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Rhabdomyosarcoma in Infancy and Childhood*
RHABDOMYOSARCOMA IN INFANCY AND CHILDHOOD* EDMUND B. SPAETH, M.D.,
AN D ALBERT F. CLEVELAND,
M.D.
Philadelphia, Pennsylvania
The literature relative to this neoplasm has never been extensive, nor has it been suffi cient to permit the inclusion of certain tumors into this category which, it now appears, should properly be included. In 1942, Cal houn and Reese 1 listed 32 references, the first of these was in 1882. In 1958, Horn and Enterline 2 listed two additional contributions prior to 1942 and 19 further references. Since then there have been three additional references in the literature.3"5 In 1946, Stout,6 in Histopathological Features of Tumors of Skeletal Muscle Origin, discussed rather definitely the microscopic pathology and the variations thereof. Since then other pathologists at various times have studied and reported on these tumors. The histopathology of them is now quite well worked out, as Horn and Enterline said, making it possible to classify certain tumors into this category, with assurance, de spite the inability to demonstrate the charac teristic cross striations of the neoplasm cells. These authors are referring to that appear ance of the tumor cells which, when present, identify the neoplasm under investigation as a rhabdomyosarcoma. This situation was present in one of our cases and confused the diagnosis early in the observation of that pa tient. This will be mentioned later herein. The work of the various pathologists over the years, based upon the histopathologic findings, has subdivided these tumors mor phologically into three types : ( 1 ) a pleomorphic rhabdomyosarcoma, (2) an alveolar type of rhabdomyosarcoma, and (3) an embry onal type of rhabdomyosarcoma. This last subdivision has, as a variant, a tumor of grapelike or polypoid pattern which was called a botryoid variety of rhabdomyosar coma. * From the Wills Eye Hospital. Presented at the annual clinical conference, Wills Eye Hospital, February, 1961.
This presentation is not discussing the his topathology of the neoplasm. This has been presented in the past in detail which needs no repetition. Such histologie presentations were made by Calhoun and Reese 1 in 1942, by Stout6"7 in 1946 and again in 1953 in the Atlas of Pathology of the Armed Forces In stitute of Pathology; by Forrest 8 and by Horn and Enterline 2 in 1958, and by Frayer and Enterline 4 in 1959. The mortality rate of this form of neo plasm is very high. Of the 39 cases in Horn and Enterline's series, one case was lost tract of, and 31 patients were known to have died. Details as to a follow-up were lacking in five cases. One patient was known to have been alive for almost three years and a second case after 12 years. Twelve of their patients with known métastases also had local recurrences. The mortality rate in the 12 cases presented by Frayer and Enterline was 75 percent, that is, in three of the 12 cases listed survival was more than three years. Horn and Enterline found that the most common site for metastatic disease was in the lung. It is rather interesting that one of the two deaths in our four cases was from pulmonary métastases. The other patient died without known métastases but from rather likely intracranial extension. In the cases presented by Calhoun and Reese1 and in the other 14 cases collected by them from the literature, all but three pa tients died between one and one and a half years after the appearance of the tumor. Of these three survivals, two were their personal patients. At the time of the presentation of their article, one of them was living two years after the exenteration of the orbit, and the other, three years after the exenteration of the orbit. Of Horn and Enterline's patients, three were less than one year of age, two were less than two years, four were less than three
464
EDMUND B. SPAETH AND ALBERT F. CLEVELAND
years, two were four or five years of age; there were three patients who, while hardly in infancy, were still in childhood and were from eight to 12 years of age. Of the seven tumors which they reported, involving the head and neck, five were orbi tal. These were all of the embryonal type. The pleomorphic type of rhabdomyosarcoma was the type found in one three-year-old pa tient and in one 12-year-old patient. This his tologie type is usually that of adult life. The embryonal form is the type most common in infancy and childhood. There is a difference in these four cases of sex incidence as compared to the sex inci dences in all previously reported cases, for in these children, two were males and two were females. Of the series of 14 cases from earlier liter ature reported by Calhoun and Reese, eight patients were male and four were female. The sex in two cases was not given. The per sonal series of Calhoun and Reese showed four males and one female. Forrest did not give a sex incidence in his four