Small round blue cell tumours: diagnostic and prognostic usefulness of the expression of B7-H3 surface molecule

• Published in: Histopathology Vol. 53; no. 1; pp. 73 - 80
• Main Authors: Gregorio, A, Corrias, M V, Castriconi, R, Dondero, A, Mosconi, M, Gambini, C, Moretta, A, Moretta, L, Bottino, C
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.07.2008; Blackwell
• Subjects: Antigens, CD - metabolism; B7 Antigens; B7-H3; Biological and medical sciences; Biomarkers, Tumor - metabolism; Brain Neoplasms - diagnosis; Brain Neoplasms - metabolism; Brain Neoplasms - mortality; diagnosis; Diagnosis, Differential; Humans; Investigative techniques, diagnostic techniques (general aspects); Italy - epidemiology; Medical sciences; Medulloblastoma - diagnosis; Medulloblastoma - metabolism; Medulloblastoma - mortality; neuroblastoma; Neuroblastoma - diagnosis; Neuroblastoma - metabolism; Neuroblastoma - mortality; Neurology; Original; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Prognosis; Receptors, Immunologic - metabolism; Rhabdomyosarcoma - diagnosis; Rhabdomyosarcoma - metabolism; Rhabdomyosarcoma - mortality; small round cell tumours; Survival Rate; Tumors of the nervous system. Phacomatoses; Italy; Nervous system diseases; Prognosis; Blue; small round cell tumours; Malignant tumor; Neuroblastoma; B7-H3 molecule; Surface; Anatomic pathology; B7-H3; Diagnosis; Autonomic neuropathy; Small cell carcinoma; Cancer
• ISSN: 0309-0167; 1365-2559
• DOI: 10.1111/j.1365-2559.2008.03070.x

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.