Mixed Gangliocytoma-Pituitary Adenoma: A Systematic Review of Diagnostic Features, Clinical Management, and Surgical Outcomes.

in World neurosurgery by Kishore Balasubramanian, Romulo Augusto Andrade de Almeida, Abdurrahman F Kharbat, Ali S Haider, Ian F Dunn, Christopher S Graffeo

TLDR

  • Mixed gangliocytoma-pituitary adenomas (MGPAs) are rare tumors that can cause a variety of symptoms, including headaches, acromegaly, and visual disturbances. The best way to treat these tumors is with a procedure called transsphenoidal resection, which removes the tumor from the pituitary gland. This procedure is usually successful, but sometimes patients need additional treatments to control the tumor. Despite the challenges, the study found that all patients who received these treatments had their tumors controlled and were still alive after a certain period of time.

Abstract

Mixed gangliocytoma-pituitary adenomas (MGPAs) are very rare sellar neoplasms. Contemporary understanding of their natural history, clinical characteristics, optimal management strategies, and prototypical outcomes remains poorly understood. Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, PubMed, EMBASE, Web of Science, and Cochrane were searched to include studies of patients with histologically confirmed Mixed Gangliocytomas (MGPAs). Clinical features, management strategies, and outcomes were analyzed. 33 studies reporting 78 patients met study criteria and were included. Median age at diagnosis was 47 years, with a female predominance (71%). Common presenting symptoms included headache (49%), acromegaly (43%), and visual disturbance (20%). Growth hormone (GH) and prolactin were the most commonly elevated hormones. Magnetic resonance imaging (MRI) identified local infiltration in 86% of patients, most frequently in the cavernous sinus. Transsphenoidal resection was the primary treatment strategy, which achieved a gross total resection (GTR) in 43%. Postoperative pituitary function was normal in 92%. Median follow-up was 21 months, during which time 14% of patients required secondary intervention for persistent hormonal hypersecretion. Following these secondary interventions and as of last follow-up, radiographic and/or biochemical control was confirmed in all patients, with an overall survival rate of 97%. MGPAs are rare, slow-growing tumors that present with a combination of endocrinological and neurological symptoms. MGPAs are optimally managed with transsphenoidal resection, which appears to achieve favorable rates of symptomatic relief and local control, despite the high incidence of local infiltration and relatively low attendant GTR rate.

Overview

  • The study aims to provide a comprehensive understanding of mixed gangliocytoma-pituitary adenomas (MGPAs).
  • The methodology used for the experiment includes searching PubMed, EMBASE, Web of Science, and Cochrane for studies of patients with histologically confirmed MGPAs. Clinical features, management strategies, and outcomes were analyzed. 33 studies reporting 78 patients met study criteria and were included.

Comparative Analysis & Findings

  • The study found that MGPAs are rare, slow-growing tumors that present with a combination of endocrinological and neurological symptoms. Growth hormone (GH) and prolactin were the most commonly elevated hormones. Magnetic resonance imaging (MRI) identified local infiltration in 86% of patients, most frequently in the cavernous sinus. Transsphenoidal resection was the primary treatment strategy, which achieved a gross total resection (GTR) in 43%. Postoperative pituitary function was normal in 92%. Median follow-up was 21 months, during which time 14% of patients required secondary intervention for persistent hormonal hypersecretion. Following these secondary interventions and as of last follow-up, radiographic and/or biochemical control was confirmed in all patients, with an overall survival rate of 97%.

Implications and Future Directions

  • The study highlights the importance of transsphenoidal resection as the primary treatment strategy for MGPAs. Further research is needed to determine the optimal management strategies for MGPAs, including the role of secondary interventions and the long-term outcomes of these interventions. The study also emphasizes the need for a multidisciplinary approach to the management of MGPAs, involving endocrinologists, neurosurgeons, and other specialists.
Read Full Article