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Retinoblastoma
American Journal of Ophthalmology PUBLISHED MONTHLY BY T H E OPHTHALMIC PUBLISHING COMPANY
EDITORIAL STAFF EDWARD JACKSON, Editor, CLARENCE LOEB, Associate Editor, 217 Imperial Bldg., Denver, Colo. 25 E. Washington St., Chicago, 111. M. URIBE-TRONCOSO, LAWRENCE T. POST, 226 W. 70th St., New York City. Metropolitan Bldg., St. Louis, Mo. MEYER WIENER, HARRY V. WURDEMANN, Carleton Bldg., St. Louis, Mo. Cobb Bldg., Seattle, Wash. Original papers, correspondence, and other scientific communications should be addressed the Editor. Books for review may be sent to any member of the editorial staff. Re ports of society proceedings should be sent to Dr. Lawrence T. Post, Metropolitan Bldg., St. Louis, Mo. Proof should be corrected, and returned wjthin forty-eight hours to the printers. Reprints may be obtained from the printers, Tucker-Kenworthy Co., 501 S. La Salle St., Chicago, III., if ordered at the time proofs are returned. But reprints to contain colored plates must be ordered when the article is accepted. Copy of advertisements must be sent to the Manager by the fifteenth of the month pre ceding its appearance. The annual subscription for the JOURNAL and the OPHTHALMIC YEAR BOOK is twelve dol
lars, in advance. Subscriptions, applications for single copies, communications with reference to advertising or other business, should be addressed to the Manager of Subscriptions and Advertising— J E A N M A T T E S O N , R O O M 1209, 7 W e s t Madison Street, Chicago, 111.
RETINOBLASTOMA. N a m e s a r e given for convenience, t o associate t h i n g s alike a n d t o dis c r i m i n a t e b e t w e e n t h o s e t h a t differ. C o m m o n and i m p o r t a n t diseases w e r e recognized a n d n a m e d b y t h e i r differ i n g clinical characteristics a n d loca tions, before a n y one h a d looked t h r u a microscope, or k n e w t h e biologic significance of a cell. W h e n V i r c h o w formulated t h e d o c t r i n e of every cell o r i g i n a t i n g from a p a r e n t cell, h e opened t h e w a y t o a b e t t e r u n d e r s t a n d i n g of t h e n a t u r e of t u m o r s . B u t h e did n o t a b r o g a t e t h e i r clinical char a c t e r s . W h e n he supposed t h a t t h e t u m o r h e found in an e y e sent t o h i m b y Graefe w a s similar t o t h e t u m o r s of t h e brain, a r i s i n g from proliferation of glial tissue, he m a d e a m i s t a k e . H e did not k n o w h o w w i d e l y t h e y differed in essential c h a r a c t e r i s t i c s ; a n d h i s mis take h a s caused g r e a t confusion as r e g a r d s t h e n a m i n g of t h e retinal t u m o r s t h a t arise in infancy. M o r e t h a n a c e n t u r y a n d a half a g o w a s published a description of a case, perfectly r e c o g n i z a b l e a s similar t o t h e one t h a t V i r c h o w called a " g l i o m a of the r e t i n a , " or t h e one t h a t F l e x n e r called a " n e u r o e p i t h e l i o m a r e t i n a e . " I n medical l i t e r a t u r e t h e r e h a d a p peared m a n y r e p o r t s d e s c r i b i n g this
one disease, b u t g i v i n g it m a n y differ ent n a m e s , before V i r c h o w in 1854, called it " g l i o m a of t h e r e t i n a , " a n d this n a m e b e c a m e a s t u m b l i n g block for s t u d e n t s of t h e s u b j e c t Becker in 1873, insisted on t h e similarities of t h e s e t u m o r s t o sarcoma, j u s t as Parker, Stokes and W a r t h i n have done this year. Becker called it a t u b u l a r a n g i o s a r c o m a , a n d figured a typical " r o s e t t e , " s u p p o s i n g his case n o t t o b e a typical o n e ; a n d h e s u g g e s t e d calling it n e u r o e p i t h e l i o m a , t r a c i n g t h e r o s e t t e to a rolled u p p a r t of t h e r o d a n d cone layer. W i n t e r s t e i n e r , 1879, s h o w e d t h a t Flexner's case w a s t y p i c a l ; a n d adopted the name "neuroepithelioma" as a b e t t e r n a m e for w h a t V i r c h o w h a d called "glioma" of the retina. Since t h e n o t h e r n a m e s h a v e been s u g g e s t e d for t h i s disease, b u t t h e m o s t i m p o r tant are retinocytoma, suggested by M a w a s ; and retinoblastoma proposed b y Verhoeff a n d favored b y Mallory and Feingold. M a l i g n a n t t u m o r s s h o w this com mon difference from all n o r m a l tissues of t h e b o d y : T h e y consist of cells which a r e w i t h o u t p o w e r of p e r f o r m i n g a n y function w h i c h is useful t o t h e b o d y a s a w h o l e . T h e y h a v e reverted to a p r i m i t i v e t y p e , in which t h e w h o l e 705
