Background: Hearing impairment and tinnitus are forgot- ten features of Parinaud Syndrome originally discussed by Henri Parinaud in 1886. Methods:A retrospective case series of 3 patients who presented with pineal region germinomas and hearing loss. Results:A 15 years old male presented with morning headaches, blurred vision, bilateral papilledema, failure of upward gaze, bilateral tinnitus and decreased hearing acuity.Post-operatively, his tinnitus disappeared completely and his hearing normalized within one week.A 16 years old male presented with complete left sided hearing impairment with cranial nerve III palsy, right eye ptosis and worsening right-sided hearing loss; he had minimal recovery of his hearing after therapy.A 46 years old with a recurrent germinoma presented with hydrocephalus, bilateral ophthalmoplegia and hearing loss; his hearing loss improved after surgical resection. Conclusion:These case reports serve as a reminder that hearing loss can also be a component of Parinaud Syndrome.
TAFRO syndrome involves a constellation of symptoms including thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly. It was first described in 2010 as a subtype of idiopathic Multicentric Castleman’s Disease (iMCD). This report describes an atypical presentation in a 12-year-old male who suffered significant morbidity and was eventually diagnosed with TAFRO syndrome. Due to overlapping characteristics with other autoimmune processes and paucity of literature in pediatrics, TAFRO syndrome could present as a diagnostic dilemma to pediatric hematologists and pediatricians. Early recognition and timely treatment can help prevent significant morbidity as noted in our patient.
Midbrain gliomas are a group of slow-growing, low-grade tumors that arise from the tectum and/or tegmentum. Clinical symptoms at diagnosis are usually due to aqueductal compression resulting in non-communicating hydrocephalus and managed by an endoscopic third ventriculostomy. In the majority of patients, diagnosis is based on imaging characteristics, and only a subset undergo biopsy or resection. As a result, little is known about the histopathological and molecular genetic spectrum of midbrain gliomas. We reviewed a series of 58 consecutive patients (age range birth-52 years) with midbrain gliomas treated at our institution over more than 30 years. Anatomically, these tumors appear to fall into two distinct radiographic categories: limited to the tectum and/or the periaqueductal gray matter of the tegmentum (Group A) and diffuse tumors that include the tectum and infiltrate the pons and/or thalamus (Group B). Twenty-one of our patients (Group A – 6, Group B – 12, unknown -- 3) had biopsies or surgical resection, and tissue was available for histologic and molecular analyses including methylation profiling and BRAF mutation analysis. Most tumors with available tissue, regardless of radiographic group, were found to be low-grade gliomas, primarily pilocytic astrocytomas (13 pilocytic astrocytomas, 7 other low grade gliomas). Most of our patients had a benign clinical course, with 10% requiring no treatment, and 34% receiving only endoscopic third ventriculostomy or ventriculoperitoneal shunt. Radiographic group was significantly associated with likelihood to progress and with progression-free survival, with only 12.5% of Group A tumors progressing but 46.4% of Group B tumors progressing (p=0.008). In addition, progression –free survival was significantly longer in Group A patients. Overall, survival in our cohort was 100% over more than 30 years, reinforcing that these are treatable tumors with generally excellent outcomes.
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