Table 5.
General guidelines for diagnosis and treatment of tumors of endocrine glands.
| Biological investigations | Paraclinical investigations | Treatment | |
|---|---|---|---|
| Pheochromocytoma/Paraganglioma | Plasmatic or urinary catecholamine metabolites (metanephrine, normetanephrine, 3-methoxytyramine) |
Imaging: Morphological: CT or MRI, adrenal-specific Functional: 18FDG PET-CT or 18F-DOPA PET-CT or 123I-MIBG scintigraphy or 68Ga-SSA PET-CT |
Surgery excision Laparoscopy preferred for abdominal location Adrenal-sparing surgery for bilateral PCC |
| Gastrointestinal neuroendocrine tumors | - Chromogranin A - Hormones (gastrin, pancreatic polypeptide, insulin, glucagon, somatostatin, VIP, etc.) - In case of carcinoid syndrome: 24-h urinary 5-HIAA, platelet serotonin |
Imaging: -CT injected with contrast agent -Abdominal-pelvic MRI injected with Gadolinium to research metastases -Nuclear imaging: 68Ga-SSA PET-CT or 18FDG PET-CT or 18F-DOPA PET-CT If pancreatic tumor -endoscopy - endoscopic ultrasound to perform biopsies |
Anti-secretory treatment: - somatostatin analogue, - telotristat (carcinoid), - PPI (gastrinoma), - diazoxide (insulinoma) Surgical excision Metastatic forms: - surgical excision (hepatic metastasis), - chemotherapy, - targeted therapy (e.g., sunitinib, everolimus), - radiometabolic therapy (177Lu-DOTATATE) |
| Primary hyperparathyroidism | - Blood calcium, phosphate, 25-hydroxy vitamin D, parathyroid hormone - Urinary calcium |
Imaging: -Morphological: Cervical US and CT -Functional: 99mTc-MIBI scintigraphy, F-choline PET |
Surgical excision using minimally invasive cervical surgery |
| Pituitary adenoma | Plasma hormones: cortisol and ACTH at 8 am, 4 pm, 12 am; IGF-1, LH, FSH, estradiol (women); testosterone, SHBG (men); prolactin, TSH, FT4 Dynamic test according to results mainly - 8 am cortisol measurement after 1 mg dexamethasone test at 12 am (if suspicion of hypercortisolism) - OGTT with GH measurement in case of acromegaly suspicion - Intravenous insulin test with GH and cortisol measurement in case of suspicion of hypopituitarism (if no cardiac or neurological impairment) |
Imaging: -Morphological: Pituitary MRI -Neuro-ophthalmological examination: e.g., visual fields, Lancaster test |
- Somatostatin analogue or dopamine antagonist - Transsphenoidal surgical excision |
CT, computed tomography scan; MRI, magnetic resonance imaging; 18FDG, 18Fluorodeoxyglucose; 18F-DOPA, 18Fluoro-dihydroxyphenylalanine; 123I-MIBG, 123I-meta-iodobenzylguanidine; 68Ga-SSA, 68Ga-somatostatin analogues; PCC, pheochromocytoma; VIP, vasoactive intestinal polypeptide; 5-HIAA, 5-hydroxyindolacetic acid; PPI, proton pump inhibitors; 177Lu-DOTATATE, lutetium (177Lu) oxodotreotide; US, ultrasound; 99mTc-MIBI, Technetium (99mTc) sestamibi; ACTH, adrenocorticotropic hormone; IGF-1, insulin-like growth factor 1; LH, luteinizing hormone; FSH, follicle-stimulating hormone; SHBG, sex hormone-binding globulin; TSH, thyroid-stimulating hormone; FT4, free thyroxine; GH, growth hormone; OGTT, oral glucose tolerance test.