Ozer Muhammet, Juneja Kirti, Mahdi Mohammed, Jannareddy Namrata, Gandhi Neel
Department of Internal Medicine, Capital Health Regional Medical Center, Trenton, NJ, USA.
Bharati Vidyapeeth Deemed University Medical College, Pune, Maharashtra, India.
Eur J Case Rep Intern Med. 2021 Mar 2;8(3):002315. doi: 10.12890/2021_002315. eCollection 2021.
Paratesticular leiomyosarcomas are uncommon malignant tumours that originate from smooth muscle and show aggressive tumour behaviour due to a high incidence of local recurrence and distant metastasis. Orbital metastasis is also rare and associated with a poor prognosis. Clinical suspicion plays an essential role in the diagnosis of orbital leiomyosarcoma because of its uncommon presentation. Comprehensive neuroimaging and histopathological work-up are warranted for definitive diagnosis and management. To date, surgical resection remains the best curative treatment. However, leiomyosarcomas are usually friable and unencapsulated, which makes surgery challenging. Also, whether adjunctive chemotherapy will alter the long-term prognosis remains to be determined. Herein, we report the case of a middle-aged man who presented with proptosis and decreased vision, who was subsequently diagnosed with metastatic orbital leiomyosarcoma from a primary paratesticular tumour. We performed orbital enucleation with negative margins. The patient is currently under systemic pazopanib chemotherapy.
Orbital metastasis from paratesticular leiomyosarcoma is rarely encountered and shows aggressive behaviour.Clinical suspicion plays an essential role in diagnosing orbital leiomyosarcoma as this is an uncommon and atypical cause of proptosis and can be easily misdiagnosed by physicians.Curative surgical treatment should be considered due to the high recurrence rate.
睾丸旁平滑肌肉瘤是一种罕见的恶性肿瘤，起源于平滑肌，由于局部复发和远处转移的发生率高，表现出侵袭性肿瘤行为。眼眶转移也很罕见，且预后不良。由于眼眶平滑肌肉瘤的表现不常见，临床怀疑在其诊断中起着至关重要的作用。需要进行全面的神经影像学和组织病理学检查以明确诊断和制定治疗方案。迄今为止，手术切除仍然是最佳的治愈性治疗方法。然而，平滑肌肉瘤通常质地脆弱且无包膜，这使得手术具有挑战性。此外，辅助化疗是否会改变长期预后仍有待确定。在此，我们报告一例中年男性病例，该患者出现眼球突出和视力下降，随后被诊断为原发性睾丸旁肿瘤转移至眼眶的平滑肌肉瘤。我们进行了切缘阴性的眼眶摘除术。该患者目前正在接受帕唑帕尼全身化疗。
睾丸旁平滑肌肉瘤的眼眶转移很少见，且表现出侵袭性行为。临床怀疑在眼眶平滑肌肉瘤的诊断中起着至关重要的作用，因为这是一种罕见且非典型的眼球突出原因，容易被医生误诊。由于复发率高，应考虑进行治愈性手术治疗。
