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Lymphosarcoma of Lacrimal and Salivary Glands*
CONGENITAL GLAUCOMA
859
40. Ashton, N., Brini, A. and Smith, R.: Anatomical studies of the trabecular meshwork of the normal human eye. Brit. J. Ophth, 40:257, 1956. 41. Heine, L.: The anatomy of the accommodation of the eye. Arch. f. Ophth., 4 9 : 1 , 1899. 42. Flocks, M., and Zweng, H. C : Studies on the mode of action of pilocarpine on aqueous flow. Am. J. Ophth., 44:380, 1957. 43. Nonnenmacher, H . : Experiences in the treatment of infantile glaucoma with atropine. Klin. Monatsbl. f. Augenh., 121:533, 1952.
LYMPHOSARCOMA RALPH
S.
OF LACRIMAL AND
HAMILTON,
M.D.,
Philadelphia, Lacrimal gland enlargements may have a v a r i e t y of e t i o l o g i e s . A n i n f r e q u e n t c a u s e of s u c h a f i n d i n g is l y m p h o s a r c o m a , as d e m o n s t r a t e d in t h e f o l l o w i n g c a s e . CASE
REPORT
C. S., a 70-year-old white woman, was admitted to Wills Eye Hospital on July 12, 1957, with a chief complaint of swellings in both upper lids and of a swelling at the angle of the right mandi ble. Her present illness dated back to one year previously, at which time she first noted a small mass in the left upper eyelid. This gradually be came larger and she consulted an ophthalmologist in her home town. X-ray studies of the orbit were negative and the tumor was excised. Pathologic studies failed to establish a diagnosis. Soon after this she developed a swelling in the right upper eyelid and, three weeks prior to her admission to the Wills Eye Hospital, she developed a swelling at the angle of the right mandible. She had been followed by her ophthalmologist for eight months prior to her referral to Wills Eye Hospital for further studies and treatment. Her past medical history appeared unrelated to her present complaints although it included a ruptured appendix in 1914, herpes zoster, acute hepatitis due to infection, and hypoproteinemia in 1951. In 1951 she had been hospitalized and had a thorough work-up of her gastrointestinal and cardiovascular systems which failed to reveal any malignancy. General physical examination showed an elderly female, well-developed and somewhat obese. Blood pressure was 150/66 mm. H g ; pulse 64. Examina tion of head and neck was significant and will be described in more detail with the eye examination. Lung fields were clear. Cardiac studies revealed P M I in fifth intercostal space in left midclavicular line with a Grade 1 aortic systolic murmur. The abdomen revealed no palpable mass. Liver and spleen were not enlarged. There were no palpable lymph nodes in axilla, supraclavicular, or inguinal regions. Breasts showed no masses. Extremities had no palpable or visible edema. * From the Wills Eye Hospital.
AND
IRVING
SALIVARY H.
LEOPOLD,
GLANDS* M.D.
Pennsylvania Eye examination. Visual acuity was: O.D., 6/30 and 6/15, with correction; O.S., 6/60 and 6/21 with correction. Intraocular pressure was: O.D., 20 mm. H g ; O.S., 20 mm. H g (Schi^ltz). External examination. There was no enophthalmos or exophthalmos. Lids revealed large tumor masses 3.0 by 2.0 cm. in the right upper lid over the lacrimal gland area and 2.5 by 1.75 cm. in the left upper lid over the lacrimal gland area. These masses were firm, nonmovable, not fixed to skin, and were painless. The tumor masses were so large that the patient had almost complete ptosis of both lids. In addition to the tumor masses was a large, firm, disc-shaped mass 4.0 by 5.0 cm. in the right periauricular and parotid region. This mass was firm, nonmovable, nontender, and not fixed to skin. Also there was a large submaxillary gland on either side measuring 2.0 by 3.0 cm. Cilia and the lacrimal drainage apparatus were normal. The conjunctiva showed some chemosis. The corneas were normal. Anterior chambers were of normal depth. Irises were normal. Pupils were round, regular, and equal, and responded to light directly and consensually. Muscle balance revealed marked limitation of lateral rotations in each eye, neither eye being able to rotate to the midline from its more or less fixed nasal displacement. The patient was unable to look up and out at all; rotations up nasally and down nasally were not restricted. She had 15 degrees of esotropia and complained of diplopia when both lids were manually elevated. She had a complete ptosis on the right and almost complete ptosis on the left. Fundus examination revealed the media to be clear, disc of good color, margins were distinct, cupping was physiologic, vessels revealed normal light reflex, there were no hemorrhages or exudates, macular reflexes were good, and there were no stress signs in the f undi. Impressions at this time were: (1) Bilateral lacrimal gland tumors with extensive preauricular lymphadenopathy on right or parotid gland swell ing on right, and bilateral submaxillary adenopathy. It was felt that this could be considered a Mikulicz's syndrome, probably based on sarcoidosis or some nonspecific granuloma. (2) The second consideration was that this could be a primary or metastatic malignancy of lacrimal glands.
