A previously healthy 8-year-old black boy developed progressive constipation over 3 years. His two-stool-a-week habit was treated conservatively and blood was never manifest in the stool. The patient was continent of urine and feces with no neurologic complaints. On presentation at the emergency department for stool disimpaction, physical examination revealed a 7×9-cm firm mobile mass inferior to the umbilicus. Neurologic and laboratory examination results were normal. Abdominal computed tomographic scan (Fig 1) and contrast enema (Fig 2) were performed.
Gross pathologic examination revealed a single, oval, encapsulated, soft-tissue mass measuring 13×8×5 cm and weighing 323 g (Fig 3). Fibrous adhesions connected the mass to the sacrococcygeal bone. Histologic findings were of a benign ganglioneuroma of the presacral region (Fig 4). At 3-month follow-up, the patient had normal bowel and bladder function and no complaints.
Denouement and Discussion
Ganglioneuroma of the peripheral nervous system was first described by Lorentz in 1870.1 Composed of nerve fibers and mature ganglion cells, ganglioneuroma is a slowgrowing neoplasm originating from autonomie nervous system ganglia.3 Ganglioneuroma is considered to be at the benign end of the neuroblastoma-ganglioneuroblastoma ganglioneuroma spectrum.2
Spirnak JP, Wood BP. SPECIAL FEATURE. Am J Dis Child. 1993;147(10):1119–1120. doi:10.1001/archpedi.1993.02160340105024
Customize your JAMA Network experience by selecting one or more topics from the list below.