title: Early surgical occipitocervical stabilization for plasma cell neoplasms at the craniocervical junction: systematic review and proposal of a treatment algorithm.
abstract: BACKGROUND
  Plasma cell neoplasms (PCNs) of the craniocervical junction (CCJ) are rare. Because of their destructive growth, PCNs may induce spinal instability and harbor the risk of sudden death. Therefore, PCNs at the CCJ require special consideration. Although the commonly used primary treatment of PCN is radiotherapy (RT), treatment guidelines are inexistent for CCJ occurrences.
  
  
  OBJECTIVE
  This study aimed to conduct a systematic review of the literature, evaluate the benefit of early and extended surgical treatment followed by RT, and outline a treatment algorithm based on the data gathered.
  
  
  METHODS
  Case series and systematic review of all reported cases in the English, Spanish and German medical literature were carried out.
  
  
  METHODS
  
  
  
  METHODS
  retrospective clinical study, tertiary care center (2004-2014). Patients with a lesion of the CCJ (C0-C2) were identified. Clinical charts, imaging data, operative reports, and follow-up data were analyzed.
  
  
  METHODS
  a systematic literature review was performed using PubMed. Further manuscripts were identified by the web search engine Google.
  
  
  RESULTS
  Our series comprised four patients (one female, three males), mean age 58 years. There was one lesion of C1 and three of C2. Two patients with neck pain received vertebroplasty (C1 and C2, respectively) and RT as primary management. Both developed secondary instability of the CCJ after 12 and 5 months, respectively, and required occipitocervical stabilization (OCS). The other two patients underwent OCS and required no additional surgery and no signs of instability at follow-up. Forty-nine cases of OCS were published previously. Spinal stability was achieved significantly more frequently by OCS than by less invasive or medical interventional treatment options (p=.001; two-sided Fisher exact test).
  
  
  CONCLUSIONS
  Plasma cell neoplasms are highly radiosensitive. However, at the CCJ, a life-threatening instability may occur early and require surgical treatment. Based on personal experience, we favor OCS in this location. A systematic review of the literature supports this approach. We present a summary of our findings in a concise treatment algorithm for PCN of the CCJ.
authors.affiliation: Department of Neurosurgery, Universitätsklinikum Düsseldorf, Moorenstr. 5, Düsseldorf 40225, Germany. Electronic address: ahmadi@med.uni-duesseldorf.de.
authors.forename: Sebastian A
authors.initials: SA
authors.lastname: Ahmadi
authors.affiliation: Department of Neurosurgery, Universitätsklinikum Düsseldorf, Moorenstr. 5, Düsseldorf 40225, Germany.
authors.forename: Philipp J
authors.initials: PJ
authors.lastname: Slotty
authors.affiliation: Department of Neurosurgery, Universitätsklinikum Düsseldorf, Moorenstr. 5, Düsseldorf 40225, Germany.
authors.forename: Christopher
authors.initials: C
authors.lastname: Munoz-Bendix
authors.affiliation: Department of Neurosurgery, Universitätsklinikum Düsseldorf, Moorenstr. 5, Düsseldorf 40225, Germany.
authors.forename: Hans-Jakob
authors.initials: HJ
authors.lastname: Steiger
authors.affiliation: Department of Neurosurgery, Universitätsklinikum Düsseldorf, Moorenstr. 5, Düsseldorf 40225, Germany.
authors.forename: Jan Frederick
authors.initials: JF
authors.lastname: Cornelius
mesh_terms: D000328:Adult; D000368:Aged; D000465:Algorithms; D005260:Female; D006258:Head and Neck Neoplasms / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010954:Plasmacytoma / Q000601:surgery*; D013123:Spinal Fusion / Q000009:adverse effects / Q000379:methods*; D054854:Vertebroplasty / Q000009:adverse effects / Q000379:methods*
keywords: Craniocervical junction; Multiple myeloma; Occipitocervical junction; Plasmacytoma; Spine; Surgical treatment