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Primary ophthalmic rhabdomyosarcoma
Reference 1. Fisher RF. The elastic constants of the human lens. J Physiol 1971;212:147– 80.
Primary Ophthalmic Rhabdomyosarcoma Dear Editor: In the report “Clinical Spectrum of Primary Ophthalmic Rhabdomyosarcoma” by Shields et al (Ophthalmology 2001;108:2284 –92), the authors reviewed their experiences with 33 consecutive cases of primary orbital rhabdomyosarcoma, demonstrating local tumor recurrence in 18% of the patients and metastasis in 6% of the patients. The authors also discussed the history of the current treatment recommendations for this disease, but we believe their historical review is incomplete. Shields et al conveyed that the great advances in the management of orbital rhabdomyosarcoma, such as the abandonment of exenteration as a favored treatment, were a result of the Intergroup Rhabdomyosarcoma Study Group, whose members first published their work in 1988. In fact, the major breakthroughs in the treatment of this disease were a result of hard work by radiation oncologists, ophthalmologists, and pediatric oncologists at Columbia Presbyterian Medical Center in New York, 20 years earlier. Their work was reported in a number of papers that were not referenced in the review by Shields et al. In 1968, Sagerman et al published the results of a study of radiation therapy used as the sole treatment modality for orbital rhabdomyosarcoma (Trans Am Acad Ophthalmol Otolaryngol 1968; 72:849 –54). An update of this study was reported in 1972 (Am J Roentgenol Radium Ther Nucl Med 1972;114:31– 4), and then a paper on 31 patients was published in 1974 (Trans Am Acad Ophthalmol Otolaryngol 1974;78:602–5). At that time, local control was obtained in 90% of patients. Research on the treatment of orbital rhabdomyosarcoma was pioneered by Drs. Ellsworth, Tretter, Wolff, and Kitchin at Columbia Presbyterian and continued throughout the 1970s. In 1978, one of us had the honor of presenting this work at the American Academy of Ophthalmology meeting; in 1979 we reported on 58 patients who were managed with combinations of external beam radiation and chemotherapy (Ophthalmology 1979;86:1330 –5). The chemotherapy regimen used included vincristine, cyclophosphamide, and dactinomycin. In that paper, we documented a 74% survival rate, with local tumor control in 91% of cases. We do not wish to minimize the value of the studies published by the Intergroup Rhabdomyosarcoma Study Group; however, the current treatment recommendations for patients with rhabdomyosarcoma of the orbit are virtually unchanged from the suggestions made at Columbia Presbyterian in the 1970s. Presently, patients with orbital rhabdomyosarcoma confined to the orbit are still treated with external beam radiation (albeit at lower dose than in the 1970s) as well as a chemotherapeutic regimen of vincristine, cyclophosphamide, dactinomycin, and etoposide in more advanced cases. Hence, the truly revolutionary changes in treatment approach—those that improved both survival rates and cosmetic results—were made not in the late 1980s, but more than 30 years ago by the physicians at Columbia Presbyterian.
We can understand how the authors of this review could omit one or two references, but the exclusion of four papers, three of which were published in this very journal and all of which were central in the history of the management of this disease, is unacceptable. I think it is appropriate to set history straight. Both the authors and the reviewers should have done a better job. DAVID H. ABRAMSON, MD ROBERT SAGEREMAN, MD New York, New York Author reply Dear Editor: Drs. Abramson and Sagerman have been instrumental in both past and present therapeutic protocols regarding many ocular neoplasms. Their contribution to the field of rhabdomyosarcoma is notable and should be included in any major review of this subject. The goal of our paper primarily was to educate the ophthalmic community on the results of the Intergroup Rhabdomyosarcoma Study Group (IRSG), a major prospective collaborative investigation of chemotherapy and radiotherapy for rhabdomyosarcoma. In the opening paragraph of our report, we discuss this study and point out that IRSG reports have been published in the pediatric, oncologic, and radiologic literature, but few have been published in the ophthalmic literature. Thus, the focus of our report was to inform the ophthalmic readers of the results from nearly three decades of work emanating from the IRSG. A secondary focus of the report was to discuss our own results and, importantly, to evaluate ophthalmic complications from therapy, a subject that had not been completely evaluated previously. As we reviewed the literature using Medline research at the National Library of Medicine, we noted that there had been 7269 published reports on rhabdomyosarcoma and 230 published reports on orbital rhabdomyosarcoma. Thus, we could not include all published literature in our report, and we referenced the 42 most relevant reports, including those on the IRSG (19 references), general pediatric oncology (five references), general ophthalmic oncology (four references), survey of orbital tumors in children (one reference), orbital imaging (two references), major survey of orbital rhabdomyosarcoma (one reference), large series of orbital rhabdomyosarcoma (one reference predating IRSG and one reference regarding refractory cases), and relevant case reports (eight references). Two of the articles that we quoted (references 16 and 17) did reflect the early experience by physicians at Columbia Presbyterian Hospital. The additional articles by Drs. Abramson and Sagerman were important contributions and likely would have added depth to our list of references. We do acknowledge their contributions to the treatment of rhabdomyosarcoma and we regret that we could not quote all of the early work from Columbia Presbyterian Hospital. However, the more recent organized studies of the IRSG, which have established the groundwork for current management, are appropriately cited. Overall, we believe we all should be pleased with our unified goal of improving survival for those children with rhabdomyosarcoma. Our joint enthusiasm from many cen-
