Clinical characteristics of cancer-associated achalasia (CAA): A 25-year review from a cancer hospital.

Abstract

e24034 Background: Achalasia is an uncommon, usually idiopathic disorder of esophageal motor function (annual incidence 1.6:100, 000). Achalasia associated with neoplasia is rarer, with an (annual incidence 1: 750,000) . Achalasia in the presence of malignancy may be by direct neoplastic infiltration (pseudo achalasia), as a paraneoplastic process, or from cancer-associated achalasia (CAA). Aim: To evaluate the clinical characteristics and management of achalasia in patients with malignancy at MD Anderson Cancer Center between 1999-2024. Methods: Electronic medical records were reviewed from January 1999 to December 2024. Patients with tumor invasion of the distal esophagus and esophagogastric junction were excluded. Results: Forty patients met the study criteria (Table). Hematologic malignancies were the most common cancers associated with achalasia, seen in 10 (25%). Stage 4 cancer was the presenting stage in 17/ 30 (56%) of solid tumors. Cancer preceded the achalasia in 38 (95%) patients. The median time interval between cancer and achalasia was 56 months (range 1-270 months). Active cancer was present at the time of achalasia in 27 (67.5%). Anti-Hu antibodies were present in one patient. Treatment with Botulinum Toxin A injection was done in 15 (37.5%) patients. 2 patients had worsening symptoms after Botox injection, and both had leukemia-associated neutropenia. Conclusions: CAA was seen in only 40 patients over 25 years, most commonly associated with a hematologic malignancy and advanced staged solid tumors. Response to achalasia treatment was favorable except for two patients with leukemia-associated neutropenia. Demographic and clinical characteristics of cancer patients with achalasia n=40. Male (%) Female 25 (62.5)15 (37.5) Race/Ethnicity (%)WhiteHispanicBlackAsianOther 29 (72.5)2 (5)6 (15)2 (5)1 (2.5) Median Age at Cancer (range) 59 (28-91) Median Age at Achalasia (range) 66 (32-93) Cancer type (%) Heme Lung Gastrointestinal Breast Genitourinary Head and Neck Others 10 (25)7 (17.5)4 (10)5 (12.5)5 (12.5)3 (7.5) 6 (15) Cancer Stage (%) I II III IV 5 (12.5)5 (12.5)4 (10)17 (42.5) Local invasion (%) 0 (0) Chemoradiation therapy (%) 5 (12.5) Cancer preceded Achalasia Dx (%) 38 (95) Active Cancer at Achalasia Diagnosis 27 (67.5) Duration between Cancer and Achalasia Onset, median (range), months 56 (1-270) Achalasia Diagnosis (%) Standard Manometry EndoFLIPBarium Swallow 36 (90)23 (57.5)4 (10) Achalasia Phenotype (%) I IIIII 1 (2.5)30 (75)9 (22.5) Achalasia Symptoms (%) Weight loss Dysphagia Regurgitation Chest pain 18 (45)39 (98)35 (88)12 (30) Eckardt Score (%)0-6 7-12 12 (30)7 (17.5) Paraneoplastic Anti-Hu Antibody (%)Positive Negative 1 (2.5)4 (10) Achalasia Treatment (%) BotoxHeller Myotomy Endoscopic DilationPOEMNone 15 (37.5)5 (12.5)5 (12.5)8 (20)7 (17.5) Achalasia treatment Response (%)Good Partial None 21 (52.5)10 (25)9 (22.5) All-cause mortality (%) 15 (37.5)