Clear cell sarcoma of the pancreas, an unusual cancer with unusual metastatic site or unusual primary site?

Abstract

Clear cell sarcoma (CCS) is a type of malignant tumor that can arise from tendons and aponeuroses. This malignant proliferation of cells with melanocytic lineage normally occurs in young patients, and it is normally identified in extremities. However, different sites including gastrointestinal organs are also described. Due difficulties in the molecular and histopathology evaluation, the diagnosis is often confused with malignant melanoma. Most cases are treated with surgical resection, but overall, the prognosis is poor. In this editorial, we will discuss a very interesting case of CCS identified in the pancreas. We will discuss the literature and controversies in the management of this type of cancer. Furthermore, we will address molecular strategies to be incorporated in those cases to better understand the primary location of the tumor. Finally, future perspectives of the field and new strategies of treatment will be described.

Keywords: Clear cell sarcoma; Soft tissue sarcoma; Metastatic cancer; Pancreatic cancer; Pancreatectomy

Core Tip: Clear cell sarcoma is a rare type of malignant tumor that can arise from tendons and aponeuroses. However, different primary sites including gastrointestinal organs are also described. Next generating sequencing evaluation can help identify molecular biomarkers that can help with the diagnosis and treatment. Although surgical resection is the mainstream treatment for most patients, the recurrence of the disease is common and normally is refractory to standard treatments including chemotherapy.