Prenatally diagnosed neuroblastoma

Abstract

Prenatally diagnosed neuroblastomas are predominantly adrenal in origin and frequently cystic. The liver is the most common site of dissemination and bone involvement is notably absent. The vast majority of these infants have a favorable stage of disease (I, II, and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patients, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression.

Outcome of suprarenal localized masses diagnosed during the perinatal period

“…In our series, as in the literature, the solid nature of the tumor after birth (13 LRNBs among 17 solid tumors) and increasing size (6 LRNBs among seven growing tumors) favored the diagnosis of LRNB. 6 Conversely, changes in the echogenicity of the mass (becoming heterogeneous with internal echoes, the late appearance of calcifications, or a continuous decrease in size on serial ultrasonography) were, as previously reported, 1,6 the only reliable signs of adrenal hemorrhage. Doppler ultrasonography, which was not used postnatally in this series, also may be helpful for the diagnosis, particularly to distinguish adrenal disease from extralobular pulmonary sequestration (systemic vessel originating from the aorta).…”

“…1 The main diagnoses are neuroblastoma and adrenal hemorrhage, but other benign masses may be found, such as extralobular pulmonary sequestration, bronchogenic cyst, and renal dysplasia. Because of the recent emergence of this patient population identified by routine imaging, the precise incidence of locoregional neuroblastoma (LRNB) among perinatally diagnosed suprarenal masses is unknown, and the optimal diagnostic and therapeutic management of these cases is not yet clearly defined.…”

“…2 Furthermore, screening increased the observed incidence of LRNB by greater than 50%, whereas many of these supplementary tumors remain asymptomatic and eventually vanish, 16,17 particularly when they are initially cystic. 1 The notion that LRNB can spontaneously regress has been confirmed by autopsy studies, and this challenges the systematic use of surgical resection. 10,17 Surgical resection cannot always prevent tumor recurrence (as in two cases in our series) and carries a risk, albeit low, of peri-or postoperative complications such as iatrogenic nephrectomy, vascular accidents resulting in vanishing kidney, and intestinal infarction.…”

“…Such a strategy could be recommended for small tumors (Ͻ 30 mm), and particularly for cystic forms, which seem to regress more frequently than solid forms. 1 Surgery then is indicated when tumors increase in size during follow-up or persist after 6 months of life. Surgical resection should be proposed initially when the suprarenal mass is larger or when the parents do not accept a wait-and-see attitude for a potentially malignant tumor.…”

“…Further-more, because screening apparently has caused an increas in the number of patients with neuroblastoma, it has been suggested that, like clinically detected Stage IV-S disease, many of these neonatal cases of neuroblastoma can regress spontaneously. 1 Yamamoto et al 3 supported this idea, because they demonstrated tumor regression in 11 of 12 neuroblastomas detected by mass screening. Conversely, when a suprarenal mass is discovered incidentally during fetal life or in the neonatal period, precise diagnosis on the basis of radiologic and laboratory examinations may be difficult.…”