[01] Ribhav Pasricha, Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India. [02] Guru Dutta Satyarthee, Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India. [03] Suvendu Purkait, Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India. [04] Mehar Chand Sharma, Department of Neuropathology, All India Institute of Medical Sciences, New Delhi, India. [05] Bhawani Shankar Sharma, Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India.

Atypical rhabdoid tumor is a malignant central nervous system tumor occurring predominantly in infants and carries high mortality and a poor prognosis. A 6-month old child who presented with posterior fossa mass lesion, was preoperatively detected to have coexistent patent foramen ovale and atrial septal defect. These congenital heart defects complicated the surgical and anesthetic management for the patient. The child underwent shunting for hydrocephalus followed by suboccipital craniectomy with gross total excision. The histopathology showed atypical teratoid/rhabdoid tumor, as confirmed by the loss of INI1 expression in the tumor cells. These tumors are highly malignant and carry very poor prognosis for the patients, most of whom are less than 2 years of age at diagnosis. The mean survival following surgery in these tumors is 11 months, although recent series suggest longer survival with trimodality therapy involving complete surgical resection; intravenous and intrathecal chemotherapy; and intensity-modulated radiotherapy.

Infant, Atypical Rhabdoid Teratoma, Congenital Heart Disease, Patent Foramen Ovale, Atrial Septal Defect, Atypical Teratoid Rhabdoid Tumor, ATRT

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