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Melanosarcoma of the Choroid
MELANOSAiíCOMA OF T H E CHOROID. FRANK E . BRAWLEY, M . D . CHICAGO, I L L . The chief features of such cases are summarized from recent Uterature. A case is re ported in which the eyeball was enucleated and radium used in the orbit. There had been no recurrence after two years. Read before the Chicago Ophthalmological Society, February li. 1925. (See p. 828.)
When one investigates the literature of this subject, a surprising number of such cases are found. T h i s may be due to the fact that pigment from the choroid is necessarily contained in most intraocular tumors, even in gliomata in children. The classical evidences of intraocu lar tumor are hypertension, engorge ment of the anterior ciliary veins, reti nal detachment, particularly if circum scribed with very limited or no motion of the retina, and a definite shadow with transillumination. S. Hagen, in the Christiania Clinic, in a survey of the subject, concludes that the glauco matous stage appears usually in one year, or less, after the first symptoms of failing vision, etc., are noted. When metastasis develops, the subject may live from one to eight years. Of twenty-eight cases operated, fifty-eight per cent were cured. Early operation is advisable. Recurrences in the orbit occur only in cases where the tumor has perforated the sclera. T h e dan ger of recurrence is greatest in the first six months after operation, and the patient is safe after four years. In cases where the growths can be seen, they are usually found to be small, circular, fairly sharply defined, and slate gray in color, and have been compared to the color of mercurial ointment. Nettleship, de Schweinitz, Shumway, and R. Foster Moore are agreed that stippling or irregular pig mentation on the surface of the growth indicates activity. The growth is usually made up of spindle cells with irregular deposits of pigment granules. T h e average age of incidence is fifty years. Injuries seem to play a role in only ten per cent of the cases. Ellett^ reported three cases very similar to the author's case. In each instance an acute glaucoma had devel oped in an eye previously blind, and each eye was found to contain a sar 790
coma, altho mention of pigment is made in only one case. I Francis^ reports a case of malignant melanoma of the choroid, seen first as a circumscribed, yellow-white, raised area, in the macular region. Two years later this region showed a small retinal detachment, with Schiotz 17. After several conflicting opinions and a reduction of tension to 5, an enuclea tion was done. The pathologic diagnosis was malignant melanoma of the choroid. T h e most significant fea tures were the gradually decreasing in traocular tension and the absence of the usual evidences of tumor, namely engorged anterior ciliary veins, hyper tension, and positive transillumination. Mr. J . M. H.: Suffered an injury to the left eye 4 years ago. Has been having severe pain in this eye and vision has gradually failed. Three months before I saw him, vision began to fail. Did not consult an oculist, but optician gave him glasses. He was first examined November 7, 1922. There was severe pain, but only slight injec tion of deep scleral vessels. Finger tension was plus 3. There was no light perception. The lens was com pletely cataractous, so that no fundus details could be seen. T h e anterior chamber was practically obliterated. Transillumination of the globe was negative. Iridotasis was performed, and on November 18, 1922, the tension with Schiotz was 25. There was no pain and the eye was quiet and drainage good. As the tension rose repeatedly and could be controlled only with massage, a possible intraocular tumor was considered and enucleation ad vised. This operation was performed December 6, 1922. T h e eyeball was examined by Dr. Edwin F . Hirsch, director of the St. Luke's Hospital Laboratory. A tumor composed chiefly of spindle cells was found near the posterior pole. T h e tumor was one
PRANK Ε . BRAWLEY
cm. in its greatest diameter, and many of the cells were black with pigment. When healing was complete radium was advised. He received radium in the following dosage, administered by Dr. W m . L . Brown of the Physician's Radium Association: December 29, 1922, the left orbit was treated with 100 mgs. radium, screened with 0.8 mm. of gold and 2.1 mm. of rubber, for two hours. January 2, 1923', the same treatment was applied. January 3, 1923, 75 mgs. radium was used at one inch distance, for four hours. Febru ary 28, 1923, 50 mgs. radium similarly screened was left in the orbit for six hours. March 1, 1923, 50 mgs. radium screened with 0.8 mm. of gold, at 12
791
mm. distance from the orbital cavity, for three hours. T h e right eye showed an irregular hyeropic astigmatism and presbyopia, which were corrected, giving normal vision. T h e right fundus showed a few fine vitreous opacities but no active uveitis. Search for a focus of in fection was negative, except that ht was at one time treated for ulcer of the stomach. T h e orbit shows no recur rence, but the radium has destroyed all the cilia. There was perhaps a larger amount of orbital and lid secretion in this case than is usual, probably due to the radium. 30 N. Michigan Ave.
