TITLE:
Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor
AUTHORS:
Tazi Charki Mohammed, Akammar Amal, Dardar Hajar, Abdellaoui Hicham, Atarraf Karima, Boubou Meryem, Afifi Moulay Abderrahmane
KEYWORDS:
Congenital, Rhabdoid Tumor, Soft Tissue, Diagnosis, Immunohistochemistry, INI-1
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.14 No.3,
May
17,
2024
ABSTRACT: Rhabdoid tumors (RTs) are a well-defined entity in the kidney or central nervous system of infants or children. However, soft-tissue involvement is uncommon. It’s an exceptional neonatal tumor of soft tissue. The imaging characteristics of this tumor are not specific. Biopsy allows diagnosis; the histomorphological characteristics of rhabdoid tumors, their immunoreactivity to epithelial markers and vimentin, and the INI-1 loss are important tools for diagnosis. RT tumors are aggressive and have a rapidly fatal clinical course in most cases. Despite multidisciplinary therapy, the survival rate is very low. We report a rare case occurring in a male neonate who presents at birth with a voluminous right axillary mass. A CT scan showed a well-limited tumor mass with lobulated contours. An ultrasound-guided biopsy was performed on day 8, showing the morphology and immunoprofile of RT. The mass showed rapid growth. The child was admitted for respiratory distress at 3 weeks. A thoraco-abdominal CT showed an increase in the size of the mass with the appearance of multiple lymph nodes and pleural, hepatic, and renal metastases. The child died two days later.