Mixed malignant mullerian tumor with neuroendocrine features in an irradiated uterus for cervical carcinoma. A unique association? A morphological, immunohistochemisty and ultrastructural study

Pathologica. 2017 Dec;109(4):392-397.

Abstract

Chemo-radiation represents an effective therapy for carcinoma of the uterine cervix. The endometrium may however receive a consistent dose of mutagenic radiations and patients may have an increased risk of secondary malignancies. Endometrial mixed malignant mullerian tumor (MMMT) is a rare, highly aggressive disease, and neuroendocrine features are even rarer. A 68 years old woman underwent radio-chemotherapy for a squamous cell carcinoma of the cervix. Follow up was uneventful until, eight years after radio-chemotherapy, imaging exams detected a diffuse enlargement of the uterine body. Radical hysterectomy revealed a multiphasic lesion with both sarcomatous and mixed carcinomatous components. The carcinomatous, component presented neuroendocrine histologic and ultrastuctural features and an intense expression of neuroendocrine immunohistochemistry markers. No residual cervical carcinoma was documented (pR0). The patient died of disease after 9 months. Reported cases further demonstrate how the irradiation of the uterus for cervical cancer carries a not negligible risk of developing a second endometrial cancer. The second cancer may develop years after initial therapy and may have aggressive histologic and clinical features. This case underlines the importance for a long follow-up in women having received radio-chemotherapy alone.

Keywords: Immunohistochemistry; MMMT; Neuroendocrine tumor; Radio-chemotherapy; Ultrastructure.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Carcinoma, Squamous Cell / diagnosis*
  • Carcinoma, Squamous Cell / drug therapy
  • Carcinoma, Squamous Cell / pathology
  • Carcinoma, Squamous Cell / surgery
  • Female
  • Humans
  • Hysterectomy
  • Immunohistochemistry
  • Mixed Tumor, Mullerian / diagnosis*
  • Mixed Tumor, Mullerian / drug therapy
  • Mixed Tumor, Mullerian / pathology
  • Mixed Tumor, Mullerian / surgery
  • Uterine Cervical Neoplasms / diagnosis*
  • Uterine Cervical Neoplasms / drug therapy
  • Uterine Cervical Neoplasms / pathology
  • Uterine Cervical Neoplasms / surgery
  • Uterine Neoplasms / diagnosis*
  • Uterine Neoplasms / drug therapy
  • Uterine Neoplasms / pathology
  • Uterine Neoplasms / surgery
  • Uterus / pathology