cases of rhab domyosarcoma, though all of his patients with this tumor were either infants or chil dren. The series of Horn and Enterline had 11 patients below the age of 12 years listed as males, and only five below the age of 12 years who were females. In a recent article in the Journal of the American Medical Association, a Buffalo group reported 16 cases of rhabdomyosar coma, none of orbital origin. In this study the age incidence was of particular interest: the average age of the 16 reported was between six and seven years. There were none in the group, however, who were actually between six and 13 years of age. In the four cases here reported (all of orbital origin), our age findings agreed very closely with those of the Buffalo group—three (all females) were aged three years or under, whereas the single male was a 14-year-old boy. The statistics of this Buffalo group showed that, although rhabdomyosarcoma is gener
ally thought to be rare as a childhood can cer, only the occurrences of leukemia and neuroblastoma are more frequent. Four additional orbital cases are being presented at this time. One of these cases was a classical pleomorphic type of rhab domyosarcoma, and three were of the embry onal type. Of the three embryonal type, one was first considered to be a schwannoma be cause, at the time of its first appearance, the section showed no striations. When Dr. Perce DeLong studied the slides of the first recur rence in the case, striations were found. These, with other cellular characteristics, convinced him that the diagnosis was an em bryonal rhabdomyosarcoma. This case also had definite characteristics of a botryoid le sion. Of the four cases of this neoplasm in in fancy or childhood, one child, in whom the lesion appeared before the age of one month, is alive and well without recurrence two years following the exenteration of the orbit. Two of the patients died six and eight months, respectively, after the surgery. The fourth patient is still alive, though it is only five months following her surgery (see later note herein). CASE REPORTS CASE 1
R. D., a boy, aged 14 years, was first seen by one of us (A. F. C.) with a swelling of the left upper lid at the inner angle of one month's duration. The lesion, the size of a small acorn, was completely excised. The microscopic report was that of a ques tionable lymphoma. The patient was discharged from the hospital several days later (November 18, 19S3). The child was readmitted on March 29, 1954, with a recurrence. This recurrence was ac companied by pain, now present for a week, and with exophthalmos. The exophthalmos was about one month's duration. Three nodules could be pal pated in the orbit through the upper lid. Exentera tion of the orbit was advised about one month prior to this last admission. The parents, however, re fused to permit this surgery. The patient had had X-ray therapy following the first surgery and prior to this admission, as an out patient, because of the original tentative diagnosis. At the time of this second admission, complete medical studies failed to show any signs of blood
RHABDOMYOSARCOMA IN CHILDREN dyscrasia or any other signs of lymphomatous dis ease. On March 31, 19S4, a complete orbital exenteration was done. The laboratory report at this time was one of rhabdomyosarcoma. On April 30, 1954, the patient was readmitted to the Wills Eye Hos pital with a large egg-shaped lesion in the depths of the orbit. Radium needles were implanted into this mass—eight needles, for 2,400 mc. hours. On May 14, 1954, the socket was in a remarkable good con dition, showing a tremendous shrinkage of this recurrence. Two months later, the patient died from a very evident intracranial recurrence or extension. CASE 2
Baby M. J. D. This case was referred by Dr. A. Davney of Trenton. The patient, at the time of the referral, was six months of age. The baby was born with an evident tumor mass in the left orbit. X-ray examination showed an over-all increase in the size of the orbit with no evidence of bone de struction. At five days of age Dr. Davney did a complete orbital exenteration, sparing the outer skin layer of the lids. The exposed orbital bone was skin grafted with a split-skin, razor-cut graft. The microscopic diagnosis was an embryonal type of rhabdomyosarcoma, this diagnosis being confirmed by Dr. A. B. Reese of New York. The child was first seen by one of us January 23, 1959. There was a tremendous amount of mucopurulent drainage from the socket, despite the fact that the skin graft had taken well. Polypoid for mation was also present. In the past year the child has been seen at several intervals. The socket was cleansed, manually, and the granulations clipped, and the site of these granulations cauterized with silver nitrate. On De cember 15, 1960, a keloidlike area on the roof of the orbit was incised and biopsied. This section showed very active granulation tissue with numerous newly formed capillaries with a varied type of inflamma tory infiltration. There was no trace of any tumor. At the present time the child is two and onehalf years of age and there have been no signs of any recurrence. There has been considerable con traction of the cicatrix within the socket. The prog nosis, while still guarded, is promising. CASE 3