860
R A L P H S. H A M I L T O N A N D I R V I N G H. L E O P O L D
Laboratory studies. Complete blood count re vealed hemoglobin of 12.5 gm. or 81 percent; red blood count 4,810,000; white blood count 4,950; differential count was segmented, neutrophils 62 percent, lymphocytes 32 percent, monocytes one percent, eosinophiles two percent, and bands three percent. Urinalysis. Albumin: negative; sugar: negative; specific gravity: 1.022; acid reaction: microscopic examination revealed two or three white blood cells, a rare red blood cell, and loaded with mucous. Blood sugar was 90 mg.; sedimenta tion rate, 8.0 mm. in 60 min.; Lee and White coagulation time: 10 min., 30 sec, bleeding time two min., 15 sec. Total serum protein: 5.1, albumin 3.5, globulin 1.6; test for Bence Jones protein, in urine negative. X-ray studies revealed calcification of internal carotid arteries, otherwise normal skull, orbits, paranasal sinuses, and optic foramina. The chest X-ray film showed an arteriosclerotic aorta and left ventricular enlargement. There was no widening of hilar or mediastinal nodes. X-ray films of hands and feet showed osteoparetic changes but nothing to suggest sarcoidosis. On July 16, 1957, on the tentative diagnosis of nonspecific granuloma, the patient was started on 20 mg. of A C T H in 500 cc. of five-percent glucose in distilled water for three days and Chloromycetin, 500 mg. every six hours. On July 20, 1957, she was put on A C T H , 40 mg. in the form of Acthar Gel intramuscularly twice a day and potassium chloride 1.0 gm. daily. She was also started on 50 mg. of prednisolone twice a day. Two days after instigation of A C T H therapy all of the masses began to resolve. Within four days there was complete resolution of both submaxillary glands and of the large mass in the right parotid region. The masses over both lacrimal glands were diminishing in size. After one week of steroid therapy, the patient could open both lids to the level of uncovering one half of each pupil. A biopsy was taken to establish the diagnosis. This was done on July 23, 1957, uneventfully under local anesthesia from the site of right lacrimal gland. This patient was discharged from the hospital on August 2, 1957, on 5.0 mg. of prednisolone four times a day and 1.0 gm. of potassium chloride daily. At the time of her discharge the masses over each lacrimal gland area were approximately one third their original size. The patient no longer had diplopia except on lateral gaze, indicating a resolu tion of the orbital extension of these masses. The ptosis was disappearing and the patient subjectively felt well. Readmission. The patient developed a fever on August 12, 1957, and sores in her mouth. She was started on mycostatin at home at this time since it seemed that she might have developed moniliasis or one of the fungus infections with the steroid treatment. She did not improve and was readmitted on August 15, 1957. At this time she complained of fever and malaise. General physical examination revealed a temperature of 102°F. orally, rales in the right lower lung fields, and percussion dullness
over this area. X-ray studies of the chest now re vealed a confluent bronchopneumonia at the base of the right lung. Routine red blood count, differ ential, and urinalysis were all normal. First and second strength Mantoux tests were again negative. Treatment consisted of prednisolone, 10 mg., three times a day, Mysteclin, 250 mg., four times a day, and Mycostatin, two tablets (1,000,000 units), four times a day. Microscopic studies of the biopsy specimen estab lished the diagnosis of lymphosarcoma. In view of this microscopic diagnosis, the patient was given 6.0 mg. of nitrogen mustard intravenously daily for four days, starting on August 21st. On August 20, 1957, Mysteclin and Mycostatin were discon tinued. By August 26th, X-ray studies of the chest revealed almost complete resolution of pneumonia and the patient was discharged home on August 27, 1957, on prednisolone, 5.0 mg. three times daily for one week, then twice a day and K Q , 1.0 gm., three times daily. The lacrimal masses were almost completely resolved in both eyes. The right eye lid was in normal position, the left was still slightly ptotic. On December 14, 1957, the patient revealed no signs of any enlargement of lacrimal glands, parot id, or cervical lymph nodes. She did have a slight ptosis of left upper lid, but no ptosis of right upper lid. She was in good spirits and apparently in good health. On July 28, 1958, the patient's family re ported her to be in good health with no evidence of recurrence of the tumor masses in either lid, preauricular, or cervical regions. DISCUSSION