706
EDITORIALS
power of the cell is devoted to increase in bulk and multiplication. This char acter marks an early stage of develop ment, an embryonic condition, of all tissues of the body. A tumor composed of "germ cells" is called a blastoma, from the Greek blastos a germ. The anlage, or founda tion of each organ is composed of such germ cells. When they have grown and multiplied enough to furnish a sufficient bulk to meet the needs of the body for that organ, they develop the normal tendency to specialize into cells that will perform its function. Thus certain cells develop to perform the function of nerve cells. If the cells that should do so fail to develop such special characters; or if some of them fail in this development, but simply continue to grow and multiply like germ cells, they constitute a tumor called a neuroblastoma. Mallory's plan of recognizing the point of origin and germ cell character istics of tumors, by calling them blastomas, is being widely adopted by pathologists. He classed the tumors we are considering as neuroblastomas. But in this class are other tumors, aris ing in other parts of the nervous sys tem and differing essentially from the retinal tumors, in their clinical course and characteristics. If it were proved that these retinal tumors developed from germ cells intended to give rise to the glial tissue of the retina they might be called glioblastomas. But this is not proved; and it is not likely that they do develop chiefly from those germ cells. If they did, the term would still be a poor one, because it would apply equally to the gliomas of the brain, optic tracts and chiasm; which are totally different in their clinical courses and tendencies. The terms neuroepithelioma and retinocytoma are not appropriate, be cause the tumors in question do not consist of cells of neuroepithelium, or of cells like those found in any part of the retina, after the fifth month of fetal life. Moreover, neuroepithelioma has been applied to tumors of wholly different character, arising in different
parts of the central nervous system. The inappropriateness of calling a tumor retinocytoma, that does not contain anything looking or staining like retinal cells, is emphasized by the persistent efforts to call these tumors some kind of sarcoma, because the mass of cells of which they are com posed, look and stain so much like those of socalled round cell sarcoma. The term retinoblastoma indicates the kinds of cells that are found in all such tumors, at all stages and in all parts of the body. The "rosettes," for which Mawas has proposed a new name, "stephanocytes" (from their re semblance to a crown) are only found in the primary tumors, developing within the eyeball; and they are well developed in less than one-third of the cases. It is the same kind of tumor, running the same course, showing the same malignancy, whether the "ro settes" are present or not. W e do not want one name for the tumor in the eyeball, and another name for the metastatic tumor following in the liver, or humerus. When Morax admits that retinoblastoma is appropriate to the tumors that do not contain "stephano cytes," he virtually admits that this is the name most appropriate to all the secondary and most of the primary tumors of this class. At its meeting this year, The Ameri can Ophthalmological Society adopted the report of its committee on this sub ject which recommended: "That the term Retinoblastoma be hereafter used to designate the tumors heretofore designated as Glioma of the Retina, Gliosarcoma, Medullary Cancer of the Retina, Neuroepithelioma Retinae, Retinocytoma, etc." and "That the Publication Committee be instructed to use it, either as the primary or sec ondary designation of such tumors, in the transactions." There is much rea son that, until the new term has be come familiar to readers, one of the old ones should accompany it by way of explanation; and this will be the editorial policy of the AMERICAN JOUR NAL OF OPHTHALMOLOGY.
