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Ganglioneuroma

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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]

Synonyms and keywords: Ganglioneuromata; GN; ganglion cell-containing tumor; ganglionic neuroma; neurofibroma gangliocellulare; neurofibroma ganglionare; neuroglioma; neuroma verum; gangliocytoma; gangliocytoma with adenoma; ganglioglioma; ganglioma

Overview

Overview

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] Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system. 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. Surgery is the mainstay of treatment for symptomatic ganglioneuroma. Adjunctive chemotherapy and radiotherapy may be required.[4]

Classification

According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[2]

Pathophysiology

Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36. 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]

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]

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. 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]

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]

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

  1. ↑ Introduction of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
  2. ↑ 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. ↑ 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. ↑ 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 D.Dx of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma
  5. ↑ 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.
  6. ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
  7. ↑ 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.
  8. ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm


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Historical Perspective

Historical Perspective

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Overview

Historical Perspective

References


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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two groups: Ganglioneuroma-maturing and ganglioneuroma-mature.[1]

Classification

According to International Neuroblastoma Pathology Classification (INPC), ganglioneuroma may be classified into two groups:[1]

  • Ganglioneuroma-maturing (intermediate)
  • Ganglioneuroma-mature (benign)

References

  1. ↑ 1.0 1.1 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.


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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Ganglioneuromas are derived from the primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36. 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.[1]

Pathophysiology

Pathogenesis

Genetics

Development of ganglioneuroma is the result of multiple genetic mutations. Genes involved in the pathogenesis of ganglioneuroma include MYCN oncogene and chromosome 1p36.

Associated Conditions

Ganglioneuromas may be associated with:[2]

Gross Pathology

  • On gross pathology, ganglioneuromas are characterized by solid, white, firm, well-circumscribed, and nodular tumors.
  • Gangliocytoma is commonly located in the following regions:[2]

Microscopic Pathology

  • 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. No atypia or mitotic activity is evident. The ganglion cells stain positive for S-100 protein.[1]
  • Ganglioneuromas are well differentiated neuronal tumors that do not contain immature elements.

Videos

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References

  1. ↑ 1.0 1.1 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.
  2. ↑ 2.0 2.1 2.2 Pathology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma


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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

There are no established causes for ganglioneuroma.[1]

Causes

There are no established causes for ganglioneuroma.[1]

References

  1. ↑ 1.0 1.1 Causes for ganglioneuroma. National Library of Meidicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm


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Differentiating Ganglioneuroma from other Diseases

Differentiating Ganglioneuroma from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Ganglioglioma must be differentiated from neuroblastoma, ganglioneuroblastoma, spinal schwannoma, spinal neurofibroma, adrenal adenoma, adrenal carcinoma, and pheochromocytoma.[1][2]

Differentiating Ganglioneuroma from other Diseases

Ganglioneuroma must be differentiated Neurofibroma must be differentiated from:[3][4][5]

Differentiating neurofibroma from other diseases
Disease entity Etiology (Genetic or others) Histopathological findings Immunohistochemical staining Risk factors Common site of involvement Clinical manifestations Other associated features
Neurofibroma[6][7][8][9][10][11][12][9][13][14][15][16]

Can be sporadic or as a part of Neurofibromatosis 1 and 2

Neurofibroma with degenerative atypia (“ancient change“) has following microscopic features:

Positive for:

Negative for:

Schwannoma[17][18][19][20][21] Positive for:

Negative for:

Symptoms of schwannoma depend on the location of the tumor:

Palisaded encapsulated neuroma (PEN) /solitary circumscribed neuroma[22] Positive for:

Negative for:

90% lesions affect the face involving:

Remaining 10% can occur anywhere in body involving:

Traumatic neuroma[23][24][25][26] Positive for: Most common oral locations are:

Rarely involves:

Also known as:
Neurotized melanocytic nevus[27][28][29][30] Positive for:

Negative for:

Can occur anywhere in body, mostly involving following areas: _
Cutaneous myxoma (Superficial angiomyxoma)[31][32][33][34] Positive for:

Negative for:

Associated with Carney’s complex/syndrome which includes following:

May be associated with NAME or LAMB syndrome

Nerve sheath myxoma[35][36][37][38][39][40] Positive for: _ Can occur anywhere in body:
Malignant peripheral nerve sheath tumor (MPNST)/malignant schwannoma[41][42][43][44][45][46]