L a c r i m a l t u m o r s h a v e b e e n s a i d to c o m p o s e f o u r t o IS p e r c e n t of all o r b i t a l t u m o r s b y F o r r e s t . 2 D u k e - E l d e r 1 s t a t e s t h a t five p e r cent of t u m o r s of t h e l a c r i m a l g l a n d a r e d e rived
from
lymphoid
tissue.
Forrest2
has
g i v e n u s a r a t h e r c o m p l e t e p a t h o l o g i c classi fication of all l a c r i m a l g l a n d t u m o r s . H e con s i d e r s f o u r m a i n g r o u p s as f o l l o w s :
A. Epitheloid lacrimal gland tumors 1. Benign or mixed tumors of lacrimal gland. 2. Malignant lacrimal gland tumors a. Adenoid cystic type b. Adenocarcinoma c. Squamous cell carcinoma d. Undifferentiated carcinoma e. Malignant mixed tumors B. Inflammatory disease 1. Sarcoid 2. Nonspecific granulomas C. Localized evidence of a lymphoma (into which class this case may be classified.)
LYMPHOSARCOMA OF LACRIMAL GLAND D. Neoplasms or inflammation unrelated to the lacrimal gland as metastatic lesions The treatment of the above lacrimal gland involvments may be summarized as follows: Group A responds only to complete and adequate removal of all the tumor and struc tures, including the bony lacrimal fossa, which are invaded by the tumor. Forrest 2 be lieves that the adenoid cystic type of carci noma under the malignant lacrimal gland tumors is radiosensitive. Group B usually responds best to systemic A C T H and steroid treatment. It is into this group that our patient was originally believed to belong. This was the rationale for the treatment with A C T H (adrenocorticotropic hormone). Attention is again called to the dramatic resolution of the tumors on this treatment. Group C is best treated with radiation therapy. Biopsy must be done for diagnostic purposes. Group D responds best to treatment di rected at the primary site of the lesion. There has been considerable overlapping and confusion as regards the classification of the lypmphomas involving the lacrimal glands. Duke-Elder 2 classifies them as fol lows: ( 1 ) Lymphoma, ( 2 ) leukemia, ( 3 ) lymphosarcoma, ( 4 ) reticulum-cell sarcoma, ( 5 ) giant follicular lymphoblastoma, ( 6 ) Hodgkin's disease. It is well known that ocular manifestations of a lymphomatous disorder may be mani festing signs of a generalized spread of the
861
disease or a prelude to development of a systemic lymphomatous disorder. These cases certainly must be very carefully followed over many years. McGavic 5 has stated that the lymphomatoid diseases are an ever-chang ing process that require repeated and care ful biopsy and blood studies. Survival rates of the lymphomatous dis orders have been pointed out by Sugarbaker and Craver 8 to be influenced by: ( 1 ) inherent qualities for growth and dissemination; (2) extent of dosage when first treated; ( 3 ) localization of primary lesion, that is, out come is much more favorable when primary site is in the head. Perhaps this is because tumors in this region present themselves to the patients early and these patients seek medical attention promptly. ( 4 ) Complicating leukemia increases mortality. ( 5 ) Age of on set, that is, the younger the patient the shorter the survival rate, the older the pa tient, the longer the survival rate. SUMMARY
In summary, a case of lymphosarcoma pre senting itself as massive bilateral lacrimal and salivary gland swellings has been de scribed. The dramatic resolution of the masses has been pointed out. While this pa tient did receive nitrogen mustard later in the course of her illness, the masses had all re solved and it is felt that the steroids and adrenocortinotropic hormones were responsi ble for this. A classification of lacrimal gland tumors has been presented and treatment has been described. 1601 Spring Garden Street (30).
