Clear cell carcinoma of the pancreas: a case report of a not so clear disease

Background: Pancreatic ductal adenocarcinoma is one of the top causes of cancer-related deaths in the United States. Many cases are diagnosed late and overall five-year survival is poor, which can be attributed to genetics as well as tumor biology. Several subtypes exist but one unique variant known as clear cell carcinoma of the pancreas (CCCP) is thought to be more aggressive. Little has been studied about the course of this malignancy and most papers in the literature consist of case reports. As a result, there is a lack of quality evidence to provide clinicians with guidance on the management and treatment of this cancer. Case Description: Here, we describe a case report of CCCP diagnosed in a 47-year-old otherwise healthy female with the unique presentation of subcutaneous nodules and abdominal pain that developed after a recent minimally invasive hysterectomy. Her initial diagnosis was thought to be acute pancreatitis but recurrence of symptoms and further imaging displayed the rapid progression of a pancreatic mass with associated metastatic lesions. Her course has been complicated by a malignant bowel obstruction requiring a diverting ileostomy. She is currently receiving palliative chemotherapy for which she is tolerating well and her disease is being monitored with surveillance imaging. Conclusions: This paper highlights the rare clear cell variant of pancreatic cancer and displays the rapidly progressive course associated with this neoplasm. Unfortunately, there is a lack of research studying this tumor’s biology, and more efforts are needed to assist clinicians in managing these patients. Further studies have the potential to uncover therapeutic strategies targeting this rare disease with the hope of prolonging life expectancy.