Electron microscopy shows:

Positive for:

In case of glandular differentiation (malignant), positive for:

Negative for:

Associated with:

May be associated with:

Bulky deep-seated tumor usually arising from major nerves in:
Dermatofibrosarcoma protuberans (DFSP) Positive for:

Negative for:

_
Spindle cell lipoma Positive for:

Spindle cells are negative for:

_
  • Multiple well-circumscribed painless nodules involving several body parts
 _
Ganglioneuroma[47][48] Genes involved in the pathogenesis of ganglioneuroma include:

Two histologic subtypes:

Positive for:

Negative for:

Ganglioneuromas may be associated with:

Located along distribution of sympathetic nervous system:

Symptoms of ganglioneuroma vary depending on the location of tumor, and include the following:

Patients with ganglioneuroma may also have paraneoplastic syndrome, which may manifest with:

Ganglioneuromas are included in the neuroblastic tumors group, which includes:

Myxoid liposarcoma[49][50][51][52][53][54][55][56][57][58]

Atypical lipomatous tumor/well differentiated liposarcoma and dedifferentiated liposarcoma are associated with:

Myxoid liposarcoma is associated with:

Pleomorphicliposarcoma is associated with:

Well-differentiated liposarcoma:

De-differentiated liposarcoma:

Atypical lipomatous tumor/well differentiated liposarcoma is positive for:

_
Leiomyoma[59][60][61][62][63][64][65][66][60][63][67]

Positive for:

Negative for:

_
Inflammatory myofibroblastic tumor(IMT)[59][60][61][62][63][64][65][66][60][63][67]

Unknown underlying etiology, may be due to inflammatory reaction to:

Mutations such as:

Positive for:

Negative for:

Also known as:

Fibroepithelial polyp/Acrochordon[68][69][70][71][72][73][74][75][76][77][78][79][80][81][82][83][84][85][86][87] Associated with: Positive for:

Negative for:

Associated with:

Also known as:


