J Korean Assoc Pediatr Surg.  2009 Jun;15(1):38-43.

Clinical Features and Surgical Outcome of Congenital Neuroblastoma of Adrenal Gland

Affiliations
  • 1Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. sukkoo.lee@samsung.com

Abstract

With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass becomes more common. Most of these masses prove to be congenital neuroblastomas (CNB) postnatally. However, the diagnosis is often confused with other benign lesions and the post-natal management remains controversial. The medical records of 13 patients that underwent primary surgical excision for an antenatally detected adrenal CNB, between January 1995 and April 2009, were reviewed retrospectively. The clinical, radiological, surgical, and pathological data on the suprarenal mass were collected. Staging evaluation was performed after histological confirmation of the CNB. Most of the CNBs were stage I (N=11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had N-myc gene amplification. The stage I patients were cured by surgery alone, and stage IV patients underwent 9 cycles of adjuvant chemotherapy and currently have no evidence of disease after 39 months of follow-up. The patient with stage IV-S is currently receiving chemotherapy. There were no post-operative complications. For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an adrenal CNB detected before birth. The surgery can be safely performed during the neonatal period and provides a cure in most cases. Surgical diagnosis and treatment of CNB is recommended in neonatal period.

Keyword

Neuroblastoma; Prenatal diagnosis; Early surgical excision

MeSH Terms

Adrenal Glands
Chemotherapy, Adjuvant
Early Diagnosis
Follow-Up Studies
Genes, myc
Humans
Medical Records
Neuroblastoma
Parturition
Prenatal Diagnosis
Retrospective Studies
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