Mubarak Mohamed, Singal Arbinder Kumar, Gawdi Ashok
College of Medicine & Medical Sciences, Arabian Gulf University, Kingdom of Bahrain.
Department of Pediatric Surgery, MGM University of Health Sciences, Kamothe & MGM's New Bombay Hospital, Vashi, Navi Mumbai, India.
Gulf J Oncolog. 2019 May;1(30):81-84.
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.
神经母细胞瘤是儿童期最常见的颅外实体瘤。4S期是神经母细胞瘤的一个特殊阶段，大多数病例可能会自发消退；然而，在某些情况下，肿瘤进展迅速，预后不良，因此需要积极治疗。本文将讨论其管理和治疗中的困境。我们报告两例4S期神经母细胞瘤病例，分别代表了这两种极端情况。第一例是一名4个月大的婴儿，最初表现为大腿肿块、阴唇和足部结节，以及肝脏和肾上腺受累。在确诊为组织学良好且N-MYC无扩增的4S期神经母细胞瘤后，选择了密切观察的方法。目前，该患者已完成两年的随访，发育正常，无异常情况。第二例是一名足月儿，表现为腹胀和呼吸窘迫。经证实为4S期神经母细胞瘤，组织学不良，伴有肝和左肾上腺转移。由于患者病情恶化，开始使用卡铂和依托泊苷进行6个周期的化疗，反应良好且迅速。该患者完成了两年的随访，无复发。
