Neuroendocrine Neoplasms: Pathogenesis, Diagnostics, and Therapy

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Cancer Therapy".

Deadline for manuscript submissions: 17 September 2025 | Viewed by 1383

Special Issue Editor

Special Issue Information

Dear Colleagues,

Neuroendocrine neoplasms (NENs) are a heterogeneous group of tumors arising from the diffuse neuroendocrine cell system. They occur in numerous different locations. Most frequently, NENs are located within the digestive tract and pancreas, followed by lungs and bronchi, and thymus, The other NEN locations include parathyroid, thyroid, adrenal, and pituitary glands. A specific group of NENs are pheochromocytomas and paragangliomas (PPGLs) arising in the various locations of the paraganglion system. Most NENs are sporadic, while some of them are associated with inherited genetic syndromes. Depending on the differentation, NENs are divided into two subgroups: well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). Depending on hormonal secretion, functional and non-functional NENs are distinguished. In patients with functional NENs, these tumors are manifested by a wide spectrum of clinical symptoms, including intermittent flushing and diarrhea, bronchospasm and wheezing, and hypertension, as well as signs secondary to the secretion of gastrointestinal hormones, i.e., insulin, glucagon, gastrin, and other peptides. In diagnostics, laboratory biochemical tests as well as radiological anatomical (computed tomography and magnetic resonance imaging) and functional imaging (somatostatin receptor scintigraphy, [18F]-FDG PET, [18F]-FDOPA PET) are used. Surgery is the treatment of choice for local or locoregional disease. The other treatment methods include stereotactic radiosurgery (SRS), external beam radiotherapy (ERBT) and hypofractioned stereotactic radiotherapy (hSRT). In advanced and metastatic disease, systemic therapy, including chemotherapy, targeted therapies, somatostatin analogs (SSAs), radiometabolic therapy, as well as loco-regional procedures (cytoreductive surgery, external beam radiotherapy, arterial embolization, cryotherapy, RF ablation) are performed. Recently, both diagnostics and treatment of neuroendocrine neoplasms have been improved, including, i.a., molecular laboratory tests, targeted and immunological oncological therapy, as well as robot-assisted surgery. This Special Issue on “Neuroendocrine Neoplasms: Pathogenesis, Diagnostics, and Therapy” will focus on all aspects regarding laboratory diagnostics as well as surgical, pharmacological, immunological, and targeted treatment of these diseases. Original research and review papers related to all aspects of diagnostics and the treatment of neuroendocrine neoplasms are welcomed.

Dr. Beata Jabłońska
Guest Editor

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Keywords

  • neuroendocrine neoplasms
  • pathogenesis
  • diagnostics
  • therapy

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Published Papers (1 paper)

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42 pages, 19095 KiB  
Review
Pheochromocytomas and Paragangliomas—Current Management
by Adam Brewczyński, Agnieszka Kolasińska-Ćwikła, Beata Jabłońska and Lucjan Wyrwicz
Cancers 2025, 17(6), 1029; https://doi.org/10.3390/cancers17061029 - 19 Mar 2025
Cited by 1 | Viewed by 1178
Abstract
Pheochromocytomas and paragangliomas (PPGLs) are infrequent neuroendocrine hypervascular neoplasms arising within different sites of the paraganglion system. They are divided into sympathetic (including pheochromocytomas and extraadrenal paragangliomas) and parasympathetic extraadrenal tumors. These tumors are usually not malignant and grow slowly; about 90% of [...] Read more.
Pheochromocytomas and paragangliomas (PPGLs) are infrequent neuroendocrine hypervascular neoplasms arising within different sites of the paraganglion system. They are divided into sympathetic (including pheochromocytomas and extraadrenal paragangliomas) and parasympathetic extraadrenal tumors. These tumors are usually not malignant and grow slowly; about 90% of them are found in the adrenal paraganglia (pheochromocytomas). Extraadrenal tumors are most frequently located in the abdominal cavity (85%), followed by the thoracic cavity (12%), and head and neck (3%). About 25% of PPGLs are related to germline mutations, which are risk factors for multifocal and metastatic disease. In PPGL diagnostics, laboratory, biochemical, and imaging (anatomical and functional) examinations are used. Surgery is the standard management choice for locoregional disease. For patients who are not candidates for surgery and who have stable, not-growing, or slow-growing tumors, active observation or other less invasive techniques (i.e., stereotactic surgery, hypofractionated stereotactic radiotherapy) are considered. In metastatic disease, systemic therapies (tyrosine kinase inhibitors [TKIs], mTORC1 inhibitor everolimus, immunotherapy, cold somatostatin analogs [biotherapy], and radioligand therapy) are used. The prognosis for PPGLs is quite good, and the 5-year survival rate is >90%. The goal of this paper is to review knowledge on the etiopathogenesis, current diagnostics, and therapy for PPGL patients. Our paper is particularly focused on the current management of PPGLs. Full article
(This article belongs to the Special Issue Neuroendocrine Neoplasms: Pathogenesis, Diagnostics, and Therapy)
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