannoma ◽  
Primary Tumor ◽  
Cerebellopontine Angle ◽  
Internal Auditory Canal ◽  
Small Cell ◽  
Poorly Differentiated ◽  
Small Intestinal

Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA.

scholarly journals Digestive Neuroendocrine Tumors about 40 Cases

2020 ◽  
Vol 5 (5) ◽  
Keyword(s):  
Neuroendocrine Tumors ◽  
Digestive System ◽  
Clinical Presentation ◽  
Medical Oncology ◽  
Unknown Origin ◽  
Distant Metastases ◽  
Stage 4 ◽  
Female Predominance ◽  
Poorly Differentiated ◽  
Well Differentiated

Neuroendocrine tumors of the digestive system account for 1% of all digestive tumors. These are a group of clinically and biologically heterogeneous neoplasms. The objective of this article is to report the epidemiological, clinical, paraclinical, histological, therapeutic, and progressive characteristics of digestive NETs in our context. Through a study of files over the period from January 2010 to September 2018, 40 cases of NET been collected in the medical oncology department of CHU HASSAN II in Fez. Clinical, paraclinical, therapeutic and evolutionary data were collected. The average age found was 50.42 years. A slight female predominance was noted with a sex ratio of 0.66. The clinical presentation was dominated by non-specific digestive symptoms. Carcinoid syndrome was found in 12.5% of patients. According to the WHO classification: 65% of patients presented well-differentiated NETs versus 30% of poorly differentiated CNE, 5% are undifferentiated NETs. The highest frequency of these tumors was observed in the D-P block (22.5%) and the small intestine (22.5%), followed by NET of unknown origin 15%.The majority of patients presented at an advanced stage (stage 4 in 65%), and the extension assessment already revealed distant metastases, particularly in the liver, and 57.5% of patients received chemotherapy.

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 Case report of atypical carcinoid tumors of the larynx

2018 ◽  
Vol 5 (9) ◽  
pp. 4155
Author(s):  
Fatimah S. Alkhaliah ◽  
Ibrahim A. Almajed ◽  
Anwar S. Alkhalifah ◽  
Abdulltaif S. Alkhalifah ◽  
Khalid A. AlYahya ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Case Report ◽  
Neuroendocrine Tumors ◽  
Histopathological Examination ◽  
Small Cell ◽  
Carcinoid Tumors ◽  
High Rate ◽  
Atypical Carcinoid ◽  
Typical Carcinoid ◽  
Laryngeal Tumors

Neuroendocrine tumors (NET) of the larynx are considered one of the rare disease; they represent only 1% of the laryngeal tumors. These tumors are very aggressive with a high rate of metastases. Diagnosis is done by computed tomography and confirmed by histopathological examination. Neuroendocrine tumors can be classified into four types based on histopathology: typical carcinoid tumors, atypical carcinoid tumors, small cell neuroendocrine tumors and paragangliomas. Atypical carcinoid tumor of the larynx is considered rare in occurrence. We report a rare laryngeal neuroendocrine tumor case.

Surgery for lung cancer as the second primary malignancy

2013 ◽  
Vol 19 (4) ◽  
pp. 421-426
Author(s):  
Arnoldas Krasauskas ◽  
Renatas Aškinis ◽  
Sigitas Zaremba ◽  
Giedrė Smailytė ◽  
Saulius Cicėnas
Keyword(s):  
Lung Cancer ◽  
Cell Carcinoma ◽  
Primary Tumor ◽  
Primary Lung Cancer ◽  
Small Cell ◽  
Second Primary ◽  
Second Primary Malignancy ◽  
Primary Malignancy ◽  
Second Primary Lung Cancer ◽  
Significant Survival

