Pediatric with

Orbital Intra—

Shigeru

FURUTA, Yoshiaki

Department

of Neurological

and

Eosinophilic

Granuloma

Extracranial

Extension

Case

Saburo

Report—

SAKAKI,

KUMON Surgery,

and

Ko

Takao

HATAKEYAMA,

NAKAMURA

Ehime University

School of Medicine,

Ehime

Abstract A rare case of eosinophilic granuloma of the orbit in a 3-year-old boy presented as right upper eyelid swelling and proptosis. Computed tomographic scanning revealed a soft-tissue dense mass in the lateral wall of the right orbit. Magnetic resonance (MR) imaging demonstrated tumor extension into the orbit, anterior and middle cranial fossae, and extracranial region. The tumor was completely removed. Histological diagnosis was eosinophilic granuloma. No evidence of recurrence was found 14 months later. MR imaging is useful for diagnosis of the lesion, and particularly for surgical manage ment. Key words:

eosinophilic

granuloma,

orbit,

Introduction Eosinophilic granuloma is the most benign tumor included in the histiocytosis X.13) Eosinophilic granuloma usually involves the skull and the long bones, and rarely affects the orbit.'1'.'9) Early diag nosis of orbital eosinophilic granuloma is essential to preserve vision as marked eyeball displacement accompanies rapid tumor growth. We describe a young boy with solitary orbital eosinophilic granu loma, and the magnetic resonance (MR) imaging of intra and extracranial tumor extensions. Case

magnetic

resonance

imaging

A plain skull x-ray film showed lytic lesions in the right posterolateral orbit and the lesser wing of the sphenoidal bone. The frontozygomatic suture was also opened (Fig. 1). Axial computed tomographic (CT) scans demonstrated a homogeneous isodense mass with moderate enhancement (Fig. 2). The mass was approximately 3 cm in diameter located in the

Report

A 3-year-old boy was admitted to our hospital on August 30, 1989, complaining of right upper eyelid swelling

and

proptosis

beginning

about

10 days

previously. Physical and neurological examinations showed tenderness and swelling in the right temporal region. Ocular movements were limited to upper and lateral directions. His visual field and acuity were normal. No liver, spleen, or systemic lymph nodes swelling was palpable. Received 21,

1991

November

29,

1990;

Accepted

January

Fig. 1

Plain skull x-ray film showing lytic bone lesions in both the lesser and greater wings of the right sphenoidal bone with no surrounding sclerosis (arrow) and opening of the frontozygomatic suture (arrowheads).

Fig.

4

Photomicrograph mononuclear eosinophils.

Fig. 2

Fig. 3

left: Precontrast axial CT scan demonstrating a homogeneous isodense (64 Hounsfield units: HU) mass lesion in the lateral wall of the right orbit extending into the orbit and the middle cranial fossa. right: Postcontrast CT scan showing the moderately enhanced mass (84-90 HU).

upper: TI (TR 500 msec, TE 29 msec) (left) and T2-weighted (TR 2000 msec, TE 92 msec) (right) MR images revealing an iso or high-in tensity mass lesion in the lateral portion of the right orbit. lower: Coronal (left) and sagittal (right) MR images with Gd-DTPA showing the moderately enhanced mass extending superior ly to the anterior cranial fossa and posteriorly to the middle cranial fossa.

lateral wall of the right orbit extending from the or bit to the middle cranial fossa. MR imaging (SMT 50, Shimadzu, Kyoto) revealed a mass lesion in the lateral orbit appearing as iso and high intensity on

demonstrating histiocytes with HE

stain,

sheets of interspersed

x 200.

TI and T2-weighted images, respectively. MR images with gadolinium diethylene-triamine-pentaacetic acid (Gd-DTPA) demonstrated a moderately en hanced mass extending superiorly into the anterior cranial fossa (Fig. 3). A right fronto-orbital craniotomy was performed on September 14, 1989. The grayish-yellow tumor, approximately 4 cm in diameter, extended from the lateral orbital wall posteromedially to near the superior orbital fissure, laterally to the temporal fossa, superiorly to the anterior cranial fossa, and posteriorly to the middle cranial fossa. The peri orbita and dura mater were intact. The tumor and involved bones were extensively removed and the lateral orbital wall reconstructed with methyl meth acrylate (cranioplastic kit, Codman and Shurtlefe, Inc., Randolph, Mass., U.S.A.). Histological exami nation showed sheets of mononuclear histiocytes with interspersed eosinophils (Fig. 4). The diagnosis was eosinophilic granuloma. Postoperative course was uneventful. Bone scinti scans found no other systemic involvement. MR images demonstrated no evidence of recurrence 14 months later. Discussion Eosinophilic granuloma accounts for only 1 % or less of all orbital tumors. '"'°"°21) The supero lateral orbit,") and extension superiorly to the anterior cranial fossa or laterally to the extracranial region' 5,6,8,10,14,22) are the most common locations. However, only two cases have demonstrated exten sion to the middle cranial fossa.3,5) In our case, the tumor extended in all directions. Initial symptoms or signs of orbital eosinophilic granuloma are upper eyelid swelling and/or prop

tosis.2-6,8,1°,12,18) CT scans demonstrate a mass of soft-tissue density moderately enhanced on postcon trast scans. CT scanning with bone window settings reveals lytic bone lesions. 1,1,1,1,21) Few reports have discussed the MR findings of histiocytosis X. Graif and Pennock9) reported that brain histiocytosis X had a slightly lower and higher intensity on T1 and T2-weighted MR images, respec tively. Moore et al.") found that skull lesions in multifocal eosinophilic granuloma were isointense on T1-weighted images and moderately high intensity on T2-weighted images. Murayama et al."' reported that calvarial monofocal eosinophilic granuloma appeared as a high-intensity mass against the low intensity calvarium on T2-weighted image. There fore, histiocytosis X has no characteristic MR fea tures. The MR appearance of orbital eosinophilic