REFERENCES
1. Duke-Elder, W. S.: Textbook of Ophthalmology. St. Louis, Mosby, 1952 and 1955, pp. 5265-5270. 2. Forrest, A. W.: Epithelial lacrimal gland tumors: Pathology as guide to prognosis. Tr. Am. Acad. Ophth., 58:848-865 (Nov.-Dec.) 1954. 3. Gruber, E.: Sarcoidosis of lacrimal glands associated with Sj^gren's syndrome. Arch. Ophth., 56: 42-47, 1956. 4. Jones, I. S., and Pfeifer, R. F.: Lacrimal gland tumors: Roentgenographic diagnosis. Tr. Am. Acad. Ophth., 58:841-847 (Nov.-Dec.) 1954. 5. McGavic, J. S.: Lymphomatoid diseases involving the eye and its adnexa. Arch. Ophth., 30:179-193, 1943. 6. Reese, A. B.: Tumors of the Eye. New York, Hoeber, 1951, pp. 464-469. 7. : The treatment of expanding lesions of the orbit with particular regard to those arising in the lacrimal gland. Am. J. Ophth., 41:3, 1956. 8. Sugarbaker, E. D., and Craver, L. F.: Lymphosarcoma: A study of 196 cases with biopsy J A M A 115:17-23 and 112-117, 1940.
859
40. Ashton, N., Brini, A. and Smith, R.: Anatomical studies of the trabecular meshwork of the normal human eye. Brit. J. Ophth, 40:257, 1956. 41. Heine, L.: The anatomy of the accommodation of the eye. Arch. f. Ophth., 4 9 : 1 , 1899. 42. Flocks, M., and Zweng, H. C : Studies on the mode of action of pilocarpine on aqueous flow. Am. J. Ophth., 44:380, 1957. 43. Nonnenmacher, H . : Experiences in the treatment of infantile glaucoma with atropine. Klin. Monatsbl. f. Augenh., 121:533, 1952.
LYMPHOSARCOMA RALPH
S.
OF LACRIMAL AND
HAMILTON,
M.D.,
Philadelphia, Lacrimal gland enlargements may have a v a r i e t y of e t i o l o g i e s . A n i n f r e q u e n t c a u s e of s u c h a f i n d i n g is l y m p h o s a r c o m a , as d e m o n s t r a t e d in t h e f o l l o w i n g c a s e . CASE
REPORT
C. S., a 70-year-old white woman, was admitted to Wills Eye Hospital on July 12, 1957, with a chief complaint of swellings in both upper lids and of a swelling at the angle of the right mandi ble. Her present illness dated back to one year previously, at which time she first noted a small mass in the left upper eyelid. This gradually be came larger and she consulted an ophthalmologist in her home town. X-ray studies of the orbit were negative and the tumor was excised. Pathologic studies failed to establish a diagnosis. Soon after this she developed a swelling in the right upper eyelid and, three weeks prior to her admission to the Wills Eye Hospital, she developed a swelling at the angle of the right mandible. She had been followed by her ophthalmologist for eight months prior to her referral to Wills Eye Hospital for further studies and treatment. Her past medical history appeared unrelated to her present complaints although it included a ruptured appendix in 1914, herpes zoster, acute hepatitis due to infection, and hypoproteinemia in 1951. In 1951 she had been hospitalized and had a thorough work-up of her gastrointestinal and cardiovascular systems which failed to reveal any malignancy. General physical examination showed an elderly female, well-developed and somewhat obese. Blood pressure was 150/66 mm. H g ; pulse 64. Examina tion of head and neck was significant and will be described in more detail with the eye examination. Lung fields were clear. Cardiac studies revealed P M I in fifth intercostal space in left midclavicular line with a Grade 1 aortic systolic murmur. The abdomen revealed no palpable mass. Liver and spleen were not enlarged. There were no palpable lymph nodes in axilla, supraclavicular, or inguinal regions. Breasts showed no masses. Extremities had no palpable or visible edema. * From the Wills Eye Hospital.
