Macroscopic aspects are variable, and final diagnosis is made from the typical histological and immunohistochemical pattern. The therapy of choice is gross total surgical resection since subtotal removal can lead to local regrowth, but the time to recurrence is unpredictable and highly variable. The role of adjuvant therapy is currently undefined.
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.
Context Discordant GH and IGF-1 values are frequent in acromegaly. Objective To evaluate the impact of different GH cut-offs on discordance rate. To investigate whether the mean of consecutive GH measurements impact discordance rate when matched to the last available IGF-1 value. Design Retrospective study. Setting Referral centre for pituitary diseases. Patients Ninety acromegaly patients with at least three consecutive evaluations for GH and IGF-1 using the same assay in the same laboratory (median follow-up 13 years). Interventions Multimodal treatment of acromegaly. Main outcome measures Single fasting GH (GHf) and IGF-1 (IGF-1f). Mean of three GH measurements (GHm), collected during consecutive routine patients’ evaluations. Results At last evaluation GHf values were 1.99 ± 2.79 µg/L and age-adjusted IGF-1f 0.86 ± 0.44 xULN (mean ± SD). Discordance rate using GHf was 52.2% (cut-off 1 µg/L) and 35.6% (cut-off 2.5 µg/L) (p=0.025). “High GH” discordance was more common for GHf <1.0 µg/L, while “high IGF-1” was predominant for GHf <2.5 µg/L (p<0.0001). Using GHm mitigated the impact of GH cut-offs on discordance (GHm <1.0 µg/L: 43.3%; GHm <2.5 µg/L: 38.9%; p=0.265). At ROC curve analysis, both GHf and GHm were poor predictors of IGF-1f normalization (AUC=0.611 and AUC=0.645, respectively). The prevalence of disease-related comorbidities did not significantly differ between controlled, discordant, and active disease patients. Discussion GH/IGF-1 discordance strongly depends on GH cut-offs. The use of GHm lessen the impact of GH cut-offs. Measurement of fasting GH levels (both GHf and GHm) is a poor predictor of IGF-1f normalization in our cohort.
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.
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