4
NEUROSARCOIDOSIS PRESENTING AS A LARGE SUPRASELLAR MASS MAGNETIC RESONANCE IMAGING FINDINGS ROHIT BAKSHI, MD, ROBERT A. FENSTERMAKER, MD, VERNICE E. BATES, MD, T. P. RAVICHANDRAN, MD, SAM GOODLOE, JR., MD, AND WILLIAM R. KINKEL, MD We present unusual magnetic resonance imaging (MRI) netic resonance imaging (MRI), although a wide vari- ety of manifestations have been noted (1–14). We findings in a case of neurosarcoidosis. MRI revealed a large solitary suprasellar mass which resembled a present an unusual case of neurosarcoidosis presenting neoplasm. The lesion was isointense and hyperintense on MRI as a large suprasellar mass; the tumor-like ap- on T1-weighted images, hypointense on T2-weighted pearance prompted a biopsy. images, and intensely homogeneously enhancing. Biopsy revealed a polymorphous inflammatory le- sion with giant cells, which extended from the hy- CASE REPORT pothalamus, consistent with neurosarcoidosis. The A 29-year-old construction worker was admitted to diagnosis of neurosarcoidosis should be considered the Millard Fillmore Hospital with visual complaints. in patients presenting with large midline tumor- He was diagnosed 3 years previously with systemic like suprasellar mass lesions. Elsevier Science sarcoidosis, without previous neurologic involvement. Inc., 1998 He had been stable on chronic prednisone therapy, when he developed an acute confusional state, in- KEY WORDS: creasing headaches, and visual complaints. Neuro- Neurosarcoidosis; Sarcoidosis; Magnetic resonance logical examination disclosed a macula-splitting left imaging; Hypothalamus; Suprasellar tumors inferior quadrantanopsia with some constriction of the left upper quadrant. The right eye demonstrated a complete left hemianopsia. These findings were consistent with a lesion superior and immediately INTRODUCTION posterior to the optic chiasm on the right. A noncon- Sarcoidosis is a disseminated granulomatous disease trast computed tomography (CT) scan disclosed a of unknown etiology which involves the central ner- heterogeneous, hyperdense suprasellar mass lesion, vous system (CNS) in approximately 10% of cases. suggesting a calcified meningioma (not shown). Con- CNS sarcoidosis, commonly known as neurosarcoido- ventional MRI of the head was performed with spe- sis, may be effectively recognized by cranial mag- cial attention to the sellar and juxta-sellar region (Figure 1). Because of the proximity of the lesion to the skull base and the visual impairment, an ex- From the Millard Fillmore Health System, Lucy Dent Imaging Center (R.B., V.E.B., T.P.R., W.R.K.), Dent Neurologic Institute tended right frontal craniotomy was performed for (R.B., V.E.B., W.R.K.), Departments of Neurology (R.B., V.E.B., biopsy and to decompress the visual pathways. At W.R.K.), Neurosurgery (R.A.F.), Pathology (S.G.), State University surgery, the optic chiasm and optic tract on the right of New York at Buffalo, School of Medicine and Biomedical Sci- ences, Buffalo, New York. were grossly abnormal and distended. Within the Address correspondence to: Rohit Bakshi, MD, Lucy Dent Im- right optic tract was a hard, rubbery yellow-tan mass aging Center, 3 Gates Circle, Buffalo, New York 14209. Received January 12, 1998; accepted March 20, 1998. which was avascular and entirely intra-axial; no exo- CLINICAL IMAGING 1998;22:323–326 Elsevier Science Inc., 1998. All rights reserved. 0899-7071/98/$–see front matter 655 Avenue of the Americas, New York, NY 10010 PII S0899-7071(98)00035-7

Neurosarcoidosis presenting as a large suprasellar mass: Magnetic resonance imaging findings

Embed Size (px)

Citation preview

Page 1: Neurosarcoidosis presenting as a large suprasellar mass: Magnetic resonance imaging findings

NEUROSARCOIDOSIS PRESENTING AS A LARGESUPRASELLAR MASSMAGNETIC RESONANCE IMAGING FINDINGS

ROHIT BAKSHI, MD, ROBERT A. FENSTERMAKER, MD,VERNICE E. BATES, MD, T. P. RAVICHANDRAN, MD,SAM GOODLOE, JR., MD, AND WILLIAM R. KINKEL, MD

We present unusual magnetic resonance imaging (MRI) netic resonance imaging (MRI), although a wide vari-ety of manifestations have been noted (1–14). Wefindings in a case of neurosarcoidosis. MRI revealed

a large solitary suprasellar mass which resembled a present an unusual case of neurosarcoidosis presentingneoplasm. The lesion was isointense and hyperintense on MRI as a large suprasellar mass; the tumor-like ap-on T1-weighted images, hypointense on T2-weighted pearance prompted a biopsy.images, and intensely homogeneously enhancing.Biopsy revealed a polymorphous inflammatory le-sion with giant cells, which extended from the hy- CASE REPORTpothalamus, consistent with neurosarcoidosis. The A 29-year-old construction worker was admitted todiagnosis of neurosarcoidosis should be considered the Millard Fillmore Hospital with visual complaints.in patients presenting with large midline tumor- He was diagnosed 3 years previously with systemiclike suprasellar mass lesions. Elsevier Science sarcoidosis, without previous neurologic involvement.Inc., 1998

