scholarly journals Neuroendocrine Tumors: Clinical, Histological and Immunohistochemical Perspectives and Case Report—Mature Teratoma in a 16-Year-Old Girl

2021 ◽  
Vol 28 (3) ◽  
pp. 373-386
Author(s):  
Elżbieta Sowińska-Przepiera ◽  
Dariusz Starzyński ◽  
Anhelli Syrenicz ◽  
Ireneusz Dziuba ◽  
Barbara Wiszniewska ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Histopathological Examination ◽  
Mature Teratoma ◽  
Young Patients ◽  
The Body ◽  
Systemic Review ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Heterogeneous Structure

A mature teratoma is a germinal neoplasm that differentiates from embryonic multipotent cells into three germ layers. There may also be glandular tissue. The literature describes a total of 658 cases of ovarian neuroendocrine neoplasms, mainly in women over 40 years of age. The authors, together with a systemic review, present a case of a 16-year-old girl diagnosed with and treated for a neuroendocrine tumor. Case description: A 16-year-old girl visited the Paediatric Gynaecology Outpatient Clinic because of abdominal pains that intensified during menstruation. Standard painkillers and diastolic drugs were ineffective. An ultrasound examination revealed a large tumor with a heterogeneous structure in her right ovary. A sparing operation was carried out. During laparotomy, the lesion was enucleated, leaving healthy tissue. Histopathological examination revealed the typical features of teratoma, as well as the coexistence of a G1 neuroendocrine tumor. Immunohistochemical examination (IHC) showed the presence of markers characteristic for this type of tumor. The patient requires constant monitoring in the Endocrinology and Oncological Gynaecology Clinic. Conclusion: Tissue of neuroendocrine neoplasm within a teratoma is rare in this age group of patients; thus, there are currently no standards for long-term follow-up. This case adds to the body of evidence and demonstrates a possible good prognosis with non-aggressive behavior in G1 neuroendocrine tumors and teratomas in young patients.

scholarly journals Case report: composite pancreatic intraductal papillary mucinous neoplasm and neuroendocrine tumor: a new mixed neuroendocrine-non-neuroendocrine neoplasm?

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jingci Chen ◽  
Pengyan Wang ◽  
Ke Lv ◽  
Weixun Zhou
Keyword(s):  
Neuroendocrine Tumor ◽  
Intraductal Papillary Mucinous Neoplasm ◽  
Histopathological Examination ◽  
Abdominal Ultrasound ◽  
Genetic Alterations ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Lesion ◽  
Partial Pancreatectomy ◽  
Mucinous Neoplasm

Abstract Background Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNEN) of the pancreas are extremely rare. Their pathogenesis and molecular landscape are largely unknown. Here, we report a case of mixed pancreatic intraductal papillary mucinous neoplasm (IPMN) and well-differentiated neuroendocrine tumor (NET) and identify its genetic alterations by next-generation sequencing (NGS). Case presentation A fifty-year-old male was admitted into the hospital for evaluation of a pancreatic lesion detected during a routine examination. Abdominal ultrasound indicated a hypoechoic mass of 2.6 cm at the head of the pancreas. Malignancy was suspected and partial pancreatectomy was performed. Thorough histopathological examination revealed a mixed IPMN-NET. In some areas, the two components were relatively separated, whereas in other areas IPMN and NET grew in a composite pattern: The papillae were lined with epithelial cells of IPMN, and there were clusters of NET nests in the stroma of papillary axis. NGS revealed shared somatic mutations (KRAS, PCK1, MLL3) in both components. The patient has been uneventful 21 months after the surgery. Conclusions Our case provides evidence of a common origin for mixed IPMN-NET with composite growth features. Our result and literature review indicate that KRAS mutation might be a driver event underlying the occurrence of MiNEN. We also recommend the inclusion of mixed non-invasive exocrine neoplasms and neuroendocrine neoplasms into MiNEN.

scholarly journals Molecular Pathology of Well-Differentiated Pulmonary and Thymic Neuroendocrine Tumors: What Do Pathologists Need to Know?

