Fetal Mediastinal Fibrosarcoma. Report of Two Cases

ÇAĞAN M., YILDIRIM S., Turkmen G. G., ÖZYÜNCÜ Ö., AKÇÖREN Z., DEREN Ö., ...More

FETAL AND PEDIATRIC PATHOLOGY, vol.41, no.5, pp.843-851, 2022 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 41 Issue: 5
  • Publication Date: 2022
  • Doi Number: 10.1080/15513815.2021.1988010
  • Journal Name: FETAL AND PEDIATRIC PATHOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, EMBASE, MEDLINE
  • Page Numbers: pp.843-851
  • Keywords: Fetal tumor, congenital fibrosarcoma, fetal ultrasound, prenatal diagnosis, ETV6-NTRK3 gene fusion, CONGENITAL-INFANTILE FIBROSARCOMA, MANAGEMENT
  • Hacettepe University Affiliated: Yes

Abstract

Introduction: One-third of fetal soft tissue tumors are malignant and include congenital fibrosarcoma (CF). We report two fetal CFs arising in the posterior mediastinum. Case Presentation: In case 1, the CF resulted in a mediastinal shift, extensive infiltration of the tumor around adjacent structures, pulmonary hypoplasia, pleural effusion, and rapid growth. The pregnancy was terminated. Case 2 had multiple intrathoracic masses, thoracic hypoplasia, pleural effusion, and fetal death. Both were diagnosed as fibrosarcoma at fetopsy. Discussion: Although congenital CF tends to be locally aggressive with a low metastatic rate, it tends to grow rapidly and the tumor location can affect fetal survival. In Case 1, the tumor demonstrated locally aggressive behavior whereas multiple distant metastases such as lung, liver, adrenals, and left eye were detected in Case 2. The tumor was directly responsible for intrauterine fetal demise in the second case.