He had been stable on chronic prednisone therapy,when he developed an acute confusional state, in-

KEY WORDS: creasing headaches, and visual complaints. Neuro-Neurosarcoidosis; Sarcoidosis; Magnetic resonance logical examination disclosed a macula-splitting left

imaging; Hypothalamus; Suprasellar tumors inferior quadrantanopsia with some constriction ofthe left upper quadrant. The right eye demonstrateda complete left hemianopsia. These findings wereconsistent with a lesion superior and immediatelyINTRODUCTIONposterior to the optic chiasm on the right. A noncon-Sarcoidosis is a disseminated granulomatous diseasetrast computed tomography (CT) scan disclosed aof unknown etiology which involves the central ner-heterogeneous, hyperdense suprasellar mass lesion,vous system (CNS) in approximately 10% of cases.suggesting a calcified meningioma (not shown). Con-CNS sarcoidosis, commonly known as neurosarcoido-ventional MRI of the head was performed with spe-sis, may be effectively recognized by cranial mag-cial attention to the sellar and juxta-sellar region(Figure 1). Because of the proximity of the lesion tothe skull base and the visual impairment, an ex-From the Millard Fillmore Health System, Lucy Dent Imaging

Center (R.B., V.E.B., T.P.R., W.R.K.), Dent Neurologic Institute tended right frontal craniotomy was performed for(R.B., V.E.B., W.R.K.), Departments of Neurology (R.B., V.E.B., biopsy and to decompress the visual pathways. AtW.R.K.), Neurosurgery (R.A.F.), Pathology (S.G.), State University

surgery, the optic chiasm and optic tract on the rightof New York at Buffalo, School of Medicine and Biomedical Sci-ences, Buffalo, New York. were grossly abnormal and distended. Within the

Address correspondence to: Rohit Bakshi, MD, Lucy Dent Im-right optic tract was a hard, rubbery yellow-tan massaging Center, 3 Gates Circle, Buffalo, New York 14209.

Received January 12, 1998; accepted March 20, 1998. which was avascular and entirely intra-axial; no exo-

CLINICAL IMAGING 1998;22:323–326 Elsevier Science Inc., 1998. All rights reserved. 0899-7071/98/$–see front matter655 Avenue of the Americas, New York, NY 10010 PII S0899-7071(98)00035-7

Page 2: Neurosarcoidosis presenting as a large suprasellar mass: Magnetic resonance imaging findings

324 BAKSHI ET AL. CLINICAL IMAGING VOL. 22, NO. 5

Figure 1 continued

phytic component extending into the subarachnoid lesions, and arteritis (1–14). Less commonly, neuro-sarcoidosis may appear as an intracranial mass le-space was noted. The diencephalon was swollen by

the mass. It was apparent that the mass was densely sion on MRI, either extra-axial or intra-parenchymal(2–13), simulating a neoplasm. The MRI-based seriesadherent to the hypothalamus and the right optic

tract. It was deemed too hazardous to separate the of Lexa and Grossman (4), noted mass lesions in 46%of patients with neurosarcoidosis. Pseudotumoralmass from the surrounding normal tissue. Therefore,

subtotal excision of the mass was performed. Patho- locations which have been reported on MRI in sar-coidosis include the cerebral convexities, anteriorlogical analysis revealed a polymorphous inflamma-

tory infiltrate diffusely involving the neuropil, con- and middle fossa, clivus, pituitary, pineal region,cerebellopontine angle, planum sphenoidale, andsistent with neurosarcoidosis. Occasional giant cells

and many epithelioid cells were present. There was cerebral lobes. The current case adds the suprasel-lar region to this list of large neurosarcoid massno evidence of neoplasm.lesions.

Signal abnormalities of pseudotumoral neurosarcoi-DISCUSSION dosis mass lesions include isointensity or hypointen-

sity on T1-weighted images and a variable appearanceNeurosarcoidosis may be difficult to diagnose be-cause of its rarity and wide spectrum of clinical man- on T2-weighted images (most commonly hypointense

or isointense). Post-contrast studies have demonstratedifestations. A myriad of abnormalities in neurosar-coidosis may be detected on cranial MRI. Common a wide variety of enhancement patterns, ranging from

homogenous to patchy-irregular appearances. Asso-presentations include leptomeningitis, periventricularwhite matter disease, hydrocephalus, diffuse atrophy, ciated findings of leptomeningeal enhancement and

multiple focal hyperintense parenchymal lesions oncranial neuritis, optic chiasm lesions, periaqeductal

Page 3: Neurosarcoidosis presenting as a large suprasellar mass: Magnetic resonance imaging findings

