Spaghetti Tumour

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, clinically characterised by abnormal uterine bleeding, pelvic pain and uterine enlargement
Management 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% of cases; metastasis is rare even after suboptimal surgical excision
Prognosis ± 85% 15-year survival, despite metastases
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