Acromegaly: Role of Surgery in the Therapeutic Armamentarium

Acromegaly is a complex disease that requires the intervention of a multidisciplinary team. The most frequent clinical manifestations are growing of distal parts of the body and some areas of the face. Patients may also present arterial hypertension, diabetes mellitus, colonic polyps, cardiomegaly, neurological and endocrine changes secondary to the presence of a GH-secreting tumor in pituitary or extrapituitary origin, or eutopic hypothalamic GHRH hypersecretion and peripheral GHRH hypersecretion. Surgery is the first treatment used for most patients, regardless of the cause. In the great majority of cases, pituitary tumor can be removed through a transsphenoidal approach. Craniotomy is reserved for those cases with giant tumors, particularly when they grow toward the middle or posterior cranial fossa. Best surgical results are obtained when the tumor is confined into the sella turcica or if it has a regular suprasellar extension. When the disease cannot be controlled with surgery, medical treatment is indicated. Somatostatin analogues are included as the first line of medication, followed by dopamine agonist and growth hormone receptors antagonists. Radiation therapy can be also indicated in two main forms for residual tumor with medically refractory patients: radiosurgery for small tumors or fractionated stereotactic radiotherapy for larger ones

Orbitocranial approach for giant middle cerebral artery bifurcation aneurysms: Is it necessary?

Background: Standard pterional approaches have largely been used as the first option for middle cerebral artery aneurysms. This approach presents several disadvantages when facing giant aneurysms: aneurysmatic neck cannot be adequately seen and perianeurysmatic architecture is not completely visualized, leading to inappropriate clipping techniques. Orbitocranial approaches have been described in anterior communicating aneurysms, but not in middle cerebral artery bifurcation ones. Methods: In this report, we present the case of a patient with subarachnoid hemorrhage secondary to a middle cerebral artery bifurcation aneurysm rupture which was clipped using an orbitocranial approach. We describe the surgical technique step-by-step, present a clinical report, and discuss the advantages and utility of this procedure. Results: This technique was performed successfully and uneventfully in our patient, and it proved to be a safe method of clipping these kind of aneurysms, providing adequate visualization for the surgeon and making it safer for the patient. Conclusions: We consider the orbitocranial approach as an adequate alternative to the conventional pterional approach when facing giant aneurysms of the middle cerebral artery bifurcations as it offers a much better visualization of the aneurysmatic neck, as well as that of the perianeurysmatic architecture

Current Approach of Functioning Head and Neck Paragangliomas: Case Report of a Young Patient with Multiple Asynchronous Tumors

Introduction. Pheochromocytomas (Pheo) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells of the adrenal medulla and from the extra-adrenal autonomic paraganglia, respectively. Only 1–3% of head and neck PGL (HNPGL) show elevated catecholamines, and at least 30% of Pheo and PGL (PCPG) are associated with genetic syndromes caused by germline mutations in tumor suppressor genes and proto-oncogenes. Clinical Case. A 33-year-old man with a past medical history of resection of an abdominal PGL at the age of eleven underwent a CT scan after a mild traumatic brain injury revealing an incidental brain tumor. The diagnosis of a functioning PGL was made, and further testing was undertaken with a PET-CT with 68Ga-DOTATATE, SPECT-CT 131-MIBG, and genetic testing. Discussion and Conclusion. The usual clinical presentation of functioning PCPG includes paroxistic hypertension, headache, and diaphoresis, sometimes with a suggestive family history in 30–40% of cases. Only 20% of PGL are located in head and neck, of which only 1–3% will show elevated catecholamines. Metastatic disease is present in up to 50% of cases, usually associated with a hereditary germline mutation. However, different phenotypes can be observed depending on such germline mutations. Genetic testing is important in patients with PCPG since 31% will present a germline mutation. In this particular patient, an SDHB gene mutation was revealed, which can drastically influence the follow-up plan and the genetic counsel offered. A multidisciplinary approach is mandatory for every patient presenting with PCPG

Current and future trends in multimodality imaging of coronary artery disease

Nowadays, there is a wide array of imaging studies available for the evaluation of coronary artery disease, each with its particular indications and strengths. Cardiac single photon emission tomography is mostly used to evaluate myocardial perfusion, having experienced recent marked improvements in image acquisition. Cardiac PET has its main utility in perfusion imaging, atherosclerosis and endothelial function evaluation, and viability assessment. Cardiovascular computed tomography has long been used as a reference test for non-invasive evaluation of coronary lesions and anatomic characterization. Cardiovascular magnetic resonance is currently the reference standard for non-invasive ventricular function evaluation and myocardial scarring delineation. These specific strengths have been enhanced with the advent of hybrid equipment, offering a true integration of different imaging modalities into a single, simultaneous and comprehensive study

On the initiation of world neurosurgery

SCOPUS: no.jinfo:eu-repo/semantics/publishe

The Genomic Landscape of Corticotroph Tumors: From Silent Adenomas to ACTH-Secreting Carcinomas

Corticotroph cells give rise to aggressive and rare pituitary neoplasms comprising ACTH-producing adenomas resulting in Cushing disease (CD), clinically silent ACTH adenomas (SCA), Crooke cell adenomas (CCA) and ACTH-producing carcinomas (CA). The molecular pathogenesis of these tumors is still poorly understood. To better understand the genomic landscape of all the lesions of the corticotroph lineage, we sequenced the whole exome of three SCA, one CCA, four ACTH-secreting PA causing CD, one corticotrophinoma occurring in a CD patient who developed Nelson syndrome after adrenalectomy and one patient with an ACTH-producing CA. The ACTH-producing CA was the lesion with the highest number of single nucleotide variants (SNV) in genes such as USP8, TP53, AURKA, EGFR, HSD3B1 and CDKN1A. The USP8 variant was found only in the ACTH-CA and in the corticotrophinoma occurring in a patient with Nelson syndrome. In CCA, SNV in TP53, EGFR, HSD3B1 and CDKN1A SNV were present. HSD3B1 and CDKN1A SNVs were present in all three SCA, whereas in two of these tumors SNV in TP53, AURKA and EGFR were found. None of the analyzed tumors showed SNV in USP48, BRAF, BRG1 or CABLES1. The amplification of 17q12 was found in all tumors, except for the ACTH-producing carcinoma. The four clinically functioning ACTH adenomas and the ACTH-CA shared the amplification of 10q11.22 and showed more copy-number variation (CNV) gains and single-nucleotide variations than the nonfunctioning tumors