AND
IRVING
SALIVARY H.
LEOPOLD,
GLANDS* M.D.
Pennsylvania Eye examination. Visual acuity was: O.D., 6/30 and 6/15, with correction; O.S., 6/60 and 6/21 with correction. Intraocular pressure was: O.D., 20 mm. H g ; O.S., 20 mm. H g (Schi^ltz). External examination. There was no enophthalmos or exophthalmos. Lids revealed large tumor masses 3.0 by 2.0 cm. in the right upper lid over the lacrimal gland area and 2.5 by 1.75 cm. in the left upper lid over the lacrimal gland area. These masses were firm, nonmovable, not fixed to skin, and were painless. The tumor masses were so large that the patient had almost complete ptosis of both lids. In addition to the tumor masses was a large, firm, disc-shaped mass 4.0 by 5.0 cm. in the right periauricular and parotid region. This mass was firm, nonmovable, nontender, and not fixed to skin. Also there was a large submaxillary gland on either side measuring 2.0 by 3.0 cm. Cilia and the lacrimal drainage apparatus were normal. The conjunctiva showed some chemosis. The corneas were normal. Anterior chambers were of normal depth. Irises were normal. Pupils were round, regular, and equal, and responded to light directly and consensually. Muscle balance revealed marked limitation of lateral rotations in each eye, neither eye being able to rotate to the midline from its more or less fixed nasal displacement. The patient was unable to look up and out at all; rotations up nasally and down nasally were not restricted. She had 15 degrees of esotropia and complained of diplopia when both lids were manually elevated. She had a complete ptosis on the right and almost complete ptosis on the left. Fundus examination revealed the media to be clear, disc of good color, margins were distinct, cupping was physiologic, vessels revealed normal light reflex, there were no hemorrhages or exudates, macular reflexes were good, and there were no stress signs in the f undi. Impressions at this time were: (1) Bilateral lacrimal gland tumors with extensive preauricular lymphadenopathy on right or parotid gland swell ing on right, and bilateral submaxillary adenopathy. It was felt that this could be considered a Mikulicz's syndrome, probably based on sarcoidosis or some nonspecific granuloma. (2) The second consideration was that this could be a primary or metastatic malignancy of lacrimal glands.
