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Paraneoplastic neurological disorders in children with benign ovarian tumors
Mei-Hsin Hsu1, Chao-Ching Huang2, Pi-Lien Hung1
1Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Insights
Paraneoplastic neurological diseases (PND) are not uncommon in children with benign ovarian tumors. Early detection and immunotherapy alongside tumor removal improve outcomes for serious PND.
Area of Science:
- Pediatric Neurology
- Gynecologic Oncology
- Neuroimmunology
Background:
- Paraneoplastic neurological diseases (PND) are rare but treatable neurological disorders.
- Paraneoplastic encephalitis is an emerging condition in children, often under-recognized.
- Benign ovarian tumors can be associated with neurological manifestations in pediatric patients.
Purpose of the Study:
- To determine the prevalence and spectrum of PND in children with benign ovarian tumors.
- To evaluate the long-term outcomes of PND in this pediatric population.
- To highlight the importance of considering ovarian tumors in pediatric patients with unexplained neurological symptoms.
Main Methods:
- Retrospective chart review of female patients under 18 with pathologically confirmed benign ovarian tumors.
- Inclusion criteria: tumor diagnosis between January 1993 and December 2010, and neuropsychiatric symptoms within 5 years of tumor diagnosis.
- Recording of clinical symptoms and relevant investigations.
Main Results:
- 133 pediatric patients with benign ovarian tumors were identified; most had mature teratoma.
- 4.5% of patients (6/133) exhibited neuropsychiatric manifestations, predominantly in those over 10 years old.
- Common symptoms included depression, headache, mutism, hypoventilation, seizures, and hallucinations. Serious PND (2.2%) included ADEM and anti-NMDAR encephalitis. All improved post-tumor removal, but one without immunotherapy had sequelae.
Conclusions:
- PND associated with benign ovarian tumors is a significant concern in pediatric patients.
- Ovarian tumor screening is crucial for adolescent females presenting with subacute, multi-level neurological involvement without a clear alternative diagnosis.
- Comprehensive treatment for serious PND includes both surgical tumor removal and immunotherapy.
Aim:
Paraneoplastic neurological diseases (PND) are rare, but potentially treatable disorders. Paraneoplastic encephalitis is rapidly emerging as an important but likely under-recognized condition in children. The aim of this study was to assess the prevalence and spectrum of PND in children with benign ovary tumor and the long-term outcome.
Patients And Methods:
We retrospectively reviewed the charts of all female patients below 18years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5years of tumor diagnosis and the related investigations were recorded.
Results:
There were total 133 children and adolescents with benign ovarian tumors, mostly mature teratoma. Six patients (4.5%) had neuropsychiatric manifestations and all but one were beyond age 10years. The most common neuropsychiatric presentations were depression or low mood (84%), headache (50%), mutism (50%), hypoventilation (50%), seizures (30%), hallucination (30%), vomiting and hypersalivation (30%). Three patients (2.2%) had serious PND including acute disseminated encephalomyelitis in 1 and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in 2. Although all of three improved after tumor removal, one without immunotherapy had neurological sequelae and prolonged ICU stay.
Conclusion:
The prevalence of PND in benign ovary tumor is not so uncommon in children. It is important to survey ovary tumors in female adolescents with subacute presentation of multiple-level involvement of neuraxis where no clear alternate diagnosis is possible. Treatment of serious PND associated with ovary tumors should include immunotherapy in addition to tumor removal.
