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Reticulum-Cell Sarcoma of the Conjunctiva*
NOTES, C A S E S , I N S T R U M E N T S
929
RETICULUM-CELL SARCOMA OF T H E CONJUNCTIVA* ROBERT DYAR,
M.D.
AND
MERRILL GRAYSON,
Indianapolis,
M.D.
Indiana
Lymphosarcoma involving the eyes is un common but not rare. Sugarbaker and Craver in a series of 196 cases of lympho ma found conjunctival involvement in 0.5% of their cases with generalized lymphosarcomatosis. Heath, in reviewing 1,600 cases of lymphoma, found 67 ocular tumors; 30 of these involved the conjunctiva; however, only three were reticulum-cell sarcomas. 1
2
McGavic reported on 21 patients with lymphomatous tumors involving the e y e ; 17 were primary tumors in the region of the eye and four had generalized lymphosarco ma before the ocular tumor was found. Reese* collected 171 cases of malignant lymphoma involving the region of the e y e ; 31 of these tumors involved the conjunctiva. He states that, since lymphoid tissue about the eye is noted in the subconjunctiva and lacrimal gland, these sites should be most commonly involved in lymphomatous dis ease. He further states that lymphoid tu mors under the conjunctiva present a typical appearance "unlike any other ocular lesion." The tumor mass is salmon-colored; the overlying conjunctiva is smooth; the mass is sharply demarcated, and the tumor usually follows the contour of the involved area, being round or oval on the bulbar conjuncti va and elongated in the fornices. 3
Fig. 1 (Dyar and Grayson). Circumscribed, raised, firm, nontender, violaceous to salmon-color nodules on face. when she consulted her family physician because of pharyngitis. A t that time, she had several fleshy nodules on the skin of her forehead which had been present for a few days. Approximately one week later, in a period of 4 8 hours, nodules similar to those on her forehead appeared throughout the skin of her entire body excluding the palms and soles. She returned to her physician because of weakness and anorexia and was admitted to a local hospital because of anemia. A bone-marrow aspi rate from the right ilium contained cells suggestive of lymphoma. The patient was then referred to the Indiana University Medical Center. Physical examination on admission revealed no abnormalities except pallor and numerous firm, raised, nontender, circumscribed, violaceous to sal mon-colored nodules throughout the skin of the en tire body (figs. 1 and 2 ) . These nodules ranged from 5.0 to 20 mm in diameter. There was no lymphadenopathy and no abdominal viscera were pal pable. One small, round nodule was noted beneath the bulbar conjunctiva nasal to the cornea of the left eye at the time of admission. Three days after admission, the corrected visual acuity was: right eye, 2 0 / 5 0 ; left eye, 20/20. A flat, salmon-colored nodule was present on the left upper lid margin. Several confluent subconjunctival
The case reported in this paper is one of reticulum-cell sarcoma involving the con junctiva of both eyes. CASE REPORT
A 52-year-old white woman was referred to the Indiana University Medical Center with a diag nosis of lymphosarcoma and aplastic anemia. She had been well until five weeks prior to admission * From the Department of Ophthalmology, Indi ana University School of Medicine. This study was supported in part by the Indiana Lions Eye Bank.
Fig. 2 (Dyar and Grayson). Nodules on patient's arm.
930
NOTES, C A S E S , I N S T R U M E N T S
Fig. 3 (Dyar and Grayson). Multiple circum scribed, salmon-patch, subconjunctival nodules. nodules were present nasal and superior to the right cornea (fig. 3 ) . A single nodule was present under the bulbar conjunctiva nasal to the cornea of the left eye (fig. 4 ) . The remainder of the ocular examination was within normal limits except for the fundus of the left eye. A small retinal exudate was present temporal to the disc and superficial ret inal hemorrhages were scattered in the region of the posterior pole (fig. 5 ) . Admitting laboratory data revealed a normocytic anemia and depressed white count with 75% lym phocytes. No tumor cells were seen in the first few smears of the peripheral blood. The admitting chest X-ray film was considered within normal lim its. Microscopic pathology of one of the cutaneous nodules and one of the subconjunctival nodules from the left eye showed dense infiltration of mon omorphous cells having large, irregular hyperchromatic nuclei with two or three nucleoli and scant cytoplasm. Numerous mitotic figures were seen. These cells were considered to be malignant reticulum cells (fig. 6 ) . Similar cells were seen in the bone-marrow aspirate. The patient remained asymptomatic for approxi mately two weeks. At that time, several epitrochlear
Fig. 4 (Dyar and Grayson). Larger subconjunc tival lesion from which biopsy was taken.