Deborah McQ., aged three years, was referred on September 12, 1959. The first sign of a patho logic process was a lesion in the upper inner corner of the upper lid, mildly inflammatory, definitely cystic and having the appearance of a foreign body cyst. On September 25, 1959, a local extirpation of this was done with a subsequent histopathologic re port of schwannoma. Recurrence appeared within six weeks. The orbit and inner medial wall, involv ing the maxillary, ethmoid and sphenoid bones and sinuses, were exenterated. An additional exentera tion was done to include the radical extirpation of all of these areas. At this time the pathologic specimens of the original lesion and of these two recurrences were carefully reviewed and studied
465
by various pathologists, including Dr. Perce DeLong. The unanimous opinion was that the case was a rhabdomyosarcoma. The patient was then transferred from the Wills Eye Hospital to the Graduate Hospital, January 14, 1960, for massive X-ray therapy. The X-ray studies done there on January 29th showed bone invasion of the frontal bone. On February 26th a recurrence appeared in the hard palate on the left side. This was removed by the faciomaxillary service at the Graduate Hospital. During this period of hospitalization the patient was continued on her X-ray therapy so that by March 31, 1960, she had had a total dosage of 3,500 r through copper filter, including all re gions of the orbital area. During this course of treatment the patient was given Cytoxin C, concomitantly with the radiation therapy. There was rapid recession of the lesion and, for a time, the prognosis seemed to be somewhat better. She was discharged April 2nd. The patient was readmitted June 29th with signs of meningial irritation and with métastases to her left femur and knee, to her superior orbital region, and to the lungs. She died on July 26th. CASE 4*
S. L., a boy, age seven years, was admitted to the U. S. Naval Hospital, Philadelphia, on Septem ber 24, 1960, by transfer from a neighboring Army Station Hospital. The condition on admission was that of a high degree of exophthalmos which had been developing for one month. The history of the case was rather confusing. One month prior to admission he had been acci dentally struck in the right eye while playing. A chemosis of the lids occurred, which persisted, with edema in the upper lid and with blurred vision. The child was treated for an orbital hemorrhage by a local physician. After two weeks of unchanged condition a needle biopsy was done of the orbital tissues, with a pathologic report of malignancy, type unknown. On admission, the right eye was in a high degree of exophthalmos. The globe was deviated inferiorly, with complete paralysis of upward movements and inability to close the lids completely. A fluctuating mass could be palpated in the upper half of the orbit. The fundus showed no intraocular pathology. The mass was easily reached through the lid and a biopsy of the mass was done. The microscopic re port was that of rhabdomyosarcoma. Exenteration of the orbit was done September 30, 1960. The tumor did not involve the bones of the orbit, neither the floor nor the medial wall. The pathologic report of the retrobulbar mass from the exenterated orbit was that of embryonal type of rhabdomyosarcoma. The patient, at this writing, is being followed at the Philadelphia Naval Hospital. The prognosis is * This patient died this past summer with intra cranial métastases and probably pulmonary méta stases as well.
466
EDMUND B. SPAETH AND ALBERT F. CLEVELAND
absolutely bad, however. Within the past month, a microscopically confirmed recurrence within the orbit has appeared as an umbrella-shaped mass. This was surgically removed and massive X-ray treatments are being used with cytotoxic agents as well. CONCLUSION
There are three factors of significance which should be emphasized at this time rela tive to this lesion. These points appear con sistently in all the literature. The first of these is the difficulty in making a correct histopathologic diagnosis. Factors related to this difficulty were emphasized by Horn and Enterline : With the embryonal, botryoid, and alveolar tu mors especially, the features that give the pa thologist assurance of the diagnosis of rhabdomyosarcoma, longitudinal and cross striations, are often found only after prolonged and painstaking search. In addition, all too frequently pathologists accept histologie preparations of something less than the best technical quality, making it futile to even at tempt to demonstrate such fine details as cross or longitudinal striations.
