Although Burkitt's lymphoma is endemic to certain regions of Africa,1 attention has in the last few years been focused on tumors occurring in the United States with striking histologica! and histoehemical similarities to the malignancy first described by Burkitt.; We describe herein a case of bilateral carcinomatous optic neuropathy, in a young North American Caucasian male, secondary to optic nerve invasion by a malignant lymphoma histologically and hi sto chemically resembling Burkitt's lymphoma.
Report of a Case
This ten-year-old male was initially evaluated at the Childrens Hospital Medical Center for a 14-cm left upper quadrant mass discovered on physical examination following a one-month history of abdominal pain and hemetemesis. At that time, chest x-ray, bone survey, brain scan and cerebral spinal fluid analysis were normal. Upper gastrointestinal series showed widening of the duodenal loop and effacement of the duodenal mucosa. Intravenous pyelography showed delayed filling and lateral displacement of the left kidney. At the time of surgery, a large mass replacing the third and fourth portion of the duodenum with Massive extension into the retroperiotenal space and root of the messentary was found. The resected tumor was composed of email, round cells with hyperchromic nuclei (Figure I). Numerous mitotic figures were present. Large, clear reticulum cells were scattered throughout the tumor. The tumorwas classified as a malignant lymphoma (Burkitt's pattern). Postoperative recovery was uneventful and the patient was begun on a course of chemotherapy consisting of vincristine, cytoxan and prednisone and radiation therapy to the abdomen.
Three months later, he was readmitted for evaluation of headaches, neck pain and numbness of the left arm. Skull x-rays, cervical spine films, brain scan, bone survey and bone marrow aspirates were normal; however, cerebrospinal fluid analysis showed atypical mononuclear cells. Blood hemograms showed a leukocytosis of 26,100 celle/mm.' During the ensuing week, both optic disc margins were reported as showing progressive blurring and at this time ophthalmic consultation was requested.
Vision was no light perception in each eye. The pupils were mid-dilated and did not react to light. The patient appeared to gaze straight ahead. Anterior segment examination was normal in each eye and there was no prop tosig. Indirect ophthalmoscopy revealed striking papilledema in both eyes (Figure 2). The optic discs were swollen to greater than six diopters with splinter hemorrhages, venous tortuousity and peripapillary retinal edema. Hemorrhages were apparent to the midequatorial zone, clinically resembling central retinal venous obstruction. Contact B-scan ultrason ography showed marked elevation of the optic disc without evidence of an orbital lesion.
Central nervous system irradiation was begun; however, the patient's hospital course was one of rapid deterioration and within two weeks the patient died. An autopsy was performed and showed metaetatic tumor spread with meningeal and peri vascular involvement of the brain and cortical atrophy. There was extensive infiltration of the cervical spinal cord and spinal nerve roots (Figure 3). Permission for ocular examination was refused; however, a section of optic nerve anterior to the chi asm was obtained for examination. In the sections reviewed, the tumor infiltrated the meningea! sheaths surrounding the entire optic nerve and invaded into the nerve tissue through the septal channels (Figure 4).
FIGURE i; Intestinal tumor composed of smali, round cells with hyperchromic nuclei and nucleoli. (Hematoxylin-eosin XlOI.
FIG URE 2: Fundus photograph of the left optic disc showing marked papilledema. A similar appearance was noted in the right eye.
FIGURE 3: Invasion of spinal cord nerve roots by tumor, iffematoxylin-easin X40).
FIGURE 4: Section of optic nerve with invasion into septal areas by tumor cells. (Hematoxylin-eosin KIGO).
Visual loss is frequent in cases of meningeal carcinomatosis and may occasionally be the presenting sign.'"* In the case reported here, rapid visual loes with progression to blindness occurred bilaterally over a oneweek period of time. Several mechanisms by which meningeal carcinomatosi s may produce blindness have been proposed including: direct tumor involvement of the optic nerve/ chronic papilledema/ and meningeal cuffing of the optic nerve by tumor cells/ " while others have proposed a vascular basis. Loss of vision in three patients secondary to retinal photoreceptor degeneration aa a remote effect of cancer was reported by Sawyer and associated A combined mechanism may have been present in the case described here since meningeal tumor cuffing and invasion into the septal areas of the optic nerve were present histologically and, clinically, the fundus picture resembled vascular obstructive disease.
