Case report: NTRK1-rearranged cervical sarcoma with fibrosarcoma like morphology presenting in a 13-year-old managed with a neo-adjuvant TRK-inhibitor and surgical excision.

This case report reviews the case of a 13-year-old patient who presented with a 9 cm NTRK1-rearranged cervical sarcoma with fibrosarcoma like morphology. At presentation the lesion filled her vagina and pelvis and any attempt at surgical removal would have been morbid and led to loss of fertility. These neoplasms are extremely rare with 18 cases of the uterine cervix reported in the literature, none of which have occurred in a paediatric patient, and none of whom have received neo-adjuvant therapy prior to excision. Based upon evidence that has shown good tolerability and responses of paediatric NTRK fusion-positive solid tumours to TRK inhibitors, both in the neo-adjuvant and upfront setting, this patient was managed with neo-adjuvant entrectinib. Following a dramatic reduction in tumour size confirmed by imaging, she underwent conservative fertility sparing surgery with final histopathology showing no residual disease.

Assessment of response to treatment in vulvar vestibulitis syndrome by means of the vulvar algesiometer.

OBJECTIVE: A pilot study was undertaken to investigate the utility of the vulvar algesiometer in correlating symptom improvement with treatment response. STUDY DESIGN: Women with a diagnosis of vulvar vestibulitis syndrome attending a specialist vulvar clinic were assessed with the vulvar algesiometer before and after treatment. RESULTS: The conditions of 25 patients had improved to the point of discharge, and readings from before and after treatment were compared. There was a statistically significant difference in the vulvar algesiometer readings before and after treatment and this improvement was reflected in patient response. Nine patients had no response to treatment and also no significant improvement in vulvar algesiometer score. CONCLUSION: The vulvar algesiometer provides an easy noninvasive means of assessing vestibular tenderness in vulvar vestibulitis syndrome that is well tolerated by the patients and provides a degree of biofeedback for them.

Cell cycle proteins as molecular markers of malignant change in vulvar lichen sclerosus.

Lichen sclerosus (LS) has a known association with the development of squamous cell carcinoma of the vulva. The purpose of this study was to investigate molecular markers, which could indicate premalignant changes. Multiple sequential vulvar biopsies were taken over a period of 11 years from a patient with longstanding LS. Immunohistochemical staining was used to demonstrate a range of molecular markers. Increased expression of p53 and Ki67 was found in areas of squamous hyperplasia (SH) and differentiated vulvar intraepithelial neoplasia (dVIN) which correlated with the subsequent development of invasive squamous cell carcinoma (SCC). Molecular changes have been found to accompany histologic changes in the progression of vulvar LS to malignancy. Such markers may prove a useful addition in the clinical management of these conditions.