Fetal Mediastinal Fibrosarcoma. Report of Two Cases

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

FETAL AND PEDIATRIC PATHOLOGY, cilt.41, sa.5, ss.843-851, 2022 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 41 Sayı: 5
  • Basım Tarihi: 2022
  • Doi Numarası: 10.1080/15513815.2021.1988010
  • Dergi Adı: FETAL AND PEDIATRIC PATHOLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, EMBASE, MEDLINE
  • Sayfa Sayıları: ss.843-851
  • Anahtar Kelimeler: Fetal tumor, congenital fibrosarcoma, fetal ultrasound, prenatal diagnosis, ETV6-NTRK3 gene fusion, CONGENITAL-INFANTILE FIBROSARCOMA, MANAGEMENT
  • Hacettepe Üniversitesi Adresli: Evet

Özet

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.