Presacral Cystic Neuroblastoma: Case Report and Review of the Literature

Abstract

Cystic neuroblastoma (CN) is highly unusual; since 2005 only 55 cases have been reported in the medical literature. The authors report a case of pelvic (presacral) CN in a 2-month-old boy. Computed tomography scan confirmed a cystic, septated, and encapsulated mass. Complete tumor excision was achieved. Histopathology diagnosed a stroma-poor and undifferentiated neuroblastoma with multiple calcifications. meta-iodobenzylguanidine scan, radionuclide bone, and a bone marrow aspiration were normal. N-myc amplification was absent. The infant was classified stage I neuroblastoma and received no further therapy (4 y of follow-up). To the authors' knowledge, this is the first report of a CN in a pelvic-presacral location.

A case of sacrococcygeal neuroblastoma

“…Teratomas, cystic lymphangiomas, meningomyeloceles and chordomas may arise from this region in infants. But today computed tomography and magnetic resonance imaging helps in diagnosis (Tanaka et al, 2005;D'Alessio et al, 2006;Watanabe et al, 2008).…”

A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant

“…The most common site for cystic neuroblastoma is the adrenal glands, and extra-adrenal occurrence is extremely unusual, with only 3 cases being previously reported in the English literature, including 2 in the thorax and 1 in the presacral region [3, 4]. Additionally, 2 cases of congenital orbital teratoma with neuroblastic features have been reported so far.…”

“…Cystic neuroblastoma, a rare variant, often presents at a lower age and is associated with lower rates of metastasis. Thus, its long-term prognosis is more favorable [3]. In our patient, in spite of the dramatic presentation of the large tumor, workup for remote metastasis was negative and there was no recurrence.…”

“…However, calcification, which is seen in most cases of solid neuroblastoma, is rarely present in cystic neuroblastoma [3, 4]. …”

“…Cystic formation of neuroblastoma is rarely described. The majority of cases of cystic neuroblastoma are located in the adrenal glands and are rarely seen in other organs [3, 4]. We report an extremely unusual case of congenital solitary orbital cystic neuroblastoma confirmed by immunohistochemical (IHC) studies in a newborn girl.…”