headache and loss of vision in the left eye. The patient was well until. 10 days prior to admission when he started to experience limited and blurry vision in the ...
Child's Nerv Syst (1991) 7:172-174
NII]NS 9 Springer-Verlag 1991
Primary intracranial extradural Burkitt-type lymphoma A unique presentation with unilateral loss of vision in a child ismail H. Tekk~k 1, Kadir Tahta 1, Aykut Erbengi 1, Miinevver Biiyiikpamuk~u 3, ~evket Ruacan 2, and Meral Top~u 4 1 Department of Neurosurgery, 2 Department of Pathology, Hacettepe University School of Medicine, 3 Pediatric Oncology Unit, and 4 Pediatric Neurology Unit, Hacettepe University Institute of Child Health, Ankara, Turkey Received February 7, 1990
Abstract. A n unusual case o f p r i m a r y extranodal Burkitt's-type l y m p h o m a o f the frontal base d u r a and adjacent b o n e with intradural invasion is reported in a 5-year-old child w h o presented with unilateral loss o f vision in the left eye. Tissue diagnosis was o b t a i n e d t h r o u g h a c r a n i o t o m y and the patient received radiotherapy plus intravenous and intrathecal c h e m o t h e r a p y . The t u m o r regression and i m p r o v e m e n t in vision was welt d o c u m e n t e d by means o f C T and visual e v o k e d potential studies (VEP). A review o f the literature on this exceptionally rare t u m o r is presented.
Key words: Burkitt-type l y m p h o m a - Central nervous system - C o m p u t e d t o m o g r a p h y - E x t r a d u r a l - Extranodal - Visual evoked potentials
P r i m a r y m a l i g n a n t l y m p h o m a s o f the central nervous system (CNS) confined exclusively to the brain and its coverings are relatively u n c o m m o n , constituting 0.3-1.5 % o f all C N S t u m o r s [9, 17] and 0.7% o f all l y m p h o m a s [7]. These t u m o r s typically occur in older adults and are exceptionally rare in childhood. It is quite r e m a r k a b l e that they rarely extend b e y o n d the C N S to p r o d u c e systemic involvement [14]. B u r k i t t ' s l y m p h o m a is a morphologically, i m m u n o p h e n o t y p i c a l l y and cytogenetically distinct rare subtype o f n o n - H o d g k i n ' s l y m p h o m a s , exhibiting highgrade malignancy. P r i m a r y involvement o f the C N S with this type o f l y m p h o m a is very rare with only eight cases reported in the literature [5, 6, 9, 10, 13, 15], all with involvement o f the brain p a r e n c h y m a . Only one case with extradural intracranial localization has been reported to date [4]. O n the other hand, C N S involvement, also k n o w n as stage IV o f the disease, occurs in 4 1 . 4 - 4 6 % o f all generalized Burkitt's-type l y m p h o m a s [1, 16]. The case presented in this report was h a r b o r i n g an extranodal in-
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tracranial Burkitt's l y m p h o m a involving the frontal base d u r a and the adjacent bone, which also e n t r a p p e d the left optic nerve intradurally, but did n o t occur elsewhere in the body. The clinical diagnostic features are given and therapeutic m o d e s and o u t c o m e are discussed.
Case report A 5-year-old boy, a Caucasian from a city located at central Asia Minor, was hospitalized for the first time in July 1988 at Hacettepe University Hospital, Department of Neurosurgery, with a history of headache and loss of vision in the left eye. The patient was well until 10 days prior to admission when he started to experience limited and blurry vision in the left eye, which rapidly progressed to total loss in 2 days. He also complained of moderate frontal headache which was not associated with nausea or vomiting. The past medical history and family history were unremarkable. On admission, his systemic findings except CNS were normal, i.e., there was no splenohepatomegaly or palpable lymph nodes. The sense of smell was normal on both sides. Pupils were equal but there was a relative afferent pupillary defect on the left. The visual acuity in the right eye was 20/20 whereas the left eye was totally blind. Examination of the fundi revealed blurred temporal margins in the left eye. Other neurological findings were within normal limits. Hemogram, urinalysis and blood biochemistry values were all normal. The CT scans demonstrated an en-plaque parasellar mass measuring 53 by 30 mm and extending symmetrically to both lesser wings of the sphenoid bone (Fig. I a, b). Anterior clinoid processes were eroded and the sella turcica was partially invaded by the tumor. No calcification or necrosis was noted. The tumor as well as the adjacent brain tissue enhanced moderately and homogeneously. The optic nerves were intact within the orbit (Fig. 1c). Visual evoked potential (VEP) studies disclosed a normal P-100 wave on the right, but no response on the left (Fig. 2). On 18 July 1988, a bifrontal craniotomy was performed. Upon exploration, an extradural en-plaque tumor was found to bulge upwards to invade and penetrate through the dura just in front of the sella. The left optic nerve was partially embedded within this intradural extention. This grayish-white tissue also infiltrated to the bone. Frozen sections of the specimen were reported to be suggestive of a malignant tumor, either a rhabdomyosarcoma or lymphoma, so no major attempt was made to remove it totally. The dura involved was excised and then repaired with temporal muscle fascia. The postoperative course was uneventful.