The second is the grave prognosis in these cases. The mortality rate, as found in the different series, varies from at least 50 per cent to close to 90 percent. The vagaries with regard to the rapidity of growth and the mortality of this pathologic condition are well illustrated by two of our cases: the one case in which procrastination followed the recommendation for radical surgery (exenteration) and the case in which immediate exenteration was permitted. It is true that in the first case, the exenteration was finally done, following a recurrence. This
may or may not be significant. The outstand ing factor is that the survival of these two patients was exactly the same—eight months following the primary diagnosis. The third factor is related to the treatment. Of all the cases quoted herein, three of the cases seemed to have made a recovery fol lowing exenteration of the orbit. Two of our cases, however, and, many of the other cases from the literature, in which the patient died, had had extensive surgery as well as exten sive irradiation therapy. Apparently, surgery and irradiation therapy may result in tempo rary improvement and temporary recession of the lesion but does not prevent métastases, nor the local spread of the pathologic proc ess. The prognosis, hence, continues to be grave in this type of neoplastic disease of in fancy and early childhood. It is, nevertheless, significant that one of the cases reported by Frayer and Enterline had, after local surgery, such extensive ir radiation therapy that necrosis occurred in the bones of the orbit. The patient died 10 years later from meningitis secondary to this osteomyelitis—but irradiation therapy in this case might well have been a factor in the de struction of the neoplasm. It must be mentioned, and emphasized, that the prognosis is not wholly hopeless, hence bold surgery and skillful irradiation therapy are to be recommended. Recoveries have occurred with these procedures. Cyto toxic agents may prove valuable in the future, though there are none now of known value. 1930 Chestnut Street (3).
REFERENCES
1. Calhoun, F. P., Jr., and Reese, A. B.: Rhabdomyosarcoma of orbit. Arch. Ophth., 27:558-578, 1942. 2. Horn, R. C, Jr., and Enterline, H. T.: Rhabdomyosarcoma: A clinico-pathological study and classifi cation of 39 cases. Cancer, 11:1 (Jan.-Feb.) 1958. 3. Blaxter, P. L., and Smith, J. L.: Rhabdomyosarcoma of the orbit. Tr. Ophth. Soc. U. Kingdom, 78:83-97, 1958. 4. Frayer, W. C, and Enterline, H. T.: Tr. Ophth. Sec. A.M.A., 1959, pp. 43-53. 5. Pinkel, D., and Pickren, J.: Rhabdomyosarcoma in children. J.A.M.A., 175:293-298, 1961. 6. Stout, A. P.: Rhabdomyosarcoma of skeletal muscles, Ann. Surg., 123:447-472, 1946. 7. : Tumors of the soft tissues; Atlas of Tumor Pathology, Sect. II, Fasc. 5, Washington, D.C. Armed Forces Institute of Pathology, 1953. 8. Forrest, A. W.: Intraorbital tumors. Arch. Ophth., 41:198-232, 1949.
AN D ALBERT F. CLEVELAND,
M.D.
Philadelphia, Pennsylvania
The literature relative to this neoplasm has never been extensive, nor has it been suffi cient to permit the inclusion of certain tumors into this category which, it now appears, should properly be included. In 1942, Cal houn and Reese 1 listed 32 references, the first of these was in 1882. In 1958, Horn and Enterline 2 listed two additional contributions prior to 1942 and 19 further references. Since then there have been three additional references in the literature.3"5 In 1946, Stout,6 in Histopathological Features of Tumors of Skeletal Muscle Origin, discussed rather definitely the microscopic pathology and the variations thereof. Since then other pathologists at various times have studied and reported on these tumors. The histopathology of them is now quite well worked out, as Horn and Enterline said, making it possible to classify certain tumors into this category, with assurance, de spite the inability to demonstrate the charac teristic cross striations of the neoplasm cells. These authors are referring to that appear ance of the tumor cells which, when present, identify the neoplasm under investigation as a rhabdomyosarcoma. This situation was present in one of our cases and confused the diagnosis early in the observation of that pa tient. This will be mentioned later herein. The work of the various pathologists over the years, based upon the histopathologic findings, has subdivided these tumors mor phologically into three types : ( 1 ) a pleomorphic rhabdomyosarcoma, (2) an alveolar type of rhabdomyosarcoma, and (3) an embry onal type of rhabdomyosarcoma. This last subdivision has, as a variant, a tumor of grapelike or polypoid pattern which was called a botryoid variety of rhabdomyosar coma. * From the Wills Eye Hospital. Presented at the annual clinical conference, Wills Eye Hospital, February, 1961.
