A rare case of bilateral stage IV adrenal neuroblastoma with multiple skin metastases in a neonate: Diagnosis, management, and outcome
ABSTRACT A neonate with stage IV bilateral adrenal neuroblastoma associated with metastases to the skin, liver, bone marrow, and right ocular conjunctiva is reported. At birth, skin metastases were present all over the body. He underwent a combination of chemotherapy, surgery, and peripheral blood stem cell transplantation (PBSCT). Histopathology was unfavorable with diploid tumor cell DNA content and low levels of TRK-A mRNA expression. Skin and conjunctival metastases disappeared after PBSCT, and there have been no signs of recurrence after 5 years of follow-up. Accurate staging of disease and histologic examination followed by intensive management are essential even in infants with neuroblastoma to ensure successful outcome.
[Show abstract] [Hide abstract]
ABSTRACT: Neuroblastoma frequently arises from the adrenal gland and has rarely been reported to arise synchronously in both adrenal glands. The majority of reports of bilateral adrenal neuroblastoma are in children under the age of 1 year. We present a case of bilateral adrenal neuroblastoma in an 8-year-old child. This is the first report of bilateral disease in a child over the age of 1 year with stage IV neuroblastoma.Journal of Pediatric Hematology/Oncology 10/2007; 29(9):652-5. DOI:10.1097/MPH.0b013e318142b788 · 0.96 Impact Factor
Article: Bilateral Adrenal Neuroblastoma[Show abstract] [Hide abstract]
ABSTRACT: Bilateral adrenal neuroblastoma is extremely rare. To date, 45 cases have been reported in the literature. We retrospectively identified and reviewed 15 cases of bilateral adrenal neuroblastoma, treated between 1988 and 2004, by the French Society of Pediatric Oncology. We then compared our cohort to the 45 cases reported in literature. Median age at diagnosis was 4 months in our cohort whereas it was 3 months in the literature. The same percentage of infants was found in both series (86.6%). Disease had generally been detected due to metastasis-related symptoms. Ten out of 15 patients in our cohort and 18/39 cases in the literature were classified as stage 4S according to the International Neuroblastoma Staging System (INSS). The incidence of stage 4S was significantly higher than that described in other neuroblastoma cohorts (P = 10(-4)). Five-year overall survival was 90% for stage 4S. In our series, bilateral neuroblastoma was neither associated with familial cases nor with any risk factors. The majority of bilateral neuroblastoma carry a favorable prognosis. Exceptional cases exhibiting risk factors, such as amplified MYCN, are comparable to high-risk unilateral neuroblastoma cases with the same poor prognostic features. The therapeutic strategy could be similar to that used against unilateral neuroblastoma, except for surgery. However, the low incidence of relapse and the risk of adrenal failure if radical surgery is performed, argue against an aggressive surgical approach.Pediatric Blood & Cancer 02/2009; 52(2):196-202. DOI:10.1002/pbc.21765 · 2.56 Impact Factor
Article: Bilateral adrenal neuroblastoma.[Show abstract] [Hide abstract]
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.European Journal of Pediatric Surgery 03/1994; 4(1):37-9. DOI:10.1055/s-2008-1066063 · 0.98 Impact Factor