2021 ◽  
Vol 32 (1) ◽  
pp. 154-168 ◽  
Author(s):  
Marco Volante ◽  
Ozgur Mete ◽  
Giuseppe Pelosi ◽  
Anja C. Roden ◽  
Ernst Jan M. Speel ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Young Patients ◽  
Carcinoid Tumors ◽  
Neuroendocrine Neoplasms ◽  
Grey Zone ◽  
Cell Hyperplasia ◽  
Ki 67 ◽  
Familial Form ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

AbstractThoracic (pulmonary and thymic) neuroendocrine tumors are well-differentiated epithelial neuroendocrine neoplasms that are classified into typical and atypical carcinoid tumors based on mitotic index cut offs and presence or absence of necrosis. This classification scheme is of great prognostic value but designed for surgical specimens, only. Deep molecular characterization of thoracic neuroendocrine tumors highlighted their difference with neuroendocrine carcinomas. Neuroendocrine tumors of the lung are characterized by a low mutational burden, and a high prevalence of mutations in chromatin remodeling and histone modification-related genes, whereas mutations in genes frequently altered in neuroendocrine carcinomas are rare. Molecular profiling divided thymic neuroendocrine tumors into three clusters with distinct clinical outcomes and characterized by a different average of copy number instability. Moreover, integrated histopathological, molecular and clinical evidence supports the existence of a grey zone category between neuroendocrine tumors (carcinoid tumors) and neuroendocrine carcinomas. Indeed, cases with well differentiated morphology but mitotic/Ki-67 indexes close to neuroendocrine carcinomas have been increasingly recognized. These are characterized by specific molecular profiles and have an aggressive clinical behavior. Finally, thoracic neuroendocrine tumors may arise in the background of genetic susceptibility, being MEN1 syndrome the well-defined familial form. However, pathologists should be aware of rarer germline variants that are associated with the concurrence of neuroendocrine tumors of the lung or their precursors (such as DIPNECH) with other neoplasms, including but not limited to breast carcinomas. Therefore, genetic counseling for all young patients with thoracic neuroendocrine neoplasia and/or any patient with pathological evidence of neuroendocrine cell hyperplasia-to-neoplasia progression sequence or multifocal disease should be considered.

scholarly journals Pancreatic Neuroendocrine Tumor with Benign Serous Cystadenoma: A Rare Entity

2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Randhir Sagar Yadav ◽  
Ashik Pokharel ◽  
Shumneva Shrestha ◽  
Ashbita Pokharel ◽  
Deepshikha Gaire ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Histopathological Examination ◽  
Epigastric Pain ◽  
Pancreatic Neuroendocrine Tumor ◽  
Serous Cystadenoma ◽  
Neuroendocrine Neoplasm ◽  
Management Approach ◽  
Cystic Neoplasms ◽  
Distal Pancreas

Mixed serous-neuroendocrine neoplasm constitutes pancreatic serous cystic neoplasms and pancreatic neuroendocrine tumor, two tumor components with different underlying pathologies. The differentiation of these tumors is important as the management and prognosis depend on the pancreatic neuroendocrine tumor component. We report a case of mixed serous-neuroendocrine neoplasm in a 47-year-old female who presented with epigastric pain abdomen for two years. Imaging studies, tumor markers, thorough systemic evaluation, surgical resection, histopathological examination, and timely follow-up constituted our management approach. A 4 cm × 4 cm mass in the distal pancreas with multiple cysts in the pancreatic parenchyma containing serous fluid on distal pancreatectomy and splenectomy was found. The histopathological examination revealed combined benign serous cystadenoma and neuroendocrine tumor. She did not have any recurrence or metastasis by four years of follow-up.

scholarly journals A Rare Case of Giant Neuroendocrine Tumor of the Thymus Treated with EN Bloc Resection

2019 ◽  
Vol 4 (02) ◽  
Author(s):  
João Isidro Fracasso ◽  
Antônio Benincá Albuquerque ◽  
André Luca Boeira Rovani ◽  
Larissa Maroni ◽  
Guilherme Baroni De Macedo ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Epigastric Pain ◽  
Neuroendocrine Differentiation ◽  
En Bloc Resection ◽  
Anterior Mediastinum ◽  
Bloc Resection ◽  
The Body ◽  
En Bloc ◽  
Tumors Of The Thymus

Neuroendocrine tumors (NETs) are epithelial neoplasms with predominant neuroendocrine differentiation that arise in most organs of the body. Neuroendocrine tumors of the thymus (tNET) and mediastinum are very uncommon. We report the case of a 49-year-old male patient with neuroendocrine tumor of the thymus, superiorly in the anterior mediastinum, asymptomatic of the respiratory tract presenting with epigastric pain, treated with the lesion's surgical resection en bloc associated with radiotherapy and chemotherapy.

scholarly journals WHO Grade 2 Neuroendocrine Tumor in a 15-Year-Old Male: A Case Report and Literature Review

