Sudden death in a female child doe to undiagnosed pleuropulmonary blastoma – An autopsy case and review of literature

Nishikata, Rie; Kato, Natsuki; Suto, Miwako; Rinno, Mami; Kuroda, Noriyuki; Hashimoto, Yuko

doi:10.1016/j.legalmed.2018.06.002

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“…Nishikata et al found the cause of sudden death of a female with PPB at autopsy to be respiratory dysfunction and that concurrent systemic inflammatory response syndrome had accelerated the process. The main causes of death in our study were respiratory failure and sepsis.…”

Section: Discussionmentioning

confidence: 99%

Clinical analysis of early death in children with pleuropulmonary blastoma in a single center in China

Chai

Zhang

et al. 2019

Pediatric Blood & Cancer

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Objective Pleuropulmonary blastoma (PPB) is a rare malignant tumor in childhood that is highly invasive and has poor prognosis. We retrospectively analyzed patients with PPB who died within 30 days in hopes of providing a basis for improving diagnosis and treatment. Methods We retrospectively reviewed six children with PPB who died within 30 days of admission at our hospital from January 2004 to March 2018, including their clinical features, pathological diagnosis, course of treatment, and major causes of death. Results Six patients (two female, four male; median age, 38 months) were included. All patients presented with respiratory symptoms. Chest imaging showed that all tumors had diameters greater than 10 cm, with varying degrees of serous effusion. Four patients underwent ultrasound‐guided fine‐needle aspiration (FNA), one patient underwent exploratory thoracotomy, and one underwent partial tumor resection. Five cases were type III, and another was type II. Four patients developed adverse events while waiting for pathological results after FNA, and four patients were treated with chemotherapy but their tumors failed to decrease in size one to two weeks later. The median hospitalization to death time was 17 days (range, 5–24 days). Conclusions PPB often presents with respiratory symptoms that rapidly develop into respiratory distress. The rapid tumor enlargement contributes to the disease's progression. Chemoreduction in such tumors is not obviously effective, and the mortality rate is high. The main causes of death were respiratory failure and sepsis. Further clinical studies will be required to evaluate the role of initial biopsy compared with upfront total excision.

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Section: Discussionmentioning

confidence: 99%