A popular term for the growth pattern of the rare (0.2% of female genital tract tumours, more common in women under age 50) intravascular leiomyomatosis, an indolent uterine stromal tumour which grows extensively within vessels–from when its trivial synonym–clinically characterised by abnormal uterine bleeding, pelvic pain and uterine enlargement
Treatment Cytoreductive surgery, i.e. bilateral annexectomy and total hysterectomy; medical management with adjuvant progestin therapy is currently the most effective treatment for curing and preventing local recurrence. Aromatase inhibitors may be effective.
Behaviour Local recurrence occurs in 70%; metastasis is rare even after suboptimal surgical excision
Pathology The cut surface is yellow-orange with bulging polypoid projections.
Prognosis ± 85% 15-year survival, despite metastases
The tumour derives its sobriquet, spaghetti from the linguini-like extensions of strands of tumour within vascular channels that may course over long distances including to the lungs
Synonyms Benign metastasising leiomyoma, endolymphatic stromal myosis, endometrial stromal sarcoma, low-grade stromal sarcoma of uterus
Reference Anticancer Res. 2008 Sep-Oct;28(5(2):2869-74