Ganglioneuroblastoma

Case contributed by Dr Abdallah Khateeb

Presentation

Abnormal chest x-ray.

Patient Data

Age: 4 years
Gender: Male
X-ray

Well defined soft tissue opacity that displaces the left posterior pleural junction line, defining its location within the posterior mediastinum. No obvious bone changes.

The lungs are unremarkable.

CT

A well defined extra-pulmonary posterior mediastinal soft tissue mass at the left paravertebral region, showing heterogeneous enhancement and few fine foci of calcification. There are no surrounding bone changes. This mass is abutting the descending thoracic aorta and the left subclavian artery.

An incidental finding is a bovine aortic arch.

Case Discussion

The differential diagnosis of a posterior mediastinal mass is fairly wide, but at this age, the sympathetic chain tumors predominate.

Histopathology

Microscopic examination shows two cell populations; the first neuroblastic cells are small round primitive cells with high nuclear to cytoplasmic ratio with scant cytoplasm, salt and pepper chromatin pattern and inconspicuous nucleoli. The second ganglionic cells are large cells with prominent nucleoli, abundant granular eosinophilic cytoplasm and distinct cell borders. The cells are arranged in an interspersed, sharply defined, an encapsulated  body with differentiate neuroblastic cells (more than 5%); surrounded by a stroma rich of spindle cells and Schwannian cells which are positive for S100. Areas of cystic degeneration, hemorrhage, dystrophic calcification and necrosis are identified.

Synaptophysin is positive in the neuroblasts and ganglionic cells, and Ki67 shows low mitotic index, S100 is positive in Schwann cells.

Diagnosis: Ganglioneuroblastoma, nodular subtype.

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Case information

rID: 45058
Case created: 12th May 2016
Last edited: 5th Sep 2016
Systems: Chest, Paediatrics
Inclusion in quiz mode: Included

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