An unusual angiofibrolipoma of the orbit: a case report
Hatim Bazhar, Moulay Omar Moustaine, Nabil Bouslous, Mohamed Lmejjati
Received: 18 Jun 2023 - Accepted: 05 Jul 2023 - Published: 24 Jul 2023
Keywords: Angiofibrolipoma, orbit, proptosis, therapeutic management, case report
©Hatim Bazhar et al. PAMJ - Clinical Medicine (ISSN: 2707-2797). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Cite this article: Hatim Bazhar et al. An unusual angiofibrolipoma of the orbit: a case report. PAMJ - Clinical Medicine. 2023;12:39. [doi: 10.11604/pamj-cm.2023.12.39.40785]
Available online at: https://www.clinical-medicine.panafrican-med-journal.com/content/article/12/39/full
An unusual angiofibrolipoma of the orbit: a case report
Angiofibrolipoma is a benign mesenchymal tumor and a rare histopathological subtype of lipoma which can be localized in several parts of the body, typically in the back, neck and shoulders. In the present report, we describe an unusual localization of this tumor through the case of a 75-year-old man, who consulted for an intermittent orbital swelling for 5 years, a radiological and an anathomopathological investigation confirmed the diagnosis of orbital angiofibrolipoma. To our best knowledge, such a case has not been previously reported in the literature.
Angiofibrolipoma is a benign mesenchymal tumor of mixed origin: fibrocytes, capillaries, and mature adipose tissue [1,2]. As a histopathological variant of lipoma, it usually develops in the fatty subcutaneous tissue, especially in the head and neck region [3,4]. Herein, we report a rare case of intraorbital angiofibrolipoma discovered following unilateral intermittent proptosis.
Patient information and timeline: a 75-year-old man with no prior medical or surgical history presented to the ophthalmology department complaining of intermittent swelling of the right eye for the past 5 years. The patient denied any other associated symptoms.
Clinical findings: the patient's best visual acuity was 20/20 in both eyes, examination of the left eye was within normal limits, and examination of the right eye revealed a painless, non-pulsatile axial proptosis of 23 mm that appears in forward bending position (Figure 1) while coughing or performing the Valsalva maneuver. The rest of the examination showed no pathological changes in both eyelids or conjunctiva except for a small-sized pterygium, extraocular movements were full without diplopia, slit lamp examination and fundoscopy were normal as was the intraocular pressure (13mmHg).
Diagnostic assessment: computed tomographic scan disclosed a noninvasive, well-defined intraconal lesion with hemorrhagic component and heterogenous enhancement. For further investigation, magnetic resonance imaging was performed and revealed a 28x20x28mm well-circumscribed intraconal tumor with poly lobed contours, heterogeneously hypointense on T1 and homogeneously hypointense on T2-weighted images. No signs of malignancy were described (no extension to the optic nerve, no invasion of extraocular muscles or bone lysis) (Figure 2). This radiological appearance was suggestive of an orbital hemangiopericytoma for which surgical excision was recommended.
Therapeutic intervention: surgically and via anterior orbitotomy, the tumor appeared as a bluish mass filled with blood with no necrosis. After macroscopic evaluation, the tumor was completely excised and sent to histopathology for examination (Figure 3).
Diagnostic assessment: the pathological examination of the tumor revealed mature adipose tissue, thick-walled anastomosing vascular channels, and fibrous material without any evidence of mitosis or cellular pleomorphism. No signs of malignancy were observed in all sections examined. This finding was consistent with the diagnosis of angiofibrolipoma (Figure 4).
Follow-up and outcome: after 6 months of follow-up, there were no clinical or radiological signs of tumor recurrence.
Informed consent: in order to respect the patient's right to privacy, informed consent was obtained before submitting the article.
Patient perspective: the patient's consent was obtained prior to the surgical procedure.
