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P. Imbach

 

 

10.10Differential Diagnosis

Besides other tumors (see above, “Pathology”):

Osteomyelitis

Rheumatoid arthritis

Signs of VIP syndrome: infectious or autoimmune intestinal disorders

In opsoclonus or ataxia, neurological disorders

In infants with hepatomegaly, storage diseases

10.11International Staging (Including the Classic Evans Staging)

International neuroblastoma staging system

Stage Description

ILocalized tumor with complete gross excision, with or without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor microscopically (nodes attached to and removed with the primary tumor may be positive)

IIA

Localized tumor with incomplete gross excision; representative, ipsilateral

 

nonadherent lymph nodes negative for tumor microscopically

IIB

Localized tumor with or without complete gross excision, with ipsilateral nonadherent

 

lymph nodes positive for tumor. Enlarged contralateral lymph nodes must be

 

negative microscopically

IIIUnresectable unilateral tumor infiltrating across the midline, with or without regional lymph node involvement; or localized unilateral tumor with contralateral regional lymph node involvement; or midline tumor with bilateral extension by infiltration (unresectable) or by lymph node involvement

IV

Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow,

 

liver, skin, or other organs (except as defined for stage 4S)

IVS

Localized primary tumor (as defined for stages I, IIA, or IIB) with dissemination

 

limited to skin, liver, or bone marrow (limited to infants aged less than 1 year)

10.11.1The International Neuroblastoma Risk Group Classification

L1 Localized tumor not involving vital structures as defined by the list of image defined risk factors and confined to one body compartement

L2 Locoregional tumor with presence of one or more imagedefinded risk factors

M Distant metastatic disease (except MS)

MS Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow

Patients with multifocal primary tumors should be staged according to the greatest extent of disease as defined above.

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