Basal Ganglia Germ Cell Tumors With or Without Sellar Involvement: A Long-Term Follow-Up in a Single Medical Center and a Systematic Literature Review

Front Endocrinol (Lausanne). 2021 Nov 10:12:763609. doi: 10.3389/fendo.2021.763609. eCollection 2021.

Abstract

Background: Basal ganglia germ cell tumors (BGGCTs) represent an extremely rare subset of tumors about which little is known. Some patients suffer from tumor dissemination, such as sellar involvement. This study aimed to evaluate the independent prognostic risk factors of patients with BGGCTs with or without sellar involvement.

Methods: Sixteen patients were diagnosed with BGGCTs at Peking Union Medical College Hospital from January 2000 to December 2020. A literature review was performed on the online databases Medline and PubMed, and 76 cases in the 19 retrieved articles were identified at the same time. The data regarding biochemical tests, radiological examinations, and outcomes during follow-up were analyzed.

Results: Of 92 patients in this study, seven patients were clinically diagnosed as germinomas, with the remaining 85 patients receiving surgery. Fifty-two patients suffered from multifocal lesions or tumor dissemination. The patients with BGGCTs demonstrated a significant male predilection. The patients with delayed diagnosis more likely had cognitive disturbance (p = 0.028), mental disturbance (p = 0.047), and diabetes insipidus (p = 0.02). Multivariate analysis demonstrated that the independent poor prognostic risk factors of patients with BGGCTs were delayed diagnosis [odd ratio (OR) 2.33; 95% CI 1.02-5.31], focal radiotherapy (OR 4.00; 95% CI 1.69-9.49), and non-pure germinoma (OR 4.64; 95% CI 1.76-12.22).

Conclusions: The delayed diagnosis, focal radiotherapy, and non-pure germinoma were associated with a poorer prognosis for patients with BGGCTs with or without sellar involvement.

Keywords: basal ganglia germ cell tumors; delayed diagnosis; independent prognostic risk factors; sellar involvement; surgical therapy.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Basal Ganglia / diagnostic imaging*
  • Brain Neoplasms / diagnostic imaging*
  • Brain Neoplasms / epidemiology
  • Brain Neoplasms / therapy
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Neoplasms, Germ Cell and Embryonal / diagnostic imaging*
  • Neoplasms, Germ Cell and Embryonal / epidemiology
  • Neoplasms, Germ Cell and Embryonal / therapy
  • Sella Turcica / diagnostic imaging*
  • Skull Neoplasms / diagnostic imaging*
  • Skull Neoplasms / epidemiology
  • Skull Neoplasms / therapy
  • Young Adult