Cervical PEComa: Challenges in diagnosis and prognosis of a rare neoplasm

• Cervical PEComas are rare neoplasms which present a diagnostic challenge. • Large tumour size is an important clinical prognosticator in cervical PEComa. • All reported cases of cervical PEComa classified as benign by Folpe criteria behaved in benign fashion clinically. • Tumours ≤ 4 cm may be assessed with cone biopsy, and simple hysterectomy considered if no adverse pathologic features. • Molecular characterization and linkage with coordinated rare tumour registries may identify novel treatments.