Spontaneous Partial Regression of Fetal Lung Interstitial Tumor With A2M::ALK Rearrangement in a Neonate

Pediatr Dev Pathol. 2024 Mar-Apr;27(2):187-192. doi: 10.1177/10935266231189929. Epub 2023 Oct 11.

Abstract

The differential diagnosis for neonatal primary lung masses includes developmental anomalies and congenital lung tumors. Fetal lung interstitial tumor (FLIT) is a rare benign mesenchymal lesion which presents either antenatally or within the first 3 months of age. FLIT is a circumscribed solid-cystic mass which histologically resembles the fetal lung during the canalicular stage at 20-24 weeks of gestation. It is composed of immature mesenchymal cells expanding the interstitium and irregular airspace-like structures. Of all published cases, only 1 identified an α2-macroglobulin (A2M)::anaplastic lymphoma kinase (ALK) fusion and all cases underwent surgical resection in the neonatal or infancy period. We present the second case of FLIT with an A2M::ALK fusion diagnosed postnatally in a neonate which partially regressed spontaneously during conservative management with interim resection at 39 months of age, and provide a review of the literature.

Keywords: A2M::ALK fusion; fetal lung interstitial tumor; neonate; pulmonary.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anaplastic Lymphoma Kinase / genetics
  • Female
  • Humans
  • Infant, Newborn
  • Lung / pathology
  • Lung Neoplasms* / congenital
  • Lung Neoplasms* / diagnosis
  • Lung Neoplasms* / genetics
  • Oncogene Proteins, Fusion* / genetics
  • Pregnancy
  • Pregnancy-Associated alpha 2-Macroglobulins*
  • alpha-Macroglobulins / genetics

Substances

  • A2M protein, human
  • alpha-Macroglobulins
  • Anaplastic Lymphoma Kinase
  • Pregnancy-Associated alpha 2-Macroglobulins
  • A2M-ALK fusion protein, human
  • Oncogene Proteins, Fusion