Ganglioneuroblastoma - retroperitoneal

Discussion:

Neuroblastic tumors are tumors of the sympathetic nervous system, They arise from embryonal cells involved in the development of the sympathetic nervous system, which are neuroblastoma, ganglioneuroblastoma, and ganglioneuroma. The most benign of these is a ganglioneuroma, ganglioneuroblastoma has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three.

Ganglioneuroblastoma is less common than neuroblastoma and ganglioneuroma and occurs in 2-4 years (rare in adults) with M:F = 1:1.

In this case, an 11 year old male presented with repeated vomiting and headache referred for imaging.

Ultrasound showed a mass, CT was used to further evaluate the mass, and we did an MRI to exclude metastasis and other causes of a headache (which was normal apart from the incidental arachnoid cyst). 

He was operated and sent for histopathology the section revealed ganglioneurocytomatous tissue interspersed sharply defined, unencapsulated nests of variably differentiated neuroblastic cells. The tumor had pushed borders predominantly with foci of infiltrative border infiltrating the surrounding fat yet the margin of the excision was tumor-free. The section from separate fatty tissue was unremarkable

The overall features go with a ganglioneuroblastoma intermixed with schwannian stroma rich-type - a favorable prognosis according to the Shimada classification and is stage I (localized) according to the International Neuroblastoma Staging System (INSS).

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