Rare Endocrine and Neuroendocrine neoplasms (NENs) are heterogeneous tumors originating from several endocrine glands, including adrenal, pituitary, thyroid and parathyroid, or from neuroendocrine cells found throughout the body, although most occur in the digestive tract, lungs, as well as paraganglias. They can be non-cancerous (benign) or cancerous (malignant) with the ability to develop distant metastasis. The peculiarity of Endocrine tumors and NENs is that they can cause not only symptoms related to the tumor mass, as it happens to other tumor types, but also symptoms related to their specific hormonal secretion, which significantly contributes to their pathological burden.

The majority of these Endocrine and Neuroendocrine neoplasms occur sporadically. However, some NENs are associated with a hereditary neoplasia syndrome such as multiple endocrine neoplasia type 1 (MEN1) or type 2 (MEN2A and MEN2B), Von Hippel-Lindau (VHL) disease, tuberous sclerosis (TS), neurofibromatosis type 1 (NF1), or Carney Complex/PPNAD.

The management of Rare Endocrine tumors and NENs raises major diagnostic challenges, sometimes with consequences for patients’ quality of life and outcome.

This Research Topic aims to provide a comprehensive picture of the molecular mechanisms underlining the development and progression of these tumors, the identification of potential diagnostic molecular markers and prognostic biomarkers of response to therapies, as well as new treatment options (including surgery, targeted therapies, chemotherapies and novel drugs), which are urgently needed to improve therapeutic results in rare Endocrine tumors and NENs.

We welcome original research, including clinical, translational, as well as molecular studies, review articles, and clinical trials on a range of topics related to rare endocrine and neuroendocrine tumors. Potential topics include, but are not limited to:

• Adrenocortical carcinoma.
• Pheochromocytoma and paraganglioma.
• Cushing’s syndrome (related to adrenocortical or ectopic tumors).
• Neuroendocrine neoplasms (gastro-entero-pancreatic NEN, lung and bronchial NEN, NEN of the thymus, NEN of genitourinary tract, Merckel cell carcinoma, NEN of other rare sites of origin).
• Thyroid tumors (medullary thyroid tumor, anaplastic tumor).
• Parathyroid carcinoma.
• Pituitary tumors (Cushing’s disease, Acromegaly, TSH-secreting pituitary tumors, pituitary carcinoma).
• Endocrine tumors related to genetic syndromes (MEN1, MEN2A, MEN2B, Neurofibromatosis type 1, von Hippel-Lindau, Tuberous sclerosis syndromes, Carney Complex/PPNAD, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome).

This Research Topic is part three of a three-part series, and focuses on the genetics and molecular aspects of endocrine and neuroendocrine tumors - please also see the collection "Rare Endocrine Tumors", which focuses on clinical studies on endocrine tumors and the third collection "Rare Neuroendocrine Neoplasms", focusing on clinical studies concerning neuroendocrine tumors

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Frontiers in Endocrinology

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