Thoracic Follicular Dendritic Cell Sarcoma

Abstract

Follicular dendritic cell sarcoma (FDCS), which makes up 0.4% of all soft tissue sarcomas, is an uncommon low-grade malignant tumor that develops from follicular dendritic cells in germinal centres of lymphoid tissue. The pathophysiology of FDCS is unknown. It can arise in both nodal and extra-nodal areas harboring FDCs and is commonly diagnosed in middle-aged individual who are in their fifth decade of life. The extranodal lymph node locations include the liver, spleen, and GI tract, where FDCS occurs most frequently (79.4%). It is quite uncommon to develop a pulmonary follicular dendritic cell sarcoma. At the microscopic scale, the FDCS displays a wide range of architectural patterns, including fascicular, storiform, whorled, and diffuse patterns. It is frequently not considered a differential diagnosis for a spindle cell neoplasm because of its rarity, misdiagnosis, and diagnostic difficulties. There is no effective treatment for this uncommon tumor, and the value of adjuvant therapy is still debatable. The median survival period following surgery for thoracic FDCS is approximately 4.41 years, with a 5-year recurrence-free survival rate of 47%. Patients diagnosed with localized disease exhibit a 5-year overall survival rate of 55%, whereas those with metastatic disease have a lower rate of 38%.