10.5.2 Microscopic Features

•High variability with various differentiation stages of sympathetic nervous tissue, ranging from undifferentiated neuroblastoma, to ganglioneuroblastoma, to differentiated ganglioneuroma

•Neuroblasts are organized in nests surrounded by fibro-vascular septae

•Differentiation with:

–Testing for intracellular catecholamines

•Histologically small round-cell tumor, which has to be differentiated from:

–Primitive neuroectodermal tumor (PNET)

–Embryonal undifferentiated rhabdomyosarcoma

–Retinoblastoma

–Ewing sarcoma

–Lymphoma

•Well-differentiated forms demonstrate islets with polymorphic nucleus separated by fibrillar material; sometimes cells are characteristically arranged as rosettes, particularly in the bone marrow

10.6Clinical Manifestations

• Occurrence can be in any area with sympathetic nervous tissue

Primary locations in order of frequency

• Rarely: primary tumor undetectable

10.6.1 Common Symptoms

•Weight loss

•Fever

•Abdominal disturbances

•Irritability

•Pain of bones and joints

•Child will not stand up, will not walk

•Pallor

•Fatigue