Malignant reninoma with pulmonary metastasis and ATRX mutation treated with olaparib: a case report of specialized treatment - Takeaways - MDSpire

Malignant reninoma with pulmonary metastasis and ATRX mutation treated with olaparib: a case report of specialized treatment

  • By

  • Guo run Zi

  • Da-jiang Zhang

  • Dong-Lin He

  • Run-lin Feng

  • Chang-xing Ke

  • May 4, 2026

  • 0 min

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  • 1

    A 32-year-old woman with malignant reninoma and an ATRX mutation was initially misdiagnosed and underwent radical nephrectomy.

  • 2

    The patient experienced local recurrence and pulmonary metastasis, leading to a revised diagnosis of malignant juxtaglomerular cell tumor.

  • 3

    Olaparib therapy resulted in the resolution of the renal recurrent tumor, but pancreatic lesions and pulmonary metastasis progressed.

  • 4

    Next-generation sequencing identified an ATRX mutation, highlighting the importance of molecular targets in managing rare tumors.

  • 5

    This case underscores the need for combination therapies to address tumor heterogeneity and drug resistance in malignant reninoma.

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