Abstract
Background Paraneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. Methods The UBC NMO clinic database was surveyed and identified 6 patients with paraneoplastic NMOSD. In 2 cases, aquaporin-4 (AQP4) immunoreactivity was assessed on patients’ tumor specimens. Results The mean age at NMOSD diagnosis was 56 years old and 5/6 patients were older than 50 years old. 4/6 patients with paraneoplastic NMOSD have positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months. Two new cancer types- serous ovarian carcinoma and adrenocortical carcinoma - were found in paraneoplastic NMOSD cases. A serous ovarian carcinoma and a thymoma, found in patients with AQP4 serological evidence, showed a positive reactivity to AQP4 immunostaining. Conclusions Our findings will increase the recognition of NMOSD as a paraneoplastic syndrome. Cancer cells can express AQP4, increasing the likelihood of a direct mechanism between cancer cells and the development of NMOSD in paraneoplastic cases.
| Original language | English |
|---|---|
| Pages (from-to) | 37-42 |
| Number of pages | 6 |
| Journal | Multiple Sclerosis and Related Disorders |
| Volume | 20 |
| DOIs | |
| Publication status | Published - Feb 1 2018 |
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Keywords
- Immunology
- Neuromyelitis optica
- Paraneoplastic Syndrome
ASJC Scopus subject areas
- Neurology
- Clinical Neurology
Cite this
Paraneoplastic Neuromyelitis Optica Spectrum Disorder : A single center cohort description with two cases of histological validation. / Beauchemin, Philippe; Iorio, Raffaele; Traboulsee, Anthony L.; Field, Thalia; Tinker, Anna V.; Carruthers, Robert L.
In: Multiple Sclerosis and Related Disorders, Vol. 20, 01.02.2018, p. 37-42.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Paraneoplastic Neuromyelitis Optica Spectrum Disorder
T2 - A single center cohort description with two cases of histological validation
AU - Beauchemin, Philippe
AU - Iorio, Raffaele
AU - Traboulsee, Anthony L.
AU - Field, Thalia
AU - Tinker, Anna V.
AU - Carruthers, Robert L.
PY - 2018/2/1
Y1 - 2018/2/1
N2 - Background Paraneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. Methods The UBC NMO clinic database was surveyed and identified 6 patients with paraneoplastic NMOSD. In 2 cases, aquaporin-4 (AQP4) immunoreactivity was assessed on patients’ tumor specimens. Results The mean age at NMOSD diagnosis was 56 years old and 5/6 patients were older than 50 years old. 4/6 patients with paraneoplastic NMOSD have positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months. Two new cancer types- serous ovarian carcinoma and adrenocortical carcinoma - were found in paraneoplastic NMOSD cases. A serous ovarian carcinoma and a thymoma, found in patients with AQP4 serological evidence, showed a positive reactivity to AQP4 immunostaining. Conclusions Our findings will increase the recognition of NMOSD as a paraneoplastic syndrome. Cancer cells can express AQP4, increasing the likelihood of a direct mechanism between cancer cells and the development of NMOSD in paraneoplastic cases.
AB - Background Paraneoplastic syndromes are remote effects of cancer caused by an autoimmune response triggered by tumor cells. Paraneoplastic Neuromyelitis Optica Spectrum Disorders (NMOSD) has been previously described, but the underlying mechanism for these rare cases is not well characterized. This paper presents a newly described case series of paraneoplastic NMOSD, including 2 new histological types of cancer and histological validation. Methods The UBC NMO clinic database was surveyed and identified 6 patients with paraneoplastic NMOSD. In 2 cases, aquaporin-4 (AQP4) immunoreactivity was assessed on patients’ tumor specimens. Results The mean age at NMOSD diagnosis was 56 years old and 5/6 patients were older than 50 years old. 4/6 patients with paraneoplastic NMOSD have positive AQP4 antibodies. The median time between NMOSD and cancer was 12 months. Two new cancer types- serous ovarian carcinoma and adrenocortical carcinoma - were found in paraneoplastic NMOSD cases. A serous ovarian carcinoma and a thymoma, found in patients with AQP4 serological evidence, showed a positive reactivity to AQP4 immunostaining. Conclusions Our findings will increase the recognition of NMOSD as a paraneoplastic syndrome. Cancer cells can express AQP4, increasing the likelihood of a direct mechanism between cancer cells and the development of NMOSD in paraneoplastic cases.
KW - Immunology
KW - Neuromyelitis optica
KW - Paraneoplastic Syndrome
UR - http://www.scopus.com/inward/record.url?scp=85039756288&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85039756288&partnerID=8YFLogxK
U2 - 10.1016/j.msard.2017.12.012
DO - 10.1016/j.msard.2017.12.012
M3 - Article
C2 - 29291482
AN - SCOPUS:85039756288
VL - 20
SP - 37
EP - 42
JO - Multiple Sclerosis and Related Disorders
JF - Multiple Sclerosis and Related Disorders
SN - 2211-0348
ER -