Sigmoid Cavernous Hemangiomas: Overlooked and Misdiagnosed

Abstract

Gastrointestinal hemangiomas and vascular malformations such as cavernous hemangiomas are benign and rare tumors. To date, approximately 100 cases have described recto-sigmoidal hemangiomas. These entities are usually misdiagnosed and have the potential to produce massive hemorrhage, with nearly 40% mortality as previously seen cases remaining untreated. A 44-year- old African American female presented with altered bowel habits for over a year, associated with a sensation of rectal itching and burning, and intermittent rectal bleeding for the past 5 years, which she attributed to hemorrhoids. Previously, she had a recto-sigmoidectomy with end colostomy for drainage of an intra-abdominal abscess. Physical examination revealed a large ventral hernia and conjunctival pallor. Laboratory analysis included a hemoglobin level of 10.2 mg/dL, MCV 80 fL, and MCHC of 30 g/dL. CT with and without contrast revealed multiple ventral hernias. The patient underwent colonoscopy and it revealed a large, 5 cm pedunculated polyp with a 3 cm long 1.5 cm wide stalk, in the sigmoid colon. Epinephrine was injected at the base of the polyp and an application of an endoloop. Hot snaring polypectomy technique achieved complete removal of the polyp, followed by the application of two endoclips. Polyp histology revealed sigmoid mucosa and submucosa demonstrating a submucosal cavernous hemangioma without evidence of epithelial dysplasia or malignancy. Inked margins of resection were benign. To date, nearly 100 cases have been described recto-sigmoidal hemangiomas. An estimated 80% of patients present with symptoms, of which nearly 60% include intraluminal rectal bleeding, 50% with chronic iron deficiency anemia, and 17% with obstructive symptoms. Unfortunately, 80% of cases undergo a minimum of one unnecessary surgical intervention as a result of misdiagnosis, as the average delay to diagnosis is approximately 19 years. Unlike our patient, some patients endorse symptoms stemming from a possible compression or invasion of adjacent structures, such as lumbar or perianal pain, hematuria, or metrorrhagia. Although CT, MRI and endorectal ultrasound may aid in the diagnosis, colonoscopy remains the procedure of choice for detection with subsequent eradication and complete resection. Clinicians must keep the differential of recurrent painless bleeding and iron deficiency anemia in the younger population broad, as entities such as sigmoidcavernous hemangiomas may remain undiagnosed for years.Figure 1Figure 2