Atypical presentation of a solid-variant orbital aneurysmal bone cyst with a literature review

Mallen, Jonathan R.; Caranfa, Jonathan T.; Zimmerman, Dale L.; Bulsara, Ketan R.; Falcone, Madina

doi:10.1016/j.neuchi.2018.10.001

Cited by 7 publications

(7 citation statements)

References 8 publications

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“…On CT, these expansile lesions have multilobulated cystic spaces, surrounded by a thin cortical shell with peripheral calcification, resulting in a soap-bubble appearance. [2][3][4]7,14,[20][21][22][23][24][26][27][28] Shifting fluid levels can be seen due to hemorrhage in various stages of lysis. 3,4,7,17,20,21,24,26 On MRI, there are multiple heterogeneous cystic spaces with fluid levels that have a hyperintense T2 and hypointense T1 signal.…”

Section: Resultsmentioning

confidence: 99%

“…ABCs appear as cystic structures divided by fibrous and osseous septations, but can also appear partially or predominantly solid. 3,7,13,27 These septae contain a cellular proliferation of bland spindle stromal cells, frequently with evidence of bone formation, with seams of osteoid associated with osteoblasts that commonly follow the contours of the fibrous septa, as well as haemosiderin laden macrophages and osteoclast-like multinucleated giant cells. 4,7,11,15,17,19,[22][23][24][25]28 There is presence of extracellular collagen, but unlike other cystic structures, there are no endothelial cells and no elastic tissues visible.…”

Section: Resultsmentioning

confidence: 99%

“…Symptoms and signs of orbital ABC4,[6][7][8][9][10][11][12][14][15][16][17][18][19][20][21][22][24][25][26][27][28][29][30][31][32][33][34][35] …”

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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%

Section: Resultsmentioning

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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“…140,141 They generally present as multicystic, hemorrhagic lesions though solid variants have been described. 142 Orbital ABCs typically present from childhood to early adulthood with rapid onset proptosis, visual loss, pain, and/or ophthalmoplegia. 140,142 Although benign, ABCs are locally aggressive, rapidly expansile tumors which, without treatment, can cause significant bony destruction, severe disfigurement, and blindness.…”

Section: Cystic Degenerationmentioning

confidence: 99%

“…142 Orbital ABCs typically present from childhood to early adulthood with rapid onset proptosis, visual loss, pain, and/or ophthalmoplegia. 140,142 Although benign, ABCs are locally aggressive, rapidly expansile tumors which, without treatment, can cause significant bony destruction, severe disfigurement, and blindness. 140,142,143 While ABCs are not considered true cysts due to their lack of epithelial lining, they demonstrate cystic features on both radiologic imaging and histopathology and are thus important differentials to consider.…”

Section: Cystic Degenerationmentioning

confidence: 99%

Cystic Lesions of the Orbit

Cameron¹,

Tong²,

Patel³

et al. 2023

International Ophthalmology Clinics

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Orbital cystic lesions encompass an array of congenital and acquired conditions with diverse aetiologies, ranging from benign, asymptomatic cysts to sight-threatening infectious or malignant disease. 1,2 They account for 14% to 21% of all orbital lesions. 2 Macroscopically, cystic lesions appear as saccular, fluid-filled structures encapsulated by a clearly demarcated wall. 1 They are commonly classified according to the cellular origin or etiology. 3,4 In a 2004 review of childhood orbital lesions, Shields and Shields 5 categorized cystic lesions by cellular origin (surface epithelium-derived, teratomatous, neural, secondary, infectious, and noncystic lesions with a cystic component). Another classification system, proposed by Kaufman et al, 6 in 1998, organizes cystic lesions by etiology; that is, congenital, acquired, or secondary cysts arising from adjacent structures. This review will expand upon the latter scheme, employing the following classifications; congenital (epithelial and nonepithelial), vascular, infectious and inflammatory, neoplastic, and miscellaneous. True cysts and cyst-like lesions, that is, those which appear cystic on imaging, will be covered, with a particular focus on the radiologic characteristics seen.Congenital lesions, such as dermoid cysts and orbital teratoma, generally present in infancy or early childhood and are usually benign; those which present later in life are more likely acquired, although lesions such as deep orbital dermoids may present in adulthood. [6][7][8] Clinical features vary depending on the size, depth, and aggressiveness of the underlying lesion. Superficial cysts often present with a painless,

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