CURATIVE IRRADIATION OF THE ENTIRE ORBIT IN RHABDOMYOSARCOMA: A CASE REPORT Walter Van den Bogaert, MD, PhD, Erik Van Limbergen, MD, Godelieve Dralands, MD, and Alfons Drochmans, MD
Rhabdomyosarcoma (RMS) is a relatively frequent tumor in children. Judicious combinations have markedly improved treatment results in recent years. Orbital localizations are treated with chemotherapy and radiotherapy. It has been advocated that radiotherapy be limited to the original tumor bed. This case report illustrates the danger of shielding part of the orbit: in a clinically tumor-free region before chemotherapy, which was shielded during radiotherapy, a local recurrence was seen while the original tumor bed remained controlled. Therefore, the whole content of the orbit should be irradiated in orbital RMS. 0 1992 John Wiley & Sons, Inc. HEAD & NECK 1992;14:392-394
Rhabdomyosarcoma (RMS), the most frequent soft tissue sarcoma in children, is a fine illustration of the advances in modern oncology and of the effectiveness of multimodality treatment in improving prognosis. RMS of the orbit is an example of a disease that has become highly curable with modern treatment modalities. Until the late 1960s, the only treatment known to be curative for RMS of the orbit was total exenteration of the orbit, and reported local
From the Departments of Oncology (Drs. Van den Bogaert, Van Limbergen, and Drochmans) and Ophthalmology (Dr. Draiands), University Hospital St. Rafael, Leuven, Belgium. Address reprint requests to Dr Van den Bogaert at the Department of Oncology, University Hospital. Kapucilnenvoer 33, 6-3000 Leuven, Belgium. Accepted for publication February 1 I , 1992
CCC 0148-64031921050392-03 $04.00 0 1992 John Wiley & Sons, Inc.
392
Orbital Rhabdomyosarcoma
control and survival were dismal.' In the late 1960s, however, reports' showed that it was possible to control RMS with radiotherapy, using fairly large fields and high doses (50-60 Gy). Although local control was achieved in 90% of orbital RMS patients, survival still was limited because of distant ~ p r e a dThe . ~ addition of primary chemotherapy to the treatment further improved the outcome in this disease, especially in patients with orbital localization^.^^^ With chemotherapy and subsequent radiotherapy, cure is achieved in more than 90% of Exenteration, or other forms of extensive surgery, are no longer the primary treatment of choice for these patients. The consequences associated with the high success rate of this treatment include a number of late effects of irradiation in long-term survivors, which are reviewed by a number of aut h o r ~ . ~ - In ' patients with orbital RMS, apart from infectious complications, several changes are found: cataract formation in a large majority of cases, corneal and conjunctival changes, and dryness, enophthalmos, and facial asymmetry. The threshold for retinal changes, which causes irreversible visual impairment, seems to be 50 G Y .The ~ concern for these late effects and complications has increased as a consequence of the excellent prognosis. It has now become important to seek the optimal dose level and treatment volume. Recently, reports have shown that doses higher than 40 Gy are probably not needed.779Reports of early ra-
HEAD & NECK
September/October 1992
diotherapy without chemotherapy for orbital RMS recommended generous fields encompassing the whole orbit. Recently, however, warnings concerning the treatment volume have become less stern. In two recent textbooks on radiotherapy, the generous covering of the primary tumor with a In one text," safety margin is it was specifically recommended to treat the initial tumor involvement with a margin, without inclusion of the entire orbit, allowing for beamshaping devices, with the obvious aim of limiting late effects as much as possible. A recent experience in our center, however, has led us to warn against such limitation of the treatment volume. In a patient with a primary tumor located at the lower inner quadrant, a recurrence was seen in the upper outer quadrant, exactly behind a block shielding the tear gland. CASEREPORT
In a teenage boy, born August 22, 1976, an RMS (poorly differentiated embryonal) was found in the right orbit, originating from the inferior rectus muscle, and forming a mass in the lower inner quadrant of the orbit (Figure 1).No regional or distant metastasis was found, and the patient was treated from June 1989 until January 1990 with six cycles of IVA (ifosfamide, vincristine, actinomycin D). After three cycles of chemotherapy in September 1989, a clinical and radiologic
FIGURE 1. NMR image of the tumor (June 1989), presenting as a mass originating from the musculus rectus inferior.
