Metastatic esophageal squamous cell carcinoma with paraneoplastic Raynaud phenomenon: a case report

Incident development of Raynaud phenomenon (RP) in older adults raises concern for secondary etiologies. Malignancy is an important, yet underrecognized, cause of RP. In particular, paraneoplastic acral vascular syndrome (PAVS) describes a spectrum of secondary vascular disorders including RP and has been described in a variety of malignancies. However, presentations with squamous cell histology are uncommon, particularly in the esophagus.

A 61-year-old Caucasian male with a history of alcohol use disorder, hypertension, and hyperlipidemia presented with 3 months of progressive dysphagia and digital ischemia. RP had developed concomitantly with dysphagia and progressed to digital ischemia prior to admission. Evaluation for rheumatologic causes of secondary RP was notable for positive antinuclear antigen with high titer. Cross-sectional imaging for evaluation of occult malignancy revealed circumferential thickening of the distal esophagus with upper abdominal lymphadenopathy. Endoscopic evaluation identified a large ulcerating mass in the distal esophagus and pathology confirmed squamous cell carcinoma (SCC). These findings resulted in a diagnosis of metastatic esophageal SCC with paraneoplastic RP.

Although paraneoplastic RP occurs most often in cases of adenocarcinoma, this case highlights the importance of considering malignancy in the evaluation of secondary RP even with underlying squamous cell histology.