Clinical and radiological characteristics of patients with retroperitoneal infantile hemangiomas.

Background: Infantile hemangiomas (IHs) are the most common tumors of infancy. The objective was to identify clinical and radiological patterns in patients with retroperitoneal IHs.

Methods: We reviewed patients from our Vascular Anomalies Center database with IHs and abdominal imaging presenting from 1999 to 2017 to identify retroperitoneal involvement.

Results: Eleven patients (10 females, one male) with retroperitoneal IHs were found. Cutaneous IHs were present in eight patients (five segmental (45%), three multifocal (27%)) and absent in 1 (9%). Segmental hemangiomas involved the face in 2/5 (40%) and lower body in 3/5 (60%). The most common symptoms were dyspnea (n = 4), hematochezia (n = 3), and/or ulceration (n = 2). Three patients were asymptomatic. Involved retroperitoneal organs included the duodenum (n = 4), pancreas (n = 3), and adrenal glands (n = 1). Non-retroperitoneal organ involvement included the liver (n = 5), non-duodenal small intestine (n = 4), and large intestine (n = 3). Perivascular retroperitoneal hemangiomas were seen in 6/11 patients (55%), most commonly surrounding the aorta (n = 5), iliac vessels (n = 2), and/or inferior vena cava (n = 2). Three of 11 patients (27%) had LUMBAR based on a segmental, sacral hemangioma with tethered cord or anorectal malformation. Follow-up information was available in 6/11 patients (55%): 5 symptomatically improved with treatment (propranolol, corticosteroids, and/or vincristine), while one succumbed from extensive hepatic involvement.

Conclusion: Retroperitoneal IHs are rare and tend to involve organs or surround vessels. Associated cutaneous IHs, if present, lack anatomical predilection and may be segmental or multifocal.