title: [Spindlecell soft tissue tumors of para- and retropharyngeal space: 5 patients, review of literature].
abstract: BACKGROUND
  The parapharyngeal space (PPR) is poorly accessible to clinical investigation due to its complex anatomy. Neoplasms in this area are rare, become lately symptomatic and are diagnosed in advanced stages. Multiple entities can be differentiated though malignant mesenchymal tumors are uncommon.
  
  
  METHODS
  We describe characteristic course of spindle cell soft tissue tumors in PPR based on 5 patients: 2 cases of synovial sarkoma (SyS), 2 cases of malignant peripheral nerve sheath tumor (MPNST) without neurofibromatosis, 1 case of Ektomesenchymoma (EM).
  
  
  RESULTS
  Both patients with SyS showed under multimodal therapy with surgery, chemotherapy and radiotherapy a rapid tumor progression and early pulmonary metastatic disease. The 2 patients with MPNST were free of recurrence after tumor resection and in one case after adjuvant radiotherapy. The patient with EM showed no recurrence after surgery.
  
  
  CONCLUSIONS
  Since malignant mesenchymal tumors of the PPR are unusual and also not have the typical behavior of metastatic epithelial neoplasms or malignant lymphoma, the diagnosis may be delayed. It is important to think in atypical lesions in PPR of the possibility of a soft tissue tumor and to arrange the necessary diagnostic steps. The imaging of choice of the PPR is the MRI, a staging adapted to the entity is necessary. The histological differentiation by immunohistochemistry and molecular genetics is complex, but is a vital to determine the optimal therapy. Diagnosis and treatment should take place in a specialized center.
authors.affiliation: Vivantes Klinikum im Friedrichshain, Hals-, Nasen- und Ohrenheilkunde, Kopf- und Halschirurgie, Plastische Operationen und Zentrum für Hörimplantate, Berlin.
authors.forename: N
authors.initials: N
authors.lastname: Prera
authors.affiliation: Hals-, Nasen- und Ohrenheilkunde, Universitätsklinikum Freiburg, Freiburg.
authors.forename: M
authors.initials: M
authors.lastname: Zorob
authors.affiliation: Klinik für Innere Medizin I, Universitätsklinikum Freiburg, Freiburg.
authors.forename: J
authors.initials: J
authors.lastname: Heinz
authors.affiliation: Klinik für Strahlenheilkunde, Universitätsklinikum Freiburg, Freiburg.
authors.forename: I
authors.initials: I
authors.lastname: Uhlig
authors.affiliation: Hals-, Nasen- und Ohrenheilkunde, Universitätsklinikum Freiburg, Freiburg.
authors.forename: W
authors.initials: W
authors.lastname: Maier
mesh_terms: D000293:Adolescent; D000328:Adult; D002277:Carcinoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D002574:Cervical Vertebrae / Q000473:pathology; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D004724:Endoscopy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D037981:Neck Dissection; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D010610:Pharyngeal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D010614:Pharynx / Q000473:pathology; D011379:Prognosis; D012509:Sarcoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*