Table of contents
Genitourinary and gynecological neuroendocrine tumors
What's new
2024 ESMO guidelines for the diagnosis and management of genitourinary and gynecological neuroendocrine tumors.
Background
Overview
Definition
GUG-NETs are rare malignancies that arise from neuroendocrine cells within the genitourinary and reproductive systems. These tumors exhibit diverse histological features and clinical behaviors, ranging from indolent growth to aggressive progression.
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Pathophysiology
The exact pathophysiology is not fully understood, with most cases being sporadic; however, associations with certain genetic syndromes have been identified, including MEN1, MEN2, von Hippel-Lindau disease, tuberous sclerosis complex, and neurofibromatosis type 1.
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Epidemiology
GUG-NETs account for 1-2% of genitourinary and gynecological malignancies, 12% of poorly differentiated NETs, and 4% of well-differentiated NETs. The incidence of these tumors in Europe is estimated at 0.5-1 per 1,000,000 person-year.
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Disease course
Some tumors may be asymptomatic and discovered incidentally, while others may present with symptoms related to hormonal hypersecretion or mass effect. The disease course can be indolent or aggressive, depending on the tumor grade and stage.
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Prognosis and risk of recurrence
Major independent prognostic factors are disease stage (TNM) and pathological features, including differentiation and WHO grade. Age, performance status, tumor burden, the presence of functional syndromes, and tumor growth rate are additional prognostic factors.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of genitourinary and gynecological neuroendocrine tumors are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2024).
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Classification and risk stratification
Diagnostic investigations
Hormone testing
As per ESMO 2024 guidelines:
Diagnose secretory syndromes based on hormone-related symptoms in patients with GUG-NETs.
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Assess somatostatin receptor status and the presence of functional syndromes before making treatment decisions.
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Molecular profiling
Imaging for staging
Medical management
General principles
As per ESMO 2024 guidelines:
Discuss diagnostic and therapeutic recommendations for patients with genitourinary and gynecological neuroendocrine neoplasms in a neuroendocrine neoplasm multidisciplinary tumor board.
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Offer enrolment in clinical trials whenever possible.
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Management of local/locoregional disease (neoadjuvant therapy)
Management of local/locoregional disease (surgical excision)
Management of local/locoregional disease (adjuvant therapy)
Management of advanced/metastatic disease (surgical excision)
Management of advanced/metastatic disease (chemotherapy)
Management of carcinoid syndrome
Management of Cushing's syndrome