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