860
R A L P H S. H A M I L T O N A N D I R V I N G H. L E O P O L D
Laboratory studies. Complete blood count re vealed hemoglobin of 12.5 gm. or 81 percent; red blood count 4,810,000; white blood count 4,950; differential count was segmented, neutrophils 62 percent, lymphocytes 32 percent, monocytes one percent, eosinophiles two percent, and bands three percent. Urinalysis. Albumin: negative; sugar: negative; specific gravity: 1.022; acid reaction: microscopic examination revealed two or three white blood cells, a rare red blood cell, and loaded with mucous. Blood sugar was 90 mg.; sedimenta tion rate, 8.0 mm. in 60 min.; Lee and White coagulation time: 10 min., 30 sec, bleeding time two min., 15 sec. Total serum protein: 5.1, albumin 3.5, globulin 1.6; test for Bence Jones protein, in urine negative. X-ray studies revealed calcification of internal carotid arteries, otherwise normal skull, orbits, paranasal sinuses, and optic foramina. The chest X-ray film showed an arteriosclerotic aorta and left ventricular enlargement. There was no widening of hilar or mediastinal nodes. X-ray films of hands and feet showed osteoparetic changes but nothing to suggest sarcoidosis. On July 16, 1957, on the tentative diagnosis of nonspecific granuloma, the patient was started on 20 mg. of A C T H in 500 cc. of five-percent glucose in distilled water for three days and Chloromycetin, 500 mg. every six hours. On July 20, 1957, she was put on A C T H , 40 mg. in the form of Acthar Gel intramuscularly twice a day and potassium chloride 1.0 gm. daily. She was also started on 50 mg. of prednisolone twice a day. Two days after instigation of A C T H therapy all of the masses began to resolve. Within four days there was complete resolution of both submaxillary glands and of the large mass in the right parotid region. The masses over both lacrimal glands were diminishing in size. After one week of steroid therapy, the patient could open both lids to the level of uncovering one half of each pupil. A biopsy was taken to establish the diagnosis. This was done on July 23, 1957, uneventfully under local anesthesia from the site of right lacrimal gland. This patient was discharged from the hospital on August 2, 1957, on 5.0 mg. of prednisolone four times a day and 1.0 gm. of potassium chloride daily. At the time of her discharge the masses over each lacrimal gland area were approximately one third their original size. The patient no longer had diplopia except on lateral gaze, indicating a resolu tion of the orbital extension of these masses. The ptosis was disappearing and the patient subjectively felt well. Readmission. The patient developed a fever on August 12, 1957, and sores in her mouth. She was started on mycostatin at home at this time since it seemed that she might have developed moniliasis or one of the fungus infections with the steroid treatment. She did not improve and was readmitted on August 15, 1957. At this time she complained of fever and malaise. General physical examination revealed a temperature of 102°F. orally, rales in the right lower lung fields, and percussion dullness
over this area. X-ray studies of the chest now re vealed a confluent bronchopneumonia at the base of the right lung. Routine red blood count, differ ential, and urinalysis were all normal. First and second strength Mantoux tests were again negative. Treatment consisted of prednisolone, 10 mg., three times a day, Mysteclin, 250 mg., four times a day, and Mycostatin, two tablets (1,000,000 units), four times a day. Microscopic studies of the biopsy specimen estab lished the diagnosis of lymphosarcoma. In view of this microscopic diagnosis, the patient was given 6.0 mg. of nitrogen mustard intravenously daily for four days, starting on August 21st. On August 20, 1957, Mysteclin and Mycostatin were discon tinued. By August 26th, X-ray studies of the chest revealed almost complete resolution of pneumonia and the patient was discharged home on August 27, 1957, on prednisolone, 5.0 mg. three times daily for one week, then twice a day and K Q , 1.0 gm., three times daily. The lacrimal masses were almost completely resolved in both eyes. The right eye lid was in normal position, the left was still slightly ptotic. On December 14, 1957, the patient revealed no signs of any enlargement of lacrimal glands, parot id, or cervical lymph nodes. She did have a slight ptosis of left upper lid, but no ptosis of right upper lid. She was in good spirits and apparently in good health. On July 28, 1958, the patient's family re ported her to be in good health with no evidence of recurrence of the tumor masses in either lid, preauricular, or cervical regions. DISCUSSION
L a c r i m a l t u m o r s h a v e b e e n s a i d to c o m p o s e f o u r t o IS p e r c e n t of all o r b i t a l t u m o r s b y F o r r e s t . 2 D u k e - E l d e r 1 s t a t e s t h a t five p e r cent of t u m o r s of t h e l a c r i m a l g l a n d a r e d e rived
from
lymphoid
tissue.
Forrest2
has
g i v e n u s a r a t h e r c o m p l e t e p a t h o l o g i c classi fication of all l a c r i m a l g l a n d t u m o r s . H e con s i d e r s f o u r m a i n g r o u p s as f o l l o w s :
A. Epitheloid lacrimal gland tumors 1. Benign or mixed tumors of lacrimal gland. 2. Malignant lacrimal gland tumors a. Adenoid cystic type b. Adenocarcinoma c. Squamous cell carcinoma d. Undifferentiated carcinoma e. Malignant mixed tumors B. Inflammatory disease 1. Sarcoid 2. Nonspecific granulomas C. Localized evidence of a lymphoma (into which class this case may be classified.)