877
Primary Ophthalmic Rhabdomyosarcoma Dear Editor: In the report “Clinical Spectrum of Primary Ophthalmic Rhabdomyosarcoma” by Shields et al (Ophthalmology 2001;108:2284 –92), the authors reviewed their experiences with 33 consecutive cases of primary orbital rhabdomyosarcoma, demonstrating local tumor recurrence in 18% of the patients and metastasis in 6% of the patients. The authors also discussed the history of the current treatment recommendations for this disease, but we believe their historical review is incomplete. Shields et al conveyed that the great advances in the management of orbital rhabdomyosarcoma, such as the abandonment of exenteration as a favored treatment, were a result of the Intergroup Rhabdomyosarcoma Study Group, whose members first published their work in 1988. In fact, the major breakthroughs in the treatment of this disease were a result of hard work by radiation oncologists, ophthalmologists, and pediatric oncologists at Columbia Presbyterian Medical Center in New York, 20 years earlier. Their work was reported in a number of papers that were not referenced in the review by Shields et al. In 1968, Sagerman et al published the results of a study of radiation therapy used as the sole treatment modality for orbital rhabdomyosarcoma (Trans Am Acad Ophthalmol Otolaryngol 1968; 72:849 –54). An update of this study was reported in 1972 (Am J Roentgenol Radium Ther Nucl Med 1972;114:31– 4), and then a paper on 31 patients was published in 1974 (Trans Am Acad Ophthalmol Otolaryngol 1974;78:602–5). At that time, local control was obtained in 90% of patients. Research on the treatment of orbital rhabdomyosarcoma was pioneered by Drs. Ellsworth, Tretter, Wolff, and Kitchin at Columbia Presbyterian and continued throughout the 1970s. In 1978, one of us had the honor of presenting this work at the American Academy of Ophthalmology meeting; in 1979 we reported on 58 patients who were managed with combinations of external beam radiation and chemotherapy (Ophthalmology 1979;86:1330 –5). The chemotherapy regimen used included vincristine, cyclophosphamide, and dactinomycin. In that paper, we documented a 74% survival rate, with local tumor control in 91% of cases. We do not wish to minimize the value of the studies published by the Intergroup Rhabdomyosarcoma Study Group; however, the current treatment recommendations for patients with rhabdomyosarcoma of the orbit are virtually unchanged from the suggestions made at Columbia Presbyterian in the 1970s. Presently, patients with orbital rhabdomyosarcoma confined to the orbit are still treated with external beam radiation (albeit at lower dose than in the 1970s) as well as a chemotherapeutic regimen of vincristine, cyclophosphamide, dactinomycin, and etoposide in more advanced cases. Hence, the truly revolutionary changes in treatment approach—those that improved both survival rates and cosmetic results—were made not in the late 1980s, but more than 30 years ago by the physicians at Columbia Presbyterian.
We can understand how the authors of this review could omit one or two references, but the exclusion of four papers, three of which were published in this very journal and all of which were central in the history of the management of this disease, is unacceptable. I think it is appropriate to set history straight. Both the authors and the reviewers should have done a better job. DAVID H. ABRAMSON, MD ROBERT SAGEREMAN, MD New York, New York Author reply Dear Editor: Drs. Abramson and Sagerman have been instrumental in both past and present therapeutic protocols regarding many ocular neoplasms. Their contribution to the field of rhabdomyosarcoma is notable and should be included in any major review of this subject. The goal of our paper primarily was to educate the ophthalmic community on the results of the Intergroup Rhabdomyosarcoma Study Group (IRSG), a major prospective collaborative investigation of chemotherapy and radiotherapy for rhabdomyosarcoma. In the opening paragraph of our report, we discuss this study and point out that IRSG reports have been published in the pediatric, oncologic, and radiologic literature, but few have been published in the ophthalmic literature. Thus, the focus of our report was to inform the ophthalmic readers of the results from nearly three decades of work emanating from the IRSG. A secondary focus of the report was to discuss our own results and, importantly, to evaluate ophthalmic complications from therapy, a subject that had not been completely evaluated previously. As we reviewed the literature using Medline research at the National Library of Medicine, we noted that there had been 7269 published reports on rhabdomyosarcoma and 230 published reports on orbital rhabdomyosarcoma. Thus, we could not include all published literature in our report, and we referenced the 42 most relevant reports, including those on the IRSG (19 references), general pediatric oncology (five references), general ophthalmic oncology (four references), survey of orbital tumors in children (one reference), orbital imaging (two references), major survey of orbital rhabdomyosarcoma (one reference), large series of orbital rhabdomyosarcoma (one reference predating IRSG and one reference regarding refractory cases), and relevant case reports (eight references). Two of the articles that we quoted (references 16 and 17) did reflect the early experience by physicians at Columbia Presbyterian Hospital. The additional articles by Drs. Abramson and Sagerman were important contributions and likely would have added depth to our list of references. We do acknowledge their contributions to the treatment of rhabdomyosarcoma and we regret that we could not quote all of the early work from Columbia Presbyterian Hospital. However, the more recent organized studies of the IRSG, which have established the groundwork for current management, are appropriately cited. Overall, we believe we all should be pleased with our unified goal of improving survival for those children with rhabdomyosarcoma. Our joint enthusiasm from many cen-
877