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Case Study of NTRK1-Rearranged Cervical Sarcoma: Focus on Neoadjuvant TRK Inhibition and Surgery

By: Jenna Carter, PhD
Posted: Friday, October 8, 2021

The first known pediatric case of NTRK-rearranged cervical sarcoma was featured recently in Gynecologic Oncology Reports. Lois J. Eva, MBBS of the National Women’s Health, Auckland City Hospital, New Zealand, and colleagues treated a 13-year-old girl, who initially presented with a short history of vaginal discharge and bleeding, fevers, and night sweats. Neoadjuvant treatment with the TRK inhibitor entrectinib and subsequent fertility-sparing surgery appeared to be successful, with final histopathology showing no residual disease.

At presentation, a pelvic ultrasound was conducted and revealed a heterogenous solid vascular mass arising from the uterine cervix, filling the vaginal cavity and displacing the uterine body anteriorly. Further CT scans confirmed a lobulated enhancing mass distending the vagina and arising from a vascular pedicle in the right low posterior cervix, measuring 9.2 x 8.2 cm. A vaginal biopsy was conducted, and histopathology and immunohistochemistry reports revealed mitotically active spindle cells with a fascicular and herring-bone architecture and diffuse S100 and patchy CD34 expression.

The patient underwent a laparoscopic right oophorectomy for ovarian cryopreservation and fertility preservation; she was treated with empiric chemotherapy but declined further chemotherapy due to toxicity. The decision was made for a trial of neoadjuvant entrectinib. The patient received a total of 27 weeks of treatment, during which time symptoms improved. Further tumor surveillance with MRI confirmed a good response to treatment; at 12 weeks post initiation, there was a reduction in tumor size, with no residual disease seen at 30 weeks. A cone biopsy was then performed to excise the tumor bed.

Based on these findings, Dr. Eva and colleagues highlighted the need for further investigation of these tumors to identify more treatment options for patients that may avoid potentially morbid extensive surgery and impair fertility.

Disclosure: The study authors reported no conflicts of interest.  



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