LYMPHOSARCOMA OF LACRIMAL GLAND D. Neoplasms or inflammation unrelated to the lacrimal gland as metastatic lesions The treatment of the above lacrimal gland involvments may be summarized as follows: Group A responds only to complete and adequate removal of all the tumor and struc tures, including the bony lacrimal fossa, which are invaded by the tumor. Forrest 2 be lieves that the adenoid cystic type of carci noma under the malignant lacrimal gland tumors is radiosensitive. Group B usually responds best to systemic A C T H and steroid treatment. It is into this group that our patient was originally believed to belong. This was the rationale for the treatment with A C T H (adrenocorticotropic hormone). Attention is again called to the dramatic resolution of the tumors on this treatment. Group C is best treated with radiation therapy. Biopsy must be done for diagnostic purposes. Group D responds best to treatment di rected at the primary site of the lesion. There has been considerable overlapping and confusion as regards the classification of the lypmphomas involving the lacrimal glands. Duke-Elder 2 classifies them as fol lows: ( 1 ) Lymphoma, ( 2 ) leukemia, ( 3 ) lymphosarcoma, ( 4 ) reticulum-cell sarcoma, ( 5 ) giant follicular lymphoblastoma, ( 6 ) Hodgkin's disease. It is well known that ocular manifestations of a lymphomatous disorder may be mani festing signs of a generalized spread of the
861
disease or a prelude to development of a systemic lymphomatous disorder. These cases certainly must be very carefully followed over many years. McGavic 5 has stated that the lymphomatoid diseases are an ever-chang ing process that require repeated and care ful biopsy and blood studies. Survival rates of the lymphomatous dis orders have been pointed out by Sugarbaker and Craver 8 to be influenced by: ( 1 ) inherent qualities for growth and dissemination; (2) extent of dosage when first treated; ( 3 ) localization of primary lesion, that is, out come is much more favorable when primary site is in the head. Perhaps this is because tumors in this region present themselves to the patients early and these patients seek medical attention promptly. ( 4 ) Complicating leukemia increases mortality. ( 5 ) Age of on set, that is, the younger the patient the shorter the survival rate, the older the pa tient, the longer the survival rate. SUMMARY
In summary, a case of lymphosarcoma pre senting itself as massive bilateral lacrimal and salivary gland swellings has been de scribed. The dramatic resolution of the masses has been pointed out. While this pa tient did receive nitrogen mustard later in the course of her illness, the masses had all re solved and it is felt that the steroids and adrenocortinotropic hormones were responsi ble for this. A classification of lacrimal gland tumors has been presented and treatment has been described. 1601 Spring Garden Street (30).
REFERENCES
1. Duke-Elder, W. S.: Textbook of Ophthalmology. St. Louis, Mosby, 1952 and 1955, pp. 5265-5270. 2. Forrest, A. W.: Epithelial lacrimal gland tumors: Pathology as guide to prognosis. Tr. Am. Acad. Ophth., 58:848-865 (Nov.-Dec.) 1954. 3. Gruber, E.: Sarcoidosis of lacrimal glands associated with Sj^gren's syndrome. Arch. Ophth., 56: 42-47, 1956. 4. Jones, I. S., and Pfeifer, R. F.: Lacrimal gland tumors: Roentgenographic diagnosis. Tr. Am. Acad. Ophth., 58:841-847 (Nov.-Dec.) 1954. 5. McGavic, J. S.: Lymphomatoid diseases involving the eye and its adnexa. Arch. Ophth., 30:179-193, 1943. 6. Reese, A. B.: Tumors of the Eye. New York, Hoeber, 1951, pp. 464-469. 7. : The treatment of expanding lesions of the orbit with particular regard to those arising in the lacrimal gland. Am. J. Ophth., 41:3, 1956. 8. Sugarbaker, E. D., and Craver, L. F.: Lymphosarcoma: A study of 196 cases with biopsy J A M A 115:17-23 and 112-117, 1940.