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| CASE REPORT |
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| Year : 2022 | Volume
: 19
| Issue : 1 | Page : 41-44 |
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A giant aneurysmal bone cyst in the sinonasal area of a 7-year-old girl
Santosh Kumar Swain
Department of Otorhinolaryngology and Head and Neck Surgery, IMS and SUM Hospital, Siksha “O” Anusandhan University, Bhubaneswar, Odisha, India
| Date of Submission | 04-Oct-2021 |
| Date of Decision | 27-Oct-2021 |
| Date of Acceptance | 30-Oct-2021 |
| Date of Web Publication | 28-Jan-2022 |
Correspondence Address:
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/am.am_109_21
Aneurysmal bone cysts (ABCs) are vascular, cystic, benign osseous tumors that can expand rapidly and cause local destruction of surrounding tissues. These tumors are typically found in tubular bones and spines. An aneurysmal bone cyst is an extremely rare bony lesion in the head-and-neck region. We present the case of a 7-year-old girl with a left side nasal block and proptosis of the left eye for 3 months. On examination with anterior rhinoscopy, it showed a mass with a smooth surface inside the left nasal cavity. Biopsy confirmed the diagnosis of ABC, and she underwent complete endoscopic excision of the sinonasal mass. A giant ABC in the sinonasal area is highly uncommon, particularly in the pediatric age group. It is often confusing in its differential diagnosis with other malignant and vascular lesions of the head-and-neck region or sinonasal tract. Surgical removal is the treatment of choice.
Keywords: Aneurysmal bone cyst, paranasal sinus, pediatric patient, sinonasal area
How to cite this article:
Swain SK. A giant aneurysmal bone cyst in the sinonasal area of a 7-year-old girl. Apollo Med 2022;19:41-4 |
| Introduction | |  |
An aneurismal bone cyst (ABC) is a benign and blood-filled lesion of the bone which often originates from the spine and metaphysis of the long bones, particularly in the femur and tibia.[1] ABC can behave locally in an aggressive manner because of its rapid growth and osteolytic nature. The lesion can increase rapidly, resulting in weakening the affected bone, resulting in pathological fractures.[2] Approximately 2%–3% of cases of ABCs are found in the head-and-neck region, with the mandible being the most common site of occurrence.[3] Diagnosis of ABC is often challenging and confusing with other vascular or malignant lesions. Radiography often shows classic osteolytic lesions, where magnetic resonance imaging (MRI) articulates blood-filled spaces and fluid-fluid levels.[4] The mainstay of treatment of ABC is surgical intervention, including excision, curettage, and bone grafting.[5] ABCs are highly uncommon tumors in the head-and-neck region, particularly in children.
| Case Report | | |
A 7-year-old girl attended the outpatient department of otorhinolaryngology for the left side nasal block for 6 months and proptosis of the left eye [Figure 1] for 1 month. She had no history of trauma to neither face nor surgery in the sinonasal area. She had no pain in the left side of the face or eye. She had no history of bleeding from the nose. On examination with anterior rhinoscopy, it showed a mass with a smooth surface inside the left nasal cavity. There was no abnormality in ocular movement and double vision. The oral cavity, oropharynx, larynx, and neck and here blood values were normal. The computed tomography (CT) scan of paranasal sinuses revealed a significant soft tissue mass in the ethmoid and maxillary sinus. It was defined from the surrounding tissue by a thin rim of dense bone-like eggshell [Figure 2]. A biopsy from the nasal cavity revealed an aneurysmal bone cyst. | Figure 1: A 7-year-old girl with sinonasal aneurysmal bone cyst presenting with left eye proptosis
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 | Figure 2: Computed tomography scan of the paranasal sinus with a coronal view showing thin, bony septations of the expansile mass in the left sinonasal area
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The patient underwent complete endoscopic excision of the sinonasal mass under general anesthesia. The mass was a firm bony lesion with a septate compartment inside. The lesion was vascular, resulting in copious bleeding during excision. The coblation technique controlled the bleeding from the area. Histopathology study shows blood-filled cystic spaces separated by cellular septa containing fibroblasts, giant cells, and woven bone [Figure 3]. A final histopathological study showed the diagnosis of the ABC. A follow-up examination at 1 month, 6 months, and 1 year showed that nasal obstruction and proptosis of the same side eye resolved. A postoperative follow-up CT scan of the paranasal sinuses was done at 1 month, which showed no evidence of residual or recurrent lesion [Figure 4]. She maintained an excellent nasal airway with markedly cosmesis of the facial area. | Figure 4: Postoperative computed tomography scan of the paranasal sinuses showing no evidence of lesion
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| Discussion | | |