Fig. 5 (Dyar and Grayson). Scattered superficial hemorrhages and exudates in posterior pole. lymph nodes were palpated and the liver became palpable and tender. A course of vinblastine sulfate, (2.5 mg per day orally) an antineoplastic drug, was begun. On the 21st day after admission the cutaneous nodules became hemorrhagic and she developed severe epistaxis. A massive gastrointesti nal hemorrhage followed and on the 24th day after admission the patient died. Pertinent provisional anatomic diagnosis at post mortem examination was: ( 1 ) reticulum-cell sarco ma involving the skin, the tracheobronchial, peripancreatic, and periaortic lymph nodes, and the stomach; (2) hemorrhages of all abdominal and thoracic viscera.
Fig. 6 (Dyar and Grayson). Dense infiltration of monomorphous cells having large, irregular hyperchromatic nuclei. These cells are malignant reticu lum cells. Bone marrow exhibited similar cells.
NOTES. C A S E S , I N S T R U M E N T S
931
SUMMARY ACKNOWLEDGMENT
Reticulum-cell sarcoma is a rare entity. Ocular involvement is noted in a case of generalized reticulum-cell sarcoma.
1100 West Michigan
Street
express our appreciation to Frank Vellios, M . D , and Michael H. Lashmet, M . D , for interpretation of the microscopic sections. W
e
w i s h
t
0
t h e i r
(7). REFERENCES
1. Sugarbaker, E. D , and Craver, L. F . : Lymphosarcoma: A study of 196 cases with biopsy. J. Am. Med. A , 1 1 5 : 1 7 , 1940. 2. Heath, P . : Ocular lymphomas. Am. J . Ophth, 32:1213, 1949. 3. McGavic, J . S . : Lymphomatoid disease involving the eye and its adnexae. Arch. Ophth, 30:179, 1943. 4. Reese, A. B.: Tumors of the Eye. New York, Harper & Row, 1963, ed. 2.
A CORNEAL
PRESSOR
BENJAMIN FRIEDMAN,
New
M.D.
York
This instrument* is intended for the assistant's use. He employs it to press against the cornea during the insertion of sutures following corneal section in those cases in which vitreous pressure pushes the lens forward and prolapses the iris through
joint. The assistant may easily position his hand so that he does not encroach upon the surgeon's field. 1 East 105th Street (29).
INVERSE
BELL'S
PHENOMENON*
A s A PROTECTIVE M E C H A N I S M
J . S. GUPTA, F.R.C.S., M.B.B.S.
ARIN CHATTERJEE, AND
K . K U M A R , M.B.B.S. Chandigarh,
Fig. 1 (Friedman). The corneal pressor.
the wound. By means of the corneal pressor, the lens-iris diaphragm is pushed backward so that suture placement can be effected without haste or difficulty. The instrument consists of a handle simi lar to that of a muscle hook; the neck is an gled at 45 degrees and terminates as a cap which has the same curvature as the cornea and covers about two thirds of the corneal surface. Two small holes are drilled through the cap to discourage suction. The cap is connected to the neck by a ball-bearing * Made by the Matalene Surgical Instrument Company, 141 East 44th Street, New York.
India
The upward movement of the eyes on normal or frustrated closure of the eyelids, popularly known as Bell's phenomenon, is perhaps a protective mechanism brought about reflexly through the connections of the 3rd and 7th nerve nuclei (fig. 1 ) . As described by Duke-Elder, Bell's phe nomenon is present in normal blinking, vol untary closure, sleep, comatosed conditions and a large number of cases of 7th nerve palsies. In supranuclear lesions affecting the fibers reaching the 3rd nerve nucleus, where voluntary upward gaze is not possible, Bell's phenomenon is retained, as can be demon1
* From the Department of Ophthalmology, Insti tute of Postgraduate Medical Education and Re search.