The predominance of involvement of abdominal and pelvic sites rather than the jaw and an older median age clinically has partially distinguished the American patient from those reported in endemic regions of Africa with this disorder.'" A case resembling Burkitt's lymphoma was described in America as early as 1928 by Brown and O'Keefe." Several reports h a ve foil o wed since that time. Arsenau and associates1"'1" reviewed the clinical features relating to survival and the pathological correlates of 30 cases of American Burkitt's lymphoma. In their series, the median age at presentation was U years and the abdomen was the most frequent site of involvement (23 caeee), as was true for the case reported here. Lymph node involvement was present in 25 of 30 cases, the cranial nerves were involved in 18% of cases, whereas malignant cella were present in the cerebrospinal fluid in two cases. Involvement of the optic nerve by tumor was found at autopsy in one case described by Cohen and co-workers."
Intraocular involvement is said to be rare in this disease, although several histologically confirmed cases havebeen described. "'"Feman and associates"1 reported an additional case; however, the retinal findings may have represented leukemic in filtration. Since permission for ocular examination was not granted at the time of autopsy in our case, the findings described here relate only to the optic nerve distal to the globe.
C arci noma tous optic neuropathy should be considered by o pth al molo gists in cases of visual loss occurring in childhood.
The clinical and hi sto pathological features of a case of carcinomatous optic neuropathy secondary to a malignant lymphoma histologically resembling "Burkitt's tumor" occurring in an 11-yeai-old American male was described. Hi etologi cai examination of the optic nerve showed meningeal involvement by tumor cells with extension into the septa] spaces of the optic nerve.
Representative sections of the intestinal tumor were also reviewed by the Armed Forces Institute of Pathology (Dr. L.E. Zimmerman).
1. Wright DH: Burkitt'a lymphoma: A review of the pathology, immunology and possible etnologie factors. Ann Path 6:337, 1971.
2. Burkitt D: A sarcoma involving the jawa in Afincan children. Br J Surgery 46:218, 1958.
3. Walch FB, Hoyt WF: Clinical Neuro-ophthalmology, ed 3. Baltimore, Williams and Wilkins, 1969. p 627.
4. Fiacher-Williama M, Bosanquet FD, Daniel PM: Carcinomatosia of the meningee. A report of three cases. Brain 78:42, 1955.
5. Katz JL, Valsamia MP, Jampel RS: Ocular signa in diffuse carcinomatous meningitis. AmJOptltfialmol52:S8l, 1961.
6. Altrocchi PA, Reinhardt PH, Eckman PB: Blindneas and meningeal carchi o m atoáis. A re A Op ht halmol 88:508, 1962.
7. Su sac JO, Smith JL, Powell JO: Carcinomatous optic neuropathy. AmJ Ophihalmol 76:672, Iff73.
8. Appen RE, DeVenecia G, Selliken JH, et al: Meningeal carcinomatoBÌs with blindness. Am J Ophthaimal 86:661, 1978.
9. Sawyer RA, Selhuret JB, Zimmerman LE, et al: Blindneas caused by photoreceptor degeneration as a remote effect of cancer. Am J Ophthalmol 81:606, 1976.
10. Arseneau JC, Canellos GP, Banks PM, et al: American Burkitt's lymphoma: A clinicopathologic study of 30 cases. I. Clinical factors relating to prolonged survival. Am J Med 58:314, 1975.
11. Brown JB, O'Keefe CD: Sarcoma of the ovary with unusual oral metastasis. Ann Surg 87:467, 1928.
12. Banks PM, Arseneau JC, Granlnick HR, et al: American Burkitt'a lymphoma: A clinicopathological study of 30 casen- ?. Pathologic correlations. Am J Med 58:322, 1975.
13. Cohen MH, Bennett JM, Berard CW, et al: Burkitt's tumor in the United Statea. Cancer 27:1259, 1969.
14. Karp LA, Zimmerman LE, Payne T: Intraocular involvement in Burkitt's lymphoma. Arch Ophthalmol 85:295, 1971,
15. Di all o J, Baaaabi S: Tumeur de Burkitt oculaire sana atteinte oaseuse orbitaire. Bull Soc Med Arf Noire Lang France XViii:154, 1973.
16. Feman SS, Niwayma G, Hepler RS, et al: "Burkitt tumor": With intraocular involvement. Surv Ophihalmol 14:106, 1969.