Gursharn Singh Narang, Jivtesh Singh Pahwa
Department of Pediatrics, Sri Guru Ram Das Institute of Medical Sciences and Research, Vallah, Amritsar, India
Address for Correspondence: Dr Gursharn Singh Narang, Associate Professor, Department of Pediatrics, SGRDMISAR, Vallah, Amritsar.
Email: jivteshpahwa@rediffmail.com
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Discussion :
It is a Rhabdomyosarcoma of the orbit. Histopathology of the mass showed embryonal rhabdomyosarcoma. According to the Intergroup Rhabdomyosarcoma Study {IRS} the tumor was staged in stageI, groupIII. Patient underwent surgery for debulking of the tumor followed by radiation therapy.
Rhabdomyosarcoma {RMS} is a rare tumor, with an annual incidence of 4.3 cases per million children. {1} The orbit is the primary site in approximately 10 percent of these tumors. {2,3} RMS is the most common malignant orbital tumor of childhood. RMS should be suspected whenever the clinical presentation of a rapidly progressive unilateral exophthalmos is observed in a child. Although the superior nasal quadrant of the orbit is the most common location of this tumor, it may also present as a palpable subconjunctival or eyelid nodule with edema of the lids and, or chemosis. Following the biopsy, the tumor should be staged according to the classification systems of the Intergroup Rhabdomyosarcoma Study {IRS} and American Joint Commission
on Cancer manual. {4} CT and MR images are fundamental in the preoperative
evaluation to determine location and size, but they are also important in evaluating residual or recurrent disease. RMS is best managed with combination treatment of chemotherapy, external-beam radiation therapy, and surgery. {5} Exenteration of the orbit is now confined to cases with recurrent disease. {6}
Last Updated: February 2011 Vol 8 Issue 2 Art # 12 | References : | - Maurya OPS, R Patel Thakur V, Singh R, Kumar M. Embryonal rhabdomyosarcoma of orbit- A case report. Indian J Ophthalmol 1990;38:202-4.
- Maurer HM, Gehan EA, Beltangady M, et al. The Intergroup Rhabdomyosarcoma Study-II. Cancer 1993; 71: 1904-1922
- Crist W, Gehan EA, Ragab AH, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol. 1995; 13: 610-630
- Shields CL, Shields JA, Honavar SG, et al. Clinical spectrum of primary ophthalmic rhabdomyosarcoma. Ophthalmology. 2001; 108: 2284-2292
- Karcioglu Z, Hadjistilianou D, Rozans M, DeFrancesco S. Orbital Rhabdomyosarcoma. Cancer Control 2004; 11: 328-333
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| Correct Answers : |  21% |
Last Shown : Feb 2011
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