E. J.
EDITORIAL STAFF EDWARD JACKSON, Editor, CLARENCE LOEB, Associate Editor, 217 Imperial Bldg., Denver, Colo. 25 E. Washington St., Chicago, 111. M. URIBE-TRONCOSO, LAWRENCE T. POST, 226 W. 70th St., New York City. Metropolitan Bldg., St. Louis, Mo. MEYER WIENER, HARRY V. WURDEMANN, Carleton Bldg., St. Louis, Mo. Cobb Bldg., Seattle, Wash. Original papers, correspondence, and other scientific communications should be addressed the Editor. Books for review may be sent to any member of the editorial staff. Re ports of society proceedings should be sent to Dr. Lawrence T. Post, Metropolitan Bldg., St. Louis, Mo. Proof should be corrected, and returned wjthin forty-eight hours to the printers. Reprints may be obtained from the printers, Tucker-Kenworthy Co., 501 S. La Salle St., Chicago, III., if ordered at the time proofs are returned. But reprints to contain colored plates must be ordered when the article is accepted. Copy of advertisements must be sent to the Manager by the fifteenth of the month pre ceding its appearance. The annual subscription for the JOURNAL and the OPHTHALMIC YEAR BOOK is twelve dol
lars, in advance. Subscriptions, applications for single copies, communications with reference to advertising or other business, should be addressed to the Manager of Subscriptions and Advertising— J E A N M A T T E S O N , R O O M 1209, 7 W e s t Madison Street, Chicago, 111.
RETINOBLASTOMA. N a m e s a r e given for convenience, t o associate t h i n g s alike a n d t o dis c r i m i n a t e b e t w e e n t h o s e t h a t differ. C o m m o n and i m p o r t a n t diseases w e r e recognized a n d n a m e d b y t h e i r differ i n g clinical characteristics a n d loca tions, before a n y one h a d looked t h r u a microscope, or k n e w t h e biologic significance of a cell. W h e n V i r c h o w formulated t h e d o c t r i n e of every cell o r i g i n a t i n g from a p a r e n t cell, h e opened t h e w a y t o a b e t t e r u n d e r s t a n d i n g of t h e n a t u r e of t u m o r s . B u t h e did n o t a b r o g a t e t h e i r clinical char a c t e r s . W h e n he supposed t h a t t h e t u m o r h e found in an e y e sent t o h i m b y Graefe w a s similar t o t h e t u m o r s of t h e brain, a r i s i n g from proliferation of glial tissue, he m a d e a m i s t a k e . H e did not k n o w h o w w i d e l y t h e y differed in essential c h a r a c t e r i s t i c s ; a n d h i s mis take h a s caused g r e a t confusion as r e g a r d s t h e n a m i n g of t h e retinal t u m o r s t h a t arise in infancy. M o r e t h a n a c e n t u r y a n d a half a g o w a s published a description of a case, perfectly r e c o g n i z a b l e a s similar t o t h e one t h a t V i r c h o w called a " g l i o m a of the r e t i n a , " or t h e one t h a t F l e x n e r called a " n e u r o e p i t h e l i o m a r e t i n a e . " I n medical l i t e r a t u r e t h e r e h a d a p peared m a n y r e p o r t s d e s c r i b i n g this
one disease, b u t g i v i n g it m a n y differ ent n a m e s , before V i r c h o w in 1854, called it " g l i o m a of t h e r e t i n a , " a n d this n a m e b e c a m e a s t u m b l i n g block for s t u d e n t s of t h e s u b j e c t Becker in 1873, insisted on t h e similarities of t h e s e t u m o r s t o sarcoma, j u s t as Parker, Stokes and W a r t h i n have done this year. Becker called it a t u b u l a r a n g i o s a r c o m a , a n d figured a typical " r o s e t t e , " s u p p o s i n g his case n o t t o b e a typical o n e ; a n d h e s u g g e s t e d calling it n e u r o e p i t h e l i o m a , t r a c i n g t h e r o s e t t e to a rolled u p p a r t of t h e r o d a n d cone layer. W i n t e r s t e i n e r , 1879, s h o w e d t h a t Flexner's case w a s t y p i c a l ; a n d adopted the name "neuroepithelioma" as a b e t t e r n a m e for w h a t V i r c h o w h a d called "glioma" of the retina. Since t h e n o t h e r n a m e s h a v e been s u g g e s t e d for t h i s disease, b u t t h e m o s t i m p o r tant are retinocytoma, suggested by M a w a s ; and retinoblastoma proposed b y Verhoeff a n d favored b y Mallory and Feingold. M a l i g n a n t t u m o r s s h o w this com mon difference from all n o r m a l tissues of t h e b o d y : T h e y consist of cells which a r e w i t h o u t p o w e r of p e r f o r m i n g a n y function w h i c h is useful t o t h e b o d y a s a w h o l e . T h e y h a v e reverted to a p r i m i t i v e t y p e , in which t h e w h o l e 705