References

  1. ↑ D.Dx of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma
  2. ↑ Adam, O; Boia, ES (2007). “ganglioneuroma” (PDF). jurnalul pediatrului. 10 (39–40). Retrieved 10 September 2015.
  3. ↑ Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2 Accessed on November 17, 2015
  4. ↑ http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/
  5. ↑ http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/
  6. ↑ Rodriguez, Fausto J.; Folpe, Andrew L.; Giannini, Caterina; Perry, Arie (2012). “Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems”. Acta Neuropathologica. 123 (3): 295–319. doi:10.1007/s00401-012-0954-z. ISSN 0001-6322.
  7. ↑ Choi, Kwangmin; Komurov, Kakajan; Fletcher, Jonathan S.; Jousma, Edwin; Cancelas, Jose A.; Wu, Jianqiang; Ratner, Nancy (2017). “An inflammatory gene signature distinguishes neurofibroma Schwann cells and macrophages from cells in the normal peripheral nervous system”. Scientific Reports. 7 (1). doi:10.1038/srep43315. ISSN 2045-2322.
  8. ↑ Liao, Chung-Ping; Booker, Reid C.; Brosseau, Jean-Philippe; Chen, Zhiguo; Mo, Juan; Tchegnon, Edem; Wang, Yong; Clapp, D. Wade; Le, Lu Q. (2018). “Contributions of inflammation and tumor microenvironment to neurofibroma tumorigenesis”. Journal of Clinical Investigation. 128 (7): 2848–2861. doi:10.1172/JCI99424. ISSN 0021-9738.
  9. ↑ 9.0 9.1 Staser, K.; Yang, F.-C.; Clapp, D. W. (2010). “Mast cells and the neurofibroma microenvironment”. Blood. 116 (2): 157–164. doi:10.1182/blood-2009-09-242875. ISSN 0006-4971.
  10. ↑ Muir, David; Neubauer, Debbie; Lim, Ingrid T.; Yachnis, Anthony T.; Wallace, Margaret R. (2001). “Tumorigenic Properties of Neurofibromin-Deficient Neurofibroma Schwann Cells”. The American Journal of Pathology. 158 (2): 501–513. doi:10.1016/S0002-9440(10)63992-2. ISSN 0002-9440.
  11. ↑ Wilkinson, Lana M.; Manson, David; Smith, Charles R. (2004). “Best Cases from the AFIP”. RadioGraphics. 24 (suppl_1): S237–S242. doi:10.1148/rg.24si035170. ISSN 0271-5333.
  12. ↑ Bernthal, Nicholas; Jones, Kevin; Monument, Michael; Liu, Ting; Viskochil, David; Randall, R. (2013). “Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect”. Cancers. 5 (4): 519–528. doi:10.3390/cancers5020519. ISSN 2072-6694.
  13. ↑ Mautner, V. F.; Friedrich, R. E.; von Deimling, A.; Hagel, C.; Korf, B.; Knöfel, M. T.; Wenzel, R.; Fünsterer, C. (2003). “Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma”. Neuroradiology. 45 (9): 618–625. doi:10.1007/s00234-003-0964-6. ISSN 0028-3940.
  14. ↑ Shen, M H; Harper, P S; Upadhyaya, M (1996). “Molecular genetics of neurofibromatosis type 1 (NF1)”. Journal of Medical Genetics. 33 (1): 2–17. doi:10.1136/jmg.33.1.2. ISSN 1468-6244.
  15. ↑ Rubin, Joshua B.; Gutmann, David H. (2005). “Neurofibromatosis type 1 — a model for nervous system tumour formation?”. Nature Reviews Cancer. 5 (7): 557–564. doi:10.1038/nrc1653. ISSN 1474-175X.
  16. ↑ Gray, Mark H. (1990). “Immunohistochemical Demonstration of Factor XIIIa Expression in Neurofibromas”. Archives of Dermatology. 126 (4): 472. doi:10.1001/archderm.1990.01670280056009. ISSN 0003-987X.
  17. ↑ Schwannoma. Dr Tim Luijkx and Dr Sara Wein et al. http://radiopaedia.org/articles/schwannoma
  18. ↑ Vestibular Schwannoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Vestibular_schwannoma Accessed on October 2 2015
  19. ↑ Giordano J, Rogers LV (1989). “Peripherally administered serotonin 5-HT3 receptor antagonists reduce inflammatory pain in rats”. European Journal of Pharmacology. 170 (1–2): 83–6. PMID 2612565. |access-date= requires |url= (help)
  20. ↑ Kolvenbach H, Lauven PM, Schneider B, Kunath U (1989). “Repetitive intercostal nerve block via catheter for postoperative pain relief after thoracotomy”. The Thoracic and Cardiovascular Surgeon. 37 (5): 273–6. doi:10.1055/s-2007-1020331. PMID 2588243. Retrieved 2015-11-20.
