BACKGROUND: Evidence-based guidelines for the management of localized perinatal adrenal neuroblastoma are not yet available. We describe our preliminary experience managing this tumor with a "wait and see" policy. METHODS: A single-center prospective study (February 2002 to December 2009) was conducted with 12 consecutive patients in whom an adrenal mass was detected antenatally or within the first 3 months of life. Diagnostic workup included the following investigations: measurement of urine catecholamine metabolites, imaging studies (ultrasonography, magnetic resonance imaging, or computed tomography), metaiodobenzylguanidine scintigraphy, and/or core needle biopsy. RESULTS: The male/female ratio was 1.4:1.0. Median tumor size at presentation was 29 mm (range 10-50 mm). Eight lesions were detected antenatally. Ten lesions were diagnosed as localized neuroblastoma. Of these ten lesions, four were excised because of parental preference (n = 2), tumor enlargement (n = 1) or tumor persistence (n = 1). The remaining six patients underwent watchful clinical observation, which showed progressive tumor shrinkage and complete regression within 10-39 months (median 12.5 months). The final two lesions were small predominantly cystic lesions without a clear-cut diagnosis. They were managed noninvasively. At an overall median follow-up of 109 months (range 30-122 months), all patients are alive and disease-free, although one patient progressed to stage 4 disease despite early excision of the primary tumor. CONCLUSIONS: Spontaneous regression of localized perinatal adrenal neuroblastoma occurs often, and a "wait and see" strategy seems justified in these small infants. Patients with enlarging or stable lesions that have persisted for several months may benefit from surgery, although prompt excision may not prevent tumor progression.
BACKGROUND: Evidence-based guidelines for the management of localized perinatal adrenal neuroblastoma are not yet available. We describe our preliminary experience managing this tumor with a "wait and see" policy. METHODS: A single-center prospective study (February 2002 to December 2009) was conducted with 12 consecutive patients in whom an adrenal mass was detected antenatally or within the first 3 months of life. Diagnostic workup included the following investigations: measurement of urine catecholamine metabolites, imaging studies (ultrasonography, magnetic resonance imaging, or computed tomography), metaiodobenzylguanidine scintigraphy, and/or core needle biopsy. RESULTS: The male/female ratio was 1.4:1.0. Median tumor size at presentation was 29 mm (range 10-50 mm). Eight lesions were detected antenatally. Ten lesions were diagnosed as localized neuroblastoma. Of these ten lesions, four were excised because of parental preference (n = 2), tumor enlargement (n = 1) or tumor persistence (n = 1). The remaining six patients underwent watchful clinical observation, which showed progressive tumor shrinkage and complete regression within 10-39 months (median 12.5 months). The final two lesions were small predominantly cystic lesions without a clear-cut diagnosis. They were managed noninvasively. At an overall median follow-up of 109 months (range 30-122 months), all patients are alive and disease-free, although one patient progressed to stage 4 disease despite early excision of the primary tumor. CONCLUSIONS: Spontaneous regression of localized perinatal adrenal neuroblastoma occurs often, and a "wait and see" strategy seems justified in these small infants. Patients with enlarging or stable lesions that have persisted for several months may benefit from surgery, although prompt excision may not prevent tumor progression.
Authors: W G Woods; M Tuchman; L L Robison; M Bernstein; J M Leclerc; L C Brisson; J Brossard; G Hill; J Shuster; R Luepker; T Byrne; S Weitzman; G Bunin; B Lemieux Journal: Lancet Date: 1996 Dec 21-28 Impact factor: 79.321
Authors: Anna Rita Gigliotti; Andrea Di Cataldo; Stefania Sorrentino; Stefano Parodi; Antonino Rizzo; Piero Buffa; Claudio Granata; Angela Rita Sementa; Anna Maria Fagnani; Massimo Provenzi; Arcangelo Prete; Carmelita D'Ippolito; Anna Clerico; Aurora Castellano; Gian Paolo Tonini; Massimo Conte; Alberto Garaventa; Bruno De Bernardi Journal: Eur J Cancer Date: 2009-09-18 Impact factor: 9.162
Authors: T Sawada; M Hirayama; T Nakata; T Takeda; N Takasugi; T Mori; K Maeda; R Koide; Y Hanawa; A Tsunoda Journal: Lancet Date: 1984-08-04 Impact factor: 79.321
Authors: Suzanne P MacFarland; Sogol Mostoufi-Moab; Kristin Zelley; Peter A Mattei; Lisa J States; Tricia R Bhatti; Kelly A Duffy; Garrett M Brodeur; Jennifer M Kalish Journal: Pediatr Blood Cancer Date: 2017-01-09 Impact factor: 3.167