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Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor

Extra Renal Rhabdoid Tumor: A Rare Cause of Congenital Soft Tissue Tumor
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摘要 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. 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.
作者 Tazi Charki Mohammed Akammar Amal Dardar Hajar Abdellaoui Hicham Atarraf Karima Boubou Meryem Afifi Moulay Abderrahmane Tazi Charki Mohammed;Akammar Amal;Dardar Hajar;Abdellaoui Hicham;Atarraf Karima;Boubou Meryem;Afifi Moulay Abderrahmane(Department of Pediatric Orthopedic and Traumatology, University Hospital Hassan II, Fez, Morocco;The Faculty of Medicine and Pharmacy, Sidi Mohamed Ben Abdellah University, Fez, Morocco;Department of Radiology, University Hospital Hassan II, Fez, Morocco)
出处 《Open Journal of Pediatrics》 2024年第3期579-584,共6页 儿科学期刊(英文)
关键词 CONGENITAL Rhabdoid Tumor Soft Tissue Diagnosis IMMUNOHISTOCHEMISTRY INI-1 Congenital Rhabdoid Tumor Soft Tissue Diagnosis Immunohistochemistry INI-1
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