Orbital Rhabdornyosarcoma
remission was seen: a discrete infiltration was seen at the bottom of the orbit on CT scan only. Irradiation was done, from September 27, 1989 to November 6, 1989: a total dose of 54 Gy was given using an anterior field with a 6 MV Linac, with blocking of the tear gland in the upper outer quadrant (Figure 2). After irradiation, chemotherapy was continued until the end of January 1990. A complete remission was obtained, documented with CT scan and nuclear magnetic imaging (NMR) in April 1990. At the end of May 1990 a recurrence developed in the upper outer quadrant, exactly underlying the shielding block (Figure 3). At the original tumor site, no recurrence was seen, the investigations with CT and NMR showed only the mass in the upper outer quadrant. A new chemotherapy regimen (AEP: Adriamycine, etoposide, and cisplatin) was given. There was tumor regression and, in December 1990, an exenteration was done. However, section margins proved to be positive and a few weeks later a local recurrence became clear, extending rapidly intracranially, and toward the sinuses. CONCLUSION
In spite of excellent complete response rates, chemotherapy alone may not always be able t o eradicate even subclinical disease in the orbit at a
FIGURE 2. Simulation film of treatment field with a block in the outer upper quadrant. A dose (at d,,) of 54 Gy was given. Portal films (not shown) were consistent. The black circle Corresponds to the original tumor mass.
HEAD & NECK
SeptemberiOctober 1992
393
outweigh even the slightest rise in the probability of recurrence, and irradiation of the whole orbit to a minimum dose of 40 Gy remains warranted.
REFERENCES
FIGURE 3. NMR image of recurrence in the upper outer quadrant (May 1990).
distance from the tumor, as demonstrated by the presented case and in several reports.436It is, therefore, suggested that irradiation of the whole orbit may be needed to control subclinical disease. Recent recommendations" have stated that limited field irradiation in orbital RMS would be as effective as whole orbit irradiation. Although this report concerns only one case, the authors feel that this is sufficient to warn against undertreatment. The consequence of shielding the tear gland (a high incidence of conjunctivitis) cannot
394
Orbital Rhabdornyosarcorna
1. Jones IS, Reese AB, Krant J. Orbital rhabdomyosarcoma: an analysis of 62 cases. A m J Ophthalrnol 1966;61:721- 736. 2. Cassady JR, Sagerman RH, Tretter P, Ellsworth RM. Radiation therapy for rhabdomyosarcoma. Radiology 1963; 91:116-120. 3. Sagerman RH, Tretter P, Ellsworth RM. The treatment of orbital rhabdomyosarcoma of children with primary radiation therapy. AJR 1972;114:31-34. 4. Tefft M, Lindberg RD, Gehan EA. Radiation therapy combined with systemic chemotherapy of rhabdomyosarcoma in children: local control in patients enrolled in the Intergroup Rhabdomyosarcoma Study. Natl Cancer Znst Monogr 1981;56:75-81. 5. Wharam M, Beltangady M, Hays D, et al. Localized orbital rhabdomyosarcoma. An interim report of the Intergroup Rhabdomyosarcoma Study Committee. Ophthalmology 1987;93:251-254. 6. Haik BG, Jereb B, Smith ME, Ellsworth RM, McCormick B. Radiation and chemotherapy of parameningeal rhabdomyosarcoma involving the orbit. Ophthalmology 1986;93:1001- 1009. 7. Mandell L, Ghairmi F, Peretz T, La Quaglia M, Exelby P. Radiocurability of microscopic disease in childhood rhabdomyosarcoma with radiation doses less than 4,000 cGy. J Clin Oncol 1990;8:1536- 1542. 8. Heyn R, Ragab A, Rancy B Jr, et al. Late effects of therapy in orbital rhabdomyosarcoma in children. Cancer 1986;9:1738- 1743. 9. Jereb B, Haik BG, Ong R, Ghavini F. Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation. Int J Radiat Oncol Biol Phys 1985; 11:2057-2065. 10. Kun LE. Childhood cancers. In: Moss WT, Cox JD,eds. Radiation Oncology. St. Louis: Mosby. 1989:744. 11. Donaldson SS. Rhabdomyosarcoma. In: Perez CA, Brady LW, eds. Principles and practice of radiation oncology. Philadelphia: J.B.Lippincott. 1987:1232.