Following a successful treatment of the first oncological disease, the second malignancy can develop. This retrospective study was designed to evaluate the efficacy of surgical treatment results for patients with the second primary lung cancer (2PLC) depending on the site of the first primary tumor (1PT). Materials and methods. From 2005 to 2009, 88 patients (pts) were treated in the Subdivision of Thoracic Surgery and Oncology, Institute of Oncology, Vilnius University, with lung cancer as the second primary malignancy. 29 patients (33%) underwent surgery: 17  pts (58.6%)  –  lobectomies, 2  pts (6.9%) – pneumonectomies, 9 pts (31%) – anatomical segmentectomies and 1 pt (3.5%) – bilobectomy. All lung resections were performed with lymph nodes dissection. We had neither major complications nor mortality 30 days after surgery. Stages of 2PLC were as follows: IA-B stage (st.) – 19 pts (65.5%), IIA-B st. – 7 pts (24.1%), and IIIA-IV st. – 3 pts (10.3%). Lung cancer morphology was the following: squamous cell – 11 pts (37.9%), adenocarcinoma – 13 pts (44.8%), large cell carcinoma – 4 pts (13.8%), small cell lung cancer – 1 pt (3.5%). First primaries of the patients were as follows: larynx – 6 pts (207%), lung – 2 pts (6.9%), stomach – 3 pts (10.3%), colon and rectum – 4 pts (13.8%), kidney – 4 pts (13.8%), prostate – 4 pts (13.8%), breast – 2 pts (6.9%), gynecology – 2 pts (6.9%) and haematologic malignancies – 2 pts (6.9%). According to 1PT localization patients were divided into 4 groups: airways, gastrointestinal, urology and other malignancies. Results. 1- and 3-year survival was 69% and 27.6% (60.9% and 30.4% for males; 27.6% and 16.7% for females). 3-year survival for the first primary cancer was 37.5% in urological cancers, 14.3% in gastrointestinal, 37.5% in airway cancers and 16.7% in other cancer cases. Survival by stage of 2PLC was as follows: IA-B st. – 33.3%, IIA-B st. – 28.6%, respectively. No patients survived for 3 years with IIIA-IV st. of 2  PLC. By morphology of 2 PLC there were adenocarcinoma cases in 14.3%, squamous cell carcinoma in 50.0%. One patient with small cell 2 PLC has lived so far. Conclusions. 1. The site of the first primary tumor is not a significant prognostic factor for surgical outcomes of the second primary lung cancer (p > 0.5). 2. Statistically significant survival rates did not differ by gender and histological type of 2PLC. 3. A statistically significant survival difference was obtained only between the patients with IA-IIB st. and IIIA-IV st. of 2PLC (p = 0.0013).

scholarly journals Primary Mesenteric Carcinoid Tumor Presenting with Carcinoid Syndrome

2018 ◽  
Vol 12 (2) ◽  
pp. 396-401
Author(s):  
Oluwaseun Shogbesan ◽  
Abdullateef Abdulkareem ◽  
Binu Pappachen ◽  
John Altomare
Keyword(s):  
Carcinoid Tumor ◽  
Primary Tumor ◽  
Carcinoid Syndrome ◽  
The Body ◽  
Carcinoid Tumors ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Diverse Group ◽  
Poorly Differentiated ◽  
Well Differentiated

Neuroendocrine neoplasms (NENs) are a diverse group of tumors arising throughout the body with a common origin from neuroendocrine cells. Well-differentiated NENs, also known as neuroendocrine tumors (NETs), are generally indolent and are often found incidentally, while poorly differentiated tumors are more aggressive. Carcinoid tumors are NETs arising from the gastrointestinal tract and less commonly from the lungs, thymus, and kidneys. NETs in the mesentery arise from metastasis from primary tumor, and carcinoid syndrome in this setting results from concomitant metastasis to the liver. Primary mesenteric carcinoid tumors are very rare. We present a 64-year-old man with carcinoid syndrome from a mesenteric carcinoid tumor without evidence of liver metastasis or other primary tumor sites.

