Paraneoplastic gastrointestinal motor dysfunction: Clinical and laboratory characteristics

OBJECTIVES: The aim of this study was to describe the clinical, manometric, and serological characteristics of 12 patients with paraneoplastic GI motor dysfunction and to assess the contributory role of diagnostic tests. METHODS: Twelve patients diagnosed with malignant tumors and GI motor dysfunction were identified at the Mayo Clinic from 1985 to 1996. RESULTS: Cancers identified were: nine small cell lung carcinoma (SCLC), one anaplastic lung adenocarcinoma, one retroperitoneal lymphoma, and one ovarian papillary serous adenocarcinoma. GI symptoms preceded the tumor diagnosis in all cases of SCLC (mean, -8.7 months, range, -1 to -24 months, n = 9). The diagnosis of a malignant tumor preceded the onset of GI symptoms in the three patients with other neoplasms (6, 12, and 24 months). Five of the nine patients found to have SCLC had no evidence of tumor on initial chest x-ray. One or more paraneoplastic autoantibodies were found in 10 of the 11 patients tested by autoimmune serology. Type 1 antineuronal nuclear antibody (ANNA-1 or anti-Hu) was detected in eight of the nine patients with SCLC (one patient was not tested). The patient with ovarian carcinoma had type 1 Purkinje cell cytoplasmic antibody (PCA-1 or anti-Yo). N-type calcium channel antibodies were found in one patient with SCLC, one with a retroperitoneal B cell lymphoma, and one with ovarian carcinoma. Gastric emptying was delayed in 89% (eight of nine tested) and 80% (four of five tested) had esophageal dysmotility. Autonomic reflex tests were abnormal in the seven patients tested. CONCLUSIONS: The diagnosis of paraneoplastic GI motor dysfunction requires a high index of clinical suspicion. A panel of serological tests for paraneoplastic autoantibodies, scintigraphic gastric emptying, and esophageal manometry are useful as first-line screening tests. Seropositivity for ANNA-1, PCA-1, or N-type calcium channel-binding antibodies should prompt further evaluation for an underlying malignancy even when routine imaging studies are negative.