title: Detection and clinical significance of disseminated tumour cells at diagnosis in bone marrow of children with localised rhabdomyosarcoma.
abstract: Identification of patients with a poor prognosis for non-metastatic rhabdomyosarcoma (RMS) remains a clinical challenge. Prospective analysis for the presence of disseminated RMS cells in bone marrow at diagnosis, using immunocytochemistry, with MyoD1 and myogenin as markers, was carried out. Thirty-seven patients treated on RMS88 and RMS96 Italian protocols underwent staging investigations, and in addition marrow examination for occult tumour cells. All patients had negative marrow involvement using cytomorphology, but 10/37 were positive with immunostaining. With a median follow-up of 46 months (range, 12-115), 7 patients had died and 30 were disease-free. Overall survival probability was 92% in patients with no occult marrow infiltration, 47% with occult marrow infiltration (P=0.001); event-free survival probability was 89% in the former and 50% in the latter (P=0.01). Disseminated tumour cells are indicative of disease spread and are significantly linked to recurrence at distant sites and poorer outcome. Marrow examination at diagnosis using immunocytochemistry may be an additional tool to modulate treatment.
authors.affiliation: Division of Oncology, RLC NHS Trust Alder Hey, Liverpool, UK.
authors.forename: Heather P
authors.initials: HP
authors.lastname: McDowell
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Giuseppe M
authors.initials: GM
authors.lastname: Milano
authors.forename: Anna
authors.initials: A
authors.lastname: Clerico
authors.forename: Olga
authors.initials: O
authors.lastname: Mannarino
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Altavista
authors.forename: Renata
authors.initials: R
authors.lastname: Boldrini
authors.forename: Raffaele
authors.initials: R
authors.lastname: Cozza
authors.forename: Alessandro
authors.initials: A
authors.lastname: Inserra
authors.forename: Carlo
authors.initials: C
authors.lastname: Dominici
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000473:pathology*; D019046:Bone Marrow Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods / Q000592:standards; D008297:Male; D017570:MyoD Protein / Q000032:analysis; D018008:Myogenin / Q000032:analysis; D011379:Prognosis; D011446:Prospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D012680:Sensitivity and Specificity
chemical_list: D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein; D018008:Myogenin