Characterization of a novel entity of high-grade well-differentiated colorectal neuroendocrine tumors.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. e15696-e15696
Author(s):  
Salman Rafi Punekar ◽  
Lena Masri-Lavine ◽  
Cristina Hajdu ◽  
Elliot Newman ◽  
Daniel Jacob Becker
Keyword(s):  
Carcinoid Tumor ◽  
Neuroendocrine Tumors ◽  
National Registry ◽  
Cox Proportional Hazards ◽  
Small Cell ◽  
Carcinoid Tumors ◽  
Low Grade ◽  
High Grade ◽  
Ki 67 ◽  
Well Differentiated

e15696 Background: Small studies suggest that a new pathologic entity of high-grade (by Ki-67 or mitotic index) well-differentiated (by histologic features) neuroendocrine tumors (NETs) exist throughout the GI tract, but the prognosis and characteristics of affected patients are unknown. We sought to further characterize the prognosis and demographics of patients with high-grade (HG) well-differentiated (WD) colorectal NET. Materials and Methods: We used the Surveillance Epidemiology and End Results (SEER) database to study patients with NETs of the colon and rectum diagnosed from 2000 to 2015. We identified patient demographics, clinical and tumor characteristics, and studied associations with tumor grade. We compared overall survival (OS) between patients with low-grade (LG)(grade 1-2) well-differentiated (ICD-0-3 = carcinoid), high-grade (grade 3-4) well-differentiated, and high-grade poorly-differentiated NETs (ICD-0-3 = small cell neuroendocrine). We used logistic regression to detect associations with grade and Cox proportional hazards models to examine predictors of survival. Results: We identified 5,894 cases with colorectal carcinoid tumor (5780 [98.1%] LG and 114 [1.9%] HG); the cohort was 68% white, 48% male, and had a median age of 54. Patients diagnosed with HG carcinoid tumors were more likely to be of older age (OR 2.23; 95% CI 1.19-4.19 for age 60-69 vs < 50) and unmarried (OR 1.56; 95% CI 1.02-2.38), and less likely to be diagnosed after 2010 (OR 0.09; 95% CI 0.06-0.15). OS for patients with HG carcinoids (median 36 m; 95% CI 13-92) fell in between OS for those with HG small cell NETs (median 7 m; 95% CI 6-8), and LG carcinoid tumor (median not reached, > 120 m). Among patients with carcinoid tumors, black patients (HR 1.31; 95% CI 1.03-1.67, older patients (HR 3.60; 95% CI 2.50-5.19 for age 60-69 vs < 50), unmarried patients (HR 1.52; 95% CI 1.24-1.87), and those with HG features (HR 3.85; 95% CI 2.88-5.15) had worse survival. Conclusions: We defined a subset of high-grade well-differentiated NETs, more commonly diagnosed in older, unmarried patients, with a prognosis between that of high grade small cell NETs and low grade carcinoid tumors. Our analysis adds the first national registry study to the literature in support of a new classification of non-pancreatic high-grade well differentiated NETs.

scholarly journals Neuroendocrine Tumors of the Breast

2017 ◽  
Vol 141 (11) ◽  
pp. 1577-1581 ◽  
Author(s):  
Lauren Elizabeth Rosen ◽  
Paolo Gattuso
Keyword(s):  
Differential Diagnosis ◽  
Breast Carcinoma ◽  
Neuroendocrine Tumors ◽  
Neuroendocrine Differentiation ◽  
Small Cell ◽  
Breast Carcinomas ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Invasive Breast Carcinomas ◽  
Metastatic Neuroendocrine Tumor

Primary neuroendocrine tumors of the breast are a rare and underrecognized subtype of mammary carcinoma. Neuroendocrine tumors of the breast occur predominately in postmenopausal women. The tumors are subclassified into well-differentiated and poorly differentiated neuroendocrine tumors, and invasive breast carcinoma with neuroendocrine features. Well-differentiated tumors show architectural similarity to carcinoids of other sites but lack characteristic neuroendocrine nuclei. Poorly differentiated neuroendocrine tumors are morphologically identical to small cell carcinoma of the lung. Neuroendocrine differentiation, seen in up to 30% of invasive breast carcinomas, is most commonly associated with mucinous and solid papillary carcinomas. The diagnosis of neuroendocrine differentiation requires expression of the neuroendocrine markers synaptophysin or chromogranin. The main differential diagnosis is a metastatic neuroendocrine tumor from an extramammary site. Neuroendocrine tumors of the breast are treated similarly to other invasive breast carcinomas. Although no consensus has been reached on the prognosis, most studies suggest a poor outcome.

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