Paraneoplastic polyneuropathy associated with inflammatory myofibroblastic tumor in a pediatric patient

Objective : We present the first case of a 14-year-old girl who developed paraneoplastic polyneuropathy associated with uterine inflammatory myofibroblastic tumor.

Method : We retrospectively analyzed the patient’s data from the database of Kocaeli University Medical Faculty Hospital.

Results : In August 2021, a 14-year-old girl who was examined for abnormal uterine bleeding for 4 months, was diagnosed with an uterine inflammatory myofibroblastic tumor. The patient was given two cycles of chemotherapy protocol including ifosfamide, vincristine, actinomycin-D anddoxorubicin. Afterwards, the patient developed febrile neutropenia and septic shock. For this reason, she was followed up in the pediatric intensive care unit for 10 days.Brain magnetic resonance imaging (MRI)was performed due to focal epileptic seizure. Brain MRI with clinical features was compatible with posterior reversible encephalopathy syndrome. Her electroencephalogram her age but no epileptiform anomaly. Electromyogram was taken when the patient developed respiratory distress in which auxiliary respiratory muscles were used.She underwent tracheotomy. Nerve conduction studies were consistent with severe sensorimotor polyneuropathy. Plasmapheresis together with IVIG was applied to the patient.

Conclusions : In the literature review, it was determined that there were cases that seen paraneoplastic pemphigus and opsoclonus-myoclonus syndrome associated with inflammatory myofibroblastic tumor.Although the paraneoplastic antibody panel studied from the patient was negative, clinical and EMG findings were supportive of paraneoplastic polyneuropathy.