This presentation is not discussing the his topathology of the neoplasm. This has been presented in the past in detail which needs no repetition. Such histologie presentations were made by Calhoun and Reese 1 in 1942, by Stout6"7 in 1946 and again in 1953 in the Atlas of Pathology of the Armed Forces In stitute of Pathology; by Forrest 8 and by Horn and Enterline 2 in 1958, and by Frayer and Enterline 4 in 1959. The mortality rate of this form of neo plasm is very high. Of the 39 cases in Horn and Enterline's series, one case was lost tract of, and 31 patients were known to have died. Details as to a follow-up were lacking in five cases. One patient was known to have been alive for almost three years and a second case after 12 years. Twelve of their patients with known métastases also had local recurrences. The mortality rate in the 12 cases presented by Frayer and Enterline was 75 percent, that is, in three of the 12 cases listed survival was more than three years. Horn and Enterline found that the most common site for metastatic disease was in the lung. It is rather interesting that one of the two deaths in our four cases was from pulmonary métastases. The other patient died without known métastases but from rather likely intracranial extension. In the cases presented by Calhoun and Reese1 and in the other 14 cases collected by them from the literature, all but three pa tients died between one and one and a half years after the appearance of the tumor. Of these three survivals, two were their personal patients. At the time of the presentation of their article, one of them was living two years after the exenteration of the orbit, and the other, three years after the exenteration of the orbit. Of Horn and Enterline's patients, three were less than one year of age, two were less than two years, four were less than three
464
EDMUND B. SPAETH AND ALBERT F. CLEVELAND
years, two were four or five years of age; there were three patients who, while hardly in infancy, were still in childhood and were from eight to 12 years of age. Of the seven tumors which they reported, involving the head and neck, five were orbi tal. These were all of the embryonal type. The pleomorphic type of rhabdomyosarcoma was the type found in one three-year-old pa tient and in one 12-year-old patient. This his tologie type is usually that of adult life. The embryonal form is the type most common in infancy and childhood. There is a difference in these four cases of sex incidence as compared to the sex inci dences in all previously reported cases, for in these children, two were males and two were females. Of the series of 14 cases from earlier liter ature reported by Calhoun and Reese, eight patients were male and four were female. The sex in two cases was not given. The per sonal series of Calhoun and Reese showed four males and one female. Forrest did not give a sex incidence in his four cases of rhab domyosarcoma, though all of his patients with this tumor were either infants or chil dren. The series of Horn and Enterline had 11 patients below the age of 12 years listed as males, and only five below the age of 12 years who were females. In a recent article in the Journal of the American Medical Association, a Buffalo group reported 16 cases of rhabdomyosar coma, none of orbital origin. In this study the age incidence was of particular interest: the average age of the 16 reported was between six and seven years. There were none in the group, however, who were actually between six and 13 years of age. In the four cases here reported (all of orbital origin), our age findings agreed very closely with those of the Buffalo group—three (all females) were aged three years or under, whereas the single male was a 14-year-old boy. The statistics of this Buffalo group showed that, although rhabdomyosarcoma is gener
ally thought to be rare as a childhood can cer, only the occurrences of leukemia and neuroblastoma are more frequent. Four additional orbital cases are being presented at this time. One of these cases was a classical pleomorphic type of rhab domyosarcoma, and three were of the embry onal type. Of the three embryonal type, one was first considered to be a schwannoma be cause, at the time of its first appearance, the section showed no striations. When Dr. Perce DeLong studied the slides of the first recur rence in the case, striations were found. These, with other cellular characteristics, convinced him that the diagnosis was an em bryonal rhabdomyosarcoma. This case also had definite characteristics of a botryoid le sion. Of the four cases of this neoplasm in in fancy or childhood, one child, in whom the lesion appeared before the age of one month, is alive and well without recurrence two years following the exenteration of the orbit. Two of the patients died six and eight months, respectively, after the surgery. The fourth patient is still alive, though it is only five months following her surgery (see later note herein). CASE REPORTS CASE 1
R. D., a boy, aged 14 years, was first seen by one of us (A. F. C.) with a swelling of the left upper lid at the inner angle of one month's duration. The lesion, the size of a small acorn, was completely excised. The microscopic report was that of a ques tionable lymphoma. The patient was discharged from the hospital several days later (November 18, 19S3). The child was readmitted on March 29, 1954, with a recurrence. This recurrence was ac companied by pain, now present for a week, and with exophthalmos. The exophthalmos was about one month's duration. Three nodules could be pal pated in the orbit through the upper lid. Exentera tion of the orbit was advised about one month prior to this last admission. The parents, however, re fused to permit this surgery. The patient had had X-ray therapy following the first surgery and prior to this admission, as an out patient, because of the original tentative diagnosis. At the time of this second admission, complete medical studies failed to show any signs of blood