325SEPTEMBER/OCTOBER 1998 SUPRASELLAR NEUROSARCOIDOSIS MASS

FIGURE 1. Single MRI examination (Picker 1.5 T scanner,Conventional spin-echo, 220 3 256, FOV-23 cm, slice thick-ness: axial, 6 mm; sagittal, 4 mm). The right side of the imagerepresents the right side of the head. A well-circumscribedround mass is noted in the suprasellar region which lies in themidline and slightly to the right. The mass measures 3 cm leftto right, 2.5 cm anterior to posterior, and 2.2 cm inferior to su-perior. (A) Non-contrast T1-weighted axial (TR/TE: 733/20).An isointense core is interspersed with and surrounded byspeckled and septated hyperintensities. Mass effect is notedon the ventral midbrain/diencephalon. (B,C) Post-contrast (ga-doteridol, 5 minute delay) T1-weighted [(B) axial, TR/TE: 733/20; (C) sagittal, TR/TE: 500/20]. Intense, homogeneous en-hancement of the mass is noted. The lesion is clearly con-fined to the suprasellar region; the sellar contents are sepa-rate from the mass and show normal enhancement. (D)Non-contrast T2-weighted axial (TR/TE: 2300/80). Themass is heterogeneous in appearance. It is largely hypoin-tense, but contains intermixed isointense regions. Peritu-moral edema is noted involving the midbrain, diencepha-lon, and medial temporal lobes.

acknowledge the tireless efforts of the Kideney Health SciencesT2-weighted images may point to sarcoidosis. How-Library staff in assisting with the literature review.ever, on occasion, such characteristic associated ab-

normalities may be absent, as in our case.The MRI appearance of the lesion in the current

case resembles a meningioma, similar to the neuro- REFERENCESsarcoid series of Wilson et al. (13). The hypointensity 1. Case records of the Massachusetts General Hospital. Case 37-

1996. N Engl J Med 1996;335:1668–1674.on T2-weighted images is in agreement with previ-ous studies (8, 13) and may relate to calcification, fi- 2. Grand S, Hoffmann D, Bost F, Francois-Joubert A, Pasquier B, Le

Bas JF. Case report: pseudotumoral brain lesion as the presentingbrous stroma, or high cellular density. In our case,feature of sarcoidosis. Br J Radiol 1996;69:272–275.

the CT and pathologic findings were consistent with3. Hayes WS, Sherman JL, Stern BJ, Citrin CM, Pulaski PD. MR andheterogeneous calcification. CT evaluation of intracranial sarcoidosis. AJNR 1987;8:841–847.

In conclusion, the diagnosis of neurosarcoidosis 4. Lexa FJ, Grossman RI. MR of sarcoidosis in the head and spine:should be considered when confronted with a young spectrum of manifestations and radiographic response to steroid

therapy. AJNR 1994;15:973–982.adult patient presenting with a large midline supra-5. Liu DPC, Sze G. Intracranial sarcoidosis on gadolinium-enhancedsellar mass lesion, as it may represent an extending

MRI. Neuroradiology 1991;33:189.hypothalamic inflammatory lesion secondary to sar-6. Miller DH, Kendall BE, Barter S, et al. Magnetic resonance im-coidosis.

aging in central nervous system sarcoidosis. Neurology1988;38:378–383.

7. Mirfakhraee M, Crofford MJ, Guinto FC, Nauta HJW, Weedn VW.We thank Evelyn Calderon, Kim Marie Malicki, Janice Tokarczyk, Virchow-Robin space: a path of spread in neurosarcoidosis. Radi-

ology 1986;158:715–720.Joan Schurr, and James Pierotti for technical assistance. We also

Page 4: Neurosarcoidosis presenting as a large suprasellar mass: Magnetic resonance imaging findings

326 BAKSHI ET AL. CLINICAL IMAGING VOL. 22, NO. 5

8. Seltzer S, Mark AS, Atlas SW. CNS sarcoidosis: evaluation with 12. Wilson JD, Castillo M. Magnetic resonance imaging of granuloma-contrast-enhanced MR imaging. AJNR 1991;12:1227–1233. tous inflammations: sarcoidosis and tuberculosis. Top Magn Res

Imag 1994;6:32–40.9. Sherman JL, Stern BJ. Sarcoidosis of the CNS: comparison of un-enhanced and enhanced MR images. AJNR 1990;11:915–923. 13. Wilson JD, Castillo M, Van Tassel P. MRI features of intracranial

sarcoidosis mimicking meningiomas. Clin Imag 1994;18:184–188.10. Stubgen J-P. Neurosarcoidosis presenting as a retroclival mass.Surg Neurol 1995;43:85–88. 14. Scott TF. Neurosarcoidosis: progress and clinical aspects. Neurol-

11. Ulmer JL, Elster AD. Sarcoidosis of the central nervous system. ogy 1993;43:8–12.Neuroimag Clin N Am 1991;1:141–158.