loading page

IMPLICATIONS OF DEFERRED DIAGNOSIS OF PAEDIATRIC INTRACRANIAL GERM CELL TUMOURS
  • +2
  • Cristina Partenope,
  • Gabriella Pozzobon,
  • Giovanna Weber,
  • Fernando Carceller,
  • Assunta Albanese
Cristina Partenope
San Raffaele Hospital

Corresponding Author:[email protected]

Author Profile
Gabriella Pozzobon
IRCCS Ospedale San Raffaele
Author Profile
Giovanna Weber
IRCCS Ospedale San Raffaele
Author Profile
Fernando Carceller
Royal Marsden Hospital Sutton
Author Profile
Assunta Albanese
Royal Marsden Hospital Sutton
Author Profile

Abstract

AIMS This study analysed the clinical features of children with intracranial germ cell tumours (IC-GCTs) treated at two European centres. We retrospectively reviewed timelag between symptoms onset, clinic-radiological findings, diagnosis and outcomes. METHODS Symptoms at diagnosis were divided into four groups: 1)raised intracranial pressure (RICP); 2)visual impairment; 3)endocrinopathy; 4)other. Total diagnostic interval (TDI), defined as the interval between symptom onset (including retrospective recall of symptoms) and definitive diagnosis of IC-GCT, was calculated and compared to survival rates. RESULTS Our cohort included 55 children with a median follow-up of 78.9 months (0.5-249.9). The majority (63.6%) had germinomas and 10.9% were metastatic at diagnosis. IC-GCTs were suprasellar (41.8%), pineal (36.4%), bifocal (12.7%) or in atypical sites (9.1%). The most common presenting symptoms were related to RICP (43.6%); however, by the time of tumour diagnosis, 50.9% of the patients had developed endocrine dysfunctions. All pineal GCTs manifested with RICP or visual impairment. All suprasellar GCTs presented with endocrinopathies. TDI ranged between 0.25-58.5 months (median 4 months). Pineal GCTs had the shortest TDI (median TDI 1 month versus 24 months in suprasellar GCTs, p<0.001). TDI >6 months was observed in 47.3% of patients and was significantly associated with endocrine presenting symptoms. No statistically significant difference was found in progression-free survival and overall survival between patients with TDI >6 months and with TDI 6 months. CONCLUSION Approximately half of our patients had TDI >6 months, mostly with endocrine deficiencies as presenting symptoms. TDI >6 months was not associated with increased relapse rate or mortality.
25 Feb 2022Submission Checks Completed
25 Feb 2022Assigned to Editor
25 Feb 2022Submitted to Pediatric Blood & Cancer
27 Feb 2022Reviewer(s) Assigned
17 Mar 2022Review(s) Completed, Editorial Evaluation Pending
21 Mar 2022Editorial Decision: Revise Major
18 Jun 20221st Revision Received
18 Jun 2022Submission Checks Completed
18 Jun 2022Assigned to Editor
26 Jun 2022Reviewer(s) Assigned
28 Jun 2022Review(s) Completed, Editorial Evaluation Pending
04 Jul 2022Editorial Decision: Revise Minor
10 Sep 20222nd Revision Received
10 Sep 2022Submission Checks Completed
10 Sep 2022Assigned to Editor
18 Sep 2022Reviewer(s) Assigned
18 Oct 2022Review(s) Completed, Editorial Evaluation Pending
24 Oct 2022Editorial Decision: Revise Major
16 Nov 2022Submission Checks Completed
16 Nov 2022Assigned to Editor
16 Nov 20223rd Revision Received
17 Nov 2022Review(s) Completed, Editorial Evaluation Pending
21 Nov 2022Editorial Decision: Revise Minor
25 Nov 2022Submission Checks Completed
25 Nov 2022Assigned to Editor
25 Nov 20224th Revision Received
26 Nov 2022Review(s) Completed, Editorial Evaluation Pending
28 Nov 2022Editorial Decision: Accept