929
RETICULUM-CELL SARCOMA OF T H E CONJUNCTIVA* ROBERT DYAR,
M.D.
AND
MERRILL GRAYSON,
Indianapolis,
M.D.
Indiana
Lymphosarcoma involving the eyes is un common but not rare. Sugarbaker and Craver in a series of 196 cases of lympho ma found conjunctival involvement in 0.5% of their cases with generalized lymphosarcomatosis. Heath, in reviewing 1,600 cases of lymphoma, found 67 ocular tumors; 30 of these involved the conjunctiva; however, only three were reticulum-cell sarcomas. 1
2
McGavic reported on 21 patients with lymphomatous tumors involving the e y e ; 17 were primary tumors in the region of the eye and four had generalized lymphosarco ma before the ocular tumor was found. Reese* collected 171 cases of malignant lymphoma involving the region of the e y e ; 31 of these tumors involved the conjunctiva. He states that, since lymphoid tissue about the eye is noted in the subconjunctiva and lacrimal gland, these sites should be most commonly involved in lymphomatous dis ease. He further states that lymphoid tu mors under the conjunctiva present a typical appearance "unlike any other ocular lesion." The tumor mass is salmon-colored; the overlying conjunctiva is smooth; the mass is sharply demarcated, and the tumor usually follows the contour of the involved area, being round or oval on the bulbar conjuncti va and elongated in the fornices. 3
Fig. 1 (Dyar and Grayson). Circumscribed, raised, firm, nontender, violaceous to salmon-color nodules on face. when she consulted her family physician because of pharyngitis. A t that time, she had several fleshy nodules on the skin of her forehead which had been present for a few days. Approximately one week later, in a period of 4 8 hours, nodules similar to those on her forehead appeared throughout the skin of her entire body excluding the palms and soles. She returned to her physician because of weakness and anorexia and was admitted to a local hospital because of anemia. A bone-marrow aspi rate from the right ilium contained cells suggestive of lymphoma. The patient was then referred to the Indiana University Medical Center. Physical examination on admission revealed no abnormalities except pallor and numerous firm, raised, nontender, circumscribed, violaceous to sal mon-colored nodules throughout the skin of the en tire body (figs. 1 and 2 ) . These nodules ranged from 5.0 to 20 mm in diameter. There was no lymphadenopathy and no abdominal viscera were pal pable. One small, round nodule was noted beneath the bulbar conjunctiva nasal to the cornea of the left eye at the time of admission. Three days after admission, the corrected visual acuity was: right eye, 2 0 / 5 0 ; left eye, 20/20. A flat, salmon-colored nodule was present on the left upper lid margin. Several confluent subconjunctival
The case reported in this paper is one of reticulum-cell sarcoma involving the con junctiva of both eyes. CASE REPORT
A 52-year-old white woman was referred to the Indiana University Medical Center with a diag nosis of lymphosarcoma and aplastic anemia. She had been well until five weeks prior to admission * From the Department of Ophthalmology, Indi ana University School of Medicine. This study was supported in part by the Indiana Lions Eye Bank.
Fig. 2 (Dyar and Grayson). Nodules on patient's arm.
930
NOTES, C A S E S , I N S T R U M E N T S
Fig. 3 (Dyar and Grayson). Multiple circum scribed, salmon-patch, subconjunctival nodules. nodules were present nasal and superior to the right cornea (fig. 3 ) . A single nodule was present under the bulbar conjunctiva nasal to the cornea of the left eye (fig. 4 ) . The remainder of the ocular examination was within normal limits except for the fundus of the left eye. A small retinal exudate was present temporal to the disc and superficial ret inal hemorrhages were scattered in the region of the posterior pole (fig. 5 ) . Admitting laboratory data revealed a normocytic anemia and depressed white count with 75% lym phocytes. No tumor cells were seen in the first few smears of the peripheral blood. The admitting chest X-ray film was considered within normal lim its. Microscopic pathology of one of the cutaneous nodules and one of the subconjunctival nodules from the left eye showed dense infiltration of mon omorphous cells having large, irregular hyperchromatic nuclei with two or three nucleoli and scant cytoplasm. Numerous mitotic figures were seen. These cells were considered to be malignant reticulum cells (fig. 6 ) . Similar cells were seen in the bone-marrow aspirate. The patient remained asymptomatic for approxi mately two weeks. At that time, several epitrochlear
Fig. 4 (Dyar and Grayson). Larger subconjunc tival lesion from which biopsy was taken.
