Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar

Abstract

Introduction There are no previous published reports on primary pediatric tumors of the central nervous system (CNS) in Qatar. We undertook this retrospective cohort study to review the diagnosis of CNS tumors in children in Qatar to analyze the presentation characteristics including symptoms, referral pathways, and time to diagnosis. Methods All children registered with Pediatric Neuro-Oncology service (PNOS) were included in the study. Data from the time of diagnosis (October 2007 to February 2020) were reviewed retrospectively. Presenting symptoms were recorded and prediagnosis symptom interval (PSI) was calculated from the onset of the first symptom to the date of diagnostic imaging. Results Of the 61 children registered with PNOS during the study period, 51 were included in the final analysis. Ten children were excluded because they were either diagnosed outside Qatar (n = 7) or were asymptomatic at the time of diagnosis (n = 3). The median age was 45 (range 1-171) months. Common tumor types included low-grade glioma (LGG) (47.1%) and medulloblastoma/primitive neuroectodermal tumors (PNET) (23.5%). Nine children had an underlying neurocutaneous syndrome. Thirty-eight patients (74.5%) had at least one previous contact with healthcare (HC) professional, but 27 (52%) were still diagnosed through the emergency department (ED). Presenting symptoms included headache, vomiting (36.2%), oculo-visual symptoms (20.6%), motor weakness (18.9%), seizures, ataxia (17.2% each), irritability, cranial nerve palsies (12% each), and endocrine symptoms (10.3%). Median PSI was 28 days (range 1-845 days) for all CNS tumors. Longest PSI was seen with germ cell tumors (median 146 days), supratentorial location (39 days), and age above 3 years (30 days). Tumor characteristics of biological behavior (high-grade tumor) and location (infratentorial) were significantly associated with shorter PSI, as were presenting symptoms of ataxia, head tilt, and altered consciousness. Conclusions Although overall diagnostic times were acceptable, some tumor types were diagnosed after a significant delay. The awareness campaign, such as the "HeadSmart" campaign in the United Kingdom (UK), can improve diagnostic times in Qatar. Further research is required to better understand the reasons for the delay.

Cervical Primitive Neuroectodermal Tumor in an Adult: Case Report and Literature Review

“…Prognostic assessment and risk of recurrence can be estimated from the analysis of Ki-67 markers and nuclear proliferation antigen (NPA), while p53 protein positivity is associated with lower survival rates disease free. 5 6 8 This explains why most of the studies 1 2 3 5 6 7 8 9 found are case reports and molecular studies, considering that new therapeutic tests are being developed, which made it difficult to correlate postoperative outcomes and long-term prognoses with those of our patient.…”

“…2,3,5,7,9 The therapeutic approach should be studied on a case-by-case basis, considering the different locations of these lesions. [2][3][4][5][6][7][8][9][10][11] However, studies 4,5,[9][10][11] suggest that radical resection followed by radiation in the entire neuroaxis is the mainstay of treatment, with better results when adding adjuvant chemotherapy. When used, the chemotherapeutic agents that have yielded the best therapeutic responses were CCNU (lomustina), vincristine, cisplatin, procarbazine, etoposide, temozolamide, cyclophosphamide, and carboplatin.…”

“…Primitive neuroectodermal tumors are rare neoplasms of the central nervous system (CNS) that can affect children and adults. 1 2 3 4 5 6 7 7 9 When compared with other tumors in the pediatric population, PNETs represent ∼ 2.5% of neoplasms, and, after medulloblastoma and astrocytoma, they are the most common embryonic tumor of childhood (the average age at diagnosis is 9 years old). 1 2 3 4 5 The incidence ratio among male and female individuals is of 1.1 and 0.8 per million inhabitants respectively.…”

“…[2][3][4][5][6][7][8] Radical resection, followed by radiation on the entire neuroaxis, are the mainstays of the treatment, with better results when adding adjuvant chemotherapy to the treatment. 4,5,[9][10][11] The models proposed to elucidate the longterm prognosis demonstrate that adults with PNETs have a survival rate that ranges from $ 50% to 60% in 5 years, and from 40% to 50% in 10 years. 5 In the present article, we report a case of PNET in an adult individual, whose infrequent presentation differs from the main data found in the literature.…”

“…Radical resection, followed by radiation on the entire neuroaxis, are the mainstays of the treatment, with better results when adding adjuvant chemotherapy to the treatment. 4 5 9 10 11 The models proposed to elucidate the long-term prognosis demonstrate that adults with PNETs have a survival rate that ranges from ∼ 50% to 60% in 5 years, and from 40% to 50% in 10 years. 5…”