Retrorectal Cystic Hamartoma with Malignant Transformation

Abstract

Retrorectal cystic hamartomas are rare congenital lesions that can undergo malignant transformation, and adenocarcinoma is the most frequently described histological type. The authors describe a case of a 53-year-old female patient with a localized well-differentiated adenocarcinoma that developed in a retrorectal cystic hamartoma. The patient was submitted to surgery (a Kraske procedure), with an R1 resection, followed by adjuvant radio-chemotherapy. After 23 months of follow up, the patient remains free from disease recurrence. Given the rarity of this entity, this case allows us to reflect on the differential diagnosis, therapeutic approach and patients’ follow-up.