RHABDOMYOSARCOMA IN CHILDREN dyscrasia or any other signs of lymphomatous dis ease. On March 31, 19S4, a complete orbital exenteration was done. The laboratory report at this time was one of rhabdomyosarcoma. On April 30, 1954, the patient was readmitted to the Wills Eye Hos pital with a large egg-shaped lesion in the depths of the orbit. Radium needles were implanted into this mass—eight needles, for 2,400 mc. hours. On May 14, 1954, the socket was in a remarkable good con dition, showing a tremendous shrinkage of this recurrence. Two months later, the patient died from a very evident intracranial recurrence or extension. CASE 2
Baby M. J. D. This case was referred by Dr. A. Davney of Trenton. The patient, at the time of the referral, was six months of age. The baby was born with an evident tumor mass in the left orbit. X-ray examination showed an over-all increase in the size of the orbit with no evidence of bone de struction. At five days of age Dr. Davney did a complete orbital exenteration, sparing the outer skin layer of the lids. The exposed orbital bone was skin grafted with a split-skin, razor-cut graft. The microscopic diagnosis was an embryonal type of rhabdomyosarcoma, this diagnosis being confirmed by Dr. A. B. Reese of New York. The child was first seen by one of us January 23, 1959. There was a tremendous amount of mucopurulent drainage from the socket, despite the fact that the skin graft had taken well. Polypoid for mation was also present. In the past year the child has been seen at several intervals. The socket was cleansed, manually, and the granulations clipped, and the site of these granulations cauterized with silver nitrate. On De cember 15, 1960, a keloidlike area on the roof of the orbit was incised and biopsied. This section showed very active granulation tissue with numerous newly formed capillaries with a varied type of inflamma tory infiltration. There was no trace of any tumor. At the present time the child is two and onehalf years of age and there have been no signs of any recurrence. There has been considerable con traction of the cicatrix within the socket. The prog nosis, while still guarded, is promising. CASE 3
Deborah McQ., aged three years, was referred on September 12, 1959. The first sign of a patho logic process was a lesion in the upper inner corner of the upper lid, mildly inflammatory, definitely cystic and having the appearance of a foreign body cyst. On September 25, 1959, a local extirpation of this was done with a subsequent histopathologic re port of schwannoma. Recurrence appeared within six weeks. The orbit and inner medial wall, involv ing the maxillary, ethmoid and sphenoid bones and sinuses, were exenterated. An additional exentera tion was done to include the radical extirpation of all of these areas. At this time the pathologic specimens of the original lesion and of these two recurrences were carefully reviewed and studied
465
by various pathologists, including Dr. Perce DeLong. The unanimous opinion was that the case was a rhabdomyosarcoma. The patient was then transferred from the Wills Eye Hospital to the Graduate Hospital, January 14, 1960, for massive X-ray therapy. The X-ray studies done there on January 29th showed bone invasion of the frontal bone. On February 26th a recurrence appeared in the hard palate on the left side. This was removed by the faciomaxillary service at the Graduate Hospital. During this period of hospitalization the patient was continued on her X-ray therapy so that by March 31, 1960, she had had a total dosage of 3,500 r through copper filter, including all re gions of the orbital area. During this course of treatment the patient was given Cytoxin C, concomitantly with the radiation therapy. There was rapid recession of the lesion and, for a time, the prognosis seemed to be somewhat better. She was discharged April 2nd. The patient was readmitted June 29th with signs of meningial irritation and with métastases to her left femur and knee, to her superior orbital region, and to the lungs. She died on July 26th. CASE 4*
S. L., a boy, age seven years, was admitted to the U. S. Naval Hospital, Philadelphia, on Septem ber 24, 1960, by transfer from a neighboring Army Station Hospital. The condition on admission was that of a high degree of exophthalmos which had been developing for one month. The history of the case was rather confusing. One month prior to admission he had been acci dentally struck in the right eye while playing. A chemosis of the lids occurred, which persisted, with edema in the upper lid and with blurred vision. The child was treated for an orbital hemorrhage by a local physician. After two weeks of unchanged condition a needle biopsy was done of the orbital tissues, with a pathologic report of malignancy, type unknown. On admission, the right eye was in a high degree of exophthalmos. The globe was deviated inferiorly, with complete paralysis of upward movements and inability to close the lids completely. A fluctuating mass could be palpated in the upper half of the orbit. The fundus showed no intraocular pathology. The mass was easily reached through the lid and a biopsy of the mass was done. The microscopic re port was that of rhabdomyosarcoma. Exenteration of the orbit was done September 30, 1960. The tumor did not involve the bones of the orbit, neither the floor nor the medial wall. The pathologic report of the retrobulbar mass from the exenterated orbit was that of embryonal type of rhabdomyosarcoma. The patient, at this writing, is being followed at the Philadelphia Naval Hospital. The prognosis is * This patient died this past summer with intra cranial métastases and probably pulmonary méta stases as well.
