Ophthalmologica, Basel 172: 412-415 (1976)
Malignant Melanoma in the Orbit A Clinicopathologlcal Case Report1 H. F anta Augenabteilung, Rudolfspital (Head: Prof. Dr. H. F anta), Wien
The purpose of this report is to present histopathologic findings of a malignant melanoma in the orbit 9 years after evisceration of the eye.
Clinical History This 72-year-old female (case No. 3135/68), has been suffering from a chronic glaucoma of both eyes for many years. The right eye had been blind for some time; the intraocular tension of the left eye was controlled by means of drops. The blind eye became more and more painful, and so the patient came for treat ment. The patient refused enucleation because she had heard that after an eviscera tion of the eye a prosthesis could be comfortably worn and finally with better cosmetic effects, as it would be more movable. The clinical diagnosis at that time was; absolute glaucoma of the right eye and chronic glaucoma of the left eye. According to the patient's wishes, an evisceration of the right eye was per formed, which healed normally. The patient then wore a prosthesis for 9 years without trouble. The left eye was continually under treatment. During the last 2 months, the patient realized that she could no longer wear the prosthesis because the socket had become smaller in size. At the examination at that time a dark mass like a tumor was seen and felt under the conjunctiva. A simple excision at this part showed a malignant melanoma. So an exenteration of the orbit was finally performed.
i With the support of the ‘Wissenschaftlicher Fonds der Stadt Wien’. Pre sented at the 13th Annual Meeting of the European Ophthalmic Pathology Society, Toulouse 1974.
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Ocular Pathology The tumor consists of dark, finely grained, pigmented and partly nodular cells. Some of them are small, some are spindel-shaped, others are larger and cu
Malignant Melanoma in the Orbit
413
boid. Parts of the tumor are necrotic and others are comprised of fibrous cords (fig. 1). At other places, follicular lymphocytes can be recognized. There are tumor cells in the blood vessels and in the lymphatic channels. The tumor grows around the optic nerve, which is surrounded by hyaline tissue, but it has not invaded the inside of the nerve. The sclera has collapsed; it is scarred and contains tumor masses (fig. 2). In the central part, there is lymphoreticular tissue; more densely distributed tumor cells are noted in some areas.
Evisceration of the eye used to be performed very frequently because it left a better base for the prosthesis. Personally, I have always preferred enucleation to evisceration because, as this case demonstrates again, enucleation is a safer operation for the patient. In this case, the eye was eviscerated only because the patient refused enucleation. A retrobulbar alcohol injection against pain had remained without result. The evis ceration was performed carefully but the contents of the eye examined only macroscopically. Finally, the sclera was so cleaned that it was completely white inside. A primary malignant melanoma of the orbit is very rare. Only some 47 cases have been reported in the literature [Reese, 1963] and in some of them the diagnosis of primary malignant melanoma was questioned. At the time of the last operation (exenteration of the orbit), the patient was in quite good health, and no metastases could be found. Even a puncture of the liver showed a normal result. But 1 year later, the patient died of generalized metastases of the tumor. In this case, it is improbable that it is a primary melanoma of the orbit which has grown into the sclera. The melanoma probably already existed at the time when the evisceration was performed but was possibly too small to be detected by macroscopic examination. It can be assumed that there were residues of the tumor in an emissary which grew slowly out of the sclera into the orbit. Remarkable is the fact that it took 9 years till the tumor grew large enough to bother the patient. This is remarkable because a tumor which grows in an emissary generally grows faster after such a period of time. A tumor can only develop in the presence of melanocytes, i.e. in the eye, on the surface of the sclera, in an emissary and in the sclera channel of a ciliary nerve; outside of the bulb, only in the conjunctiva and caruncle. The last two cannot be considered because the tumor would not have grown inside
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Discussion
F anta
Fig. 1. Different cell types and necrotic parts outside the scarred sclera. Fig. 2. Tumor masses in the scarred sclera.
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414
Malignant Melanoma in the Orbit
415
the scarred sclera. The same applies for metastases after a primary malig nant melanoma of the skin. If the tumour were not inside the eye when it was eviscerated it might have developed later from a melanocyte in an emissary or in the sclera channel around a ciliary nerve [L eopoldsberger, 1940; V ogel, 1970]. It is not possible to draw a definite conclusion because the necessary observations could not be made. This case again shows that it is very difficult to diagnose a primary malignant melanoma of the orbit. Even though the period of 9 years is remarkably long, I believe that the primary tumor was not inside the eye, but in an emissary or in a sclera channel of a ciliary nerve.
References L eopoldsberger , O. W.: Zur Frage der neurogenen Herkunft der Aderhautsarkome.
Graefes Arch. Ophthal. 142: 229-240 (1940). R eese , A. B.: Tumor of the eye (Hoeber, New York 1963). V ogel, M. H.: Malignes Aderhautsarkom und Ziliarnerv. Klin. Mbl. Augenheilk.
Prof. Dr. H. F anta, Ferstelgasse 4, A-1090 Wien (Austria)
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157: 215-227 (1970).
