Rare Fibroid Syndromes

There are a number of rare fibroid syndromes that may present clinical challenges to the practicing gynecologist. This chapter provides information that will be helpful to the clinician managing rare fibroid disorders. Distinguishing characteristics of each syndrome are reviewed as these may provide important diagnostic clues to such cases. In addition to family history, rare fibroid syndromes present with a constellation of physical and systemic findings that can be distinguished by genetic testing, specific imaging modalities, and physical examination. Because of the rarity of these disorders, management cannot be based on large clinical trials but rather small case series and anecdotal reports. Most patients will benefit from a multidisciplinary approach, as well as multimodal imaging. Medical management is often based on extrapolation of drug studies for the treatment of common fibroid tumors. Combination medical therapy may stabilize disease in some cases.