title: Primitive neuroectodermal tumor of the jejunum; a case report and literature review.
abstract: OBJECTIVE
  The aim of this paper is to report an unusual presentation of extranodal follicular dendritic cell tumor of neck with spinal metastasis. Follicular dendritic cells are nonlymphoid immune accessory cells present in the germinal centers of lymphoid follicles and play a crucial role in the induction and maintenance of the humoral immune response. Tumors from these cells are rare and treatment modality poorly defined.
  
  
  METHODS
  A 37-year-old lady presented with recurrent neck swelling which was initially reported as malignant paraganglioma. The primary disease was treated with surgery and radiotherapy. Eleven years later, the patient presented with metastasis to the spinal cord. Subsequent immunohistochemical analysis of the primary site tumor and the metastatic deposits revealed it to be a follicular dendritic cell tumor.
  
  
  METHODS
  The patient was treated with surgery followed by radiotherapy to spine, and one and half year after treatment, the patient is doing well and has regained complete motor functions.
  
  
  CONCLUSIONS
  Metastasis to spinal cord for follicular dendritic cell tumor is very rare, and to the best of our knowledge, no such case has been previously reported in the scientific literature so far. In the present case, good local control was achieved with initial surgery and radiotherapy but resulted in distant failure after 11 years. This underlines the need for adjuvant systemic therapy, and understanding the biology of the tumor may help in formulating targeted therapy in the future for this rare disorder.
authors.affiliation: Department of Radiation Oncology Unit I, Christian Medical College Vellore, Vellore, 632004 Tamil Nadu, India.
authors.forename: Saikat
authors.initials: S
authors.lastname: Das
authors.forename: Thomas Samuel
authors.initials: TS
authors.lastname: Ram
authors.forename: Raju T
authors.initials: RT
authors.lastname: Chacko
authors.forename: Simon
authors.initials: S
authors.lastname: Pavamani
authors.forename: Perungulam N
authors.initials: PN
authors.lastname: Viswanathan
authors.forename: Faith R
authors.initials: FR
authors.lastname: Viswanathan
authors.forename: Susy
authors.initials: S
authors.lastname: Kurian
mesh_terms: D000293:Adolescent; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D013505:Digestive System Surgical Procedures; D005260:Female; D006801:Humans; D007580:Jejunal Neoplasms / Q000473:pathology* / Q000628:therapy; D009364:Neoplasm Recurrence, Local / Q000473:pathology; D018242:Neuroectodermal Tumors, Primitive / Q000473:pathology* / Q000628:therapy; D011878:Radiotherapy
chemical_list: D000970:Antineoplastic Agents