Ganglioneuroma
Khin Thway, BSc, MBBS, FRCPath
Key Facts
Terminology
Benign, well-differentiated tumor of neurogenic origin
Etiology/Pathogenesis
Usually develop de novo
Clinical Issues
Most commonly located in posterior mediastinum
Also in retroperitoneum (extraadrenal)
Affects older population than in neuroblastoma
Mostly children > 10 years
Microscopic Pathology
Abundant moderately cellular, uniform spindle cell matrix
Intermixed ganglion cells of variable number and maturation
Primitive neuroblasts absent
 Needle core biopsy specimen of ganglioneuroma shows small numbers of mature ganglion cells singly  and in clusters, within a moderately cellular schwannian stroma-rich background. |
 The number of ganglion cells varies in this case of ganglioneuroma. They are numerous and mature with abundant cytoplasm. The stroma is composed of bland, Schwann cell-like spindle cells. |
TERMINOLOGY
Abbreviations
Ganglioneuroma (GN)
Definitions
Benign, well-differentiated tumor of neural crest origin
Arises from sympathetic or peripheral nerves
ETIOLOGY/PATHOGENESIS
Associated Conditions
Within intestinal tract, lesions often associated with tumor syndromes
Polypoid GN in GI tract has associations with Cowden syndrome, juvenile polyposis, and tuberous sclerosis
Ganglioneuromatous polyposis reported in patients with neurofibromatosis type 1 and MEN2B
Most develop de novo
Some develop from maturation of neuroblastoma
CLINICAL ISSUES
Epidemiology
Incidence
Rare compared to other benign nerve sheath tumors
Age
Significantly older population than in neuroblastoma
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