MEETING No. 20, HELD ON MAY 17th, 2000 AT HUMANITAS CLINICAL INSTITUTE, ROZZANO (MILANO)

Case 1
Case 2
Case 3
Case 5
Case 6

Case 4

"RETROPERITONEAL MASS IN A 24 yr. OLD WOMAN"
GIANLUIGI ARRIGONI - S. Raffaele Hospital - Milano
(Pictures by dr. A. Faravelli)

Clinical history: a 24y.o. girl presents with a retroperitoneal mass that displaces the kidney and the adrenal gland. No clinical history for NF. Macroscopic description: 18x16x8.5 cm mass; on cut section it is formed by whitish tissue with hemorrhagic and necrotic areas. Attached to the mass, but not infiltrated by it, are a 7 cm segment of the vena cava and the kidney, which measures 11 cm in maximum dimension and shows compressed hilar structures. Microscopic description: sections show areas with relatively small spindled cells ( pict. 1) - ( pict. 2) and areas with pleomorphic, ganglion – like cells arranged in nests ( pict. 3) - ( pict. 4) - ( pict. 5).
Further sections show areas with epithelioid-like or spindled atypical cells, arranged in storiform pattern with focal necrosis and increased mitotic activity ( pict. 6) - ( pict. 7).
There are also nests of small, hyperchromatic neuroblast-like cells with scanty cytoplasm ( pict. 8) - ( pict. 9) - ( pict. 10)associated with calcium deposits and lymphoid infiltrate.
Immunohistochemical staining results: Synaptophysin: + ( pict. 11) (picture n.11), Chromogranin: +/- ( pict. 12) and S100: +, in the areas with ganglial and neurofibroma-like differentiation; focal Desmin (+), focal Actin (+/-), Chromogranin (-), Synaptophysin (-), S100 (-) in the areas with atypical spindled cells.

Diagnosis

pict. 1	pict. 2
pict. 3	pict. 4
pict. 5	pict. 6
pict. 7	pict. 8
pict. 9	pict. 10
pict. 11	pict. 12

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