Fig. 5 (Dyar and Grayson). Scattered superficial hemorrhages and exudates in posterior pole. lymph nodes were palpated and the liver became palpable and tender. A course of vinblastine sulfate, (2.5 mg per day orally) an antineoplastic drug, was begun. On the 21st day after admission the cutaneous nodules became hemorrhagic and she developed severe epistaxis. A massive gastrointesti nal hemorrhage followed and on the 24th day after admission the patient died. Pertinent provisional anatomic diagnosis at post mortem examination was: ( 1 ) reticulum-cell sarco ma involving the skin, the tracheobronchial, peripancreatic, and periaortic lymph nodes, and the stomach; (2) hemorrhages of all abdominal and thoracic viscera.
Fig. 6 (Dyar and Grayson). Dense infiltration of monomorphous cells having large, irregular hyperchromatic nuclei. These cells are malignant reticu lum cells. Bone marrow exhibited similar cells.
NOTES. C A S E S , I N S T R U M E N T S
931
SUMMARY ACKNOWLEDGMENT
Reticulum-cell sarcoma is a rare entity. Ocular involvement is noted in a case of generalized reticulum-cell sarcoma.
1100 West Michigan
Street
express our appreciation to Frank Vellios, M . D , and Michael H. Lashmet, M . D , for interpretation of the microscopic sections. W
e
w i s h
t
0
t h e i r
(7). REFERENCES
1. Sugarbaker, E. D , and Craver, L. F . : Lymphosarcoma: A study of 196 cases with biopsy. J. Am. Med. A , 1 1 5 : 1 7 , 1940. 2. Heath, P . : Ocular lymphomas. Am. J . Ophth, 32:1213, 1949. 3. McGavic, J . S . : Lymphomatoid disease involving the eye and its adnexae. Arch. Ophth, 30:179, 1943. 4. Reese, A. B.: Tumors of the Eye. New York, Harper & Row, 1963, ed. 2.
A CORNEAL
PRESSOR
BENJAMIN FRIEDMAN,
New
M.D.
York
This instrument* is intended for the assistant's use. He employs it to press against the cornea during the insertion of sutures following corneal section in those cases in which vitreous pressure pushes the lens forward and prolapses the iris through
joint. The assistant may easily position his hand so that he does not encroach upon the surgeon's field. 1 East 105th Street (29).
INVERSE
BELL'S
PHENOMENON*
A s A PROTECTIVE M E C H A N I S M
J . S. GUPTA, F.R.C.S., M.B.B.S.
ARIN CHATTERJEE, AND
K . K U M A R , M.B.B.S. Chandigarh,
Fig. 1 (Friedman). The corneal pressor.
the wound. By means of the corneal pressor, the lens-iris diaphragm is pushed backward so that suture placement can be effected without haste or difficulty. The instrument consists of a handle simi lar to that of a muscle hook; the neck is an gled at 45 degrees and terminates as a cap which has the same curvature as the cornea and covers about two thirds of the corneal surface. Two small holes are drilled through the cap to discourage suction. The cap is connected to the neck by a ball-bearing * Made by the Matalene Surgical Instrument Company, 141 East 44th Street, New York.
India
The upward movement of the eyes on normal or frustrated closure of the eyelids, popularly known as Bell's phenomenon, is perhaps a protective mechanism brought about reflexly through the connections of the 3rd and 7th nerve nuclei (fig. 1 ) . As described by Duke-Elder, Bell's phe nomenon is present in normal blinking, vol untary closure, sleep, comatosed conditions and a large number of cases of 7th nerve palsies. In supranuclear lesions affecting the fibers reaching the 3rd nerve nucleus, where voluntary upward gaze is not possible, Bell's phenomenon is retained, as can be demon1
* From the Department of Ophthalmology, Insti tute of Postgraduate Medical Education and Re search.