Acromegaly is a rare and severe disease caused by an increased and autonomous secretion of growth hormone (GH), thus resulting in high circulating levels of insulin-like growth factor 1 (IGF-1). Comorbidities and mortality rate are closely related to the disease duration. However, in most cases achieving biochemical control means reducing or even normalizing mortality and restoring normal life expectancy. Current treatment for acromegaly includes neurosurgery, radiotherapy and medical therapy. Transsphenoidal surgery often represents the recommended first-line treatment. First-generation somatostatin receptor ligands (SRLs) are the drug of choice in patients with persistent disease after surgery and are suggested as first-line treatment for those ineligible for surgery. However, only about half of patients treated with octreotide (or lanreotide) achieve biochemical control. Other available drugs approved for clinical use are the second-generation SRL pasireotide, the dopamine agonist cabergoline, and the GH-receptor antagonist pegvisomant. In the present paper, we revised the current literature about the management of acromegaly, aiming to highlight the most relevant and recent therapeutic strategies proposed for patients resistant to first-line medical therapy. Furthermore, we discussed the potential molecular mechanisms involved in the variable response to first-generation SRLs. Due to the availability of different medical therapies, the choice for the most appropriate drug can be currently based also on the peculiar clinical characteristics of each patient.

Posterior cranial fossa tumours frequently develop hydrocephalus as first presentation in up to 80% of paediatric patients and 21.4% of adults, although it resolves after tumour removal in 70-90% and 96%, respectively. New onset hydrocephalus is reported in about 2.1% of adult and 10-40% of paediatric patients after posterior fossa surgery. There is no consensus concerning prophylactic external ventricular drainage (EVD) placement that is frequently used before posterior fossa lesion removal, as well in those cases without clear evidence of hydrocephalus. The aim of the study was to define the most correct management for patients who undergo posterior fossa tumour surgery, thus identifying cohorts of patients who are at risk of persistent hydrocephalus prior to surgery. A systematic review of literature has been performed, following PRISMA guidelines. Most of the studies reported CSF shunt only in the presence of hydrocephalus, whereas only a few authors suggested its prophylactic use in the absence of signs of ventricular dilatation. Predictive factors for postoperative hydrocephalus has been identified, including young age (< 3 years), severe symptomatic hydrocephalus at presentation, EVD placement before surgery, FOHR index > 0.46 and Evans index > 0.4, pseudomeningocele, CSF leak and infection. The use of pre-resection CSF shunt in case of signs and symptoms of hydrocephalus is mandatory, although it resolves in the majority of cases. As reported by several studies included in the present review, we suggest CSF shunt also in case of asymptomatic hydrocephalus, whereas it is not indicated without evidence of ventricular dilatation.

Posterior interosseous nerve distal entrapment is a rare condition; therefore, further investigation is needed when radiological images at the arcade of Frohse do not show any entrapment.

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Purpose Spinal aneurysms are rare vascular malformations, commonly associated with spinal AVMs. AVM-associated spinal aneurysms are burdened by significant morbidity. The purpose of our study is to evaluate the best treatment strategy for these uncommon vascular lesions and to report an illustrative case. Methods We reviewed clinical and radiological data of a patient surgically treated at our institution for a spinal AVM with an associated prenidal aneurysm. According to PRISMA guidelines, a systematic literature review has been performed in order to discuss the best management AVM-associated prenidal aneurysms. Results In the reported case, the aneurysm showed spontaneous regression at follow-up after surgical removal of the AVM. Only 6 articles reported management of spinal prenidal AVM-associated aneurysms. Basing on our experience and data from literature, surgical treatment of the aneurysm may be indicated along with the resection of the AVM if the aneurysm is close to the nidus. Conversely, if the aneurysm is far away from the nidus or in an unfavorable position, resection of the nidus only may lead to aneurysm regression as in the reported case. Conclusions The treatment strategy for AVM-associated spinal aneurysms should be tailored on the single patient. In presence of large aneurysms that cause mass-effect symptoms, when rupture of the aneurysm is suspected or when treatment of the AVM is not proposable, direct treatment of the aneurysm should be considered. Otherwise, when complete resection of the nidus is performed, the eventually associated unruptured aneurysms located in challenging positions can be safely managed conservatively.

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