Multidisciplinary management of difficult/aggressive growth-hormone pituitary neuro-endocrine tumors

Abstract

Growth Hormone-secreting adenomas exhibits variable biological behavior and heterogeneous natural history, ranging from small adenomas and mild disease, to invasive and aggressive neoplasms with more severe clinical picture. Patients not cured or controlled after neurosurgical and first-generation somatostatin receptor ligands (SRL) therapy could require multiple surgical, medical and/or radiation treatments to achieve disease control. To date, no clinical, laboratory, histopathological, or neuroradiological markers are able to define the aggressiveness or predict the disease prognosis in patients with acromegaly. Therefore, the management of these patients requires careful evaluation of laboratory assessments, diagnostic criteria, neuroradiology examinations, and neurosurgical approaches to choose an effective and patient-tailored medical therapy. A multidisciplinary approach is particularly useful in difficult/aggressive acromegaly to schedule multimodal treatment, which includes radiation therapy, chemotherapy with temozolomide and other, recent emerging treatments. Herein, we describe the role of the different members of the multidisciplinary team according to our personal experience; a flow-chart for the therapeutic approach of difficult/aggressive acromegaly patients is proposed.

Multidisciplinary Team Care in Pituitary Tumours

“…The pursuit of excellence in delivering the best care to patients with pituitary tumours and other sellar lesions, including craniopharyngiomas or Rathke’s cleft cysts, has led to the implementation of Pituitary MDT/PTCOE. Pituitary MDT/PTCOE are now widely recommended by different guidelines and medical societies relevant in the field [ 2 , 10 , 11 , 22 , 23 ]. The definition criteria of PTCOE have been proposed [ 10 ], and further validated in a survey of several internationally recognised tertiary centres [ 11 ].…”

“…The multidisciplinary management of functioning pituitary tumours, including prolactin, growth hormone (GH)- or adrenocorticotropic hormone (ACTH)-secreting tumours, is crucial for improving clinical outcomes and the prognosis of patients [ 22 , 23 , 44 , 45 , 46 , 47 , 48 ]. Experienced neurosurgeons working in Pituitary MDT/PTCOE have better results [ 49 , 50 , 51 , 52 ].…”

“…Moreover, many pituitary disorders are primarily managed by endocrinologists, such as prolactinomas, congenital hypopituitarism, or acquired hypopituitarism (e.g., after trauma or radiotherapy). Endocrinologists are also crucial in providing support in peri- and post-operative settings, specifically in hormone replacement and water–sodium imbalances [ 2 , 10 , 11 , 23 , 39 , 42 , 47 , 69 ].…”

“…Positron emission tomography-computed tomography (PET-CT) with 18 F-fluorodeoxyglucose may be helpful for differential diagnosis and pre-operative characterization of some sellar lesions. Other tracers are currently used in the pituitary setting [23]. PET-CT with 68 Galabeled somatostatin analogues may localize and determine response to surgical, medical, or radiation treatment, as well as aid in selecting aggressive or metastatic pituitary cases suitable for peptide receptor radionuclide therapy [31].…”