An unusual cause of dyspepsia: oesophageal cavernous haemangioma

A 73-year-old gentleman presented to his local general practitioner with a sudden onset history for 24 h of dyspepsia. The symptoms were exacerbated with food over the period. He was referred to the medical assessment unit at The Horton Hospital, Banbury, UK. His medical history included: hypertension (on enalapril) and asthma (on a salbutamol inhaler as required). He was a retired construction manager who had worked for a long period in The Middle East and Japan. He took 2–3 cans of beer and 2–3 glasses of wine per week. He had been noted to have longstanding mildly abnormal liver function tests 12 years prior to admission with a predominantly elevated γ-GT. A chronic liver disease screen and ultrasound arranged previously at a private clinic had been unremarkable. In the medical assessment unit, clinical examination was unremarkable. His blood tests (including a Troponin to exclude significant cardiac event), electrocardiograph and chest X-ray were normal. He was started on a proton pump inhibitor with an outpatient gastroscopy requested. A gastroscopy was carried out 2 weeks later performed by a general practitioner (GP) endoscopist with the squamocolomnar junction seen at 36 cm with a 4-cm hiatus hernia. An abnormal area was found at 38 cm (Figure 1). The area was biopsied and subsequently bled briskly settling without intervention. Biopsies subsequently revealed only normal oesophageal squamous mucosa. The findings were felt to be likely oesophageal varices; however, the images were reviewed by the senior consultant gastroenterologist who noted an absence of variceal cords above the lesion and suggested an alternative diagnosis. As a result, a computed tomography (CT) scan was arranged to further delineate the lesion. Lesion noted on endoscopy of lower oesophagus. A CT showed a soft tissue abnormality containing calcification encompassing the lower oesophagus (Figure 2), which appeared to be vascular. An endoscopic ultrasound (EUS) was performed thereafter once again finding tortuous vessels in the oesophageal wall from 24 to 40 cm with numerous phleboliths noted. The diagnosis of cavernous haemangioma of the lower oesophagus was made. No further intervention was undertaken and the patient remains well 3 years later. CT scan of lesion showing soft tissue abnormality containing calcification encompassing the lower oesophagus. Haemangiomas are well known to arise from organs such as: skin, liver, kidney and brain; however, oesophageal haemangioma is a relatively a rare diagnosis, and although is known to be a submucosal tumour of the oesophagus it has been reported to be <5% of oesophageal benign tumours.1–3 The majority of cases are found incidentally at post-mortem or during a surgical operation; however, they can cause haematemesis or dysphagia due to their size.4–8 It occurs in men predominantly between the fourth and seventh decades.9–11 Benign haemangioma of the esophagus can be grouped histologically into cavernous, capillary and hamartomatous and arteriovenous malformations (AVMs) subtypes. A cavernous haemangioma is defined by the size of venous channels that are larger than capillaries. The differential diagnosis includes: malignant haemangioma, Kaposi’s sarcoma, benign metastasizing haemangioma and angiosarcoma.12 The findings at endoscopy have been described as a blue coloured protruding submucosal-like tumour appearing anywhere in the oesophagus and can also have atypical appearances including a reddish discolouration, a normal-appearing mucosa or an ulcerated appearance.4 In our case, the lesion looked very much like an oesophageal varix, and however did not appear in cords or have other areas suspicious of varcies on the contralateral wall. Also the patient had no clinical signs of portal hypertension and preserved synthetic liver function. On CT, oesophageal cavernous haemangiomas appear homogenous and isodense mass with calcification found.13 EUS can be used to further delineate the anatomy of the tumour. On EUS, haemangiomas range from anechoic to hypoechoic to isoechoic and involve the mucosal and submucosal layers with an intact muscularis propria.7,8 EUS can also confirm the absence of continuity with major blood vessels.7,8 Further imaging may be carried out with the use of magnetic resonance imaging (MRI) techniques. Characteristic MRI findings have included an isointense signal from the mass on T1-weighted images and a high signal on T2-weighted images.6,7 Other reports have described atypical features such as a high-intensity signal from the mass on both T1- and T2-weighted images.7 Diagnosis can be aided with a biopsy of the mass; however, this is often deferred due to the potential risk of haemorrhage (a rare occurrence),7 and the often confusion between the tumour with oesophageal varices leads to caution on the endoscopist’s part. Malignancy has been reported in some tumours predicating the need for surgical evaluation of the tumour; however, the risk of malignancy is greater with tumours >3 cm in size.14,15 Fluorodeoxyglucose-position emission tomography imaging can be of aid to differentiate the type of tumour assessing for the degree of oesophageal radiotracer. Radiotracer uptake is believed to be a prerogative of cancer in which there is an increased expression of glucose transport enzymes in cell membranes and enzymes of the first steps of glycolytic pathways.16,17 If diagnosis is certain, endoscopic resection is recommended, only if the tumour measures <2.5 cm in diameter, promising good clinical results.18 Treatment when indicated for a patient with symptomatic dysphagia, bleeding, for clarification of diagnosis12 or to exclude malignancy is via the surgical approach. Treatment options include resection by thoracoscopic approach or laparoscopic approach. Endoscopic polpectomy and ligation,7 or enucleation have been used with success for this tumour, especially when pedunculated and small in size (<25 mm).8,19 Esophagectomy has been described as a surgical procedure often carrying great morbidity and mortality, especially when for a benign lesion. Another form of therapy is potassium titanyl/yttrium aluminium garnet (KTP/YAG) laser therapy.20 The larger the size of the tumour along with any locally invasive tissue8 may predicate the need for surgical resection. Conflict of interest: None declared.