J Neurosurg 47:776-780, 1977

Pineoeytomas presenting as subaraehnoid hemorrhage R e p o r t of two cases

PAUL STEINBOK, M.D., CLARISSE L. DOLMAN, M.D., AND KEN KAAN, M.D. Departments of Neurosurgery, Pathology, and Radiology, Vancouver General Hospital and University of British Columbia, Vancouver, British Columbia, Canada

~" Two cases of pineocytoma are reported in patients whose initial symptoms resembled a subarachnoid hemorrhage. The underlying tumor became obvious only later in the disease. Several episodes of subarachnoid bleeding occurred. These are the first documented cases of pineocytoma with this type of clinical presentation. KEY W O R D S

9 pineocytoma

pineal tumor

9 brain tumor

RAIN tumors occasionally simulate primary intracranial hemorrhages. We report two cases of pineocytoma in patients who were first diagnosed as having subarachnoid hemorrhage (SAH).

B

C a s e Reports

Case 1

At the age of 13 years this previously healthy boy suffered an acute bout of headache, vomiting, and lethargy, which resolved within a month. Shortly afterward, however, severe headache recurred, leading to hospitalization. Course. He had no neurological signs except for neck stiffness. Lumbar puncture yielded bloody cerebrospinal fluid (CSF) with xanthochromia. Carotid angiograms and ventriculograms were reported as normal. A diagnosis of SAH was made. Further episodes of headache and vomiting followed, 776

9 subarachnoid hemorrhage

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and 6 months later he was found to have chronic papilledema but no other abnormalities. Pneumoencephalogram demonstrated enlarged ventricles; left vertebral arteriogram was normal. A ventriculoatrial shunt was inserted and periodically revised. Three months later, he fell without striking his head, and developed vomiting, a stiff neck, and pain in his legs. Examination revealed meningismus, poor reaction of the pupils to light, and loss of upward conjugate gaze. Lumbar puncture again yielded bloody CSF. Carotid and vertebral angiograms were normal, but a ventriculogram via the shunt demonstrated a mass in the posterior part of the third ventricle. He was given a course of radiation to the pineal region and the gaze abnormalities disappeared. At the age of 17 years the patient had another SAH, proved by lumbar puncture. He recovered and was well for 11/2years, when he developed increasing hearing loss, ataxia J. Neurosurg. / Volume 47 / November, 1977

Subarachnoid hemorrhage in pineocytomas of gait, dysfunction of horizontal and vertical gaze, and decreased sensation of pain and temperature on the left side of the body. His illness slowly progressed over the next 11/2 years with the signs of increased intracranial pressure. Finally he lost consciousness, and died at the age of 20 years. Postmortem Examination. A tumor about 4 cm in diameter was found in the pineal region, invading and destroying the tectum of the midbrain, and infiltrating the anterior lobe of the cerebellum (Fig. 1). Superiorly the tumor was hard and white, but in the tectum it was hemorrhagic and the compressed third ventricle contained a blood cast; a small quantity of blood was present in the fourth ventricle and in the basal subarachnoid space. A 1-cm nodule of partly gelatinous, hemorrhagic, partly hard, white tumor was situated in the left parietooccipital area where a shunting procedure had been attempted. The lateral ventricles were not dilated. The ependyma was roughened. Pathological E x a m i n a t i o n . Microscopically, the tumor consisted of small-to-large cells with distinct eosinophilic cytoplasm and large irregular, sometimes reniform nuclei, occasionally with prominent nucleoli. Anteriorly, the cells lay in groups in a very dense hyaline stroma (Fig. 2). Posteriorly, cell processes extended to vessels of the abundant reticulum present. No mitoses were seen. Silver impregnations for axons, stains for glial fibers, amyloid, Nissl substance, and mucin were negative. There was evidence of recent and old hemorrhage into the tumor, extensive gliosis surrounding it, and dense nodular scarring of the ependyma. The pineal gland could not be identified. The appearance of the tumor was that of a pineocytoma. The hyalinization of the stroma, and perhaps even the somewhat atypical appearance of the tumor cells, may have been due to the radiation.

