Ganglioneuroma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Classification
According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two subtypes/groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[1]
Pathophysiology
On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors. On microscopic histopathological analysis, ganglioneuromas are characterized by spindle-shaped cells with cell borders in a fibrillar matrix containing ganglion cells with large round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm.[2] Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.
Causes
There are no established causes for ganglioneuroma.[3]
Differentiating brain tumors from other diseases
Ganglioglioma must be differentiated from neuroblastoma, ganglioneuroblastoma, spinal schwannoma, spinal neurofibroma, adrenal adenoma, adrenal carcinoma, and pheochromocytoma.[3][4]
Epidemiology and Demographics
The incidence of ganglioneuroma is approximately 1 per 100,000 children in the United States.[4] Ganglioneuroma is a rare disease that tends to affect children and young adults.[3] Females are more commonly affected with ganglioneuroma than males.[4] There is no racial predilection to ganglioneuroma.
Risk factors
There are no established risk factors for ganglioneuroma.[3]
Screening
Screening for ganglioneuroma is not recommended.[5]
Natural History, Complications and Prognosis
Common complications of ganglioneuroma include paralysis, metastases, and local recurrence.[6] The prognosis of ganglioneuroma is excellent with treatment.[3]
Diagnosis
Staging
According to the International Neuroblastoma Staging System (INSS), there are six stages of ganglioneuroma based on clinical, radiologic, and surgical features.[4]
History and Symptoms
Symptoms of ganglioneuroma include dyspnea, chest pain, abdominal pain, bloating, pain and numbness in limbs, paresis, diarrhea, diaphoresis, and hirsuitism.[3]
Physical examination
Common physical examination findings of ganglioneuroma include dyspnea, stridor, motor loss, sensory loss, diaphoresis, scoliosis, clitoromegaly, hirsuitism, and hypertension.[3]
Laboratory Findings
Some patients with ganglioneuroma may have elevated concentrations of VMA (vanillylmandelic acid) or HVA (homovanillic acid) in urine.[3][4]
X Ray
On chest x-ray, ganglioneuroma is characterized by a posterior mediastinal mass, which may cause rib spreading and foraminal erosion. Plain x-rays may show a mass in the retroperitoneum, pelvis, or neck indicating the presence of metastasis.[3]
CT
Chest, abdomen, and pelvic CT scan may be helpful in the diagnosis of ganglioneuroma. Findings on CT scan suggestive of ganglioneuroma include a solid, well-circumscribed, and encapsulated mass that is iso to hypoattenuating to muscle.[3][4]
MRI
Chest and abdominal MRI scan may be helpful in the diagnosis of ganglioneuroma. Findings on MRI scan suggestive of ganglioneuroma include a well circumscribed and encapsulatated mass characterized by low signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images.[3]
Ultrasound
On ultrasound, ganglioneuroma is characterized by a homogeneous, hypoechoic, well circumscribed mass.[4]
Other Imaging Findings
Other imaging tests for ganglioneuroma include scintigraphy or medaiodobenzylguanidine (MIBG) scan.[4]
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
References
- ↑ Okamatsu C, London WB, Naranjo A, Hogarty MD, Gastier-Foster JM, Look AT; et al. (2009). "Clinicopathological characteristics of ganglioneuroma and ganglioneuroblastoma: a report from the CCG and COG". Pediatr Blood Cancer. 53 (4): 563–9. doi:10.1002/pbc.22106. PMC 2730988. PMID 19530234.
- ↑ Vasiliadis K, Papavasiliou C, Fachiridis D, Pervana S, Michaelides M, Kiranou M; et al. (2012). "Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report". Int J Surg Case Rep. 3 (11): 541–3. doi:10.1016/j.ijscr.2012.07.008. PMC 3437388. PMID 22907039.
- ↑ 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 Causes for ganglioneuroma. National Library of Meidicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 Adam, O; Boia, ES (2007). "ganglioneuroma" (PDF). jurnalul pediatrului. 10 (39–40). Retrieved 10 September 2015.
- ↑ Fiori E, Pozzessere C, Lamazza A, Leone G, Borrini F, Schillaci A; et al. (2012). "Endoscopic treatment of ganglioneuroma of the colon associated with a lipoma: a case report". J Med Case Rep. 6: 304. doi:10.1186/1752-1947-6-304. PMC 3469395. PMID 22978818.
- ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm