Pranav J, Bansal Shivam, Barman Saptarshi, Kumawat Nivesh, Gowda Rohan, Dhingra Mohit, Kumar Arvind
Department of Orthopaedics, All India Institute of Medical Sciences, Rishikesh, India.
Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Rishikesh, India.
Indian J Orthop. 2024 Jun 7;58(8):1166-1169. doi: 10.1007/s43465-024-01193-5. eCollection 2024 Aug.
Deep angiomyxoma (DAM) is a slow-growing benign tumor with high risk of local recurrence after surgical resection. Although DAM in a lower extremity is extremely rare, clinicians must be aware of its possible occurrence. Suspicion can be made based on clinical examination and radiological imaging but final diagnosis is confirmed on histopathological examination and immunohistochemistry. We intend to present an extremely rare case of DAM in the knee, managed successfully with marginal excision.
A 4-year-old male child presented with the complains of a painless, progressively increasing, soft, non-tender, and fluctuant swelling in his right knee. The plain radiograph showed a non-calcified soft-tissue swelling and MRI revealed a multi-loculated cystic lesion with multiple septations. A pre-operative diagnosis of a benign cystic lesion was made. It was managed by marginal excision of the tumor and a histological diagnosis of DAM was made. IHC staining showed positivity for SMA, CD34 and vimentin were focally positive, while desmin and calponin were negative. At 12 months of follow-up, the patient had a normal painless gait and full knee ROM, without any local recurrence.
DAM is a rare tumor which is often misdiagnosed. In this report, we present a rare case of benign cystic lesion which turned out to be DAM on HPE of resected specimen. Marginal excision of this lesion revealed good outcomes with no recurrence until 12 months of final follow-up. With this, we conclude that surgical excision should be the gold standard in cases of DAM.
深部血管黏液瘤（DAM）是一种生长缓慢的良性肿瘤，手术切除后局部复发风险高。尽管下肢DAM极为罕见，但临床医生必须意识到其可能发生。可根据临床检查和影像学检查进行怀疑，但最终诊断需经组织病理学检查和免疫组织化学确认。我们打算介绍一例极其罕见的膝关节DAM病例，通过边缘切除成功治疗。
一名4岁男童因右膝无痛、逐渐增大、柔软、无压痛且有波动感的肿胀前来就诊。X线平片显示非钙化的软组织肿胀，磁共振成像（MRI）显示一个多房性囊性病变，有多个分隔。术前诊断为良性囊性病变。通过肿瘤边缘切除进行治疗，并作出了DAM的组织学诊断。免疫组织化学（IHC）染色显示平滑肌肌动蛋白（SMA）、CD34阳性，波形蛋白局灶性阳性，而结蛋白和钙调蛋白阴性。随访12个月时，患者步态正常无痛，膝关节活动范围正常，无局部复发。
DAM是一种罕见肿瘤，常被误诊。在本报告中，我们介绍了一例罕见的良性囊性病变病例，切除标本的组织病理学检查结果显示为DAM。该病变的边缘切除显示出良好的效果，直到最后随访12个月均无复发。据此，我们得出结论，手术切除应是DAM病例的金标准。
