Myopericytoma Masquerading as Dupuytren’s Disease: A Case Report and Systematic Literature Review

TL;DR

A rare case of myopericytoma mistaken for Dupuytren’s disease is reported, highlighting diagnostic challenges and the need for detailed imaging and histology.

Contribution

The paper presents a unique case of coexisting myopericytoma and Dupuytren’s disease, emphasizing the importance of accurate diagnosis through imaging and histopathology.

Findings

Myopericytoma was confirmed via MRI and histopathology in a patient with Dupuytren’s disease.

A systematic review identified 41 cases of myopericytoma in the hand and upper extremity.

Coexistence with Dupuytren’s disease is rare and requires detailed diagnostic evaluation.

Abstract

Background: 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. Case Presentation: 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. Methodology: 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. Conclusions: 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.

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