ORIGINAL RESEARCH article
Front. Oncol.
Sec. Hematologic Malignancies
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1544803
Follicular Dendritic Cell Sarcoma: A Great Mimicker with Unpredictable Clinical Course-Experience from a Tertiary Care Cancer Centre in India
Provisionally accepted
Ajas Ibrahim
Mohmad Hussain Mir*
Farhana SirajMushtaq Ahmad SofiAbrar Rasool KhandaySuhail Ahmad WaniSunil RegmiMudasir Hamid
Nisar Ahmad SyedFaisal GuruUlfat Ara WaniSumaira Qadri- Sher-I-Kashmir Institute of Medical Sciences, Srinagar, India
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Background: Follicular dendritic cell sarcoma (FDCS) is a rare mesenchymal malignant tumor derived from follicular dendritic cells. FDCS arises mainly from lymph nodes and rarely are extra nodal. Diagnostic dilemma occurs due to same micromorphology as other sarcomas and lymphomas. Curative radical resection is the standard therapy and adjuvant treatment is not defined. For unresectable disease, chemotherapy and radiotherapy is indicated with variable response rates. Due to its rarity standard treatment is not yet defined.Objective: To analyse the clinico pathological features, treatment patterns and survival outcome of FDCS cases in our Institution.Methodology: The study was conducted in the Department of Medical Oncology State Cancer Institute, Sher I Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Jammu and Kashmir, India. Biopsy proven FDCS patients were identified through the hospital based registry from 1st January 2020 to 31st December 2023.Results: A total of six patients were diagnosed during the study period. Median age was 28 years (range 21-51 years). There were four males and two females with male to female ratio of 2:1. Common symptoms were Abdominal pain (50%) and cough and dyspnoea (33.3%). Four patients (66.6%) had nodal involvement with retroperitoneum and mediastinum in two cases each. Three patients had extra nodal involvement with colonic in two and one with liver. Five patients were initially misdiagnosed as non-hodgkins lymphoma, soft tissue sarcoma,neurogenic tumor and carcinoma. Treatment offered was surgery, chemotherapy, targeted therapy, radiotherapy and observation. Four patients were alive at a median follow up of 12 months with three patients having no evidence of disease and one case living with disease. Two patients had succumbed to the disease.Conclusion: The study described clinicopathological characteristics, diagnostic challenges and management difficulties in FDCS patients. Due to rarity of this disease, high expertise is needed to diagnose FDCS otherwise it usually gets delayed.
Keywords: Dentritic cell, Follicular dentritic cell sarcoma, mimicker, histopathology, Immunohisthochemistry
Received: 13 Dec 2024; Accepted: 12 May 2025.
Copyright: © 2025 Ibrahim, Mir, Siraj, Sofi, Khanday, Wani, Regmi, Hamid, Syed, Guru, Wani and Qadri. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Mohmad Hussain Mir, Sher-I-Kashmir Institute of Medical Sciences, Srinagar, India
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