Ruptured intracranial aneurysm associated with cerebral reticulum cell sarcoma

Ruptured intracranial aneurysm associated with cerebral reticulum cell sarcoma

Journal of Computed Tomography 6:151-159, 1982 RUPTURED INTRACRANIAL ANEURYSM ASSOCIATED WITH CEREBRAL RETICULUM CELL SARCOMA JOHN STILLER, WEN C...

10MB Sizes 0 Downloads 182 Views

Journal

of Computed

Tomography

6:151-159,

1982

RUPTURED INTRACRANIAL ANEURYSM ASSOCIATED WITH CEREBRAL RETICULUM CELL SARCOMA JOHN STILLER, WEN C. YANG, MAX RUDANSKY, HERBERT RUBINOWITZ, AND JONATHAN G. SARLIN

A case of ruptured anterior communicating aneurysm with association of reticulum cell sarcoma and aTteriovenous malformation is reported. Sequential CT documented the evolution of a cerebral reticulum cell sarcoma, which added complexity to the clinical course. Cranial CT, cerebral angiograms, and postmortem examination are presented. KEY WORDS: Reticulum

Cell

Sarcoma,

Aneurysm,

Cerebral

Angiography

INTRODUCTION There have been numerous reports in the literature of the association of intracranial saccular aneurysms and brain tumors [l-3]. Meningiomas, gliomas, and pituitary adenomas constitute the majority of the cases 11, 21. Taylor [3] concluded that the two pathological conditions could be explained on the basis of chance alone. Regardless of whether or not there is an increased incidence of arterial aneurysms with intracranial tumors, their coexistence is important with respect to diagnosis and treatment because of the high operative risk when these two lesions are present [l].An unusual clinical case of the association of a ruptured saccular aneurysm and

From the Departments of Neurology U.S., M.R., H.R.), Radiology (W.C.Y), and Pathology (J.G.S.), Beth Israel Medical Center and Mount Sinai School of Medicine, New York. Address reprint requests to Wen C. Yang, M.D., Department of Radiology, Beth Israel Medical Center, New York, NY 10003. Received April 1982; accepted May 3, 1982. 0 1982 by Elsevier Science Publishing Co., Inc. 52 Vanderbilt Ave., New York, NY 10017 0149-936x/82/020151-09$2.75

evolving primary reticulum cell sarcoma of the brain is presented with sequential CT and postmortem correlation.

CASE REPORT A 75year-old woman was admitted to Beth Israel Medical Center on November 24, 1980, with the abrupt onset of severe headache and brief loss of consciousness. Her past medical history was significant for hypertension, a left lower pole renal cyst diagnosed by ultrasonography several years previously, and resection of a benign parotid gland tumor in 1959. Examination on admission revealed only nuchal rigidity and no other neurologic findings. Routine laboratory studies and a chest x-ray were within normal limits. The electrocardiogram showed nonspecific ST-T wave changes. Spinal puncture yielded grossly bloody fluid under a pressure of 190 mmH,O. Cerebrospinal fluid contained 100 mg% glucose, 348 mg% protein, 269,000 red blood cells, and 850 white blood cells (64 polymorphonuclear cells, 36 lymphocytes). Cranial CT (GE CT/T 8800 unit, 120 KVF’, 614 mA, lo-mm scan slice] revealed evidence of subarachnoid hemorrhage with a suggestion of an anterior communicating artery aneurysm on the postcontrast scan (Figure 1). One week after admission, the patient developed mild aphasia and right-sided weakness. At that time steroid medication was started. A cranial CT demonstrated an area of decreased attenuation in the left frontal lobe, which was interpreted as an infarct (Figure 2). The patient developed transient urinary incontinence, which was followed by gradual improvement of the aphasia and right-sided weakness. On

FIGURE 1. (11/24/80) (A) Precontrast CT scan. Subarevident here (arrows). hemorrhage is achnoid (B,C,D) Postcontrast CT scans. The vascular structures at

brain base are enhanced with suggestive evidence of an aneurysm in the anterior communication artery (arrows].