FIG. 1. Case 1, autopsy specimen. The tumor, which is inferiorly hemorrhagic, occupies the pineal region and infiltrates midbrain and cerebellum. rhages. Lumbar puncture yielded bloody CSF containing 11,000 red blood cells (RBC)/cu mm. Brain scan and four-vessel angiography were negative. The patient was kept in bed for 4 weeks, during which time the papilledema improved and she returned to normal health.

Case 2

This woman had become obese at age 18 years and from that time on had frequent headaches. At 33 years of age she suddenly developed very severe occipital headache which spread over the top of the head. She was dizzy and nauseated, and had photophobia. Course. On examination there was blurring of the disc margins and subhyaloid hemorJ. Neurosurg. / Volume 47 / November, 1977

FIG. 2. Case 1. Photomicrograph shows triangular tumor cells lying in dense hyaline stroma. H & E , x 150. 777

P. Steinbok, C. L. Dolman and K. Kaan

FIG. 3. Case 2. Pneumoencephalogram showing the mass in the posterior third ventricle (outlined by arrows). A little over a year later, she was readmitted with a 1-week history of bifrontal headache. She had bilateral chronic papilledema with early optic atrophy in the right eye. Kernig's sign was positive. Lumbar puncture yielded xanthochromic fluid containing 5170 RBC/cu mm, no white cells, and 124 mg% protein. Four-vessel angiograms were again negative as was a complete myelogram. She was discharged but had to be readmitted within 3 months because of persisting headache. By now she had bilateral optic atrophy with concentric visual field losses

FIG. 4. Case 2. Autopsy specimen shows encapsulated, partly necrotic tumor in the pineal region. 778

but normal acuity. A pneumoencephalogram was reported as demonstrating a communicating hydrocephalus only, and a right ventriculoatrial shunt was placed. Postoperatively, she developed a left hemiplegia. Four years later the patient had a further SAH, proved by lumbar puncture. Her pupils were now dilated and nonreactive and she could not look upward, but the remainder of the visual examination was identical with the previous one. A week later, the CSF was yellow and cloudy, and contained 72 RBC/cu mm and 700 mg% protein. Brain scan was negative. Four-vessel angiograms demonstrated a pineal tumor and review of the pneumoencephalogram of 4 years before showed a mass in the posterior third ventricle (Fig. 3). She was again kept in bed for 4 weeks and discharged on a diet, since it was felt that her obesity precluded surgery. Three months later another SAH made the patient semicomatose with conjugate deviation of the eyes to the right and bilateral Babinski reflexes. A brain scan showed increased uptake in the pineal region. A needle biopsy of the tumor was taken stereotaxically and a pineocytoma was diagnosed. Since the patient was still deemed inoperable, she was treated with a course of radiation, but with little benefit. She was discharged home where she died of bronchopneumonia, a little over 6 years after onset of her illness. Postmortem Examination. The arachnoid was markedly fibrosed at the base of the brain and over the roof of the fourth ventricle, and contained deposits of golden blood pigment. The optic nerves were gray and atrophic. An encapsulated tumor, about 3 cm in diameter, gray, but with large yellow areas of necrosis, lay in the region of the pineal gland and compressed the midbrain (Fig. 4). Although the shunt was present and patent, the lateral and third ventricles were enlarged. The fourth ventricle was also enlarged and the ependyma was roughened and stained brown. Microscopically, the tumor replaced the pineal gland and consisted of well differentiated neural cells forming rosettes (Fig. 5) with processes which were impregnated with silver. Large portions of the tumor were necrotic, perhaps due to the radiation, and the adjacent brain tissue was gliosed and contained numerous hemosiderin-laden histiocytes. The ependyma was studded by glial nodules. J. Neurosurg. / Volume 47 / November, 1977

Subarachnoid hemorrhage in pineocytomas

FIG. 5. Case 2. Photomicrograph shows small regular cells lying in a fibrillary background. H & E , • 150.

