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. 2008 Jul;53(1):73-80.
doi: 10.1111/j.1365-2559.2008.03070.x.

Small Round Blue Cell Tumours: Diagnostic and Prognostic Usefulness of the Expression of B7-H3 Surface Molecule

Free PMC article

Small Round Blue Cell Tumours: Diagnostic and Prognostic Usefulness of the Expression of B7-H3 Surface Molecule

A Gregorio et al. Histopathology. .
Free PMC article


Aims: To assess whether the expression of B7-H3 surface molecule could improve differential diagnosis of small cell round tumours.

Methods and results: One hundred and one well-characterized paraffin-embedded small round cell tumours, stored in the pathology archive of the Gaslini Institute, were immunohistochemically analysed with the 5B14 monoclonal antibody, which recognizes the surface molecule B7-H3. All lymphoblastic lymphomas and the blastematous component of Wilms' tumours were completely negative and a few Ewing's sarcoma and Burkitt's lymphoma specimens showed focal positivity, whereas 74% of neuroblastomas, 67% of rhabdomyosarcomas and 100% of medulloblastomas were positive. The pattern of immunoreactivity of 5B14 mAb observed in rhabdomyosarcoma, neuroblastoma and medulloblastoma specimens was limited to the cytoplasmic membrane, and in neuroblastomas areas of rosette formation or of ganglion differentiation were preferentially stained. Interestingly, in neuroblastoma patients high expression of the antigen recognized by the 5B14 mAb was associated with a worse event-free survival.

Conclusions: The 5B14 mAb represents an additional tool for the differential diagnosis of small round cell tumours and might be useful in identifying neuroblastoma patients at risk of relapse who may take advantage of more careful follow-up.


Figure 1
Figure 1
Reactivity of 5B14 mAb in small round blue cell tumours. Lymphoblastic lymphomas (A) and blastematous components of Wilms’ tumours (B) were always negative. Three out of 11 Burkitt's lymphoma specimens showed focal positivity (C) Several rhabdomyosarcomas (D), as well as medulloblastomas (not shown) and neuroblastomas (NB) (see Figure 2), were positive. *Area corresponding to the inset. Original magnification ×100, insets ×400.
Figure 2
Figure 2
Different score and grade of 5B14 staining in neuroblastoma (NB). Representative NB with score 3 (A), score 1 (B), grade +++ (C) and grade + (D). Original magnification ×100 (A–B), ×40 (C–D).
Figure 3
Figure 3
Rosettes of neuroblastoma (NB) cells (A) and differentiating NB cells (B) stained with 5B14 antibody. Original magnification ×100.
Figure 4
Figure 4
Kaplan–Meyer plots of event-free survival of neuroblastoma (NB) patients stratified according to absence/presence in their tumours of score 3 positivity (all patients, A); grade +++/++ (all patients, B); score 3 positivity (stage 4 patients excluded, C); score 3 positivity (only stage 1, 2 and 3 patients, D).

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