SEPTEMBER

1982

FIGURE 2. (12/2/80) CT scans without contrast. (A,B,C) Residual hemorrhage is evident in the pericallosal cistern [arrowheads). In addition, there is an area of decreased attenuation, thought to represent an infarct (arrows).

mild 24, the third cranial CT revealed ventricular dilatation. The area of decreased attenuation in the left frontal lobe remained unchanged improvement in [Figure 3). There was continuous the patient’s clinical status and steroids were gradually discontinued. The patient refused cerebral angiography and she was discharged on January 15, 1981, with no residual neurological deficit. The patient continued to do well until early May, 1981, when the family noticed increasing difficulty with memory and speech over a Z-week period. She was readmitted to the hospital and examination revealed an organic mental syndrome with aphasic elements and a mild right hemiparesis. Physical examination was otherwise normal. Complete blood count, routine chemistries, coagulation studies, and chest x-ray were all normal. On May 11, 1981, a CT scan showed an enhancing December

RUPTURED

INTRACRANIAL

ANEURISM

153

154

STILLER ET AL.

THE JOURNAL OF COMPUTED

TOMOGRAPHY

VOL. 6 NO. 2

FIGURE 3. (12124180) CT scans without contrast. (A,B,C) The ventricles are slightly dilated. The area of

decreased tinct.

attenuation

in the left frontal

lobe is less dis-

mass lesion in the left frontal lobe, left basal ganglia, left frontal horn, and left atrium (Figure 4). Steroid medication was reinstituted and a mild clinical improvement was noted over the next 2 weeks. The repeat cranial CT on May 23 demonstrated a minimal decrease in the size of the enhancing lesion in the left frontal lobe basal ganglia and left frontal horn (Figure 5). Cerebral angiography on May 29 demonstrated an aneurysm arising from the anterior communicating artery and vascular stain in the left atria1 mass (Figure 6). The patient became progressively more confused and lethargic, and a CT scan performed on June 7 revealed enlargement of the mass lesions [Figure 7). The patient had a respiratory arrest and expired on June 11, 1981. On postmortem examination, a 7-mm saccular aneurysm of the left anterior cerebral artery was present arising from the anterior communicating ar-

SEPTEMBER

1982

FIGURE 4. (5/11/81) (A) Precontrast and (B,C) postcontrast CT scans. An enhanced mass is noted in the left frontal lobe, left basal ganglia with extension into left frontal horn. A separate enhanced mass is noted in left antrium (arrows).

tissue surrounding tery (Figure 8). The connective the aneurysm contained considerable quantities of hemosiderin. The cerebral tissues were markedly swollen, particularly the anterior left cerebral hemisphere. The region of the left corpus striatum and thalamus was replaced by a poorly circumscribed, soft, dusky rose tumor (Figure 8), which on histological examination was a reticulum cell sarcoma (malignant lymphoma, histiocytic type), consisting of sheets of large lymphoid cells with hyperchromatic irregular nuclei (Figure 9). There was much nuclear debris and a tendency toward a perivascular distribution. A small arteriovenous malformation was noted on the surface of right cerebellar hemisphere without evidence of rupture. Other autopsy findings included a large simple cyst of the left kidney, generalized atherosclerosis, and a focal acute bronchopneumonia.

RUPTURED

INTRACRANIAL

ANEURISM

155

156

FIGURE 6. (5/29/81) Left carotid angiograms in (A) anteroposterior and (B) lateral projections. An ante-

rior communicating artery aneurysm (arrows) is demonstrated. There is vascular stain [open arrows) in left atria1 region supplied by left anterior choroidal artery [arrowheads) and left lateral posterior choroidal artery (double arrowheads). An arterial branch (triple arrowheads) from the right internal occipital artery is noted entering the atrium and giving rise to vascular stain. (C) Left vertebral angiogram in lateral projection. A small arteriovenous malformation (arrows) is noted in the distal territory of right superior cerebellar artery.

FIGURE 5. (5/23/81) (A,B) Postcontrast CT scans. There is minimal shrinkage of the enhanced mass in left frontal lobe and basal ganglia as compared with Figure 4B.

SEPTEMBER

1982

RUPTURED

INTRACRANIAL

ANEURISM

157

FIGURE 6. B FIGURE 6. C

FIGURE 7. (6/7/81) CT scan without contrast. The mass lesions are much larger.

DISCUSSION In 116 cases of cerebral aneurysm with associated brain tumors reviewed by Pia et al. [2], 69% presented with symptoms attributed to the tumor and 22% with symptoms owing to the aneurysm. Our patient’s initial presentation was a common one for a ruptured intracranial aneurysm. The onset of focal symptoms in 1 week is not uncommon with subarachnoid hemorrhage and is often attributed to focal ischemia or infarction secondary to vasospasm. In our case, an unenhanced CT scan at the time the patient developed focal findings revealed an area of decreased attenuation in the left frontal lobe, which appeared to support the diagnosis of infarction.

