Clinical Case Summary
An 80-year-old man, with no history of alcohol or tobacco use, presented with a six-month history of oral mucosal erosions, odynophagia, dysphagia, dysphonia, and a significant weight loss. Physical examination revealed an abdominal mass. A CT and MRI showed a normal sized liver with regular contours and a large nodular hepatic lesion suggestive of hepatocellular carcinoma (HCC), along with two small satellite nodules and one large lymphadenopathy at the hepatic hilum. Labs showed elevated -fetoprotein levels of 190 ng/mL. He was referred to Hepatic Oncology and underwent an ultrasound-guided biopsy of the liver lesion, confirming well-differentiated HCC. The patient also developed erythematous-brown scaly plaques on the trunk and limbs. A skin biopsy was performed, and along with clinical and serological findings, confirmed the diagnosis of paraneoplastic pemphigus. High-dose Prednisolone was started and led to partial improvement. Two cycles of IVIG followed, with significant remission of skin lesions. He also began Sorafenib for HCC. After six months, follow-up imaging showed progression of the hepatic lesion, as well as tumor seeding in the thoracic wall along the needle tract of the liver biopsy. Treatment was escalated to Regorafenib, which was discontinued after two months. He died 16 months after diagnosis ,from traumatic brain injury. Paraneoplastic pemphigus is a rare autoimmune disease typically linked to hematologic cancers, with only four cases of associations with HCC described in the literature. This rare combination limits treatment options, particularly immune checkpoint inhibitors. In this case, immunosuppression may have impaired antitumor immunity, contributing to rapid tumor progression and biopsy needle tract dissemination.
References
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