From the Guidelines
The treatment plan for patients with Multiple Endocrine Neoplasia (MEN) presenting with multiple neuroendocrine tumors should prioritize a multidisciplinary approach, focusing on surgical resection of tumors when possible, and medical management with somatostatin analogs and targeted therapies to control hormone secretion and tumor growth, as recommended by the most recent guidelines 1.
Key Considerations
- Surgical resection is the primary treatment for localized tumors, with the goal of achieving complete removal of the tumor and regional lymph nodes, as outlined in the guidelines for neuroendocrine tumors 1.
- Medical management includes somatostatin analogs like octreotide LAR or lanreotide to control hormone secretion and tumor growth, with peptide receptor radionuclide therapy (PRRT) with lutetium-177-DOTATATE as an option for somatostatin receptor-positive tumors 1.
- Targeted therapies such as everolimus or sunitinib may be used for progressive pancreatic NETs, and alpha-blockade with phenoxybenzamine or doxazosin is initiated 1-2 weeks before surgery for pheochromocytomas, followed by beta-blockade if needed.
Multidisciplinary Approach
- A multidisciplinary team (MDT) should be involved in the management of patients with MEN, including specialist physicians, surgeons, radiologists, nuclear medicine specialists, histopathologists, and clinical nurse specialists, as recommended by the guidelines for gastroenteropancreatic neuroendocrine tumors 1.
- Regular biochemical screening and imaging surveillance are essential components of long-term management, with screening intervals determined by tumor type and MEN subtype.
Recent Guidelines
- The most recent guidelines for multiple endocrine neoplasia and hyperparathyroid-jaw tumor syndromes recommend a comprehensive approach to management, including clinical and biochemical markers to detect disease and follow evolution through the course of observation and treatment 1.
- These guidelines emphasize the importance of early intervention to mitigate morbidity and mortality, and highlight the need for a multidisciplinary approach to care.
From the Research
Treatment Plan for Multiple Endocrine Neoplasia (MEN)
The treatment plan for patients with Multiple Endocrine Neoplasia (MEN) presenting with multiple neuroendocrine tumors involves a comprehensive approach. Key aspects of the treatment plan include:
- Early genetic diagnosis and periodic screening to detect tumors at an early stage 2
- Surgical resection for loco-regional disease, as well as liver-directed and targeted chemotherapies for advanced progressive disease 3
- Management of pancreatic neuroendocrine tumors (PNETs) through diagnosis, surveillance, adequate staging, and interdisciplinary, multimodal treatments to optimize patient outcome 3
Diagnosis and Screening
Diagnosis of MEN1 can be made by genetic screening, which allows for early detection and treatment of MEN1-associated disease 4, 2. Screening methods include:
- Ultrasonography
- Endoscopic ultrasonography (EUS)
- CT
- MRI
- Selective arterial angiography
- Somatostatin receptor scintigraphy
Treatment Modalities
Treatment modalities for MEN1-related tumors include:
- Surgery: subtotal parathyroid gland resection for primary hyperparathyroidism, and selective tumour enucleation for neuroendocrine tumours 2
- Medical therapy: watchful waiting and medical therapy for MEN1-associated pituitary and adrenal adenomas 2
- Biotherapy: somatostatin analogues, such as octreotide or lanreotide, for functional tumors 5
- Chemotherapy: streptozotocin and 5-fluorouracil, possibly combined with D,L-folinic acid, for progressive tumor disease 5
Importance of Early Detection
Early detection and treatment of MEN1-associated disease are crucial to improve patient outcomes. Genetic testing can identify patients harboring an MEN1 mutation before the development of clinical signs or symptoms of endocrine disease 4. Biochemical screening can detect evidence of neoplasia an average of 10 years before clinically evident disease, allowing for early surgical intervention 4.