Sacrococcygeal Teratoma Presenting as Perineal and Periumbilical Discoloration (Cullen Sign)
Article Outline
A 15-month-old Caucasian boy had a several-month history of perineal and scrotal bruising. The bruised area was sharply demarcated, occurring to the left without crossing the midline (Figure 1). It was indurated, but non-tender. Similar bruising developed later in the periumbilical region (Cullen sign).
Figure 1
Left-sided perineal bruising.
Past medical history was notable for constipation at 6 months of age manifested by hard stools and straining. Stool consistency improved with osmotic laxative, but straining persisted.
The results of an abdominal examination were normal, without detectable masses. The results of a rectal examination initially were considered to be normal with soft stool in the vault, although with a subsequent examination a possible mass was revealed. The results of the remainder of the patient’s physical examination were normal.
With pelvic ultrasound scanning, a cystic and solid mass was revealed. A complex cystic sacrococcygeal teratoma (SCT) was confirmed with magnetic resonance imaging with enhancement of the surrounding ischiorectal fat (Figure 2). Alpha fetoprotein level was high-normal.
Figure 2
Complex cystic sacrococcygeal teratoma with surrounding ischiorectal fat enhancement.
At the time of operation, the teratoma was successfully excised in entirety. By means of histology, a mature type IV teratoma, measuring 6.5 cm in diameter, was revealed. Three months after resection, the results of repeated computed tomography of the abdomen and pelvis were negative for recurrent disease. The patient currently has no symptoms and has normal voiding and stooling patterns.
Skin discoloration can occur because of intraperitoneal and extraperitoneal hemorrhage. The Cullen sign represents bluish discoloration around the umbilicus and was first described by Thomas Cullen in 1918 as evidence of ruptured ectopic pregnancy.1 It is also seen with hemorrhagic pancreatitis and abdominal trauma. In our case, the apparent bruising was caused by tumor involvement and surrounding ischiorectal fat necrosis resulting in hemorrhage.
Approximately 10% of all SCTs are type IV. Types I, II, and III have an external component and are visible at birth; type IV is entirely intra-abdominal, often leading to delayed diagnosis. When resected before the age of 2 months, risk of malignancy with type IV SCT is ≤10%, increasing to 50% by 1 year of age.2 In this case, the perineal changes and Cullen sign prompted an early diagnosis.
References
PII: S0022-3476(10)00680-3
doi:10.1016/j.jpeds.2010.08.011
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