HEAD & NECK
Septernber/October 1992
Rhabdomyosarcoma (RMS) is a relatively frequent tumor in children. Judicious combinations have markedly improved treatment results in recent years. Orbital localizations are treated with chemotherapy and radiotherapy. It has been advocated that radiotherapy be limited to the original tumor bed. This case report illustrates the danger of shielding part of the orbit: in a clinically tumor-free region before chemotherapy, which was shielded during radiotherapy, a local recurrence was seen while the original tumor bed remained controlled. Therefore, the whole content of the orbit should be irradiated in orbital RMS. 0 1992 John Wiley & Sons, Inc. HEAD & NECK 1992;14:392-394
Rhabdomyosarcoma (RMS), the most frequent soft tissue sarcoma in children, is a fine illustration of the advances in modern oncology and of the effectiveness of multimodality treatment in improving prognosis. RMS of the orbit is an example of a disease that has become highly curable with modern treatment modalities. Until the late 1960s, the only treatment known to be curative for RMS of the orbit was total exenteration of the orbit, and reported local
From the Departments of Oncology (Drs. Van den Bogaert, Van Limbergen, and Drochmans) and Ophthalmology (Dr. Draiands), University Hospital St. Rafael, Leuven, Belgium. Address reprint requests to Dr Van den Bogaert at the Department of Oncology, University Hospital. Kapucilnenvoer 33, 6-3000 Leuven, Belgium. Accepted for publication February 1 I , 1992
CCC 0148-64031921050392-03 $04.00 0 1992 John Wiley & Sons, Inc.
392
Orbital Rhabdomyosarcoma
control and survival were dismal.' In the late 1960s, however, reports' showed that it was possible to control RMS with radiotherapy, using fairly large fields and high doses (50-60 Gy). Although local control was achieved in 90% of orbital RMS patients, survival still was limited because of distant ~ p r e a dThe . ~ addition of primary chemotherapy to the treatment further improved the outcome in this disease, especially in patients with orbital localization^.^^^ With chemotherapy and subsequent radiotherapy, cure is achieved in more than 90% of Exenteration, or other forms of extensive surgery, are no longer the primary treatment of choice for these patients. The consequences associated with the high success rate of this treatment include a number of late effects of irradiation in long-term survivors, which are reviewed by a number of aut h o r ~ . ~ - In ' patients with orbital RMS, apart from infectious complications, several changes are found: cataract formation in a large majority of cases, corneal and conjunctival changes, and dryness, enophthalmos, and facial asymmetry. The threshold for retinal changes, which causes irreversible visual impairment, seems to be 50 G Y .The ~ concern for these late effects and complications has increased as a consequence of the excellent prognosis. It has now become important to seek the optimal dose level and treatment volume. Recently, reports have shown that doses higher than 40 Gy are probably not needed.779Reports of early ra-
HEAD & NECK
September/October 1992
diotherapy without chemotherapy for orbital RMS recommended generous fields encompassing the whole orbit. Recently, however, warnings concerning the treatment volume have become less stern. In two recent textbooks on radiotherapy, the generous covering of the primary tumor with a In one text," safety margin is it was specifically recommended to treat the initial tumor involvement with a margin, without inclusion of the entire orbit, allowing for beamshaping devices, with the obvious aim of limiting late effects as much as possible. A recent experience in our center, however, has led us to warn against such limitation of the treatment volume. In a patient with a primary tumor located at the lower inner quadrant, a recurrence was seen in the upper outer quadrant, exactly behind a block shielding the tear gland. CASEREPORT
In a teenage boy, born August 22, 1976, an RMS (poorly differentiated embryonal) was found in the right orbit, originating from the inferior rectus muscle, and forming a mass in the lower inner quadrant of the orbit (Figure 1).No regional or distant metastasis was found, and the patient was treated from June 1989 until January 1990 with six cycles of IVA (ifosfamide, vincristine, actinomycin D). After three cycles of chemotherapy in September 1989, a clinical and radiologic
FIGURE 1. NMR image of the tumor (June 1989), presenting as a mass originating from the musculus rectus inferior.
