Krebswissenschaft und -therapie

Rhabdoid Tumors in Children: About a Moroccan Pediatric Population

Abstract

Hajar Bettach*, Meriam Haloua, Nizar El Bouardi, Badr Alami, Moulay Youssef Alaoui Lamrani, Mustapha Maâroufi and Meryem Boubbou

Background: Rhabdoid tumor (RT) is a rare, highly malignant tumor that occurs primarily in the central nervous system (CNS), also known as atypical Rhabdoid/teratoid tumor (AT/RT), kidney (malignant Rhabdoid tumor of the kidney) or soft tissue (malignant extra-renal Rhabdoid tumor extra-cerebral). Infants, usually in their first year of life, may also have a dual location of primary RT, consistent with a genetic predisposition to cancer.

Materials and Methods: This is a retrospective study of a series of RT collected at the Mother and Child Radiology Department of the Hassan II hospital in Fez during a period of 7 years (janvier 2016 to December 2021). Epidemiological, clinical radiological, pathological and therapeutic results were analyzed by Excel.

Results: RT represents a frequency of 1.16 new cases per year followed in the Mother and Child Radiology Department. The median age of the study population was 23 months (01 month-5 years) and the sex ratio was 1.33 (F/M). Abdominal volume increase was the most common cause of consultation for the kidney locations followed by an intracranial hypertension syndrome for the brain locations. Majority of cases were diagnosed at locally advanced and metastatic stages (62.5%of cases). 57% was a renal RT, 14% was a temporal supratentorial cerebral localization, 14% was an orbital localization and 14% was a localization of the soft parts of the left axillary, among these cases two patients had a bifocal localization with an atypical AT/RT at the level of the posterior cerebral fossa.

Four cases in this study underwent surgery; it was complete ans without microscopic residue. One patient received an exclusive radiotherapy. Chemotherapy neoadjuvant was prescribed in four patients and mostly in palliative setting in one patient. Four patients were cured and three patients were died from disease.

Conclusion: RT is rare, highly malignant tumors. The radiologist therefore has an important role for in the management of these patients, both in the diagnostic phase, to affirm or deny the various signs of malignancý as well as in the therapeutic and follow-up phases, to evaluate the efficacý of the treatment and detect signs of recurrence.