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Atypical Teratoid/Rhabdoid tumor (ATRT) of the Cervical Spinal Cord in a neonate: An unusual case 2006

General Spine

Megan E Deacon-Casey, MD, Non ASSR Member
Deborah A Lee, MD, PhD, Non ASSR Member
Daniel F Broderick, MD, Non ASSR Member

Excerpta Extraordinaire


A 2 day old neonate was delivered to an 18 year old woman G1P0. Her pregnancy was complicated by multiple sclerosis and she delivered via C-section secondary to failure to progress and prolonged rupture of membranes after attempt with vacuum extraction. At the time of birth the patient was noted to have macrosomia, meconium stained amniotic fluid and respiratory distress. Initially the patient was floppy and unresponsive at birth requiring resuscitated. Within the first 24 hours the patient was able to move all four extremities; 48 hours later the neonate presented with seizures, minimal movement of the right upper extremity and decreased tone, which later progressed to bilateral upper extremity hypotonicity.

The neonate was initially evaluated with Computed Tomography (CT) of the head revealing no definite abnormality and probably a subtle hypodense region in the brainstem. Further evaluation with Magnetic Resonance Imaging (MRI) revealed a heterogeneous, T1 hypointense and T2 hyperintense, expansile mass in the cervical spinal cord. The mass demonstrated heterogeneous enhancement and contained several well-defined loculated components. There was contiguous cephalad extension of the lesion with involvement of the medulla. A surgical biopsy was performed revealing a histologic diagnosis of an extrarenal rhabdoid cervical spinal cord tumor.

Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive primitive central nervous system (CNS) neoplasm of infancy. The reported cases in the literature are commonly seen in the posterior fossa and upper cervical spinal cord. To our knowledge, this is the first reported case of ATRT presenting in a neonate. The imaging characteristics are not specific. The differential diagnosis includes other more common malignant tumors such as primitive neuroendocrine tumors (PNET). Rhabdoid tumor (RT) was first described as an aggressive neoplasm of unknown histogenesis affecting the kidneys of infants and young children, but has since been reported in all ages and in many other primary sites, including the central nervous system. The most common extrarenal location involves the posterior fossa. Although malignant ATRT are rare and the imaging features are nonspecific, it should be included in the differential diagnosis of childhood intracranial and spinal neoplasms.

ATRT is a rare, high grade tumor occurring most commonly in children younger than 2 years. It is generally found in the cerebellum. These tumors tend to be aggressive and frequently spread through the central nervous system. This diagnosis of ATRT has only been classified in the last three to five years; the tumor is a subset of medulloblastomas. They occur in about 1-2 percent of children with brain tumors. These tumors are often mistaken for medulloblastoma (MB) or central primitive neuroectodermal tumor (PNET) due to similar imaging features. It is important to distinguish ATRT from MB and PNET, since ATRT is associated with a particularly poor prognosis and an unrelenting clinical course when conventional therapies for MB and PNET are used. Treatment generally involves surgical removal of the tumor followed by chemotherapy. Radiation therapy may be considered depending on the age of the child and whether the tumor has recurred.