Despite its rarity, tumors of the orbit are a vast entity that encompasses a wide spectrum of primary lesions arising from all components of this rich anatomical structure . The orbital localization of lipoma is uncommon, yet when it does occur, it may result in local and non-specific symptoms such as displacement of the globe (proptosis) and limitation of ocular motility [6,7]. Histologically, lipomas are categorized as fibro-lipomas, angiolipomas, infiltrating angiolipomas, angiomyolipomas, and angiofibrolipomas based on their fat, muscle, blood vessel, and connective tissue content [1,3,4]. Angiofibrolipoma is a mixture of mature adipocytes, vascular tissue, and collagenous connective tissue, generally non-aggressive and well-separated from the surrounding tissues in spite of being non-encapsulated [1,8]. Its diagnosis can be assessed preoperatively by magnetic resonance imaging (MRI), except that it can be misled by other differential diagnoses such as hemangiopericytoma in our case, which highlights the importance of histopathological examination for confirmation [8,9]. As with any lipoma, the primary treatment for angiofibrolipoma is complete surgical excision. Long-term follow-up is recommended despite the low recurrence rate [2,3,8].
Angiofibrolipoma is a benign "mixed" neoplasm and a rare histopathologic variant of lipoma. Its orbital localization is unprecedented. Magnetic resonance imaging is efficient preoperatively for tissue characterization and extension assessment. However, complete surgical excision and histopathologic examination remain the most adequate tools for both diagnosis and treatment. The authors have described an extremely rare case of angiofibrolipoma of the orbit that has never been reported in the literature. This tumor should be considered as one of the possible diagnoses to be evoked in any patient presenting with a positional proptosis.
The authors declare no competing interests.
Hatim Bazhar drafted the initial article. Moulay Omar Moustaine, Nabil Bouslous and mohamed Lmejjati developed the idea of this scientific work and critically revised the article. All the authors have read and agreed to the final manuscript.
Figure 1: clinical presentation: axial proptosis of the right eye observed from below (onset after 1 minute in head down position)
Figure 2: magnetic resonance imaging: an intraconal lobulated mass, not infiltrating the surrounding structures observed within the right superolateral orbit; this mass appears heterogeneously hypointense on T1-weighted images (A) and homogeneously hypointense on T2-weighted images (B); after contrast injection (gadolinium), it enhances heterogeneously on both T1 and T2-weighted sequences (C, D)
Figure 3: the appearance of the lesion after surgical resection
Figure 4: (A, B, C, D) histological view of the lesion (hematoxylin and eosin stain) showing both fibreous and adipose tissue with numerous thick-walled vascular vessels
- Liu QL, Tian B, Zhang H, Qiao DS. Angiofibrolipoma of the spermatic cord. Asian J Androl. 2009 Nov;11(6):746-7. PubMed | Google Scholar
- Kucuker I, Keles MK, Yosma E, Polat V. Angiofibrolipoma of the tendon sheath. Case Reports Plast Surg Hand Surg. 2014 Oct 16;1(1):11-2. PubMed | Google Scholar
- Çetin MA, İkincioğulları A, Göktürk UG, Baran H, Ensari S, Hatipoğlu HG et al. Intranasal angiofibrolipoma. The Turkish Journal of Ear Nose and Throat. 2013 Sep 1;23(5):291-4. PubMed | Google Scholar
- Attia M, Megdiche I, Neji H, Belhadj A, Baccouche I, Zannad SH, M'rad KB. Angiofibrolipoma of posterior mediastinum with transforaminal extension. Rare Tumors. 2017 Mar 29;9(1):6811. PubMed | Google Scholar
- Kannan S, Hasegawa M, Yamada Y, Kawase T, Kato Y. Tumors of the orbit: Case report and review of surgical corridors and current options. Asian J Neurosurg. 2019 Jul-Sep;14(3):678-685. PubMed | Google Scholar
- Aljerian K, Fathaddin A, Almajed N, Kalantan H. Angiolipoma of the Orbit. Ophthalmic Plast Reconstr Surg. 2019 May/Jun;35(3):e81-e82. PubMed | Google Scholar
- Feinfield RE, Hesse RJ, Scharfenberg JC. Orbital angiolipoma. Arch Ophthalmol. 1988 Aug;106(8):1093-5. PubMed | Google Scholar
- Brkic A, Özçamur Ç, Gürkan-Köseoglu B, Olgac V. Angiofibrolipoma of the buccal mucosa: a case report. J Oral Sci. 2010 Mar;52(1):173-6. PubMed | Google Scholar
- Jacob A, Kneile J, Welling DB. Angiofibrolipoma of the ear canal. Laryngoscope. 2005 Aug;115(8):1461-2. PubMed | Google Scholar