Journal of Cancer Research and Therapeutics Medknow Publications on behalf of the Association of Radiation Oncologists of India (AROI) ISSN: 0973-1482 EISSN: 1998-4138 Vol. 5, Num. 4, 2009, pp. 318-320

Journal of Cancer Research and Therapeutics, Vol. 5, No. 4, October-December, 2009, pp. 318-320

Case Report

Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child

Agrawal Amit, Bhake Arvind, Cincu Rafael

Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha

Correspondence Address:Department of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha- 442 004, Maharashtra
dramitagrawal@gmail.com

Code Number: cr09077

PMID: 20160373

DOI: 10.4103/0973-1482.59906

Abstract

Keywords: Atypical teratoid/rhabdoid tumor, spinal tumors, paraspinal tumors, computerized tomography, pediatric spinal tumor, rhabdoid tumor

Introduction

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive and uncommon tumor of the central nervous system, primarily affecting young children; it has a rapidly progressive course and a fatal outcome. ^{[1],[2],[3],[4]} AT/RT of the paraspinal region, the spine, and the spinal cord is extremely rare, with only few case reports in the literature. ^{[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11]} We report an unusual case of a giant lumbar paraspinal AT/RT with intraspinal extension in a child. To the best of our knowledge, this kind of presentation has not been reported previously in the English literature.

Case Report

A previously healthy 18-month-old female child presented with a progressively increasing painful swelling over the left lumbar paraspinal region. There was no history of hematuria and fever. Her general and systemic examinations were normal. Local examination revealed a large, firm, tender lump over the left lumbar paraspinal region [Figure - 1]. The lump was non-ballotable and was not palpable per abdomen. Plain computerized tomography (CT) scan showed a large, well-defined, isodense, lobulated mass in the left paraspinal area, with erosion of the transverse process of the 2 ^nd lumbar vertebra [Figure - 2] A-C. The mass showed wavy contrast enhancement [Figure - 2] B and C. The left kidney was seen well separated from the mass and was pushed anteriorly and laterally [Figure - 2]B. The patient underwent surgery and near-total excision of the tumor was performed [Figure - 3]A-F. Histopathological examination showed a tumor containing primitive neuroectodermal and rhabdoid cells. The rhabdoid component was characterized by medium-sized, round to oval cells with eccentric nuclei, prominent nucleoli, and eosinophilic cytoplasm. There were frequent aberrant mitotic activity [Figure - 4] A and B. Based on all these histomorphological features, a diagnosis of atypical teratoid rhabdoid tumor (AT/RT WHO grade IV) was made. The patient was started on chemotherapy. Two months after surgery the patient presented with worsening of pain and weakness of both lower limbs. A repeat CT scan revealed extensive recurrence of the lesion.

Discussion

Rorke et al. termed these lesions as ′atypical teratoid/rhabdoid tumors′ (AT/RT) to highlight the fact that these are distinct central nervous system (CNS) neoplasms with a disparate combination of rhabdoid, primitive neuroepithelial, epithelial, and mesenchymal components. ^[1],[12] AT/RT arising from the paraspinal region and involving the spine is rare and is usually seen in young children (< 3 years), with no predilection for either sex. ^{[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11]} Imaging findings in intracranial AT/RT have been well documented; ^{[1],[2],[3],[4]} however, the imaging findings in paraspinal and spinal AT/RT are nonspecific. ^{[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11]} On imaging, these lesions appears as a large-sized, heterogeneous masses, which correlates well with their histopathologic complexity and aggressive nature. ^[5],[7] Warmuth-Metz et al. have described a distinct and unusual pattern of a wavy, band-like, enhancement surrounding a central hypointensity [Figure - 2] B and C, a typical feature of AT/RTs; ^[3] this pattern, although uncommon, was seen in the present case also. It has been recommended that, despite their nonspecific imaging features, rhabdoid tumor be included in the differential diagnosis of childhood intracranial and paraspinal region neoplasms. ^[2] Histologically, AT/RTs contain nests or sheets of rhabdoid tumor cells. In addition, they show the multiple-lineage developmental characteristics of malignant teratomas of neuroectoderm, mesoderm, and endoderm. AT/RT also express a wide range of immunohistochemical markers, including vimentin, epithelial membrane antigen, cytokeratin, synaptophysin, glial fibrillary acidic protein, and smooth muscle actin. ^{[1, 2, 6, 11, 12]} It has been suggested that recognition of the rhabdoid element is critical because this phenotype correlates with a significantly worse prognosis than the classic primitive neuroectodermal (PNET)/medulloblastoma (MB). ^[13],[14] The treatment of patients with AT/RT is complex and many current treatment regimens for AT/RTs are derived from the strategies used for management of PNET/ MB (i.e., radical surgery, chemotherapy, and radiotherapy). ^{[4],[13],[14],[15]} The outcome of patients with AT/RT is uniformly poor despite aggressive surgical and adjuvant radiochemotherapy, ^{[1],[2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[13],[14],[15]} and this was true in the present case also.

References

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