Indian Journal of Pathology and Microbiology
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Year : 2019  |  Volume : 62  |  Issue : 3  |  Page : 499-501
A rare case of recurrent vascular aneurysmal bone cyst of the orbit in a 4-year-old child

1 Department of Histopathology, Max Super Speciality Hospital, Saket, New Delhi, India
2 Department of Pathology, Max Super Speciality Hospital, Saket, New Delhi, India
3 Department of Neurosurgery, Max Super Speciality Hospital, Saket, New Delhi, India

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Date of Web Publication26-Jul-2019

How to cite this article:
Mukherjee U, Tyagi A, Khurana A, Saroha A, Arora H. A rare case of recurrent vascular aneurysmal bone cyst of the orbit in a 4-year-old child. Indian J Pathol Microbiol 2019;62:499-501

How to cite this URL:
Mukherjee U, Tyagi A, Khurana A, Saroha A, Arora H. A rare case of recurrent vascular aneurysmal bone cyst of the orbit in a 4-year-old child. Indian J Pathol Microbiol [serial online] 2019 [cited 2021 Jun 13];62:499-501. Available from: https://www.ijpmonline.org/text.asp?2019/62/3/499/263476


Aneurysmal bone cyst (ABC) is a benign cystic lesion of the bone, defined in histopathological terms by blood-filled spaces separated by connective tissue septa along with fibroblasts, osteoclast-type giant cells, and reactive bone formation. Nearly 50% of ABCs arise in the long bones. Orbital involvement has been reported rarely (less than 1% of all presenting sites).[1] The usual age group ranges from 10 to 30 years old, with 80% of the patients below 20 years of age.[2]

Clinically, ABC can be distinguished into three types. Conventional type (95%) presents as a rapidly growing, destructive lesion progressing to cortical perforation and soft-tissue invasion. The solid type (5%) may present as an incidentally detected radiolucency on a routine radiograph.[3]

A 4-year-old girl presented with a 6-month history of painless progressive proptosis of the right eye, with history of excessive tear, redness, and occasional headache. She was operated twice in the past 1 year for the same complaints. There was no history of trauma. On evaluation, there was 5 cm of proptosis with restriction of extraocular motility in all the directions in the right eye along with downward displacement of the right eye ball [Figure 1]a.
Figure 1: (a) Marked proptosis with the downward displacement of right eye ball. (b) Coronal CT image of the orbit demonstrates a mass with fluid levels in the right orbit extending into the ethmoid sinus. (c) Photomicrograph showing hemorrhagic cystic spaces lined by connective tissue containing fibroblasts and osteoclast-type giant cells with neo-osteoid formation (H and E, ×200). (d) Photomicrograph showing multinucleated giant cells in the fibrous tissue (H and E, ×400)

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Computed tomography (CT) demonstrated a large mass with fluid levels in the right orbit extending into the ethmoid sinus and right basifrontal region [Figure 1]b. There were inferior and lateral displacement of the right globe, extraocular soft tissue thickening, and hematoma seen along the superior aspect of the globe and superior orbital margin. Peripheral calcification was seen in the right basifrontal region. The differential diagnosis included an ABC, a giant-cell tumor (osteoclastoma), or a lymphangioma. Magnetic resonance imaging (MRI) demonstrated a large cystic mass with enhancing septations and extending posteriorly to erode the medial wall of the right orbit and extending into the right ethmoid and sphenoid sinuses and superiorly causing an extradural bulge into left basifrontal region.

The mass was explored through right fronto-orbito-zygomatic craniotomy; the tumor was completely extradural, originating from the surrounding bone of the roof of the orbit. The globe and cone of intraocular muscles could not be visualized, as it was pushed inferiorly and laterally. The previously performed surgery was evident, and previously placed plates and screws were removed along with the supraorbital rim. Gross total excision of the tumor was done after dissecting the tumor meticulously, and tumor tissue was sent for histopathological examination.

Pathologic examination revealed cystic spaces separated by fibrous septa. The fibrous septae were composed of a moderately dense cellular proliferation of fibroblast, with scattered multinucleated osteoclast-type giant cells and reactive woven bone rimmed by osteoblasts. Calcification and siderophage collection were noted [Figure 1]c and [Figure 1]d. The diagnosis was consistent with the ABC. Postoperative course was satisfactory and the patient's eye symptoms improved and proptosis reduced significantly.

Since the first reported orbital aneurysmal bone cyst in 1953, few cases have been documented in literature. Proptosis is a common presenting sign in ABCs of orbit. In our patient, history of rapidly progressive proptosis was probably a result of hemorrhage into the surrounding bony elements. Other presenting complaints include visual changes, chemosis, and periorbital heaviness.

Modalities such as CT and MRI are necessary to help in diagnosis and for surgical planning. CT may show the presence of an expansile, osseous lesion with bone remodeling and cortical loss.[4] Fluid levels are more apparent on MRI,[5] and features may include the presence of multiple internal septations and a “soap bubble” appearance due to the projection of small cysts exophytically from larger cysts.

A recurrence rate of 10%–30% has been reported, most of which have been detected within 2 years of the initial treatments. This underlines the need to review these patients at regular intervals paying careful attention to the optic nerve function and ocular motility. To the best of our knowledge, this is the first reported case of recurrent ABC in a 4-year-old child.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Cakirer S, Cakirer D, Kabukcuoglu F. Aneurysmal bone cyst of the orbit: A case of rare location and review of the literature. Clin Imaging 2002;26:386-91.  Back to cited text no. 1
Darsaut TE, Lanzino G, Lopes MB, Newman S. An introductory overview of orbital tumors. Neurosurg Focus 2001;10:E1.  Back to cited text no. 2
Pelo S, Gasparini G, Boniello R, Moro A, Amoroso PF. Aneurysmal bone cyst located in the mandibular condyle. Head Face Med 2009;5:8.  Back to cited text no. 3
Hashemi SM, Heidarpour M, Eshaghian A, Ansari P, Hashemi MS, Yaghoobi M, et al. A rare case of aneurysmal bone cyst in the paranasal sinus. Iran J Otorhinolaryngol 2015;27:395-9.  Back to cited text no. 4
Senol U, Karaali K, Akyüz M, Gelen T, Tuncer R, Lüleci E. Aneurysmal bone cyst of the orbit. AJNR Am J Neuroradiol 2002;23:319-21.  Back to cited text no. 5

Correspondence Address:
Aarti Tyagi
Department of Pathology, Max Super Speciality Hospital, Saket, New Delhi - 110 017
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_317_18

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