RESUMEN
Craniofacial duplication (diprosopus) is a rare form of conjoined twins. A case of monocephalus diprosopus with anencephaly, cervicothoracolumbar rachischisis, and duplication of the respiratory tract and upper gastrointestinal tract is reported. The cardiovascular system remained single but the heart showed transposition of the great vessels. We present this case due to its rarity, and compare our pathologic findings with those already reported.
Asunto(s)
Adulto , Femenino , Humanos , Recién Nacido , Masculino , Embarazo , Anomalías Múltiples/patología , Anomalías Craneofaciales , Gemelos Siameses/patología , Anencefalia , Sistema Digestivo , Cara , Resultado Fatal , Defectos del Tubo Neural , Polihidramnios , Anomalías del Sistema Respiratorio , Disrafia EspinalRESUMEN
The clinicopathological features and immunohistochemistry profile of desmoplastic cerebral astrocytoma of infancy are discussed in a 4 month old male infant who presented with an increasing head circumference more pronounced in the last two weeks prior to admission to the University Pediatric Hospital. This is a rare tumor that occurs in infants within the first two years of life and it is characterized by a massive, often cystic, supratentorial lesion usually in the frontoparietal region. It has a biphasic histologic pattern with an astrocytic and desmoplastic component and a good prognosis after total or near total surgical resection. This patient represents the first case of desmoplastic cerebral astrocytoma of infancy diagnosed in the Puerto Rico Medical Center.