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Objective  The critical mediastinal mass syndrome (CMMS) is a life-threatening condition and is challenging for physicians. We analyse the clinicopathological profile and outcome of CMMS from a large tertiary-care pediatric oncology center in Pakistan.  Methods  We retrospectively reviewed the medical record of a tertiary-care hospital in Pakistan from April 2017 to September 2019 for all children (1 month-16 years) who presented with an anterior mediastinal mass (AMM). A CMMS case is defined as a child with an AMM presenting with cardiorespiratory compromise and needing intensive care support. Demographic data, clinical profile, pathological diagnosis, and outcome of all such children were recorded. Descriptive statistics were applied using the Statistical Package for the Social Sciences (SPSS), version 22 (IBM Corp., Armonk, NY).  Results  Of the total 221 mediastinal masses, 61 children were diagnosed as CMMS and enrolled in the study. The mean age was 9 ± 3.3 years, and 68.9%% were male; 65.6% of patients had a weight for age less than the fifth percentile. A total of 49.2% of patients had a duration of illness of more than one month before diagnosis. Fever (97.6%) and lymphadenopathy (82%) were the most common findings, along with respiratory and cardiovascular signs and symptoms; 9.8% had superior vena cava syndrome. The pericardial effusion was present in 54.6% and 27.9% had pleural effusion. Peripheral blood flow cytometry made the diagnosis in 59%, peripheral lymph node biopsy in 13%, mediastinal core biopsy in 5%, and pleural fluid flow cytometry in one case; 62.3% had a white blood cell count of >100,000/mm 3 . A total of 72.1% (n=44) cases were diagnosed as T-cell acute lymphoblastic leukemia in our cohort. Clinical and laboratory tumor lysis syndrome developed in 10% and 73% of cases, respectively. Mechanical ventilation was required in 9.8% of the cohort. Mortality was reported in 10 (16.4%) patients.  Conclusion  We found that the 100% fatality rate with controlled positive pressure ventilation and spontaneous breathing is ideal. Tumour lysis syndrome was the most common morbidity in our cohort.

Morbidity and Mortality Associated With Pediatric Critical Mediastinal Mass Syndrome