Article
A primary intracranial neuroendocrine carcinoma in a 2½-year-old boy: an extremely rare tumor entity
Ein primär intrakranielles neuroendokrines Karzinom bei einem 2½ Jahre alten Jungen: Beschreibung einer seltenen Tumorlokalisation
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Published: | May 4, 2005 |
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Outline
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Objective
Neuroendocrine neoplasms arising outside the appendix are very rarely observed in the pediatric population. Some authors describe primary sites of this tumor type in the lung or the liver. A neuroendocrine tumor with its primary location in the cranium has not been described so far.
Methods
A 2½-year-old boy was admitted to our hospital with the clinical sign of swollen lymph nodes in the cervical region. Initially he was diagnosed as an infective disease and was subsequently treated with antibiotics. During this treatment he deteriorated and a cranial MRI was performed. This showed a large tumor of the skullbase with an intracerebral cystic portion in the left frontal lobe. Tumor staging of the chest and the abdomen revealed no other tumors. An endoscopic guided biopsy of the tumor demonstrated an undifferentiated neuroendocrine carcinoma. The boy was treated with 3 cycles of chemotherapy and already after the second one the tumor showed retractable regression.
Results
Because of the largely destroyed skull base an operation of the remaining parts was performed and the skull base was reconstructed 16 weeks after first diagnosis. The post operative course was uncomplicated and a further chemotherapy was attached. Several MRI scans during the following month showed a complete tumor regression. A last MRI one year after first diagnosis showed a wide meningeal spread of the tumor and in approval with the parents further therapy was cancelled.
Conclusions
In the actual literature a neuroendocrine carcinoma with its primary site at the skull base and a large intracerebral part isn’t described so far. Regarding to the different types of chemotherapy for different tumors 6 we highly recommend that even in young patients with a skull base tumor with difficulties in histological classification a neuroendocrine tumor should be considered as a differential diagnosis in such type of lesion.