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
Ganglioneuroma is a rare and benign tumor of the peripheral nervous system that tends to affect children and young adults.[1] Ganglioneuroma may be classified into two subtypes/groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[2] 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.[3] Ganglioglioma must be differentiated from neuroblastoma, ganglioneuroblastoma, spinal schwannoma, spinal neurofibroma, adrenal adenoma, adrenal carcinoma, and pheochromocytoma.[4][5] Females are more commonly affected with ganglioneuroma than males.[5] Common complications of ganglioneuroma include paralysis, metastases, and local recurrence.[6] The prognosis of ganglioneuroma is excellent with treatment.[4] Symptoms of ganglioneuroma include dyspnea, chest pain, abdominal pain, bloating, pain and numbness in limbs, paresis, diarrhea, diaphoresis, and hirsuitism.[4] Common physical examination findings of ganglioneuroma include dyspnea, stridor, motor loss, sensory loss, diaphoresis, scoliosis, clitoromegaly, hirsuitism, and hypertension.[4] On chest x-ray, ganglioneuroma is characterized by a posterior mediastinal mass, which may cause rib spreading and foraminal erosion.[4] On CT scan, ganglioneuroma is characterized by a solid, well-circumscribed, and encapsulated mass that is iso to hypoattenuating to muscle.[4][5] On MRI scan, ganglioneuroma is characterized by a well circumscribed and encapsulatated mass with low signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images.[4] Surgery is the mainstay of treatment for symptomatic ganglioneuroma.[4] Adjunctive chemotherapy and radiotherapy may be required.[4]
Classification
According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two subtypes/groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[2]
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.[3] Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.
Causes
There are no established causes for ganglioneuroma.[4]
Differentiating brain tumors from other diseases
Ganglioglioma must be differentiated from neuroblastoma, ganglioneuroblastoma, spinal schwannoma, spinal neurofibroma, adrenal adenoma, adrenal carcinoma, and pheochromocytoma.[4][5]
Epidemiology and Demographics
The incidence of ganglioneuroma is approximately 1 per 100,000 children in the United States.[5] Ganglioneuroma is a rare disease that tends to affect children and young adults.[4] Females are more commonly affected with ganglioneuroma than males.[5] There is no racial predilection to ganglioneuroma.
Risk factors
There are no established risk factors for ganglioneuroma.[4]
Screening
Screening for ganglioneuroma is not recommended.[7]
Natural History, Complications and Prognosis
Common complications of ganglioneuroma include paralysis, metastases, and local recurrence.[8] The prognosis of ganglioneuroma is excellent with treatment.[4]
Diagnosis
Staging
According to the International Neuroblastoma Staging System (INSS), there are six stages of ganglioneuroma based on clinical, radiologic, and surgical features.[5]
History and Symptoms
Symptoms of ganglioneuroma include dyspnea, chest pain, abdominal pain, bloating, pain and numbness in limbs, paresis, diarrhea, diaphoresis, and hirsuitism.[4]
Physical examination
Common physical examination findings of ganglioneuroma include dyspnea, stridor, motor loss, sensory loss, diaphoresis, scoliosis, clitoromegaly, hirsuitism, and hypertension.[4]
Laboratory Findings
Some patients with ganglioneuroma may have elevated concentrations of VMA (vanillylmandelic acid) or HVA (homovanillic acid) in urine.[4][5]
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.[4]
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.[4][5]
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.[4]
Ultrasound
On ultrasound, ganglioneuroma is characterized by a homogeneous, hypoechoic, well circumscribed mass.[5]
Other Imaging Findings
Other imaging tests for ganglioneuroma include scintigraphy or medaiodobenzylguanidine (MIBG) scan.[5]
Other Diagnostic Studies
Other diagnostic studies for ganglioneuroma include biopsy, which demonstrates spindle-shaped cells with cell borders in a fibrillar matrix containing ganglion cells with large round nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm.[4]
Treatment
Medical Therapy
The predominant therapy for symptomatic ganglioneuroma is surgical resection. Adjunctive chemotherapy and radiotherapy may be required.[4]
Surgery
Surgery is the mainstay of treatment for symptomatic ganglioneuroma.[4]
References
- ↑ Introduction of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
- ↑ 2.0 2.1 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.
- ↑ 3.0 3.1 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.
- ↑ 4.00 4.01 4.02 4.03 4.04 4.05 4.06 4.07 4.08 4.09 4.10 4.11 4.12 4.13 4.14 4.15 4.16 4.17 4.18 4.19 4.20 4.21 4.22 D.Dx of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma
- ↑ 5.00 5.01 5.02 5.03 5.04 5.05 5.06 5.07 5.08 5.09 5.10 Adam, O; Boia, ES (2007). "ganglioneuroma" (PDF). jurnalul pediatrului. 10 (39–40). Retrieved 10 September 2015.
- ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
- ↑ 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