Clinical Characteristics of Malignancy-associated Secondary Achalasia: An 18-Year Assessment at a Cancer Hospital

Abstract

Introduction: Achalasia is an unusual disorder of esophageal motor abnormalities with an incidence of 1.6:100, 000 individuals. Achalasia secondary to neoplasia is extremely rare, the incidence is approximately 1:750,000 people. Achalasia in malignancy may be caused by direct infiltration of the lower esophageal sphincter, as a neoplastic infiltration of the myenteric plexus, pseudoachalasia, (PA) or as a paraneoplastic process. The purpose of this study is to evaluate the characteristics of achalasia associated with malignancy. Methods: Electronic medical records were retrospectively reviewed from January 1999 to May 2017 to identify patients with a diagnosis of achalasia and a malignancy. We identified 19 patients who had a nonesophageal malignancy and a diagnosis of achalasia. Age at diagnosis of cancer, at diagnosis of achalasia, sex, type of cancer and its stage, time interval between cancer and diagnosis of achalasia, treatment and responses were reviewed. Results: The median age at diagnosis of Achalasia was 56 years (19-85). There were 11 (58%) male and 8 (42%) female patients. The most common associated malignancy was Breast cancer, N=5 (27%), and Lymphoma, N=4 (21%). Other cancers included Lung, N=3 (16%), Carcinoid tumor, N=2 (11%), Myeloma, N=1 (5%), CLL, N=1 (5%), AML, N=1 (5%), Thymoma, N=1(5%) and Sarcoma, N=1 (5%). The median time interval between the diagnosis of cancer and a diagnosis of Achalasia was 58 months (range 2-120 months). In most patients the cancer diagnosis preceded the achalasia. All patients underwent confirmation by esophageal manometry. In 15/19 patients barium studies were available. All patients underwent endoscopy to rule out psuedoachalasia. In four patients achalasia was diagnosed before the cancer was diagnosed. The median time interval between Achalasia and onset of a primary malignancy in these patients was 24 months (range 12-72 months). Patients were treated for their primary cancers with chemotherapy, radiation and/or surgery. Eight patients underwent endoscopic dilatations, 11/19 had Botulinum Toxin A injection, 6/19 had both. Two underwent Heller myotomy. Response to treatment was favorable for 6 months after treatment, except in three patients. The median overall survival time was 3 years (0-16) after the achalasia onset. Conclusion: Malignancy associated secondary achalasia is very rare. The most common malignancy according to the literature is gastric but in our population breast cancer and lymphoma were seen.Table: Table. Clinical presentation of achalasia in cancer patients