REFERENCES.
1.
Ellett, E. C. American Journal of Ophthalmology, October, 1920, p. 732. Francis, Lee M. American Journal of Ophthalmology, December, 1920, p. 872.
A CRITICAL SURVEY OF N E O P L A S M S O F T H E CHOROID. MARY S. K N I G H T , M . D .
ROCHESTER, MINNESOTA.
Reviewing recent work on the origin of melanin it is concluded that tihe chromatophores of the choroid and skin cannot produce pigment but are only phagocytic cells that ingest the melanin. A case is reported of bupbthalmos for which the eye was removed, and studied microscopically. From the study o f this specimen and accounts of others it is concluded that the choroid in this and similar cases shows the structure described in neurofibromatosis, the lesion being developmental and not malignant. This work was done at the Mayo Founda tion and the report of it submitted as part of the requirements for the Degree of Master of Science in Ophthalmology in the University of Minnesota.
One of the fundamental principles of the science of oncology is that the varieties of tumor to which any type of tissue may give rise will correspond to all the developmental variations which that tissue may assume in the body. Consequently, various types of car cinomas arise from epithelium, and tumors ranging from the fibromas to the varying forms of sarcomas, with many intermediate groups, arise from connective tissue. On this hypothesis a study of the primary tumors of the choroid, usually considered a connec tive tissue structure, would be ex pected to include examples of most, if not all, the varieties of neoplasms which might arise in other connective tissue structures of the body. Neither the literature on this subject nor the examination of a considerable number of actual tumors of the choroid has shown this as clearly as would be ex
pected. I t is my purpose, therefore, to review this evidence and to report my experience- in the examination of the tumors of the choroid which have oc curred in the Mayo Clinic, and to fur nish some ba.sis for the assertion that perhaps in the final analysis, the mel anomas represent the sole type of pri mary malignant tumors of this struc ture. T h e names that have been given to tumors of the choroid are legion. Fuchs described fourteen varieties of sarcomas and other writers have recommended the inclusion of other types. Parsons seems to refer only to tumors containing melanin or those po tentially capable of producing the pig ment. H e does refer briefly to the pos sibility of the occurrence of metastatic sarcomas, but is unable to cite an authentic case. In a review of the lit erature I was unable to find a well
When one investigates the literature of this subject, a surprising number of such cases are found. T h i s may be due to the fact that pigment from the choroid is necessarily contained in most intraocular tumors, even in gliomata in children. The classical evidences of intraocu lar tumor are hypertension, engorge ment of the anterior ciliary veins, reti nal detachment, particularly if circum scribed with very limited or no motion of the retina, and a definite shadow with transillumination. S. Hagen, in the Christiania Clinic, in a survey of the subject, concludes that the glauco matous stage appears usually in one year, or less, after the first symptoms of failing vision, etc., are noted. When metastasis develops, the subject may live from one to eight years. Of twenty-eight cases operated, fifty-eight per cent were cured. Early operation is advisable. Recurrences in the orbit occur only in cases where the tumor has perforated the sclera. T h e dan ger of recurrence is greatest in the first six months after operation, and the patient is safe after four years. In cases where the growths can be seen, they are usually found to be small, circular, fairly sharply defined, and slate gray in color, and have been compared to the color of mercurial ointment. Nettleship, de Schweinitz, Shumway, and R. Foster Moore are agreed that stippling or irregular pig mentation on the surface of the growth indicates activity. The growth is usually made up of spindle cells with irregular deposits of pigment granules. T h e average age of incidence is fifty years. Injuries seem to play a role in only ten per cent of the cases. Ellett^ reported three cases very similar to the author's case. In each instance an acute glaucoma had devel oped in an eye previously blind, and each eye was found to contain a sar 790