Malignant Melanoma in the Orbit A Clinicopathologlcal Case Report1 H. F anta Augenabteilung, Rudolfspital (Head: Prof. Dr. H. F anta), Wien
The purpose of this report is to present histopathologic findings of a malignant melanoma in the orbit 9 years after evisceration of the eye.
Clinical History This 72-year-old female (case No. 3135/68), has been suffering from a chronic glaucoma of both eyes for many years. The right eye had been blind for some time; the intraocular tension of the left eye was controlled by means of drops. The blind eye became more and more painful, and so the patient came for treat ment. The patient refused enucleation because she had heard that after an eviscera tion of the eye a prosthesis could be comfortably worn and finally with better cosmetic effects, as it would be more movable. The clinical diagnosis at that time was; absolute glaucoma of the right eye and chronic glaucoma of the left eye. According to the patient's wishes, an evisceration of the right eye was per formed, which healed normally. The patient then wore a prosthesis for 9 years without trouble. The left eye was continually under treatment. During the last 2 months, the patient realized that she could no longer wear the prosthesis because the socket had become smaller in size. At the examination at that time a dark mass like a tumor was seen and felt under the conjunctiva. A simple excision at this part showed a malignant melanoma. So an exenteration of the orbit was finally performed.
i With the support of the ‘Wissenschaftlicher Fonds der Stadt Wien’. Pre sented at the 13th Annual Meeting of the European Ophthalmic Pathology Society, Toulouse 1974.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/7/2018 9:57:02 PM
Ocular Pathology The tumor consists of dark, finely grained, pigmented and partly nodular cells. Some of them are small, some are spindel-shaped, others are larger and cu
Malignant Melanoma in the Orbit
413
boid. Parts of the tumor are necrotic and others are comprised of fibrous cords (fig. 1). At other places, follicular lymphocytes can be recognized. There are tumor cells in the blood vessels and in the lymphatic channels. The tumor grows around the optic nerve, which is surrounded by hyaline tissue, but it has not invaded the inside of the nerve. The sclera has collapsed; it is scarred and contains tumor masses (fig. 2). In the central part, there is lymphoreticular tissue; more densely distributed tumor cells are noted in some areas.
Evisceration of the eye used to be performed very frequently because it left a better base for the prosthesis. Personally, I have always preferred enucleation to evisceration because, as this case demonstrates again, enucleation is a safer operation for the patient. In this case, the eye was eviscerated only because the patient refused enucleation. A retrobulbar alcohol injection against pain had remained without result. The evis ceration was performed carefully but the contents of the eye examined only macroscopically. Finally, the sclera was so cleaned that it was completely white inside. A primary malignant melanoma of the orbit is very rare. Only some 47 cases have been reported in the literature [Reese, 1963] and in some of them the diagnosis of primary malignant melanoma was questioned. At the time of the last operation (exenteration of the orbit), the patient was in quite good health, and no metastases could be found. Even a puncture of the liver showed a normal result. But 1 year later, the patient died of generalized metastases of the tumor. In this case, it is improbable that it is a primary melanoma of the orbit which has grown into the sclera. The melanoma probably already existed at the time when the evisceration was performed but was possibly too small to be detected by macroscopic examination. It can be assumed that there were residues of the tumor in an emissary which grew slowly out of the sclera into the orbit. Remarkable is the fact that it took 9 years till the tumor grew large enough to bother the patient. This is remarkable because a tumor which grows in an emissary generally grows faster after such a period of time. A tumor can only develop in the presence of melanocytes, i.e. in the eye, on the surface of the sclera, in an emissary and in the sclera channel of a ciliary nerve; outside of the bulb, only in the conjunctiva and caruncle. The last two cannot be considered because the tumor would not have grown inside
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/7/2018 9:57:02 PM
Discussion
F anta
Fig. 1. Different cell types and necrotic parts outside the scarred sclera. Fig. 2. Tumor masses in the scarred sclera.
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/7/2018 9:57:02 PM
414
Malignant Melanoma in the Orbit
415
the scarred sclera. The same applies for metastases after a primary malig nant melanoma of the skin. If the tumour were not inside the eye when it was eviscerated it might have developed later from a melanocyte in an emissary or in the sclera channel around a ciliary nerve [L eopoldsberger, 1940; V ogel, 1970]. It is not possible to draw a definite conclusion because the necessary observations could not be made. This case again shows that it is very difficult to diagnose a primary malignant melanoma of the orbit. Even though the period of 9 years is remarkably long, I believe that the primary tumor was not inside the eye, but in an emissary or in a sclera channel of a ciliary nerve.
References L eopoldsberger , O. W.: Zur Frage der neurogenen Herkunft der Aderhautsarkome.
Graefes Arch. Ophthal. 142: 229-240 (1940). R eese , A. B.: Tumor of the eye (Hoeber, New York 1963). V ogel, M. H.: Malignes Aderhautsarkom und Ziliarnerv. Klin. Mbl. Augenheilk.
Prof. Dr. H. F anta, Ferstelgasse 4, A-1090 Wien (Austria)
Downloaded by: Univ. of California Santa Barbara 128.111.121.42 - 3/7/2018 9:57:02 PM
157: 215-227 (1970).
