Extracerebral choroid plexus papilloma in the pharynx with airway obstruction in a newborn: a case report

Abstract

Background: Choroid plexus papillomas (CPPs) are rare, usually benign, neoplasms originating in the central nervous system. In this study, we present the first case of a giant airway-obstructing CPP in the pharynx of a newborn. Case presentation: A cystic mass located in the pharynx was noted in a fetus at the 29th week of gestation. Elective cesarean section was performed at the 38th week of gestation with successful intubation and ex utero intrapartum treatment. On computed tomography, there was a huge airway-obstructing cystic mass in the choana and pharynx. Elective surgery with total excision was performed, and histological examination confirmed the diagnosis of CPP. Conclusion: We report the first case of an extracerebral airway-obstructing CPP in the pharynx of a newborn. Radiologic examinations are not enough for the diagnosis of CPPs, and complete excision of the tumor with histological confirmation is indispensable for accurate diagnosis and treatment.

Extracerebral choroid plexus papilloma in a newborn: a rare presentation

“…CPPs are positive for cytokeratin, S-100 and vimentin and negative for epithelial membrane antigen and glial fibrillary acidic protein. CPPs are negative for p53 as was also seen in our case 5…”

“…There is a central core consisting of fibrous tissue with vascular component. This projection forms the papilla, which is lined by ventricular ependyma, a single layer of cuboidal or columnar cells 5. Our patient had the characteristic findings of the same on histopathological examination.…”

“…Heterotopic extracranial CPP is an extreme rarity, and there is just one case report available in the English literature 5. In our patient, swelling was laterally placed, and there was no intracranial extension noted in the MRI.…”

“…CPPs are, theoretically, an important cause of hydrocephalus by increasing the production of cerebrospinal fluid (CSF) and were first reported by Guerard in 1932 in a 3-year-old child 5. CPPs are low-grade rare neoplasms of the central nervous system (CNS), accounting for 0.4%–0.6% of all intracranial CNS neoplasms 6 7.…”

“…In most cases, an increased intracranial pressure is the most common presentation, and other symptoms are dependent on the site of the lesion 4. Extracranial CPP is only once reported in the English literature in a newborn 5…”