Background
Atypical choroid plexus papilloma is a recently introduced entity with intermediate pathological characteristics. These tumors are relatively rare and the optimal management of these tumors is a matter of debate. Therefore, we performed a systematic review and pooled analysis about the effects of adjuvant therapies upon outcome measures of these patients. We also compared these effects upon totally and partially resected tumors and pediatric and adult populations.
Methods
A systematic search of three databases based on inclusion/exclusion criteria was performed. Data extraction was separately performed by two authors, and the summarized data were presented in the form of tables. Pooled estimates of different outcome measures were calculated for each adjuvant therapy and presented separately for studies with pediatric, adult, or mixed populations.
Results
A review of 14 included studies consisting of 144 patients revealed the effect of adjuvant treatment on reduction of tumor recurrence, metastasis, and reoperation rates and increasing survival rates in patients with subtotal tumor resection. This advantage was not seen in the case of gross total tumor resection. Almost all outcome measures were more favorable in the pediatric population.
Conclusions
It can be concluded that whenever gross total resection is not feasible, the implementation of adjuvant therapy can improve the outcome and prognosis. In other cases, it should be decided on an individual basis. Also, more aggressive behavior and higher rates of recurrence and mortality in the adult population suggests the consideration of more aggressive adjuvant treatments for adult patients.
Germinoma is the most common type of intracranial germ cell tumors (GCTs). Pineal gland and suprasellar region are the most frequent sites of central nervous system (CNS) involvement. Intracranial masses caused by Langerhans cell histiocytosis (LCH) mimics features of CNS GCTs. LCH frequently involve spine and is the most common cause of vertebra plana in children. A 15-year-old boy presented with progressing symptoms of polydipsia, polyuria, general headache, nausea and severe back pain. Brain MRI showed brain tumor with simultaneous involvement of suprasellar region and pineal gland. An excisional biopsy of suprasellar mass was done. The pathologic assessment confirmed the diagnosis of germinoma. Patient's treatment continued accordingly. A spine MRI, done due to persistent backache, showed a vertebra plana. We reevaluated the primary diagnosis suspecting LCH. Germinoma of CNS was confirmed and a biopsy of vertebral lesion resulted in hemangioma. Thus we report a case of CNS germinoma with co-occurrence of vertebra plana. We emphasized the importance of histopathologic diagnosis of pineal/suprasellar masses and primary investigation of other CNS regions including spine for possible metastasis or comorbidities.
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