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Presented by Peter Illei, M.D. and prepared by Shien Micchelli, M.D.
Case 4: This 12-month-old infant girl was born term via vaginal delivery who at 9 months of age was noted to have macrocephaly.
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Week 304: Case 4
This 12-month-old infant girl was born term via vaginal delivery who at 9 months of age was noted to have macrocephaly. CT scan and MRI demonstrated a 1.7 cm third ventricle tumor with ventriculomegaly.images/3_12_07_4a.jpg
images/3_12_07_4b.jpg
images/3_12_07_4c.jpgCorrect
Answer: Choroid plexus papilloma
Histology: This papillary neoplasm is composed of delicate fibrovascular stalks that are lined by a single layer of bland appearing cuboidal to columnar epithelium with round to oval, basally situated monomorphic nuclei. No mitotic figures are readily identified. These features are diagnostic of a choroid plexus papilloma.
Discussion: Choroid plexus tumors make up less than 1% of all brain tumors, but they represent 2-4% of brain tumors in children, and 10-20% of brain tumors in the first year of life. In general, choroids plexus papillomas are five times more common than choroids plexus carcinomas. Approximately 80% of choroid plexus carcinomas arise in children representing 20-40% of pediatric choroid plexus tumors. The majority of choroids plexus tumors arise in the lateral ventricle (50%), followed by the fourth (40%) and third ventricles (5%).
Histologically, the differential diagnosis of choroids plexus papilloma includes villous hyperthrophy (diffuse enlargement of histologically normal choroid plexus in both lateral ventricles), choroid plexus carcinoma (histologically malignant appearing tumor with solid areas, high mitotic activity, nuclear pleomorphism, necrosis and often brain invasion), and metastatic carcinoma.
Incorrect
Answer: Choroid plexus papilloma
Histology: This papillary neoplasm is composed of delicate fibrovascular stalks that are lined by a single layer of bland appearing cuboidal to columnar epithelium with round to oval, basally situated monomorphic nuclei. No mitotic figures are readily identified. These features are diagnostic of a choroid plexus papilloma.
Discussion: Choroid plexus tumors make up less than 1% of all brain tumors, but they represent 2-4% of brain tumors in children, and 10-20% of brain tumors in the first year of life. In general, choroids plexus papillomas are five times more common than choroids plexus carcinomas. Approximately 80% of choroid plexus carcinomas arise in children representing 20-40% of pediatric choroid plexus tumors. The majority of choroids plexus tumors arise in the lateral ventricle (50%), followed by the fourth (40%) and third ventricles (5%).
Histologically, the differential diagnosis of choroids plexus papilloma includes villous hyperthrophy (diffuse enlargement of histologically normal choroid plexus in both lateral ventricles), choroid plexus carcinoma (histologically malignant appearing tumor with solid areas, high mitotic activity, nuclear pleomorphism, necrosis and often brain invasion), and metastatic carcinoma.
