title: Clinicopathological Profile and Outcomes of Follicular Dendritic Cell Sarcoma of the Head and Neck Region - A Study of 10 Cases with Literature Review.
abstract: BACKGROUND
  Follicular Dendritic Cell Sarcomas (FDCS) are rare disorders of the lymph node and soft tissues. Accurate characterization of these neoplasms is important in planning optimal treatment given its potential for recurrence and metastasis.
  
  
  OBJECTIVE
  To analyse the clinicopathological profile and outcomes of a series of 10 cases of follicular dendritic cell sarcoma arising in the head and neck region diagnosed at our regional cancer centre.
  
  
  METHODS
  A series of 10 cases of FDCS of the head and neck region from the hospital registry of head and neck cancers diagnosed between 2007 and 2013 were collected and analysed retrospectively. Clinical details, pathologic features, immuno-phenotypic profile, treatment approach and outcomes over a period of 5 years were noted. The Recurrence Free Survivals (RFS) of all the patients were recorded.
  
  
  RESULTS
  The median age of the patients was 57 years and the mean age was 50.9 years. Male: female ratio was 7:3. In eight of the 10 patients, the tumours were located in the tonsils. All the tumour cells showed diffuse cytoplasmic CD21 and CD23 positivity by IHC. All the 10 cases underwent surgical excision of the tumour and three cases underwent additional neck dissection for cervical lymph node enlargement. All the patients with high grade tumours were uniformly managed with Tri-modality treatment (Surgery followed by radiotherapy and chemotherapy). The overall recurrence rate was 70%. The mean and median recurrence free survival was 39.6 and 44 months, respectively. Two of the three patients who remained recurrence free at the end of the 60 months had low grade tumours.
  
  
  CONCLUSIONS
  Early recognition of follicular dendritic cell sarcomas requires a high index of suspicion and bi-modality or tri-modality treatment may cure a subset of low and high grade tumours respectively and prolong recurrence in a large subset of patients. Surgery is the mainstay and the definitive modality of treatment; the advantages and benefit of adjuvant radiotherapy and chemotherapy are yet to be established. Tri-modality management may have a role in high grade patients which needs to be substantiated in future studies.
authors.affiliation: Associate Professor, Department of Pathology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Usha
authors.initials: U
authors.lastname: Amirtham
authors.affiliation: Fellow, Department of Pathology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Vidhya
authors.initials: V
authors.lastname: Manohar
authors.affiliation: Resident, Department of Medical Oncology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Mangesh Padmanabha
authors.initials: MP
authors.lastname: Kamath
authors.affiliation: Associate Professor, Department of Pathology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Premalatha Chennagiri
authors.initials: PC
authors.lastname: Srinivasamurthy
authors.affiliation: Professor and HOD, Department of Medical Oncology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Lakshmaiah Kuntegowdenahalli
authors.initials: LK
authors.lastname: Chennagiriyappa
authors.affiliation: Professor and HOD, Department of Head and Neck Oncology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Ashok Mohan
authors.initials: AM
authors.lastname: Shenoy
authors.affiliation: Professor and HOD, Department of Radiation Oncology, Kidwai Memorial Institute of Oncology , Bangalore, Karnataka, India .
authors.forename: Pramod Kallur Paramesh
authors.initials: PK
authors.lastname: Renuka
authors.affiliation: Professor and HOD, Department of Pathology, Kidwai Memorial Institute of Oncology, Bangalore, Karnataka, India.
authors.forename: Rekha Vijay
authors.initials: RV
authors.lastname: Kumar
keywords: Immunophenotype; Trimodality treatment