Orbital Rhabdornyosarcoma
remission was seen: a discrete infiltration was seen at the bottom of the orbit on CT scan only. Irradiation was done, from September 27, 1989 to November 6, 1989: a total dose of 54 Gy was given using an anterior field with a 6 MV Linac, with blocking of the tear gland in the upper outer quadrant (Figure 2). After irradiation, chemotherapy was continued until the end of January 1990. A complete remission was obtained, documented with CT scan and nuclear magnetic imaging (NMR) in April 1990. At the end of May 1990 a recurrence developed in the upper outer quadrant, exactly underlying the shielding block (Figure 3). At the original tumor site, no recurrence was seen, the investigations with CT and NMR showed only the mass in the upper outer quadrant. A new chemotherapy regimen (AEP: Adriamycine, etoposide, and cisplatin) was given. There was tumor regression and, in December 1990, an exenteration was done. However, section margins proved to be positive and a few weeks later a local recurrence became clear, extending rapidly intracranially, and toward the sinuses. CONCLUSION
In spite of excellent complete response rates, chemotherapy alone may not always be able t o eradicate even subclinical disease in the orbit at a
FIGURE 2. Simulation film of treatment field with a block in the outer upper quadrant. A dose (at d,,) of 54 Gy was given. Portal films (not shown) were consistent. The black circle Corresponds to the original tumor mass.
HEAD & NECK
SeptemberiOctober 1992
393
outweigh even the slightest rise in the probability of recurrence, and irradiation of the whole orbit to a minimum dose of 40 Gy remains warranted.
REFERENCES
FIGURE 3. NMR image of recurrence in the upper outer quadrant (May 1990).
distance from the tumor, as demonstrated by the presented case and in several reports.436It is, therefore, suggested that irradiation of the whole orbit may be needed to control subclinical disease. Recent recommendations" have stated that limited field irradiation in orbital RMS would be as effective as whole orbit irradiation. Although this report concerns only one case, the authors feel that this is sufficient to warn against undertreatment. The consequence of shielding the tear gland (a high incidence of conjunctivitis) cannot
394
Orbital Rhabdornyosarcorna
1. Jones IS, Reese AB, Krant J. Orbital rhabdomyosarcoma: an analysis of 62 cases. A m J Ophthalrnol 1966;61:721- 736. 2. Cassady JR, Sagerman RH, Tretter P, Ellsworth RM. Radiation therapy for rhabdomyosarcoma. Radiology 1963; 91:116-120. 3. Sagerman RH, Tretter P, Ellsworth RM. The treatment of orbital rhabdomyosarcoma of children with primary radiation therapy. AJR 1972;114:31-34. 4. Tefft M, Lindberg RD, Gehan EA. Radiation therapy combined with systemic chemotherapy of rhabdomyosarcoma in children: local control in patients enrolled in the Intergroup Rhabdomyosarcoma Study. Natl Cancer Znst Monogr 1981;56:75-81. 5. Wharam M, Beltangady M, Hays D, et al. Localized orbital rhabdomyosarcoma. An interim report of the Intergroup Rhabdomyosarcoma Study Committee. Ophthalmology 1987;93:251-254. 6. Haik BG, Jereb B, Smith ME, Ellsworth RM, McCormick B. Radiation and chemotherapy of parameningeal rhabdomyosarcoma involving the orbit. Ophthalmology 1986;93:1001- 1009. 7. Mandell L, Ghairmi F, Peretz T, La Quaglia M, Exelby P. Radiocurability of microscopic disease in childhood rhabdomyosarcoma with radiation doses less than 4,000 cGy. J Clin Oncol 1990;8:1536- 1542. 8. Heyn R, Ragab A, Rancy B Jr, et al. Late effects of therapy in orbital rhabdomyosarcoma in children. Cancer 1986;9:1738- 1743. 9. Jereb B, Haik BG, Ong R, Ghavini F. Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation. Int J Radiat Oncol Biol Phys 1985; 11:2057-2065. 10. Kun LE. Childhood cancers. In: Moss WT, Cox JD,eds. Radiation Oncology. St. Louis: Mosby. 1989:744. 11. Donaldson SS. Rhabdomyosarcoma. In: Perez CA, Brady LW, eds. Principles and practice of radiation oncology. Philadelphia: J.B.Lippincott. 1987:1232.
HEAD & NECK
Septernber/October 1992
