Madelung's Disease as the First Presentation of an Occult Gastric Adenocarcinoma

Abstract

Madelung's disease is a rare disorder defined as cervical benign symmetric lipomatosis. Its association to alcoholism, pulmonary pathology, and neck cancer has been reported. We first describe the occurrence of this syndrome during the course of a gastric cancer. A 65-year-old white Latino male patient was referred to our service for investigation of dyspeptic symptoms, weight loss, and small bilateral symmetric and painless cervical masses. Upper endoscopy revealed a pyloric ulcer, treated with a 3-month regimen of antibiotics and omeprazole. A new endoscopy revealed persistence of the gastric ulcer, and biopsy pathology report showed chronic inflammatory changes but no malignant cells. Two months after the biopsy, the patient was admitted to the Emergency unit for gastric obstruction. Gastric wall enlargement and distention were found at the operation. Intraoperative frozen section gastric biopsy was inconclusive. Resection was not performed and a gastroenteric anastomosis was constructed. Definitive pathology report revealed gastric adenocarcinoma with signet-ring cells. Thus, the patient underwent subtotal gastrectomy. Definitive pathology report confirmed diffuse signet-ring cell gastric cancer with duodenal invasion; 1 lymph node was positive for cancer. Postoperative course was uneventful, and adjuvant chemotherapy was performed. Gastric cancer recurred and the patient died 2 years after the operation. Madelung's disease did not show any progress during the disease-free period. Madelung's disease has been associated with head and neck cancers and now with gastric adenocarcinoma. We suggest that patients presenting with cervical lipomatosis also should be screened for occult gastric cancer and abdominal malignancy.