血管内乳头状内皮增生 (IPEH)，也称为马森瘤，是一种罕见的旺盛的组织血栓形式，可能发生在血管、血管肿瘤或血肿内，并可能改变成像外观以模仿侵袭性过程。必须在病理上与血管肉瘤相鉴别。它们最常见于浅表软组织肿瘤，但很少描述其快速生长和对骨骼的影响。我们介绍了一名患有软组织血管瘤并伴有骨内扩展的 IPEH 患者的病例，该患者表现为右肱骨病理性骨折，并伴有侵袭性骨病变。 CT 和 MRI 显示整个右上肢存在多灶性不明确的软组织肿块，其下有皮质隧道和肱骨近端扇形。类似的影像学结果也出现在肱骨远端和同侧肩胛骨中，并在她住院期间演变。经皮活检显示血管瘤具有乳头状内皮增生并骨内扩展的特征，患者因无关的感染性休克在 ICU 中死亡。尸检证实了诊断。原发性和继发性 IPEH 通常被描述为边界明确的孤立性肿块，最常见于浅表软组织。这例深部软组织血管瘤伴有II型IPEH、骨内扩展以及局部多室受累的影像学表现是非常不寻常的。在文献中报告该病例应该有利于与类似混杂肿块的病理相关性，并提出软组织血管瘤骨内扩展的可能机制。© 2024。作者。

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumor, is an uncommon exuberant form of organizing thrombus that may occur within a vessel, vascular tumor, or hematoma and may change the imaging appearance to mimic an aggressive process. It must be distinguished pathologically from angiosarcoma. They have been most commonly reported within superficial soft tissue tumors, and rapid growth and effect on bone are rarely described. We present a case of a patient with a soft tissue hemangioma with IPEH with intraosseous extension that presented with a pathologic fracture of her right humerus with an aggressive appearing osseous lesion. CT and MRI demonstrated a multifocal ill-defined soft tissue mass throughout the right upper extremity with underlying cortical tunneling and scalloping of the proximal humerus. Similar imaging findings were also present in the distal humerus and ipsilateral scapula and evolved during her hospitalization. Following percutaneous biopsy revealing hemangioma with features of papillary endothelial hyperplasia with intraosseous extension, the patient died in the ICU secondary to unrelated septic shock. Diagnosis was confirmed at autopsy. Primary and secondary IPEH have been generally characterized as well-defined solitary masses, most often in the superficial soft tissues. This case of a deep soft tissue hemangioma with type II IPEH, intraosseous extension, and imaging findings of regional multicompartmental involvement is very unusual. Reporting of this case in the literature should be beneficial for pathologic correlation with similar confounding masses as well as propose a possible mechanism for intraosseous extension of soft tissue hemangiomas.© 2024. The Author(s).