coma, altho mention of pigment is made in only one case. I Francis^ reports a case of malignant melanoma of the choroid, seen first as a circumscribed, yellow-white, raised area, in the macular region. Two years later this region showed a small retinal detachment, with Schiotz 17. After several conflicting opinions and a reduction of tension to 5, an enuclea tion was done. The pathologic diagnosis was malignant melanoma of the choroid. T h e most significant fea tures were the gradually decreasing in traocular tension and the absence of the usual evidences of tumor, namely engorged anterior ciliary veins, hyper tension, and positive transillumination. Mr. J . M. H.: Suffered an injury to the left eye 4 years ago. Has been having severe pain in this eye and vision has gradually failed. Three months before I saw him, vision began to fail. Did not consult an oculist, but optician gave him glasses. He was first examined November 7, 1922. There was severe pain, but only slight injec tion of deep scleral vessels. Finger tension was plus 3. There was no light perception. The lens was com pletely cataractous, so that no fundus details could be seen. T h e anterior chamber was practically obliterated. Transillumination of the globe was negative. Iridotasis was performed, and on November 18, 1922, the tension with Schiotz was 25. There was no pain and the eye was quiet and drainage good. As the tension rose repeatedly and could be controlled only with massage, a possible intraocular tumor was considered and enucleation ad vised. This operation was performed December 6, 1922. T h e eyeball was examined by Dr. Edwin F . Hirsch, director of the St. Luke's Hospital Laboratory. A tumor composed chiefly of spindle cells was found near the posterior pole. T h e tumor was one
PRANK Ε . BRAWLEY
cm. in its greatest diameter, and many of the cells were black with pigment. When healing was complete radium was advised. He received radium in the following dosage, administered by Dr. W m . L . Brown of the Physician's Radium Association: December 29, 1922, the left orbit was treated with 100 mgs. radium, screened with 0.8 mm. of gold and 2.1 mm. of rubber, for two hours. January 2, 1923', the same treatment was applied. January 3, 1923, 75 mgs. radium was used at one inch distance, for four hours. Febru ary 28, 1923, 50 mgs. radium similarly screened was left in the orbit for six hours. March 1, 1923, 50 mgs. radium screened with 0.8 mm. of gold, at 12
791
mm. distance from the orbital cavity, for three hours. T h e right eye showed an irregular hyeropic astigmatism and presbyopia, which were corrected, giving normal vision. T h e right fundus showed a few fine vitreous opacities but no active uveitis. Search for a focus of in fection was negative, except that ht was at one time treated for ulcer of the stomach. T h e orbit shows no recur rence, but the radium has destroyed all the cilia. There was perhaps a larger amount of orbital and lid secretion in this case than is usual, probably due to the radium. 30 N. Michigan Ave.
REFERENCES.
1.
Ellett, E. C. American Journal of Ophthalmology, October, 1920, p. 732. Francis, Lee M. American Journal of Ophthalmology, December, 1920, p. 872.
A CRITICAL SURVEY OF N E O P L A S M S O F T H E CHOROID. MARY S. K N I G H T , M . D .
ROCHESTER, MINNESOTA.
Reviewing recent work on the origin of melanin it is concluded that tihe chromatophores of the choroid and skin cannot produce pigment but are only phagocytic cells that ingest the melanin. A case is reported of bupbthalmos for which the eye was removed, and studied microscopically. From the study o f this specimen and accounts of others it is concluded that the choroid in this and similar cases shows the structure described in neurofibromatosis, the lesion being developmental and not malignant. This work was done at the Mayo Founda tion and the report of it submitted as part of the requirements for the Degree of Master of Science in Ophthalmology in the University of Minnesota.
One of the fundamental principles of the science of oncology is that the varieties of tumor to which any type of tissue may give rise will correspond to all the developmental variations which that tissue may assume in the body. Consequently, various types of car cinomas arise from epithelium, and tumors ranging from the fibromas to the varying forms of sarcomas, with many intermediate groups, arise from connective tissue. On this hypothesis a study of the primary tumors of the choroid, usually considered a connec tive tissue structure, would be ex pected to include examples of most, if not all, the varieties of neoplasms which might arise in other connective tissue structures of the body. Neither the literature on this subject nor the examination of a considerable number of actual tumors of the choroid has shown this as clearly as would be ex
pected. I t is my purpose, therefore, to review this evidence and to report my experience- in the examination of the tumors of the choroid which have oc curred in the Mayo Clinic, and to fur nish some ba.sis for the assertion that perhaps in the final analysis, the mel anomas represent the sole type of pri mary malignant tumors of this struc ture. T h e names that have been given to tumors of the choroid are legion. Fuchs described fourteen varieties of sarcomas and other writers have recommended the inclusion of other types. Parsons seems to refer only to tumors containing melanin or those po tentially capable of producing the pig ment. H e does refer briefly to the pos sibility of the occurrence of metastatic sarcomas, but is unable to cite an authentic case. In a review of the lit erature I was unable to find a well