A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant
Neuroblastoma, a relatively common tumor in infants, is rarely present at birth. A solid mass is the typical appearance of this neoplasm, while cystic formation is uncommon. Congenital cystic neuroblastoma is extremely rare and no previous report exists of its occurrence in the orbit. A newborn girl was referred with an extremely large mass on the right side of her face on the third day of her life. Prenatal ultrasonography had revealed a large mass (80 × 80 mm) in the frontal area. MRI revealed a heterogeneous mass composed of cystic and solid, contrast-enhancing components. Excisional biopsy was successfully performed and histopathologic diagnosis of neuroblastoma was confirmed. Systemic workup was negative for involvement of other organs and levels of catecholamine metabolites of serum and urine (homovanillic acid and vanillylmandelic acid) were normal. Postoperative course was uneventful with complete healing of the anophthalmic socket and normal physical and mental growth and development of the infant.
This article presents a rare case of congenital cystic neuroblastoma occurring in the orbit of a newborn girl. Neuroblastoma is a common tumor in infants, but it rarely presents at birth, and cystic formation is uncommon. This case is unique because it is the first reported instance of congenital cystic neuroblastoma primarily arising in the orbit. The newborn presented with a large mass on the right side of her face, which prenatal ultrasonography revealed to be a large mass in the frontal area.
MRI confirmed a heterogeneous mass with cystic and solid components. An excisional biopsy was performed, and histopathological examination confirmed the diagnosis of neuroblastoma. Further systemic workup showed no involvement of other organs, and catecholamine metabolite levels were normal. The postoperative course was uneventful, with complete healing and normal development of the infant.
The authors emphasize the rarity of cystic neuroblastoma, especially outside the adrenal glands, and highlight the diagnostic challenges posed by this unusual presentation. The successful resection of the large orbital mass and the lack of systemic involvement in this case contribute valuable information to the limited existing literature on congenital cystic neuroblastoma. The case underscores the importance of considering neuroblastoma in the differential diagnosis of congenital orbital masses, even when cystic features are present.
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