2019
DOI: 10.4103/ijri.ijri_148_19
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Adenocarcinoma and neuroendocrine tumor arising within presacral teratoma associated with Currarino syndrome: A case report

Abstract: We present a case of a 59-year-old woman with a malignant tumor arising within presacral teratoma associated with Currarino syndrome (CS). A characteristic crescent-shaped sacrum was detected on preoperative image examination and the presacral mass was pathologically diagnosed as a malignant tumor associated with CS. To our knowledge, this is the first case report of presacral teratoma associated with CS coexisting with both adenocarcinoma and a neuroendocrine tumor.

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Cited by 4 publications
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“…In the literature, an association of presacral NENs with tailgut cysts and teratomas has been described. Additionally, an association with Currarino syndrome can be observed, an autosomal-dominant disorder caused by mutations in the motor neuron and pancreas homeobox 1 (MNX1) gene and characterized by presacral mass, sacral dysgenesis, anorectal anomalies (21,49,53). Most remarkably, whereas tailgut cysts or at least partially cystic primary tumors were observed in some of our patients, only one association with teratoma and none of other abnormalities like anorectal malformations or Currarino syndrome were present.…”
Section: Discussionmentioning
confidence: 63%
“…In the literature, an association of presacral NENs with tailgut cysts and teratomas has been described. Additionally, an association with Currarino syndrome can be observed, an autosomal-dominant disorder caused by mutations in the motor neuron and pancreas homeobox 1 (MNX1) gene and characterized by presacral mass, sacral dysgenesis, anorectal anomalies (21,49,53). Most remarkably, whereas tailgut cysts or at least partially cystic primary tumors were observed in some of our patients, only one association with teratoma and none of other abnormalities like anorectal malformations or Currarino syndrome were present.…”
Section: Discussionmentioning
confidence: 63%
“…The presacral lesion is usually a benign entity such as anterior meningocele, teratoma, hamartoma, enteric cyst, or dermoid/epidermoid cyst. Malignant presacral mass degeneration is rare, approximately 1% of all cases with neuroendocrine tumor predominance 4–8 . Cross-sectional imaging features of CS are well known with a presacral mass associated with sacral malformations also known as “scimitar sacrum” or “sickle-shaped sacrum.” 3 In instances of rising concern for malignant degeneration, PET/CT provides the ability to assess the metabolism and degree of aggressive behavior of the presacral lesions, to detect the extent of their potential metastasis, and to monitor their therapeutic response 7,8 …”
mentioning
confidence: 99%