Bilateral Adrenal Neuroblastoma

Abstract

Two cases of bilateral adrenal neuroblastoma in infants are reported. The first patient presented with abdominal distension due to metastatic hepatomegaly and the second patient was found because of elevated urinary VMA by VMA mass-screening system. Both were under one year of age and were classified as stage IVs neuroblastoma. In each case unilateral adrenalectomy was performed and the contra-lateral adrenal tumor was observed. The remaining tumors slowly decreased in size and clinical progress was good. A review of published cases of bilateral adrenal neuroblastoma suggests that the characteristics of this disease are age less than 1 year, stage IVs, and good prognosis.

Bilateral Adrenal Neuroblastoma is Different

“…Both of our cases with amplification died. Our experience and that of other authors [8,14] suggests that preservation of adrenal function is convenient and that bilateral total adrenalectomy must be restricted to cases with unsalvageable glands, so that unilateral adrenalectomy with contralateral enucleation or partial adrenalectomy is an acceptable option [8,10,17]. In summary, bilateral adrenal neuroblastomas are different from regular neuroblastomas to the point of making staging, treatment and prognosis uncertain.…”

Metastatic, Bilateral Adrenal Neuroblastoma Presenting With Blindness Without Proptosis

“…9 In most cases, a satisfactory outcome suggests vigorous treatment, including bilateral adrenalectomy, intense chemotherapy, and radiation therapy, which are unnecessary for bilateral neuroblastoma. 6 Our patient has adverse prognostic features, including (a) metastatic disease, (b) age above 18 months, and (c) N-MYC amplification. We plan to treat with the backbone of HR-NB1/SIOPEN protocol.…”

“…Clinical features of bilateral adrenal neuroblastoma are often similar to those of stage IVs neuroblastoma. 6,7 Compared with patients with unilateral disease, patients with bilateral adrenal neuroblastoma are younger, more likely to have distant metastasis, and a favorable outcome. 8 Exceptional cases exhibiting risk factors, such as amplified N-MYC, are comparable with high-risk unilateral neuroblastoma cases with similar poor prognostic features.…”

“…The bilateral involvement may either be because of the multifocal origin of the tumor or contralateral metastasis. Clinical features of bilateral adrenal neuroblastoma are often similar to those of stage IVs neuroblastoma 6,7. Compared with patients with unilateral disease, patients with bilateral adrenal neuroblastoma are younger, more likely to have distant metastasis, and a favorable outcome 8.…”

“…Exceptional cases exhibiting risk factors, such as amplified N-MYC, are comparable with high-risk unilateral neuroblastoma cases with similar poor prognostic features 9. In most cases, a satisfactory outcome suggests vigorous treatment, including bilateral adrenalectomy, intense chemotherapy, and radiation therapy, which are unnecessary for bilateral neuroblastoma 6…”