  21. ↑ Opaleva-Stegantseva VA, Ivanov AG, Gavrilina IA, Khar’kov EI, Ratovskaia VI (1986). “[Incidence of sudden death cases in acute coronary insufficiency and acute myocardial infarction at the pre-hospital stage in Krasnoyarsk]”. Kardiologiia (in Russian). 26 (5): 23–6. PMID 3735913. |access-date= requires |url= (help)
  22. ↑ Misago N, Inoue T, Narisawa Y (2007). “Unusual benign myxoid nerve sheath lesion: myxoid palisaded encapsulated neuroma (PEN) or nerve sheath myxoma with PEN/PEN-like features?”. Am J Dermatopathol. 29 (2): 160–4. doi:10.1097/01.dad.0000256688.91974.09. PMID 17414438.
  23. ↑ Lee EJ, Calcaterra TC, Zuckerbraun L (1998). “Traumatic neuromas of the head and neck”. Ear Nose Throat J. 77 (8): 670–4, 676. PMID 9745184.
  24. ↑ Hanna SA, Catapano J, Borschel GH (2016). “Painful pediatric traumatic neuroma: surgical management and clinical outcomes”. Childs Nerv Syst. 32 (7): 1191–4. doi:10.1007/s00381-016-3109-z. PMID 27179535.
  25. ↑ Foltán R, Klíma K, Spacková J, Sedý J (2008). “Mechanism of traumatic neuroma development”. Med Hypotheses. 71 (4): 572–6. doi:10.1016/j.mehy.2008.05.010. PMID 18599222.
  26. ↑ Yao C, Zhou X, Zhao B, Sun C, Poonit K, Yan H (2017). “Treatments of traumatic neuropathic pain: a systematic review”. Oncotarget. 8 (34): 57670–57679. doi:10.18632/oncotarget.16917. PMC 5593675. PMID 28915703.
  27. ↑ Gray MH, Smoller BR, McNutt NS, Hsu A (1990). “Neurofibromas and neurotized melanocytic nevi are immunohistochemically distinct neoplasms”. Am J Dermatopathol. 12 (3): 234–41. PMID 1693815.
  28. ↑ Chen Y, Klonowski PW, Lind AC, Lu D (2012). “Differentiating neurotized melanocytic nevi from neurofibromas using Melan-A (MART-1) immunohistochemical stain”. Arch Pathol Lab Med. 136 (7): 810–5. doi:10.5858/arpa.2011-0335-OA. PMID 22742554.
  29. ↑ Singh N, Chandrashekar L, Kar R, Sylvia MT, Thappa DM (2015). “Neurotized congenital melanocytic nevus resembling a pigmented neurofibroma”. Indian J Dermatol. 60 (1): 46–50. doi:10.4103/0019-5154.147789. PMC 4318062. PMID 25657396.
  30. ↑ Gray MH, Smoller BR, McNutt NS, Hsu A (1990). “Immunohistochemical demonstration of factor XIIIa expression in neurofibromas. A practical means of differentiating these tumors from neurotized melanocytic nevi and schwannomas”. Arch Dermatol. 126 (4): 472–6. PMID 1690969.
  31. ↑ https://www.sciencedirect.com/topics/medicine-and-dentistry/cutaneous-myxoma
  32. ↑ Alaiti, Samer; Nelson, Fern P.; Ryoo, Jei W. (2000). “Solitary cutaneous myxoma”. Journal of the American Academy of Dermatology. 43 (2): 377–379. doi:10.1067/mjd.2000.101878. ISSN 0190-9622.
  33. ↑ Carney, J. Aidan (1986). “Cutaneous Myxomas”. Archives of Dermatology. 122 (7): 790. doi:10.1001/archderm.1986.01660190068018. ISSN 0003-987X.
  34. ↑ Iida, Ken; Egi, Takeshi; Shigi, Masato; Sogabe, Yusuke; Ohashi, Hirotsugu (2019). “Cutaneous Myxoma of Multiple Lesions”. Plastic and Reconstructive Surgery – Global Open. 7 (2): e2040. doi:10.1097/GOX.0000000000002040. ISSN 2169-7574.
  35. ↑ Fetsch JF, Laskin WB, Miettinen M (2005). “Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate”. Am J Surg Pathol. 29 (12): 1615–24. PMID 16327434.
  36. ↑ Yadav SK, Singh S, Sarin N, Naeem R, Pruthi SK (2019). “Nerve Sheath Myxoma of Scalp: A Rare Site of Presentation”. Int J Trichology. 11 (1): 34–37. doi:10.4103/ijt.ijt_45_18. PMC 6385516. PMID 30820132.
  37. ↑ Bhat A, Narasimha A, C V, Vk S (2015). “Nerve sheath myxoma: report of a rare case”. J Clin Diagn Res. 9 (4): ED07–9. doi:10.7860/JCDR/2015/10911.5810. PMC 4437072. PMID 26023558.
  38. ↑ Avninder S, Ramesh V, Vermani S (2007). “Benign nerve sheath myxoma (myxoid neurothekeoma) in the leg”. Dermatol Online J. 13 (2): 14. PMID 17498433.
  39. ↑ Kim BW, Won CH, Chang SE, Lee MW (2014). “A case of nerve sheath myxoma on finger”. Indian J Dermatol. 59 (1): 99–101. doi:10.4103/0019-5154.123526. PMC 3884944. PMID 24470676.
  40. ↑ Pulitzer DR, Reed RJ (1985). “Nerve-sheath myxoma (perineurial myxoma)”. Am J Dermatopathol. 7 (5): 409–21. PMID 4091218.
