Marcaccini Gianluca, Seth Ishith, Novo Jennifer, Bautista Marcus, Ruhunage Lakal, Bhat Saiuj, Cuomo Roberto, Rozen Warren M
Department of Plastic and Reconstructive Surgery, University of Siena, 53100 Siena, Italy.
Faculty of Medicine and Surgery, Peninsula Clinical School, Monash University, Clayton, VIC 3199, Australia.
J Clin Med. 2025 May 25;14(11):3703. doi: 10.3390/jcm14113703.
Myopericytoma is a rare benign vascular tumour characterised by concentric spindle cell proliferation around blood vessels, often misdiagnosed due to its resemblance to other soft tissue masses. Dupuytren's disease (DD), a fibroproliferative disorder of the palmar fascia, causes progressive contractures, typically affecting the ring and little fingers. While these conditions are well-documented individually, their coexistence in the same region is rare and diagnostically challenging. This report highlights a 67-year-old male with longstanding DD and a recurrent palmar mass initially attributed to fibrosis. Magnetic resonance imaging revealed hallmark vascular features suggestive of myopericytoma, confirmed by histopathological analysis showing spindle cell proliferation and immunohistochemical positivity for alpha-smooth muscle actin and h-caldesmon. Concurrent DD, characterised by fibrosis and activated myofibroblasts, further complicated the clinical picture. PubMed, Scopus, Web of Science, and Embase databases were searched from January 1901 to December 2024, and 20 studies were found, reporting 41 cases of myopericytoma in hand and upper extremity. Histopathological analysis consistently showed spindle cell proliferation and smooth muscle actin positivity. Coexistence with DD was rare, highlighting the need for detailed imaging and histological evaluation for accurate diagnosis. This case emphasises the complexity of differentiating overlapping pathologies. Surgical excision of myopericytoma and tailored DD management yielded favourable outcomes. Further research into shared fibroinflammatory pathways, including tumour necrosis factor-alpha and interleukin-6, may enhance diagnostic accuracy and treatment strategies for overlapping conditions.
肌周细胞瘤是一种罕见的良性血管肿瘤，其特征是血管周围出现同心性梭形细胞增生，常因与其他软组织肿块相似而被误诊。掌腱膜纤维增生症（DD）是一种掌腱膜的纤维增生性疾病，可导致进行性挛缩，通常影响环指和小指。虽然这些疾病分别都有充分的文献记载，但它们在同一区域共存的情况很少见，诊断也具有挑战性。本报告重点介绍了一名67岁男性，患有长期的掌腱膜纤维增生症，手掌部有一复发性肿块，最初被认为是纤维化所致。磁共振成像显示了提示肌周细胞瘤的典型血管特征，组织病理学分析证实为梭形细胞增生，α-平滑肌肌动蛋白和h-钙调蛋白免疫组化呈阳性。同时存在的掌腱膜纤维增生症以纤维化和活化的肌成纤维细胞为特征，使临床情况更加复杂。检索了1901年1月至2024年12月的PubMed、Scopus、科学网和Embase数据库，共找到20项研究，报告了41例手部和上肢肌周细胞瘤病例。组织病理学分析一致显示梭形细胞增生和平滑肌肌动蛋白阳性。与掌腱膜纤维增生症共存的情况很少见，这凸显了进行详细的影像学和组织学评估以准确诊断的必要性。本病例强调了鉴别重叠病理情况的复杂性。肌周细胞瘤的手术切除和针对性的掌腱膜纤维增生症治疗取得了良好效果。对包括肿瘤坏死因子-α和白细胞介素-6在内的共同纤维炎症途径进行进一步研究，可能会提高对重叠病症的诊断准确性和治疗策略。