Discussion

Pineal tumors classically present with signs of hydrocephalus due to obstruction of the aqueduct, restriction o f upward gaze (Parinaud's syndrome) due to involvement of the pretectal region, ataxia secondary to pressure on the cerebellum, and mental or endocrine disturbances caused by extension into the third ventricle. Smith and Estridgd mentioned that pineal neoplasms may present as SAH, but cited no specific cases and gave no references. Poppen and Marino, 8 in a study of 45 patients, and De Girolami and Schmidek, 3 in a review of 53 cases of tumors in the pineal region, did not find any with a hemorrhage. Cerebrospinal fluid removed from these patients in no instance contained red blood cells. Apuzzo, et al., 1 recently reported a case of apoplexy due to hemorrhage into a pineal cyst in a patient on anticoagulant therapy, and on reviewing the literature were unable to find any other cases of hemorrhage from a pineal tumor. Miller 7 reported a hemorrhage J. Neurosurg. / Volume 47 / November, 1977

from a cavernous hemangioma situated in the posterior third ventricle. Although hemorrhage into a variety of brain tumors is common, bleeding from these into the subarachnoid space is rare. Locksley, et al., 5 found only 28 cases among 5836 patients with SAH. In various reports 2,~-7,9,1~ gliomas, pituitary adenomas, vascular tumors, plexus papillomas, metastases, acoustic neuromas, and meningiomas have been implicated. We believe that our cases are the first examples of pineocytomas presenting in such fashion. Both patients had repeated S A H before the tumor became evident. Repeated S A H from other types of tumors is the rule, since Glass and Abbott 4 in their review of such cases found it in 30 of 36 patients, one having as many as nine separate episodes. Our first patient had three, our second patient four hemorrhages. In the second patient, considerable arachnoid scarring and obstruction had resulted. The tumors behaved in a comparatively benign fashion: the course of illness was 7 years in Case 1 and 6 years in Case 2. In the latter, the tumor was still encapsulated and nonin~asive. Radiation therapy had presumably led to marked hyalinization of the t u m o r in one case and partial necrosis in the other. Long survival is not uncommon in well differentiated pineocytomas. References

1. Apuzzo MLJ, Davey LM, Manuelidis EE: Pineal apoplexy associated with anticoagulant therapy. Case report. J Neurosurg 45:223-226, 1976 2. Daum S, Navarro-Artiles G: Les tumeurs c6r6brales r6vel6es par un syndrome m6ning6 aigu. Hemorrhagie sousarachno~denne ou m6ningite aseptique. Sere Hop Paris 46: 544-549, 1970 3. De Girolami U, Schmidek HH: Clinicopathological study of 53 tumors of the pineal region. J Neurosurg 39:455-462, 1973 4. Glass B, Abbott KH: Subarachnoid hemorrhage consequent to intracranial tumors. Review of literature and report of seven cases. Arch Neurol Psychiatry 73:369-379, 1955 5. Locksley HB, Sahs AL, Sandier R: Report on the cooperative study of intracranial aneurysms and subarachnoid hemorrhage. Section III: Subarachnoid hemorrhage unrelated to intracranial aneurysm and arteriovenous malformation. J Neurosurg 24: 1034-1056, 1966 6. McCoyd K, Barron KD, Cassidy R J: Acoustic neurinoma presenting as subarachnoid 779

P. Steinbok, C. L. Dolman and K. Kaan hemorrhage. Case report. J Neurosurg 41:391-393, 1974 7. Miller RH: Spontaneous subarachnoid hemorrhage: a presenting symptom of a tumor of the third ventricle. Surg Clin North Am 41 (No 4):1043-1048, 1961 8. Poppen JL, Marino R Jr: Pineal tumors of the posterior portion of the third ventricle. J Neurosurg 28:357-364, 1968 9. Smith RA III, Estridge MN: Pineal tumors, in Vinken P J, Bruyn GW (eds)" Handbook of

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Clinical Neurology, Volume 17. Tumours of the Brain and Skull, Part II. Amsterdam: North Holland Publishing, 1974, pp 648-665 10. Walton JN: Subarachnoid hemorrhage of unusual etiology. Neurology 3:517-543, 1953

Address reprint requests to: Clarisse L. Dolman, M.D., Pathology Laboratory, 855 West 12th Avenue, Vancouver, British Columbia, Canada V5Z 1M9.

J. Neurosurg. / Volume 47 / November, 1977

Pineocytomas presenting as subarachnoid hemorrhage. Report of two cases.

J Neurosurg 47:776-780, 1977 Pineoeytomas presenting as subaraehnoid hemorrhage R e p o r t of two cases PAUL STEINBOK, M.D., CLARISSE L. DOLMAN, M...
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