  41. ↑ Valeyrie-Allanore, L.; Ismaili, N.; Bastuji-Garin, S.; Zeller, J.; Wechsler, J.; Revuz, J.; Wolkenstein, P. (2005). “Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1”. British Journal of Dermatology. 153 (1): 79–82. doi:10.1111/j.1365-2133.2005.06558.x. ISSN 0007-0963.
  42. ↑ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (2002). “Malignant peripheral nerve sheath tumours in neurofibromatosis 1”. J Med Genet. 39 (5): 311–4. PMC 1735122. PMID 12011145.
  43. ↑ Panigrahi S, Mishra SS, Das S, Dhir MK (2013). “Primary malignant peripheral nerve sheath tumor at unusual location”. J Neurosci Rural Pract. 4 (Suppl 1): S83–6. doi:10.4103/0976-3147.116480. PMC 3808069. PMID 24174807.
  44. ↑ Ferrari A, Bisogno G, Carli M (2007). “Management of childhood malignant peripheral nerve sheath tumor”. Paediatr Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
  45. ↑ Neville H, Corpron C, Blakely ML, Andrassy R (2003). “Pediatric neurofibrosarcoma”. J Pediatr Surg. 38 (3): 343–6, discussion 343-6. doi:10.1053/jpsu.2003.50105. PMID 12632346.
  46. ↑ Zehou, Ouidad; Fabre, Elizabeth; Zelek, Laurent; Sbidian, Emilie; Ortonne, Nicolas; Banu, Eugeniu; Wolkenstein, Pierre; Valeyrie-Allanore, Laurence (2013). “Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review”. Orphanet Journal of Rare Diseases. 8 (1): 127. doi:10.1186/1750-1172-8-127. ISSN 1750-1172.
  47. ↑ Vasiliadis, K.; Papavasiliou, C.; Fachiridis, D.; Pervana, S.; Michaelides, M.; Kiranou, M.; Makridis, C. (2012). “Retroperitoneal extra-adrenal ganglioneuroma involving the infrahepatic inferior vena cava, celiac axis and superior mesenteric artery: A case report”. International Journal of Surgery Case Reports. 3 (11): 541–543. doi:10.1016/j.ijscr.2012.07.008. ISSN 2210-2612.
  48. ↑ https://radiopaedia.org/articles/ganglioneuroma
  49. ↑ Khin Thway, Rashpal Flora, Chirag Shah, David Olmos & Cyril Fisher (2012). “Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors”. The American journal of surgical pathology. 36 (3): 462–469. doi:10.1097/PAS.0b013e3182417330. PMID 22301498. Unknown parameter |month= ignored (help)
  50. ↑ J. Rosai, M. Akerman, P. Dal Cin, I. DeWever, C. D. Fletcher, N. Mandahl, F. Mertens, F. Mitelman, A. Rydholm, R. Sciot, G. Tallini, H. Van den Berghe, W. Van de Ven, R. Vanni & H. Willen (1996). “Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group)”. The American journal of surgical pathology. 20 (10): 1182–1189. PMID 8827023. Unknown parameter |month= ignored (help)
  51. ↑ Dal Cin, Paola; Kools, Patrick; Sciot, Raf; De Wever, Ivo; Van Damme, Boudewijn; Van de Ven, Wim; Van Den Berghe, Herman (1993). “Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors”. Cancer Genetics and Cytogenetics. 68 (2): 85–90. doi:10.1016/0165-4608(93)90001-3. ISSN 0165-4608.
  52. ↑ Dei Tos, Angelo P.; Doglioni, Claudio; Piccinin, Sara; Sciot, Raf; Furlanetto, Alberto; Boiocchi, Mauro; Dal Cin, Paola; Maestro, Roberta; Fletcher, Christopher D. M.; Tallini, Giovanni (2000). “Coordinated expression and amplification of theMDM2,CDK4, andHMGI-C genes in atypical lipomatous tumours”. The Journal of Pathology. 190 (5): 531–536. doi:10.1002/(SICI)1096-9896(200004)190:5<531::AID-PATH579>3.0.CO;2-W. ISSN 0022-3417.
  53. ↑ Dei Tos, A (2000). “Liposarcoma: New entities and evolving concepts”. Annals of Diagnostic Pathology. 4 (4): 252–266. doi:10.1053/adpa.2000.8133. ISSN 1092-9134.
  54. ↑ M. D. Kraus, L. Guillou & C. D. Fletcher (1997). “Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma”. The American journal of surgical pathology. 21 (5): 518–527. PMID 9158675. Unknown parameter |month= ignored (help)
  55. ↑ P. Argani, F. Facchetti, G. Inghirami & J. Rosai (1997). “Lymphocyte-rich well-differentiated liposarcoma: report of nine cases”. The American journal of surgical pathology. 21 (8): 884–895. PMID 9255251. Unknown parameter |month= ignored (help)
  56. ↑ H. L. