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

Abstract

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.

Treatment and prognosis of pleuropulmonary blastoma: A single‐center report of 31 cases

“…However, a core needle biopsy cannot always yield the correct diagnosis because it may penetrate necrotic tissue. Furthermore, PPB progresses very rapidly and patients may not be able to wait for pathological results; for example, Xi Chai described how four of six patients developed adverse events while waiting for pathological results after core needle biopsy 12 . For these reasons, we have not adopted core needle biopsy, but rather upfront excision.…”

“…Furthermore, PPB progresses very rapidly and patients may not be able to wait for pathological results; for example, Xi Chai described how four of six patients developed adverse events while waiting for pathological after core needle biopsy. 12 For these reasons, we have not adopted core needle biopsy, but rather upfront excision. In our group, no preoperative adverse events occurred, but the overall survival rate was still relatively low.…”

Treatment and prognosis of pleuropulmonary blastoma: A Single-Center Report of 31 Cases

“…Furthermore, PPB progresses very rapidly and patients may not be able to wait for pathological results; for example, Xi Chai described how four of six patients developed adverse events while waiting for pathological results after core needle biopsy. 12 For these reasons, we have not adopted core needle biopsy, rather upfront excision.…”