BACKGROUND CONTEXT: 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. PURPOSE: 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. STUDY DESIGN/ SETTING: Case series and systematic review of all reported cases in the English, Spanish and German medical literature were carried out. CASE SERIES: 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. REVIEW: 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.
BACKGROUND CONTEXT: 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. PURPOSE: 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. STUDY DESIGN/ SETTING: Case series and systematic review of all reported cases in the English, Spanish and German medical literature were carried out. CASE SERIES: 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. REVIEW: 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.