Ganglioneuroblastoma: retroperitoneal

Case contributed by Dr Abbas M. Mahmood

Presentation

Repeated vomiting for three days associated with mild abdominal discomfort and mild headache for a one-week duration.

Patient Data

Age: 11 years
Gender: Male

There is well defined heterogenous soft tissue lesion at the right side of the retroperitoneal cavity located at the posterolateral aspect of the right kidney measuring (78mm length x 54mm width x 55mm AP diameter).

The lesion has heterogenous consistency and enhancing heterogeneously after administration of IV contrast.

There are multiple focal areas of liquefaction and few fatty locules at the peripheral aspect of the lesion.

The lower pole of the right kidney displaced medially & upward with a preserved fat plan between the lesion and the surrounding structures (lower border of the right lobe of the liver.the lower border of the right kidney, bowel loops, chest wall muscles, and paravertebral muscles. 

There is little fatty stranding surrounding the posterior border of the lesion.

Pathology

The mass already bisected in two different containers measuring (9x6.5x6 cm) cut section faint grey-white mass nodular at periphery associated with two separate pieces of fatty tissue.

Case 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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Case information

rID: 59704
Published: 22nd Apr 2018
Last edited: 14th Aug 2019
Inclusion in quiz mode: Included
Institution: The Arab Board Of Health Specializations

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