DIPG-28. BURDEN OF DIFFUSE INTRINSIC PONTINE GLIOMA DIAGNOSIS IN A DEVELOPING COUNTRY. Issue 2 (22nd June 2018)

• Record Type: Journal Article
• Authors: Al Zebin, Zebin; Sawidan, Maisa; Ibrahimi, Ahmed; Halalsheh, Hadeel; Khalifeh, Kawther; Ramlawi, Raed; Amayiri, Nisreen
• Is Part Of: Neuro-oncology. Volume 20:Issue 2(2018)supplement 2
• Page Start: i54
• Page End: i54
• Publication Date: 2018-06-22
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noy059.121
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: Diffuse Intrinsic Pontine Glioma (DIPG) is a universally fatal tumor with significant burden on families. Experiences from a developing country are rarely reported. We reviewed the medical charts of children treated for DIPG at KHCC/Jordan between 1/2008 and 9/2015 and fulfilled the radiological diagnostic criteria. We evaluated their clinical characteristics and disease burden. Twenty-eight patients were identified (50% females, 20 Jordanians). Median age 7.9years (3.1–17.6years). Twenty-three patients (82%) had 2/3 symptoms of long tracts, ataxia and cranial nerve deficits at median duration 7weeks (2-24weeks). Two patients had initial spinal metastasis; one was paralyzed. Five (19%) had attempted tumor biopsy. Three patients didn't receive radiotherapy; two refused and one intubated earlier. Median time to begin radiation was 15days (2-35days) with median duration 42days (35-64days). All patients except 3 received dexamethasone at diagnosis, mostly high dose >8mg/day for > 60days. Fifteen patients (63%) improved with radiation and 4 stabilized. Seventeen follow-up MRIs showed tumor shrinkage (5), stability (8) and progression (4). Seventeen patients required 1–5 hospitalizations (median 4 total days/ patient, 1-150days) most due to respiratory distress, altered-consciousness and near-death. All patients had DNR code except two died intubated (parental refusal). Median duration between DNR code and death was 127days (25-478days). While ten patients lost follow up, eighteen died at median 7.9months (1.3-16months); sixteen died hospitalized. With the disease rapid course and dismal prognosis, oncologists in developing countries should avoid unnecessary interventions (biopsy, follow-up MRIs, intubation) and advocate for palliative home care.