However, in retrospect, with the subsequent demonstration of an enhancing mass in this same region several months later, it is likely that the initial lesion in the left frontal area was tumor. Although the clinical course of a complete resolution of the neurological deficit is more consistent with infarction, clinical remissions in cerebral reticulum cell sacoma are known to occur with the administration of steroids [4, 51. Some of these remissions were sustained long after the steroids had been discontinued [4, 51. Of interest is the minimal decrease in size of the enhancing lesion on the scan of May 23 (Figure 5) compared to May 11 (Figure 41, which was approximately 2 weeks after steroids had been reinstituted. Williams et al. [4] noted similar apparent radiologic improvement in cerebral reticulum cell sarcoma treated with steroids. Although CT findings of cerebral reticulum cell sarcoma are not specific, certain characteristics regarding the anatomic location and tumor appearance are usually noted [6] and were present in our case. These characteristics are involvement of the basal ganglia and periventricular region, and ho-

158

STILLER

ET AL.

THE IOURNAL

OF COMPUTED

TOMOGRAPHY

VOL.

6 NO.

2

A

FIGURE 8. [A) Saccular aneurysm of left anterior communicating artery (H&E, 5 x ). (B) Representative coronal

section of brain with poorly defined tumor in the region of left corpus striatum. (C) Reticulum cell sarcoma of brain (H&E, 490 x ).

mogeneous contrast enhancement of the tumor. The angiographic appearance of primary cerebral reticulum cell sacroma has been well documented [i’]. Features such as arterial encasement and dilated deep medullary veins known to be suggestive of the lesion are lacking in this case. However, tumor staining, an uncommon finding, is clearly evident in the territory of the anterior choroidal artery (Figure 6B).

The present case is unique as CT demonstrated the evolution of reticulum cell sarcoma after the episode of subarachnoid hemorrhage. Moreover, a coincident arteriovenous malformation was noted inthe posterior fossa (Figure 6C). To our knowledge, no case of ruptured cerebral aneurysm with association of brain tumor and arteriovenous malformation has ever been documented.

SEPTEMBER

1982

IODINATED RADIOGRAPHIC CONTRAST MEDIA

We are grateful to Dr. Stefan Shanzer for review of this manuscript and to Ms. Susan Cowan for her help in the preparation of this paper.

REFERENCES 1. Handa J, Matsuda

intracranial

I, Handa H: Association of brain tumor and aneurysms. Surg Neurol 6:25-29, 1976

2. Pia H, Obrador,

arterial 1971

S, Martin JG: Association of brain tumors and intracranial aneurysms. Acta Neurochir 27:189-204,

3. Taylor PE: Delayed

nial aneurysms

post operative hemorrhage from intracraafter craniotomy for tumor. Neurology 11:225-

231, 1961 4. Williams

RS, Crowell RM, Fischer CM, Davis K, Lavyne MH, Ropper AH, Bremer AM: Clinical and radiological remission in reticulum cell sarcoma of the brain. Arch Neurol 36:206210,1979

5. Wallack EM, Reavis WM, Hall CD: Primary brain stem reticu-

lum cell sarcoma causing demetia. Dis Nerv Syst 38:744-747 1977 6. Enzmann

DR, Krikorian J, Norman D, Kramer R, Pollock J, Faer M: Computed tomography in primary reticulum cell sarcoma of the brain. Radiology 130:165-170, 1979

7. Kishikawa

T, Numaguchi Y, Fukiu M, Komaki S, Ikeda J, Kitamura K, Matuura K: Primary intracranial sarcomas: Radiologic diagnosis with emphasis on arteriography. Neuroradiology 21:25-31,198l

159

CONTINUING MEDICAL EDUCATION QUESTIONS Choose the correct answer(s) correct)

[more than one answer

may be

In patients with associated cerebral aneurysm and cerebral tumors a. The clinical presentation is usually that which is attributed to the tumor b. There is high operative mortality c. The tumor is rarely a meningioma With steroid treatment of cerebral reticulum cell sarcoma a. Clinical remission may be achieved during treatment b. Clinical remission is never sustained after steroids are discontinued c. Improvement on CT scans may occur CT findings of cerebral reticulum cell sarcoma a. Are specific b. Often show involvement of the basal ganglia c. Most often reveal homogeneous contrast enhancement of the tumor d. Usually spare the periventricular area Angiographic features of cerebral reticulum coma often include a. Arterialencasement b. Dilated deep medullary veins c. Aneurysmal dilatation of the vessel

cell

sar-