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<p>Primary hepatic lymphangioleiomyoma is an exceptionally rare neoplasm that poses a considerable diagnostic challenge due to its non-specific radiological features, often mimicking common hepatic tumors such as hemangioma. We present the case of a 39-year-old asymptomatic woman with an incidentally detected and progressively enlarging liver mass. Preoperative magnetic resonance imaging demonstrated a well-defined, lobulated lesion with enhancement patterns suggestive of hemangioma. The patient underwent successful open partial hepatectomy. Histopathological analysis revealed characteristic spindle-shaped smooth muscle cells proliferating around lymphatic channels. The diagnosis was confirmed immunohistochemically by co-expression of smooth muscle actin (SMA), HMB45, and Melan-A. The patient recovered well with no evidence of recurrence at 6-month follow-up. This case highlights that primary hepatic lymphangioleiomyoma, although rare, should be considered in the differential diagnosis of vascular liver masses in young women. Accurate diagnosis hinges on histopathology and characteristic immunohistochemical staining, with complete surgical resection offering an excellent prognosis for localized disease.</p>

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Lymphangioleiomyoma presenting as an isolated hepatic mass: a case report and literature review

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年8月4日

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  • Kun Liu,
  • Jin-Kai Feng,
  • Kun-Ye Luan,
  • Meng Li,
  • Da-Wei Guo,
  • Lin Gong

摘要

Primary hepatic lymphangioleiomyoma is an exceptionally rare neoplasm that poses a considerable diagnostic challenge due to its non-specific radiological features, often mimicking common hepatic tumors such as hemangioma. We present the case of a 39-year-old asymptomatic woman with an incidentally detected and progressively enlarging liver mass. Preoperative magnetic resonance imaging demonstrated a well-defined, lobulated lesion with enhancement patterns suggestive of hemangioma. The patient underwent successful open partial hepatectomy. Histopathological analysis revealed characteristic spindle-shaped smooth muscle cells proliferating around lymphatic channels. The diagnosis was confirmed immunohistochemically by co-expression of smooth muscle actin (SMA), HMB45, and Melan-A. The patient recovered well with no evidence of recurrence at 6-month follow-up. This case highlights that primary hepatic lymphangioleiomyoma, although rare, should be considered in the differential diagnosis of vascular liver masses in young women. Accurate diagnosis hinges on histopathology and characteristic immunohistochemical staining, with complete surgical resection offering an excellent prognosis for localized disease.