Stage 4S Neuroblastoma: A Report of Two Cases Presenting with Extremes of Biological Behavior

Neuroblastoma is the most common extracranial solid tumor in childhood. Stage 4S is a special stage of neuroblastoma in which majority of cases may have spontaneous regression; however, in some cases the tumor is rapidly progressive with poor prognosis and thus requires aggressive therapy. Dilemmas in its management and therapy will be discussed. We report two cases of stage 4S neuroblastoma exemplifying these two extreme behaviors. The first case is of a four-month-old baby who initially presented with a thigh lump, labial and foot nodules as well as hepatic and adrenal involvement. Following the confirmation of stage 4S Neuroblastoma with favorable histology and N-MYC negative amplification, a watchful observation approach was elected. Currently, the patient has completed two years of uneventful follow-ups with normal development. The second case is of a full-term new born baby who presented with abdominal distention and respiratory distress. Stage 4S Neuroblastoma was confirmed with an unfavorable histology and metastasis to the liver and the left adrenal gland. Due to the deterioration of the patient's condition, chemotherapy with carboplatin and etoposide was initiated for six cycles with a good and rapid response. The patient completed two years of follow up without recurrence.