Presacral tumor associated with the Currarino triad in an adolescent

Abstract

A 17-year-old woman presented with pain over the sacral region. Plain radiographs of the sacrum demonstrated a bony deformity of the sacrococcygeal region in the shape of a scimitar. Magnetic resonance imaging showed a cystic mass of the presacral region which appeared to be continuous with the dural sac. An anteroposterior view myelogram revealed caudal elongation of the dural sac, and on the lateral view it was recognized as an anterior meningocele. At surgery, we confirmed a connection between the presacral mass and the rectum. In light of the combination of a sacral bony deformity, presacral mass including meningocele, and mass-rectum connection, we made the diagnosis of the Currarino triad, which is a rare complex of congenital caudal anomalies. The patient underwent excision of the presacral mass. Histologic examination of the resected specimen revealed features of an epidermoid cyst.

New clinical and therapeutic perspectives in Currarino syndrome (study of 29 cases)

“…Most series concern only a few cases [20][21][22][23][24][25][26][27]. In the 3 main series of the literature, the authors focused on phenotypic and genetic disorders, but therapeutic management was not described [6,18,19].…”

Recurrent Meningitis Associated With Complete Currarino Triad in an Adult-Case Report-

“…11,[14][15][16][17]19,20) The most frequent symptom is constipation (67%) followed by urination difficulty (9%) in pediatric cases, but various symptoms seen in adult cases. The frequency of constipation associated with anorectal malformation is lower in adult cases.…”

“…4,7) However, we should also note that some cases are idiopathic meningitis and include three cases of epidermoid cysts containing pus. 10,11,19) The mechanism of infection remains uncertain. In our case, no neurenteric or skin fistula was detected.…”

“…4) The presacral mass is most frequently a teratoma (18% of cases) or an anterior sacral meningocele (69%), whereas neurenteric cysts or dermoid (3%) and epidermoid cysts (1%) are unusual. 10,11,[14][15][16][17]19,20) The mass is associated with a hemisacral or scimitar sacral defect, but segmentation anomalies are less common. The anorectal malformation is usually either anal stenosis or atresia.…”

Degeneración tumoral en meningocele no intervenido: Descripción de dos casos

“…Incluso se conoce un caso publicado en que coinciden meningocele sacro con un teratocarcinoma intracraneal 7 . Existe una agrupación sindrómica catalogada como Síndrome de Currarino, caracterizada por la existencia de múltiples anomalías caudales congénitas destacando malformaciones ano-rectales, anomalías del sacro y masas presacras 2,4,8 . Publicaciones posteriores de los años 80 sugieren una relación del tumor de Wilms (nefroblastoma) con pacientes afectos de espina bífida oculta o con estigmas cutáneos de disrafia espinal, pudiendo ser demostrada estadísticamente a comienzos de la década actual 14 .…”