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Eric Johannesen ◽  
Van Nguyen
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Neuroendocrine Differentiation ◽  
Failure To Thrive ◽  
Proliferation Index ◽  
Neuroendocrine Neoplasms ◽  
Who Grade ◽  
Increased Risk ◽  
History Of ◽  
Well Differentiated

Neuroendocrine tumors, distinguished from adenocarcinomas by their neuroendocrine differentiation, are the most common pediatric epithelial malignancy that most often occurs in the appendix. In 2010, the WHO classified neuroendocrine neoplasms into three grades based on morphology, mitotic count, and Ki67 proliferation index. A 15-year-old male with a history of anemia and failure to thrive was diagnosed with a well-differentiated neuroendocrine tumor in the jejunum that invaded into the subserosal soft tissue and metastasized to four lymph nodes. Pediatric neuroendocrine tumors frequently arise within hereditary tumor syndromes with pancreatic neuroendocrine tumors being the most common. Several studies also indicate an elevated risk of small intestinal neuroendocrine tumors in which children born to a parent with a history of neuroendocrine tumors in the small intestine have a significant increased risk of developing one.

scholarly journals Unmet Needs in Functional and Nonfunctional Pancreatic Neuroendocrine Neoplasms

2018 ◽  
Vol 108 (1) ◽  
pp. 26-36 ◽  
Author(s):  
Robert T. Jensen ◽  
Lisa Bodei ◽  
Jaume Capdevila ◽  
Anne Couvelard ◽  
Massimo Falconi ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Unmet Needs ◽  
Advisory Board ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated

Recently, the European Neuroendocrine Tumor Society (ENETS) held working sessions composed of members of the advisory board and other neuroendocrine neoplasm (NEN) experts to attempt to identify unmet needs in NENs in different locations or with advanced/poorly differentiated NENs. This report briefly summarizes the main proposed areas of unmet needs in patients with functional and nonfunctional pancreatic NENs.

Neuroendocrine Tumors of Larynx

1992 ◽  
Vol 101 (8) ◽  
pp. 710-714 ◽  
Author(s):  
Adel K. El-Naggar ◽  
John G. Batsakis ◽  
Mario A. Luna
Keyword(s):  
Survival Rate ◽  
Neuroendocrine Tumors ◽  
Small Cell ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Neural Basis ◽  
Small Cell Neuroendocrine Carcinoma ◽  
Typical Carcinoid ◽  
Clinical Behavior ◽  
Neuroendocrine Carcinomas

Neuroendocrine neoplasms of the larynx have either an epithelial or a neural basis. The former are more numerous and are classified as typical or atypical carcinoids and small cell neuroendocrine carcinomas. Paraganglioma is the sole type of neural neuroendocrine neoplasm. There is a significant worsening of prognosis from typical carcinoid to small cell neuroendocrine carcinoma, with the latter having a dismal 5-year survival rate regardless of therapy. Paragangliomas are the most benign of laryngeal neuroendocrine neoplasms, but their clinical behavior may not be predictable on the basis of their histologic appearance.

scholarly journals An atypical type I gastric neuroendocrine tumor

2017 ◽  
Vol 55 (4) ◽  
pp. 253-256 ◽  
Author(s):  
Andrada-Viorela Gheorghe ◽  
Mihai Rimbas ◽  
Octav Ginghina ◽  
Andrada Spanu ◽  
Theodor Alexandru Voiosu
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Polypoid Lesion ◽  
Muscularis Propria ◽  
Distant Metastases ◽  
The Body ◽  
Gastric Body ◽  
Pathology Report ◽  
Type I ◽  
Muscularis Mucosa

AbstractBackground. Gastric neuroendocrine tumors (GI-NETs) are rare lesions, usually discovered incidentally during endoscopy. Based on their pathology, there are 4 types of GI-NETs. Type I are multiple small polypoid lesions with central ulceration located in the gastric body or the fundus, associated with atrophic gastritis usually noninvasive and very rarely metastatic. We report on a rare case of a gastric NET arising from the muscularis propria layer of the pyloric ring.Case report. We present the case of a 65-year old woman with a history of alcoholic cirrhosis, investigated for melena. Upper endoscopy revealed a 30 mm submucosal pedunculated polypoid lesion located on the pylorus protruding in the duodenum, with normal overlying mucosa, fundic gastric atrophy and multiple small polyps at this level, with no active bleeding. CT scan did not reveal any distant metastases. An ultrasound endoscopy was performed, and a round hypoechoic heterogeneous solitary mass, evolving from the pyloric muscle was described. Considering a 30-mm tumor evolving from the gastric muscle layer in the absence of local invasion and with no distant metastases we decided against an endoscopical resection and we referred the patient to surgery. A laparoscopic wedge resection was performed. The pathology report described a 30/25 mm welldifferentiated neuroendocrine tumor invasive in the muscularis mucosa (pT3).Conclusions. Usually, type I neuroendocrine tumors are located in the body or the fundus of the stomach without submucosal invasion. The interesting feature in our case was that the tumor originated from the pylorus, making it an atypical presentation for a neuroendocrine tumor.