466
EDMUND B. SPAETH AND ALBERT F. CLEVELAND
absolutely bad, however. Within the past month, a microscopically confirmed recurrence within the orbit has appeared as an umbrella-shaped mass. This was surgically removed and massive X-ray treatments are being used with cytotoxic agents as well. CONCLUSION
There are three factors of significance which should be emphasized at this time rela tive to this lesion. These points appear con sistently in all the literature. The first of these is the difficulty in making a correct histopathologic diagnosis. Factors related to this difficulty were emphasized by Horn and Enterline : With the embryonal, botryoid, and alveolar tu mors especially, the features that give the pa thologist assurance of the diagnosis of rhabdomyosarcoma, longitudinal and cross striations, are often found only after prolonged and painstaking search. In addition, all too frequently pathologists accept histologie preparations of something less than the best technical quality, making it futile to even at tempt to demonstrate such fine details as cross or longitudinal striations.
The second is the grave prognosis in these cases. The mortality rate, as found in the different series, varies from at least 50 per cent to close to 90 percent. The vagaries with regard to the rapidity of growth and the mortality of this pathologic condition are well illustrated by two of our cases: the one case in which procrastination followed the recommendation for radical surgery (exenteration) and the case in which immediate exenteration was permitted. It is true that in the first case, the exenteration was finally done, following a recurrence. This
may or may not be significant. The outstand ing factor is that the survival of these two patients was exactly the same—eight months following the primary diagnosis. The third factor is related to the treatment. Of all the cases quoted herein, three of the cases seemed to have made a recovery fol lowing exenteration of the orbit. Two of our cases, however, and, many of the other cases from the literature, in which the patient died, had had extensive surgery as well as exten sive irradiation therapy. Apparently, surgery and irradiation therapy may result in tempo rary improvement and temporary recession of the lesion but does not prevent métastases, nor the local spread of the pathologic proc ess. The prognosis, hence, continues to be grave in this type of neoplastic disease of in fancy and early childhood. It is, nevertheless, significant that one of the cases reported by Frayer and Enterline had, after local surgery, such extensive ir radiation therapy that necrosis occurred in the bones of the orbit. The patient died 10 years later from meningitis secondary to this osteomyelitis—but irradiation therapy in this case might well have been a factor in the de struction of the neoplasm. It must be mentioned, and emphasized, that the prognosis is not wholly hopeless, hence bold surgery and skillful irradiation therapy are to be recommended. Recoveries have occurred with these procedures. Cyto toxic agents may prove valuable in the future, though there are none now of known value. 1930 Chestnut Street (3).
REFERENCES
1. Calhoun, F. P., Jr., and Reese, A. B.: Rhabdomyosarcoma of orbit. Arch. Ophth., 27:558-578, 1942. 2. Horn, R. C, Jr., and Enterline, H. T.: Rhabdomyosarcoma: A clinico-pathological study and classifi cation of 39 cases. Cancer, 11:1 (Jan.-Feb.) 1958. 3. Blaxter, P. L., and Smith, J. L.: Rhabdomyosarcoma of the orbit. Tr. Ophth. Soc. U. Kingdom, 78:83-97, 1958. 4. Frayer, W. C, and Enterline, H. T.: Tr. Ophth. Sec. A.M.A., 1959, pp. 43-53. 5. Pinkel, D., and Pickren, J.: Rhabdomyosarcoma in children. J.A.M.A., 175:293-298, 1961. 6. Stout, A. P.: Rhabdomyosarcoma of skeletal muscles, Ann. Surg., 123:447-472, 1946. 7. : Tumors of the soft tissues; Atlas of Tumor Pathology, Sect. II, Fasc. 5, Washington, D.C. Armed Forces Institute of Pathology, 1953. 8. Forrest, A. W.: Intraorbital tumors. Arch. Ophth., 41:198-232, 1949.