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Fig. 1 a - c . Preoperative CT scans showing a hyperdense frontobasal mass, b enhancement of the adjacent brain tissue, and c intact optic nerves within the orbit Preoperotive
The definite histopathological diagnosis was a malignant Burkitt-type lymphoma (Fig. 3). The atypical lymphoid cells were arranged in a "starry sky" pattern. The bony structures were also invaded. The tumor cells demonstrated intense cytoplasmic pyroninophilia with methyl-green pyronine stain. Investigations for systemic involvement were started on the 4th postoperative day. Radiological examination of the chest was normal. The bone marrow aspirate was interpreted as normal, while ultrasonography and CT of the abdomen showed no enlargement of abdominal lymph nodes. CSF cytology was found twice to be negative. A combined chemotherapy regimen was started on the 7th postoperative day, consisting of: intrathecal methotrexate, 15 mg/m 2 (milligrams per square meter body surface); cytosine arabinoside, 60 mg/m2; prednisolone, 30 mg/m 2, plus intravenous cyclophosphamide, 1200 rag/ mZ; vincristine, 1.4mg/m2; adriamycin, 45mg/m2; and prednisolone 40 mg/sq 2. On postoperative day 11 craniospinal irradiation was begun. His vision was better by the 15th postoperative day, i.e., he was able to count fingers from 1 m, although vision was limited to the lower temporal quadrant. This improvement was verified by VEPs done on the 18th day, showing an abnormal but present P-J00 response in the left (Fig. 2). Craniospinal radiation with steroid cover was completed in 27 days with a total dose of 35 Gy, using a conventional 6~ source. The chemotherapy planned was subsequently administered. At the first follow-up examination 2 months later, his vision was no different except for the fact that the fundus on the left was atrophic. A C T scan showed shrinkage of the tumor. A maintenance chemotherapy program was administered for 12 months postoperatively. The child is now well 18 months later.
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Fig. 2. Pre- and postoperative VEPs of the left eye demonstrating the reappearance of P-100 response
Discussion Since the first d e s c r i p t i o n o f a distinctive p a t t e r n o f l y m p h o m a b y B u r k i t t in 1958 [2], the n e o p l a s m n o w b e a r ing his n a m e h a s been t h o r o u g h l y studied, p r o v i d i n g a n u m b e r o f a n s w e r s to the early q u e s t i o n s a b o u t its etiology, g e o g r a p h i c a l d i s t r i b u t i o n o r p r i m a r y site. A l t h o u g h the b r a i n is an o r g a n n o r m a l l y d e v o i d o f l y m p h o i d tissue, the b r a i n a n d its c o v e r i n g s can well be the origin o f a l y m p h o i d t u m o r , w h i c h is o f B-cell origin in the m a j o r i t y o f cases, like the B u r k i t t - t y p e [6, 8, 14].
Fig. 3. Photomicrograph illustrating a "starry sky" pattern, x 150
T h e f o r m s o f B u r k i t t t u m o r t h a t o r i g i n a t e f r o m the a b d o m e n on j a w are m o s t c o m m o n in the late first a n d e a r l y s e c o n d d e c a d e , b u t t h e y c a n o c c u r in adults, too. It is quite i n t e r e s t i n g t h a t all b u t o n e o f the B u r k i t t - t y p e
174
primary CNS lymphomas reported were in adults. This may suggest a change in predilection by age, but the total number is too small to permit conclusions. The brain is generally involved in two ways: either as localized tumor masses resembling certain gliomas or as diffusely invasive neoplasms [16]. Accordingly, all previously documented patients with primary Burkitt-type lymphoma of the CNS were reported to present with indistinct symptoms and signs of focal neurological deficit rather than raised ICP. Gawish reported a primary Burkitt-type lymphoma of the frontal bone presenting with a steadily growing mass in a patient who subsequently developed hemiplegia [4]. To our knowledge, the optic nerve compression through intradural invasion of a primary extradural Burkitt-type lymphoma has not been reported previously. When visual function is impaired, the oculocerebral type of malignant lymphoma should also be taken into consideration. The ocular findings in our patient excluded such a possibility even preoperatively. The visual function of our case was interpreted by means of VEPs both pre- and postoperatively in the hope of recording the slightest improvement. As shown in a recent study, the presence of a P-100 wave is a good indicator of visual recovery and the absence of a P-100 wave in sequential VEPs was indicative of a poor outcome [11]. We would thus like to stress the predictive value of this test, especially in children presenting with loss of vision. Unfortunately, the preoperative CT diagnosis for our patient was an optic glioma or a meningioma, although the latter was not likely for his age. However, a primary lymphoma was never suspected. As was the case in many of the reports on primary Burkitt-type lymphoma [5, 6, 9, 13, 15], most of which were diagnosed at autopsy. We would also like to stress the fact that in a new era with an increasing number of immunocompromised patients, primary intracranial lymphoma of any kind should be kept in mind. As predicted by Rosenblum recently, the occurrence of primary intracranial lymphomas associated with AIDS will exceed the incidence of low-grade astrocytomas and approach the incidence of meningiomas by the year 1991 [12]. Biopsy, either open or stereotactic, should be the choice in tumors around the optic nerve that are highly infiltrative, or when expectations that the tumor is amenable to adjunctive therapy exist. We were satisfied with multibiopsy and excision of the dura involved. Burkitt-type lymphoma is known as a highly radiosensitive tumor, and for that reason priority was given to early irradiation. Although CSF pathways were clear of neoplastic cells, spinal irradiation was still done. The chemotherapy regimen administered in this case was recently documented as being highly effective in pa-
tients with non-Hodgkin's lymphomas, excluding the Burkitt type [3]; however, it seemed quite satisfactory for a Burkitt-type intracranial lymphoma, as judged after an ] 8-month follow-up period during which there has been no recurrence or any further symptoms.
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