447 Clinical Characteristics of Secondary Achalasia Associated With a Malignancy: A 20-Year Review From a Cancer Hospital

Abstract

INTRODUCTION: Achalasia is an uncommon neurodegenerative disorder of esophageal motor function with an annual incidence of 1.6:100, 000. Most cases are idiopathic. Achalasia secondary to neoplasia is extremely rare, with an incidence of 1: 750,000. AIM: To evaluate the incidence and characteristics of achalasia in patients with a malignancy at our institution between 1999-2019. METHODS: Electronic medical records were retrospectively reviewed from January 1999 to May 2019 to identify patients with a diagnosis of achalasia and a malignancy. Patiets with direct tumor invasion of the distal esophagus were excluded. We identified 28 patients who had a malignancy and a diagnosis of achalasia. RESULTS: The median age at diagnosis of achalasia was 58 years (19-85). There were 14 (50%) male and 14 (50%) female patients. The most common associated cancer was a hematologic malignancy (N = 9, 32%: lymphoma N = 4, leukemia N = 4, myeloma N = 1), followed by breast cancer, (N = 7, 25%). Other cancers included lung, (N = 3, 11%), carcinoid tumor, (N = 2, 7%), and one each of thymoma, sarcoma, esophagus, stomach, thyroid, larynx, and prostate. The median interval between the diagnosis of cancer and achalasia was 78 months (range 2- 364). In 21 patients, the cancer preceded the diagnosis of achalasia. All patients underwent endoscopy to rule out psuedoachalasia. Where high resolution manometry was done (N = 10/28, 36%), type 2 achalasia was seen in 8/10 patients and type 1 achalasia in 2/10 patients. In seven patients, achalasia was diagnosed before the cancer. The median time interval between diagnosis of achalasia and diagnosis of a primary malignancy in these patients was 72 months (range 12-354 months). Anti-Hu antibodies were present in 2/8 patients who were checked for it, suggestive of a paraneoplastic process. Eight patients underwent endoscopic dilatations, 12 had Botulinum Toxin A injection into the lower esophageal sphincter and 6 had both procedures. 7 patients underwent a Heller myotomy. One is awaiting surgery. Response to treatment was favorable when assessed for improvement in dysphagia and eating ability for 6 months after diagnosis in 24/ 28 patients (85%). CONCLUSION: Malignancy associated secondary achalasia is very rare. In our population heme associated malignancy and breast cancer were the 2 cancers most commonly associated with achalasia. Patients with cancer who develop dysphagia should be screened for achalasia, as response to treatment is favorable.