IS case 441: Ganglioneuroblastoma

Meena Moorthy, MD, MBA

University of Rochester


Imaging Sciences URMC 2010
Publication Date: 2010-08-30

History

Patient is a 4-year-old female who began having vague abdominal pain 8 months ago. At that time she was noted to have difficulty urinating and decreased urine output (UOP). Two months later, during a visit to the Emergency Department for abdominal pain, a radiograph of her abdomen was significant for mass effect in the pelvis, which was displacing the bowel. The next evaluation of this patient with imaging was six months later, during which time an abdomen radiograph was again notable for a pelvic mass effect. Follow-up with CT demonstrated an extensive pelvic mass, which was then biopsied. Biopsy was significant for ganglioneuroblastoma, with no evidence of metastatic disease in the bone marrow. The patient has been undergoing treatment with surgical resection and chemotherapy.

Findings

Initial abdomen radiograph six months ago showed a mass effect in the pelvis, which is displacing the bowel from the pelvis and mid-abdomen. CT of the abdomen and pelvis shows a large pelvic mass arising from the sacral area and extending out through the neural foramina and into the right buttock.

Diagnosis

Ganglioneuroblastoma

Discussion

Ganglioneuroblastoma is one of the neuroblastic (neurogenic) tumors, which includes neuroblastoma and ganglioneuroma. It is a "mature" or differentiated tumor of the sympathetic nervous system; a slightly more benign counterpart of neuroblastoma, which is the 3rd most common malignancy of childhood. In contrast, ganglioneuroblastoma is very rare, with an incidence of about 5/1,000,000.

It is considered an intermediate or transitional tumor, in the spectrum of benign (ganglioneuroma) to malignant (neuroblastoma), and often presents as an abdominal mass in young children. Histologically, a ganglioneuroblastoma contain both neuroblasts and mature ganglion cells. Almost 95% of these tumors secrete catecholamines, such as VMA and HVA.

Radiographically they appear as well circumscribed, smooth or lobulated, with or without calcifications. CT is the preferred imaging method when identifying tumor size, organ of origin, tissue invasion, vascular encasement, adenopathy, and calcifications. MR is often used to define the extent of spinal involvement.

Ganglioneuroblastoma can metastasize, most commonly to bone or liver; if no metastatic disease is present at time of diagnosis, the prognosis is generally good. Treatment includes surgical resection and chemotherapy.

References

  1. Blickman JG, Parker BR, Barnes PD. Pediatric Radiology: The Requisites, 3rd ed. Mosby, 2009.
  2. Donnelly LF. Fundamentals of Pediatric Radiology. Saunders, 2001.

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