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<p>Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as CD4+/CD56 + hematodermic neoplasm or blastic natural killer cell lymphoma, is a rare tumor classified as a dendritic cell neoplasm. Here, we report a case of BPDCN with pelvic invasion arising from the right buttock that was treated with radiation therapy alone. A 79-year-old Japanese man presented with symptoms that resembled those of an anal fistula during defecation. He noticed a mass in his right buttock 2 months earlier. Biopsy of the lesion confirmed the diagnosis of BPDCN. Physical examination revealed a 10 × 8&#xa0;cm ulcerated, dark red tumor extending from the right buttock to the anus. Computed tomography revealed a bulky mass infiltrating the skin of the right buttock into the rectum and prostate, accompanied by metastasis to the left inguinal lymph node. Radiation therapy alone, delivered at a total dose of 45&#xa0;Gy in 18 fractions to both the primary lesion and metastatic lymph node, achieved an excellent therapeutic response. Five months after irradiation, the patient died of sepsis resulting from the progression of an out-of-field lesion in the left pelvis; however, local control within the irradiated area was well maintained. This case highlights the efficacy of radiation therapy for BPDCN with bulky tumor formation and pelvic invasion. We also discussed the optimal radiation dose for BPDCN.</p>

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Radiation therapy for blastic plasmacytoid dendritic cell neoplasm with pelvic invasion: case report and review of literature

  • 期刊论文

  • 发表日期：

    2026年7月24日

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  • Masahide Anada,
  • Shigeo Takahashi,
  • Osamu Imataki,
  • Tomoya Ishida,
  • Takamasa Nishide,
  • Toshifumi Kinoshita,
  • Norimitsu Kadowaki,
  • Toru Shibata

摘要

Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as CD4+/CD56 + hematodermic neoplasm or blastic natural killer cell lymphoma, is a rare tumor classified as a dendritic cell neoplasm. Here, we report a case of BPDCN with pelvic invasion arising from the right buttock that was treated with radiation therapy alone. A 79-year-old Japanese man presented with symptoms that resembled those of an anal fistula during defecation. He noticed a mass in his right buttock 2 months earlier. Biopsy of the lesion confirmed the diagnosis of BPDCN. Physical examination revealed a 10 × 8 cm ulcerated, dark red tumor extending from the right buttock to the anus. Computed tomography revealed a bulky mass infiltrating the skin of the right buttock into the rectum and prostate, accompanied by metastasis to the left inguinal lymph node. Radiation therapy alone, delivered at a total dose of 45 Gy in 18 fractions to both the primary lesion and metastatic lymph node, achieved an excellent therapeutic response. Five months after irradiation, the patient died of sepsis resulting from the progression of an out-of-field lesion in the left pelvis; however, local control within the irradiated area was well maintained. This case highlights the efficacy of radiation therapy for BPDCN with bulky tumor formation and pelvic invasion. We also discussed the optimal radiation dose for BPDCN.