Clinical presentation and treatment of four children with pulmonary mucoepidermoid carcinoma

Ther Adv Respir Dis. 2024 Jan-Dec:18:17534666241258679. doi: 10.1177/17534666241258679.

Abstract

Primary lung cancer in childhood is extremely rare, with an incidence rate of less than 2/100,0000, and pulmonary mucoepidermoid carcinoma (PMEC), is even rarer. Their symptoms are usually not specific, and there are no guidelines for their management, which makes their clinical management a challenge for pediatricians. The purpose of this report is to discuss the clinical presentation, positive signs, examinations, pathological characteristics, surgical modalities, chemotherapy regimens, and prognosis in children. The clinical data of four patients diagnosed with PMEC at the Children's Hospital of Chongqing Medical University from June 2021 to November 2022 were retrospectively analyzed, and their clinical features, treatment, and prognosis were summarized. Among them, two were male and two were female; their ages ranged from 3 years and 10 months to 10 years and 11 months, and all were staged according to tumor node metastasis classification (TNM). Immunohistochemical tests were performed in all children, among which four cases were positive for cytokeratin (CK), two cases were positive for CK7, four cases were positive for p63, about 5-10% of tumor cells were positive for Ki67. Among the four children, three had surgery alone and one had surgery + chemotherapy. All four children are presently living, with no evidence of tumor recurrence or metastasis. PMEC in children is very rare, and its age of onset and symptoms are not specific, and there is no obvious correlation with gender. Its diagnosis mainly relies on pathomorphological diagnosis, and immunohistochemical detection has no specific performance. The prognosis of children with PMEC is related to the clinical stage and whether surgery is performed. Whether further chemotherapy or radiotherapy is needed for patients who cannot undergo surgical resection and for those who have a combination of distant metastases requires further clinical studies.

Keywords: children; prognosis; pulmonary mucoepidermoid carcinoma; surgery.

Plain language summary

Clinical presentation and treatment of 4 children with pulmonary mucoepidermoid carcinomaLung cancer in childhood is extremely rare, occurring at a rate of less than 2/1000000, and a type of lung cancer called pulmonary mucoepidermoid carcinoma (PMEC), is even rarer. The symptoms are usually not specific, and there are no guidelines for its management, which is a challenge for doctors. The purpose of this report is to discuss the signs and symptoms medical examinations, disease characteristics, surgical procedures, chemotherapy regimens and prognosis in children with pulmonary mucoepidermoid carcinoma. The clinical data of four patients diagnosed with pulmonary mucoepidermoid carcinoma at the Children’s Hospital of Chongqing Medical University from June 2021 to November 2022 were analyzed, and their clinical features, treatment and prognosis were summarized. All four children are currently alive, and there is no recurrence or spread of the tumor after treatment. We have discussed various aspects of the disease, such as the rate of occurrence, causes, signs and symptoms, the way in which it might be diagnosed and treated, and the survival rate after operation, hoping to provide some insights for future work.

MeSH terms

  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / metabolism
  • Carcinoma, Mucoepidermoid* / diagnosis
  • Carcinoma, Mucoepidermoid* / pathology
  • Carcinoma, Mucoepidermoid* / therapy
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Lung Neoplasms* / diagnosis
  • Lung Neoplasms* / pathology
  • Lung Neoplasms* / therapy
  • Male
  • Neoplasm Staging
  • Pneumonectomy
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Biomarkers, Tumor