Chylothorax and Chylous Ascites - A Rare Manifestation of Signet Ring Cell Carcinoma

Abstract

Introduction: Chylous ascites is the accumulation of milk-like peritoneal fluid rich in triglycerides in the abdominal cavity. Chylothorax is the accumulation of this lipid-rich fluid in the thoracic cavity, and has rarely been reported in the literature as a consequence of malignancy. We present a case of a patient with signet ring cell carcinoma presenting with chylothorax and chylous ascites. Case Presentation: A 68 year old man with severe COPD and atrial fibrillation presented with shortness of breath. A chest x-ray (Figure 1A) and CT Chest (Figure 1B) revealed a right pleural effusion. Multiple thoracenteses revealed cloudy, white fluid. Infectious and cytologic studies on the fluid, as well as a whole body PET-CT were negative for malignancy.Figure 1Three months later the patient was admitted for reaccumulating hydrothorax, as well as new-onset ascites. A diagnostic paracentesis revealed milky, white chylous fluid (Figure 1C). Ascitic fluid analysis revealed Triglycerides of 500, WBC 605 (76% lymph), Amylase 13, Total Protein 3.1, Albumin 1.6 and a SAAG of 2.1. Blood work was not consistent with cirrhosis. A triple phase CT revealed normal liver size and parenchyma, with no identifiable hepatic or intra-abdominal lesions. EGD revealed a small area of mucosal nodularity in the 2nd portion of the duodenum (Figure 2A). A colonoscopy revealed mucosal thickening in the rectosigmoid colon (Figure 2B). Biopsies of both lesions were positive for poorly differentiated adenocarcinoma with signet ring cells.Figure 2The patient initiated therapy with FOLFOX regimen, however ultimately opted for hospice and expired three months later. Discussion: Chylous ascites is rare, with an incidence of approximately 1 per 20,000 admissions over the last 20 years. In the setting of malignancy, chylous fluid extravasation is thought to result from obstruction of the lymphatic channels by either direct tumor cell invasion or mass effect. Chylous ascites and chylothorax are often with lymphoma. There are only a few reported cases of chylous ascites or chylothorax from signet ring cell carcinoma. All reported cases depict this finding as a late manifestation of metastatic disease. Conclusion: Chylous ascites and chylothorax are exceedingly rare manifesations of signet ring cell carcinoma. All reported cases have been diagnosed in the setting of late stage metastatic disease. These findings should prompt further evaluation for an underlying gastrointestinal malignancy with EGD and colonoscopy.