scholarly journals Incidence of Gastrointestinal Neuroendocrine Tumor: Case Series, Armed Forces Hospital Southern Region, Hospital-Based Tumor Board Registry

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Abdullah Mohammed Albishi ◽  
Ahmed Mostafa Mohamed Mostafa ◽  
Hatim Mahgoub Ali ◽  
Yahia Atiah Alhagawi ◽  
Mohamed F. Bazeed ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Case Series ◽  
Armed Forces ◽  
The Body ◽  
Tumor Board ◽  
Knowing That ◽  
Almost Everywhere ◽  
Time Period ◽  
Gastrointestinal Neuroendocrine Tumor

Neuroendocrine tumors are aggressive and rare tumors which can occur almost everywhere in the body. The annual incidence of neuroendocrine tumors is 2.5-5 per 100000. We report seven cases of gastrointestinal neuroendocrine tumors which were diagnosed and treated at our hospital from the time period of 2016-2018 knowing that the total number of our hospital tumor board cases registry during the same period was 444 cases.

scholarly journals Peptide receptor radionuclide therapy controls inappropriate calcitriol secretion in a pancreatic neuro-endocrine tumor: a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Maarten Haemels ◽  
Thierry Delaunoit ◽  
Koen Van Laere ◽  
Eric Van Cutsem ◽  
Chris Verslype ◽  
...  
Keyword(s):  
Liver Metastases ◽  
Neuroendocrine Tumor ◽  
Neuroendocrine Tumors ◽  
Serum Calcium ◽  
Radionuclide Therapy ◽  
Active Form ◽  
Peptide Receptor Radionuclide Therapy ◽  
Pancreatic Neuroendocrine Tumor ◽  
Neuroendocrine Neoplasm ◽  
Peptide Receptor

Abstract Background Hypercalcemia of malignancy is not uncommon in patients with advanced stage cancer. In rare cases the cause of the hypercalcemia is excessive production of calcitriol, the active form of vitamin D. Although inappropriate tumoral secretion of calcitriol is typically associated with lymphomas and some ovarian germ cell tumors, we present a case of calcitriol overproduction-induced hypercalcemia due to a pancreatic neuroendocrine tumor. The high expression of somatostatin receptors on this neuroendocrine neoplasm opened up the opportunity to treat the patient with radiolabelled somatostatin analogs, which successfully controlled the refractory hypercalcaemia and calcitriol levels. This case documents a rare finding of refractory hypercalcaemia of underlying malignancy due to a calcitriol-producing pancreatic neuroendocrine tumor, responding to peptide receptor radionuclide therapy (PRRT). Case presentation A 57 years-old patient presented with back pain, general discomfort, polydipsia, polyuria, fatigue and recent weight loss of 10 kg. Clinical examination was normal and there was no relevant medical history. Biochemical evaluation showed hypercalcemia with markedly increased calcitriol levels. CT-thorax-abdomen and ultrasound guided biopsy revealed a pancreatic neuroendocrine tumor with multifocal liver metastases, suggesting that excessive overproduction of calcitriol by this neuroendocrine tumor was the cause of the refractory hypercalcemia. The patient was eligible for PRRT. Four cycles of 177Lu-DOTATATE PRRT resulted in a morphological response and a normalization of serum calcium levels, confirming the hypothesis of a calcitriol producing pancreatic neuroendocrine tumor. Progression of liver metastases warranted further therapy and temozolomide-capecitabine was started with morphological and biochemical (serum calcium, calcitriol) stabilization. Conclusion Although up to 30–40% of gastroenteropancreatic neuroendocrine tumors are known to be functional (i.e. producing symptoms associated with the predominant hormone/peptide secreted), calcitriol secreting pancreatic neuroendocrine tumors are very rare. However, treatment with PRRT resulted in normalization of calcium and calcitriol levels, strongly supporting the hypothesis of a calcitriol-producing pancreatic neuroendocrine tumor.

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