706
EDITORIALS
power of the cell is devoted to increase in bulk and multiplication. This char acter marks an early stage of develop ment, an embryonic condition, of all tissues of the body. A tumor composed of "germ cells" is called a blastoma, from the Greek blastos a germ. The anlage, or founda tion of each organ is composed of such germ cells. When they have grown and multiplied enough to furnish a sufficient bulk to meet the needs of the body for that organ, they develop the normal tendency to specialize into cells that will perform its function. Thus certain cells develop to perform the function of nerve cells. If the cells that should do so fail to develop such special characters; or if some of them fail in this development, but simply continue to grow and multiply like germ cells, they constitute a tumor called a neuroblastoma. Mallory's plan of recognizing the point of origin and germ cell character istics of tumors, by calling them blastomas, is being widely adopted by pathologists. He classed the tumors we are considering as neuroblastomas. But in this class are other tumors, aris ing in other parts of the nervous sys tem and differing essentially from the retinal tumors, in their clinical course and characteristics. If it were proved that these retinal tumors developed from germ cells intended to give rise to the glial tissue of the retina they might be called glioblastomas. But this is not proved; and it is not likely that they do develop chiefly from those germ cells. If they did, the term would still be a poor one, because it would apply equally to the gliomas of the brain, optic tracts and chiasm; which are totally different in their clinical courses and tendencies. The terms neuroepithelioma and retinocytoma are not appropriate, be cause the tumors in question do not consist of cells of neuroepithelium, or of cells like those found in any part of the retina, after the fifth month of fetal life. Moreover, neuroepithelioma has been applied to tumors of wholly different character, arising in different
parts of the central nervous system. The inappropriateness of calling a tumor retinocytoma, that does not contain anything looking or staining like retinal cells, is emphasized by the persistent efforts to call these tumors some kind of sarcoma, because the mass of cells of which they are com posed, look and stain so much like those of socalled round cell sarcoma. The term retinoblastoma indicates the kinds of cells that are found in all such tumors, at all stages and in all parts of the body. The "rosettes," for which Mawas has proposed a new name, "stephanocytes" (from their re semblance to a crown) are only found in the primary tumors, developing within the eyeball; and they are well developed in less than one-third of the cases. It is the same kind of tumor, running the same course, showing the same malignancy, whether the "ro settes" are present or not. W e do not want one name for the tumor in the eyeball, and another name for the metastatic tumor following in the liver, or humerus. When Morax admits that retinoblastoma is appropriate to the tumors that do not contain "stephano cytes," he virtually admits that this is the name most appropriate to all the secondary and most of the primary tumors of this class. At its meeting this year, The Ameri can Ophthalmological Society adopted the report of its committee on this sub ject which recommended: "That the term Retinoblastoma be hereafter used to designate the tumors heretofore designated as Glioma of the Retina, Gliosarcoma, Medullary Cancer of the Retina, Neuroepithelioma Retinae, Retinocytoma, etc." and "That the Publication Committee be instructed to use it, either as the primary or sec ondary designation of such tumors, in the transactions." There is much rea son that, until the new term has be come familiar to readers, one of the old ones should accompany it by way of explanation; and this will be the editorial policy of the AMERICAN JOUR NAL OF OPHTHALMOLOGY.
E. J.