"Mucormycosis" the Emerging Global Threat; Overview and Treatment Outcome Among Pediatric Cancer Patients in Egypt

Background: Mucormycosis is a serious emerging fungal infection. Unlike invasive aspergillosis, the prognosis and outcome of hematologic malignancy patients who develop invasive Mucormycosis have not significantly improved over the past decade.The aim of the study was to evaluate clinical characteristics and treatment outcome in pediatric oncology patients with Mucormycosis in children cancer hospital 57357, Cairo, Egypt. Patients and Methods: A retrospective study during the period from 2007 to 2016. Data collected included patients' demographics, diagnosis, risk factors, diagnostic work up, antifungal treatment given and treatment outcome. Diagnosis was made according to the EORTC/MSG modified criteria (2008). Results: During the study period, 45 patients developed proven Mucormycosis. Mean age was 8 years (range 2-17). Male to female ratio 1:1. Diagnosis of Mucormycosis was highest among patients with hematological malignancies (90%). Twenty-two cases of acute myeloid leukemia (49%), 17 cases acute lymphoblastic leukemia (37%), and 1 case of CML post-transplant. The other 10% were having solid malignancies. Sinus involvement has been documented in (55.5%) representing the sole localization in 16 cases. The other 9 patients had secondary localizations; 2 central nervous system (Rhino-cerebral) and 7 lung (Sino-pulmonary) cases. Other primary sites of infection were: Pulmonary, Cutaneous and Gastrointestinal in (13.3%), (8.8%) and (11%) respectively. Disseminated disease was seen in 5 cases (11%). All patients had infection documented by histopathology or/and culture. Diagnosis was reported by histopathology in 38 patients (84%), blood culture in 2 patients (4 %) and both culture and histopathology in 5 patients (12%). At time of diagnosis, 15 patients (33%) were on Voriconazole prophylaxis (n =13) or Micafungin (n= 2). Liposomal Amphotercin -B 5mg/kg/day was the mainstay of treatment with duration ranging from 3 to 4 weeks. Posaconazole was used as secondary prophylaxis in 35 % of cases mainly for patients with hematological malignancies. Combination of liposomal amphotericin B with caspofungin was used in 3 cases with progressive Mucormycosis. Surgical intervention was achievable in 50 % of cases; sinus debridement in 11 cases, lung lobectomy in 3 cases, intestinal resection anastomosis in 5 cases and debridement of necrotic lesion in 4 cases. Therapy was successful in 30 patients (66%). Co-Morbidities related to Mucormycosis was seen in 5 cases with disfigurement, and perforated hard palate. Chemotherapy delay with subsequent relapse of primary malignancy was reported in 1 case. Mucormycosis was the primary cause of death in 15 cases (34%). Gastrointestinal and disseminated Mucormycosis was independent risk factors for mortality. Conclusion: Early diagnosis of Mucormycosis infection, with rapid initiation of appropriate antifungal therapy and surgical intervention, whenever feasible, are the back bone of decreasing Mucormycosis-related mortality and morbidity among pediatric cancer patients. Disseminated and gastrointestinal Mucormycosis had higher risk for mortality.