Aneurysmal bone cyst of the ethmoid on fibrous dysplasia: A usual association within a rare location

Mortaji, H. El; Elghazi, Mohamed; Belhadj, Z.; Boutakioute, B.; Ouali, Meriem; Ganouni, N. Chérif Idrissi El

doi:10.1016/j.radcr.2019.07.015

Cited by 4 publications

(11 citation statements)

References 5 publications

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“…8 Radiotherapy and chemotherapy are not used in treatment of ABC due to the risk of developing secondary cancers, in particular osteosarcoma in the pediatric population. 2,3,7,8,12,13,16 Recurrence Rates. Most commonly ABCs recur within 2 years, with a higher frequency within the first year.…”

Section: Resultsmentioning

confidence: 99%

“…5,7,8,10,11 Although surgical excision is still widely accepted as the gold standard treatment, sclerotherapy, arterial embolization, denosumab, and interferon alpha-2a have been demonstrated as viable treatment options. [1][2][3]5,7,8,[12][13][14][15][16][17] We present a case of orbital ABC in a 17-month-old infant with confirmed UPS6 gene rearrangement, and review the literature with an emphasis on new developments in adjuvant therapies and diagnostic genetics.…”

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confidence: 99%

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Aneurysmal Bone Cyst of the Orbit With USP6 Gene Rearrangement

Phan

Tong

Krivanek

et al. 2022

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Purpose: Aneurysmal bone cyst (ABC) of the orbit is a very rare tumor, occurring mostly in the pediatric population, and can result in sight threatening complications and disfigurement. This review discusses previously reported cases with a focus on evolving treatment options and molecular genetics.Methods: We report the youngest case of an orbital ABC with a confirmed gene fusion: a 17-month-old girl, with confirmed FGFR-UPS6 (Fibroblast Growth Factor Receptor 1-ubiquitin specific peptidase 6/tre-2). A literature search for relevant publications on the topic was performed via Medline and PubMed, with the appropriate data extracted.Results: Thirty-two cases of orbital aneurysmal bone cyst were identified in the literature. Presentations are varied and can include pain, proptosis, decreased vision, and extraocular motility disturbance. Typical imaging and histopathology findings are discussed, in particular the usefulness of identifying USP6 gene arrangements. Treatment modalities are reviewed including surgery, embolization, and receptor activator of nuclear factor kappa-B ligand (RANKL) inhibitors. Recurrences can occur, usually within 2 years.Conclusions: Orbital ABC is a neoplasm that presents unique diagnostic and treatment challenges. Gene rearrangements can confirm primary ABC and rule out other underlying pathology. Disfigurement and sight threatening complications can occur due to both the disease process and with treatment. Outcomes may be improved with the use of systemic therapy.

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Section: Resultsmentioning

confidence: 99%

mentioning

confidence: 99%

Aneurysmal Bone Cyst of the Orbit With USP6 Gene Rearrangement

Phan

Tong

Krivanek

et al. 2022

Ophthalmic Plastic &Amp; Reconstructive Surgery

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“… 2 ABCs in the head and neck area are present in only 2% of cases. 4 The mandible and maxilla are the most frequently involved sites with a mandible predilection of 2:1 over the maxilla 4 ; with over 90% of cases reported above 30 years of age. 3 The involvement of the ethmoid bone as in our case is rare.…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, presentation depends on location, size and compressive effects of the mass on surrounding structures. 4 Neurologic deficits are more likely to happen when the skull base is involved. No neurologic abnormality was found in our patient where painless proptosis was the presenting symptom.…”

Section: Discussionmentioning

confidence: 99%

Aneurysmal bone cyst on top of fibro-osseous lesion of the ethmoid sinus with orbital and intracranial extension in a child

et al. 2022

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Objective: Aneurysmal bone cysts (ABCs) rarely involve the cranium. We report a case arising in the ethmoid sinus with orbital and intracranial invasion. Imaging suggested an associated fibro-osseous lesion. The lesion was completely resected. Histology confirmed the imaging diagnosis of ABC on top of an ossifying fibroma. A multi-disciplinary approach is essential for optimal surgical outcomes. Methods: We report a case of an 8-year-old boy with a 5 week history of painless, increasing prominence of the left eye. Clinical examination revealed non-pulsatile left proptosis. Visual acuity and ocular movements were normal. CT and MRI scans of the maxillofacial regions showed a large space-occupying lesion involving the left ethmoid air cells with left orbital and left inferior frontal intracranial extension. Multiple fluid levels with blood products were seen. Areas of the bony component of the lesion showed ground glass density on CT. Imaging was consistent with an aneurysmal bone cyst with an underlying fibro-osseous lesion; probably fibrous dysplasia which was confirmed after surgical removal of the lesion. A multi disciplinary team of maxillo-facial, neurosusugery and ENT surgeons performed the surgery. Results: Aneurysmal bone cyst arising from an osseous fibroma of the skull is rare. Total resection can be achieved with a multi-disciplinary surgical approach. Post-operative histology confirmed by the imaging findings.

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“…On a computed tomography scan, it appears usually osteolytic unilocular or multilocular radiolucent lesions. [ 7 8 ]…”

Section: Discussionmentioning

confidence: 99%

Unusual case presentation of an aneurysmal bone cyst of maxilla in geriatric patient

Doddawad

Shivananda

Ravi

et al. 2022

Journal of Oral and Maxillofacial Pathology

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An aneurysmal bone cyst (ABC) is a non-neoplastic, rapidly expanding pathologic bone lesion that mostly affects the mandible and is most commonly found in the first to third decade of life. The most of the ABC cases are locally aggressive osteolytic lesion with a high recurrence rate. As a result, we present a swelling with pus discharge at the maxillary alveolus in a 68-year-old female who was diagnosed with ABC after a computed tomography scan and histological analysis. The conservative surgical excision was performed without considerable bleeding, and no recurrence was observed even after a five-year follow-up period.

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Aneurysmal bone cyst of the ethmoid on fibrous dysplasia: A usual association within a rare location

Cited by 4 publications

References 5 publications

Aneurysmal Bone Cyst of the Orbit With USP6 Gene Rearrangement

Aneurysmal Bone Cyst of the Orbit With USP6 Gene Rearrangement

Aneurysmal bone cyst on top of fibro-osseous lesion of the ethmoid sinus with orbital and intracranial extension in a child

Unusual case presentation of an aneurysmal bone cyst of maxilla in geriatric patient

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