Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx.
The nasopharynx is the upper part of the pharynx (throat) behind the nose. The pharynx is a hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus. The nostrils lead into the nasopharynx....
Attempting aggressive surgical resection is the first step in the management of newly diagnosed CNS PNETs, although studies have yet to demonstrate that the extent of resection is predictive of outcome.[1,2,3]
CNS PNETs are often amenable to resection; in reported case series, 50% to 60% of patients were totally or near-totally resected.[1,2]
After surgery, children with CNS PNETs usually receive treatment similar to that received by children with high-risk medulloblastoma.
Conventionally, patients are treated with radiation to the entire neuraxis with local boost radiation therapy, as given for medulloblastoma. However, the local boost radiation therapy may be problematic because of the size of the tumor and its location in the cerebral cortex. Also, there is no definitive evidence that craniospinal radiation therapy is superior to radiation to the primary tumor site alone in children with nondisseminated lesions.[1,2,3]
The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with high-risk medulloblastoma. Three-year to 5-year overall survival rates of 25% to 50% have been noted.[1,2,3]; [Level of evidence: 2A]; [Level of evidence: 3iiiB]
Children Aged 3 Years and Younger
Standard treatment options
Treatment of children aged 3 years and younger with CNS PNETs is similar to that outlined for children aged 3 years and younger with medulloblastoma. (Refer to the medulloblastoma Children Aged 3 Years and Younger section of this summary for more information).
With the use of chemotherapy alone, outcome has been variable, with survival rates at 5 years ranging between 0% and 50%.[6,7,8]; [Level of evidence: 2Di] The addition of craniospinal irradiation to chemotherapy-based regimens may successfully treat some children but with anticipated neurodevelopmental decline.[Level of evidence: 2A]