PELVIC MASS IN VON RECKLINGHAUSENS DISEASE

BACKGROUND Neurofibromatosis type 1 (NF 1) or von Recklinghausens disease is associated with leiomyoma of gastrointestinal tract and urinary tract. NF 1 is rarely associated with uterine leiomyoma. However literature suggests that the association of NF 1 and uterine leiomyoma are not coincidental. CASE REPORT 42 years old unmarried woman with NF 1, presented with mass abdomen for past 6 months. Per abdomen examination revealed mass corresponding to the size of 30 weeks pregnant uterus .MRI and CT of abdomen and pelvis suggested heterogeneous space occupying lesion of 19x16x11 cm most likely fibroid uterus and least likely solid ovarian mass. Planned for laparotomy and proceed. Intraoperative diagnosis was fibroid uterus and proceeded with total abdominal hysterectomy and bilateral salphingo - oophorectomy. Microscopic examination confirmed multiple uterine leiomyoma. CONCLUSION Here is a rare case of NF1 associated with uterine leiomyoma. This is to emphasize that when patient with NF1 complaints of mass abdomen gastrointestinal leiomyoma and uterine leiomyoma should be ruled out. The incidence of uterine leiomyoma with NF 1 is rare and deserves to be explored in a large series of female patients with NF 1.