Background: Benign peritoneal cystic mesothelioma (BPCM) is a rare tumor of unknown origin, most frequently encountered in women of reproductive age. Etiology is unknown; definitions and terminology are confusing, and preoperative diagnosis is difficult. Several differential diagnoses must be considered.
Methods: Based on our own clinical experience and a review of the relevant literature, we address clinical challenges and controversies of importance.
Results: Current literature on BPCM is mostly based on small case reports. Complete surgical resection is recommended if possible. Nevertheless, recurrent disease is not uncommon. Clinical positive effects of various adjuvant medical treatments remain to be shown.
Conclusions: Lack of consistent definitions, various treatment approaches, and mostly short follow-up times make it difficult to draw any firm conclusions from published reports. The natural history of this rare disease is less than well clarified. When possible, in an individual patient, surgical resection with curative intent seems to be the treatment of choice.