Pathological complete response to gefitinib in a 10-year-old boy with EGFR-negative pulmonary mucoepidermoid carcinoma: a case report and literature review

Clin Respir J. 2017 May;11(3):346-351. doi: 10.1111/crj.12343. Epub 2015 Aug 6.

Abstract

Background and aims: Pulmonary Mucoepidermoid carcinoma (MEC) accounts for 0.1-0.2% of all lung cancer. It occurred in the 3-78 years old, and 50% patients younger than 30. MEC has no standard treatment, but recently reports indicated MEC without epidermal growth factor receptor (EGFR) mutations sensitive to gefitinib.

Objectives: To explore a new standard treatment strategy for MEC, after reviewed literature related to MEC, we used Gefitinib to treatment a patient with EGFR-negative MEC, and observe its effects.

Methods: 10-year-old boy was diagnosed with low-grade MEC by bronchial lung biopsy, EGFR gene mutation test was negative. Gefitinib was administered as neoadjuvant therapy at 125 mg daily.

Results: The patient underwent right middle lobe, lower lobe resection and mediastinal lymph node dissection. After surgery, the patient had gained weight (5 kg) after 18 days of gefitinib therapy. A CT scan of the chest 1 month after surgical resection showed no recurrence, and followed for 22 months after treatment without tumour recurrence, suggesting that the patient was completely cured.

Conclusion: Gefitinib has potential to become a standard treatment for pulmonary MEC patients, including pediatric patients. However, the mechanisms need further investigation.

Keywords: CRTC1-MAML2; EGFR-TKI; lung cancer; mucoepidermoid carcinoma; pediatric.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Carcinoma, Mucoepidermoid / diagnostic imaging
  • Carcinoma, Mucoepidermoid / drug therapy*
  • Carcinoma, Mucoepidermoid / genetics*
  • Carcinoma, Mucoepidermoid / surgery
  • Child
  • DNA-Binding Proteins / genetics
  • ErbB Receptors / antagonists & inhibitors
  • ErbB Receptors / genetics*
  • Gefitinib
  • Humans
  • Lung Neoplasms / drug therapy*
  • Lung Neoplasms / pathology
  • Lung Neoplasms / surgery
  • Male
  • Mutation
  • Neoadjuvant Therapy / methods
  • Neoplasm Staging
  • Oncogene Proteins, Fusion / genetics
  • Protein Kinase Inhibitors / therapeutic use
  • Quinazolines / administration & dosage
  • Quinazolines / pharmacology*
  • Tomography, X-Ray Computed / methods
  • Transcription Factors
  • Treatment Outcome

Substances

  • CRTC1 protein, human
  • DNA-Binding Proteins
  • MAML1 protein, human
  • Oncogene Proteins, Fusion
  • Protein Kinase Inhibitors
  • Quinazolines
  • Transcription Factors
  • EGFR protein, human
  • ErbB Receptors
  • Gefitinib