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| LETTER TO EDITOR |
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| Year : 2012 | Volume
: 60
| Issue : 1 | Page : 126-128 |
Giant pediatric aneurysmal bone cyst of the occipital bone: Case report and review of the literature
Alok Umredkar, Rakshith Srinivasa
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| Date of Submission | 02-Nov-2011 |
| Date of Decision | 05-Nov-2011 |
| Date of Acceptance | 07-Nov-2011 |
| Date of Web Publication | 7-Mar-2012 |
Correspondence Address:
Alok Umredkar
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.93620
How to cite this article:
Umredkar A, Srinivasa R. Giant pediatric aneurysmal bone cyst of the occipital bone: Case report and review of the literature. Neurol India 2012;60:126-8 |
Sir,
Aneurysmal bone cyst (ABC) is a rare vascular and benign tumor-like lesion of bone. [1] They account for 1-2% of all primary bone tumors and cranial location is described in 3-6% of all cases, the occiput location is very rare. [2],[3]
An eight-year-old male presented with pain and bony swelling of the suboccipital region of one-year duration. On examination there were no neurologic deficits except an 8 × 10cm bony swelling in the suboccipital region. Computed tomography (CT) of head showed mixed density multi-loculated mass in the suboccipital region with bone destruction [Figure 1]a. Magnetic resonance imaging (MRI) of the brain revealed a large extra-axial 6 × 7 × 8cm bony mass with multiple small cystic spaces and fluid-fluid level with total bone destruction and peripheral wall enhancement after gadolinium, suggestive of aneurysmal bone cyst [Figure 1]b-d. He underwent midline sub-occipital craniotomy with total excision. The whole bony tumor was encapsulated and extradural. The tumor was defined all around and cut with midas rex drill with 5 mm normal bony margin and taken out in toto. The tumor was composed of multiple small cysts filled with liquefied brown blood in varying stages. Postoperative period was uneventful and CT head showed complete excision [Figure 1]e and f. Histopathology showed aneurysmal bone cyst. On follow-up at six months, he is doing fine and MRI brain showed no recurrence. | Figure 1: (a) Contrast CT head showing multi-loculated aneurysmal bone cyst of the occipital bone with total destruction of bone and peripheral wall enhancement. (b) Sagittal section of contrast MRI brain showing giant aneurysmal cyst with multiple cystic cavities with fluid-fluid level inside and wall enhancement. (c) Axial section of contrast MRI brain showing same tumor with occipital bone destruction and compression of cerebellum and brainstem. (d) Fluid attenuated inversion recovery FLAIR sequence of MRI brain showing same tumor with fluid-fluid level. (e, f) CT head showing total excision of aneurysmal bone cyst
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Only 16 cases of occipital aneurysmal bone cysts were described in the literature and the gender distribution was equal and the median age was 15 years [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] [Table 1]. The precise origin of ABCs remains uncertain, but most authors feel that ABCs are a reactive process secondary to trauma or a vascular disturbance. In some cases, ABCs are associated with an underlying lesion like giant cell tumors (19-39%), chondroblastomas, osteoblastomas, osteosarcomas, chondromyxoid fibroma, and fibrous dysplasia. Although the lesion is classified as benign, malignant transformation has been reported in 3% of patients. [5],[12] Recent genetic and immunohistochemical studies suggest that primary ABCs are tumors and not reactive tumor-simulating lesions. Moreover, a neoplastic basis for primary ABC was suggested by the demonstration of clonal chromosome band 17p13 translocations, placing the USP6 oncogene under the regulation of the highly active CDH11 promoter. [17]  | Table 1: Reported cases of occipital aneurysmal bone cysts in the literature
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In the cranium, ABCs can present with ptosis, [3] loss of vision, cranial nerve palsies, [5] signs of raised intracranial pressure, [4] seizures, and cerebellar signs. [6] Rarely, spontaneous intracerebral hemorrhage has been described. [12] Axial CT head can reveal a multi-loculated, expansive, osseous lesion with occasional fluid levels that may enhance peripherally after contrast. [6] MRI scan reveals multiple fluid levels within multiple cysts resulting from unclotted blood, separate from the soft tissue and medullary bone. Some cysts appear hyperintense on T1-weighted sequences due to methemoglobin. [18] In our patient, MRI showed similar findings. The staging of ABCs according to Enneking classification is: Stage I (latent), ABC remains static or heals spontaneously; Stage II (active), grows progressively but without cortical destruction; Stage III (aggressive), progressive growth with cortical destruction. [18] Pathologically, ABC usually involves both the inner and outer tables of the skull symmetrically and almost always has intracranial extension. [2] ABC may be divided into: Classic (containing cysts; 95%) and solid (5%). [18] Microscopically, ABCs appear as blood-filled cavernous spaces with a paucity of endothelial cells, separated by septa composed of spindle-celled fibrous tissue. [18] En-bloc resection is the treatment of choice with the lowest risk of recurrence. Reported recurrence rates vary from 20-70% with incomplete resection. [2],[18] But, the primary challenge in the management of cranial ABCs involves the inherent difficulty in accessing and completely excising the lesion, especially involving the skull base, orbit, and the paranasal sinuses. [2],[18] In these cases, partial excision or intralesional curettage with adjunctive therapy like embolization, cryotherapy or radiotherapy, should be considered. [2] In our patient, as ABC was restricted to the occipital bone only, total excision was possible.
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[Figure 1]
[Table 1]
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