Cervical Rhabdomyosarcoma in an Endocervical Polyp of a 50-year-Old Patient with Intermenstrual Bleeding

Case Reports in Gynecologic Oncology
15 Apr, 2019 ,

A 50 year old Afro-Caribbean premenopausal female presented to her gynecologist with complaints of malodorous vaginal discharge, intermenstrual bleeding, and passing fleshy tissue per vagina for one month. She had no significant past medical nor family history, and denied loss of appetite, bloating, abdominal pain, or weight loss. On physical exam her cervix appeared grossly normal and the uterus was mobile and enlarged to 16 weeks size with palpable fibroids. During her workup, an endocervical polyp was visualized on hysteroscopy. Pathology review of endometrial curettings from a dilation and curettage (D&C) showed polypoid fragments of the endometrium, myometrium and cervical tissue with foci of primitive rhabdoid differentiation, suspicious for rhabdomyosarcoma. Immunohistochemistry of the primitive cells was positive for p53, myogenin, desmin, and SALL4 with high Ki-67 proliferation index of 40–80%.

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A 50 year old Afro-Caribbean premenopausal female presented to her gynecologist with complaints of malodorous vaginal discharge, intermenstrual bleeding, and passing fleshy tissue per vagina for one month. She had no significant past medical nor family history, and denied loss of appetite, bloating, abdominal pain, or weight loss. On physical exam her cervix appeared grossly normal and the uterus was mobile and enlarged to 16 weeks size with palpable fibroids. During her workup, an endocervical polyp was visualized on hysteroscopy. Pathology review of endometrial curettings from a dilation and curettage (D&C) showed polypoid fragments of the endometrium, myometrium and cervical tissue with foci of primitive rhabdoid differentiation, suspicious for rhabdomyosarcoma. Immunohistochemistry of the primitive cells was positive for p53, myogenin, desmin, and SALL4 with high Ki-67 proliferation index of 40–80%.

She subsequently underwent surgical staging including a hysterectomy, bilateral salpingo-oophorectomy, and pelvic and para-aortic lymph node dissection. Final pathology confirmed embryonal rhabdomyosarcoma arising in a 1.5 cm polyp in the anterior endocervix. The tumor was composed of nodules of hyaline cartilage, small blue cells in sheaths, and cells with rhabdoid morphology. The endometrium, ovaries, fallopian tubes, and ovaries were unremarkable. However, there was focal lymphovascular space and perineural invasion present. Immunohistochemistry of the final tumor specimen was positive for desmin (100%), myogenin (40–50%) and CD56 (100%) with high Ki-67 proliferation index of 50–60%. It was negative for AE1/AE3, synaptophysin, chromogranin, TTF-1 and CD10.

Systemic therapy with Vincristine, Dactinomycin and Cyclophosphamide (VAC) for forty weeks was recommended based on the data published by the Children's Oncology Group (COG). Based on this data, concurrent external beam radiation to 5040 cGy was given concurrently during weeks 6–10. The patient finished her treatment without any delays. She experienced grade 1 diarrhea and constipation that resolved with changes in diet and grade 1 mucositis that resolved with Nystatin oral suspension. Computed tomography scans of the chest, abdomen, and pelvis after completion of chemotherapy showed no evidence of disease. Six months after finishing treatment the patient is without any signs or symptoms of recurrence.