An aneurysmal bone cyst (ABC is a nonneoplastic, expansile lesion of the bone consisting of sponge-like cavities of different sizes, which contain blood or serum. Jaffe and Lichtenstein first reported ABC in 1942.[6] Aneurysmal bone cyst terminology was not derived from its histopathological pictures but from its similarity to an aneurysm on the radiographic findings. It appears like an aneurysm because of the contours of the affected bone, which expands. However, it has no pulsation and is not considered an actual aneurysm. ABCs are often uncommon clinical entities in the head-and-neck region. The exact pathogenesis of ABC is not clear, and it is thought that the lesion is due to a reactive process secondary to injury or vascular disturbances. It may occur secondary to raised venous pressure, which leads to bleeding followed by osteolysis. This osteolysis may promote more hemorrhage causing amplification of the cyst. ABCs are often associated with and thought to occur secondary to underlying pathology.[7] ABCs are usually found in patients younger than 20 and typically present with a painless mass in the head-and-neck region.[8] The clinical manifestations depend on their location in the head-and-neck region. The symptoms may be a headache, loss of vision, diplopia, proptosis, tooth mobility, hearing loss, and facial swelling.[8] Crucial clinical symptoms of ABC in the sinonasal tract are nasal obstruction, hyposmia, occasional nasal bleeding, headache, orbital swelling, and diplopia.[9],[10] ABC is more commonly seen in females and manifests in the first few decades of life.[10] ABCs at the skull base area are often present with neurological deficits such as anosmia or gait ataxia.[11] ABCs can also be asymptomatic or sometimes present with vague local pain and swelling, which cause delaying of the diagnosis. In this case, the patient was presenting with nasal block and proptosis in the affected side. Typically, patients with ABC in the sinonasal tract present with headaches and rapid swelling in the affected region. Based on the location and extension, the clinical presentations vary, and the symptoms may include diplopia, altered vision, loosening of teeth, and facial paresthesia.[7]
We need first to establish the diagnosis of the ABC, and other lesions with bleeding history should be ruled out. Lesions of the sinonasal area, such as vascular lesions such as angiofibroma or hemangioma, and malignant lesions often confuse the clinicians.[12],[13] The diagnosis of ABCs is often challenging because of several reasons.[14] This tumor is responsible for only 1%–2% of all the primary bone tumors.[15] Hence, many clinicians are unaware or ill informed about the ABCs. The initial diagnosis of ABC can be made based on a radiograph with a CT scan and MRI. The radiological features of ABC include cystic bone expansion, honeycomb, or soap bubble-like inner structure. Sometimes, it shows destruction of the bony cortex and periosteal reaction.[16] CT scan reveals the characteristics of the ABC. The lesion is seen as an expansile soft tissue mass. It is usually well defined from the surrounding tissue by a thin rim of dense bone-like eggshell due to periosteal bone formation, suggesting a slowly growing benign lesion. Sometimes, internal septations and fluid-fluid levels are seen. The internal septa are enhanced by contrast medium. MRI is considered more diagnostic, and it shows the clear internal structure of the ABC. In MRI, the lesion is seen as a mass surrounded by a thin, well-defined, low-signal rim, and the inside is loculated by low signal septa. The low signal rim is thought to be fibrous tissue. In MRI, the signals of one loculation differ from other adjacent loculation, and some loculations have the formation of fluid-fluid levels. In a T2-weighted image, fluid-fluid levels lead to loculations and cause the upper hypertense and lower hypointense parts.[17] The definitive diagnosis needs a histopathological examination of the surgical specimen. The gross appearance of ABC is sponge-like, consisting of blood-filled cavities separated by a thin fibrous septum. ABC has a multicystic structure, and the cavities are not lined by endothelium and, therefore, not a true cyst. Macroscopically, ABC is often described as blood filled sponge. Cytogenetic characterization along with histological study is expected to refine the diagnostic method for ABC.[18]
Preoperative angiography and embolization followed by complete surgical excision of the lesion is the effective mode of treatment in majority of cases. However, in some instances, this may be problematic. In recalcitrant type, other treatment options include radiotherapy and interferon. However, radiation therapy, particularly in young patients, has a high risk for causing long-term secondary sarcoma development. When weighing the risks and benefits of various treatment options in complex or unresectable cases, we need to consider the impact of late malignancy. ABC may occur secondary to underlying bone disease, for example, osteoblastoma, resulting in refractory to treatment. The nonsurgical treatment options include injection agents, embolization, radiation, and chemotherapy. These treatment options may act as a primary or adjuvant role with surgery, as they are less invasive and can target unresectable lesions because of the sites. One study showed 86% of patients with ABC were treated with an injection of pure alcohol solution into the tumor.[18] In this case, the sinonasal ABC was entirely excised by endoscopic approach, and the bleeding was controlled by coblation.
| Conclusion | | |
Aneurysmal bone cysts are benign but locally destructive lesions. They are uncommon clinical entity in the sinonasal tract of the head-and-neck region. In children, ABCs of the sinonasal area pose a diagnostic challenge, as they are rare and the symptoms are nonspecific. CT and MRI are essential screening tools for visualizing the ABCs and show characteristic unicystic or multilocular lesions with contrast enhancement of the vascular stroma and septa. Histopathological examination confirms the diagnosis. Complete surgical excision remains the gold standard treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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