8)

9)

10)

Glover AT, Grove AS Jr: Eosinophilic granuloma of the orbit with spontaneous healing. Ophthalmology 94: 1008-1012, 1987 Graif M, Pennock JM: MR imaging of histiocytosis X in the central nervous system. AJNR 7: 21-23, 1986 Jakodiec FA, Trokel SL, Aron-Rosa D, Iwamoto T, Doyon D: Localized eosinophilic granuloma (Langerhans' cell histiocytosis) of the orbital frontal bone. Arch Ophthalmol 98: 1814-1820, 1980

11) König B, Vojácek K, Zapletal B: Eosinophiles Granulom der Orbita. Klin Monatsbl Augenheilkd 145: 212-221, 1964 12)

Kwak R, Saso S, Ushirobata H: Clinical and pathological study of the cranial histiocytosis X: Report of 10 cases. No To Shinkei 25: 1029-1040, 1973 (in Japanese) 13) Lichtenstein L: Histiocytosis X. Integration of eosinophilic granuloma of bone, “Letterer-Siwe granuloma in our case was an isointensity mass on disease,” and “Schüller-Christian disease” as related T1-weighted images and a high-intensity mass on T2 manifestations of a single nosologic entity. Arch weighted images. Gd-DTPA clearly demonstrated Path (Chicago) 56: 84-102, 1953 the tumor extension. MR imaging was valuable for 14) Moore AT, Pritchard J, Taylor DSI: Histiocytosis X: the diagnosis and management of this tumor. An ophthalmological review. Br J Ophthalmol 69: 7 Curettage is often performed for solitary eosino -14, 1985 15) Moore JB, Kulkarni R, Crutcher DC, Bhimani S: philic granuloma.2,6,10) More extensive lesions may MRI in multifocal eosinophilic granuloma: Staging require adjuvant radiation therapy. 11,21)However, disease and monitoring response to therapy. Am J some reports indicate only local predonisolone ad Pediatr Hematol Oncol 11: 174-177, 1989 ministration 22) or spontaneous healing.") Our case 16) Murayama S, Numaguchi Y, Robinson AE, Rechard received total removal with extensive craniotomy to son DE: Magnetic resonance imaging of calvarial relieve eyeball compression and reconstruction of eosinophilic granuloma. J Comput Assist Tomogr the severely eroded orbital wall, with successful 12: 251-252, 1988 results. 17) Nauert C, Zoronoza J, Ayala A, Harle TS: Eosinophilic granuloma of bone: Diagnosis and References management. Skeletal Radiol 10: 227-235, 1983 18) Nover A, Ohmer B: Eosinophiles Granulom der Or bita. Klin Monatsbl Augenheilkd 171: 283-286, 1977 1) Arnow SJ, Notz RG: Eosinophilic granuloma of the 19) Rowlings CE III, Wilkins RH: Solitary eosinophilic orbit. Trans Pa Acad Ophthalmol Otolaryngol 36: 41-48, 1983 granuloma of the skull. Neurosurgery 15: 155-161, 2) Baghdassarian SA, Shammas HF: Eosinophilic 1984 20) Smith DG, Nesbit ME Jr, D'Angio GJ, Levitt SH: granuloma of the orbit. Ann Ophthalmol 9: 1247 -1251, 1977 Histiocytosis X: Role of radiation therapy in manage 3) Beller AJ, Kornblueth W: Eosinophilic granuloma of ment with special reference to dose levels employed. the orbit. Br J Ophthalmol 35: 220-225, 1951 Radiology 106: 419-422, 1973 4) Dornow H, Dausch D: Histiozytosis X der Orbita. 21) Wende S, Aulich A, Nover A, Lanksch W, Kazner E, Klin Monatsbl Augenheilkd 169: 78-84, 1976 Steinhoff H, Meese W, Lange S, Grumme T: Com 5) Erhart G, Malbrel C, Talmud M, Behar C, puted tomography of orbital lesions. A cooperative Rousseaux P, Lacour P: Un nouveu cas de study of 210 cases. Neuroradiology 13: 123-134, 1977 22) Wirtschafter JD, Nesbit M, Anderson P, McClain K: granulome eosinophile de l'orbite. Bull Soc Ophtalmol Fr 83: 1369-1372, 1983 Intralesional metylpredonisolone for Langerhans' cell histiocytosis of the orbit and cranium. J Pediatr 6) Feldman RB, Moore DM, Hood CI, Hiles DA, Ophthalmol Strabismus 24: 194-197, 1987 Romano PE: Solitary eosinophilic granuloma of the lateral orbital wall. Am J Ophthalmol 100: 318-323, 1985 Address reprint requests to: S. Furuta, M.D., Department 7) Forbes GS, Earnest F N, Waller RR: Computed of Neurological Surgery, Ehime University School of tomography of orbital tumors, including late-genera Medicine, Shitsukawa, Shigenobu-cho, Onsen-gun, tion scanning techniques. Radiology 142: 387-394, Ehime 791-02, Japan. 1982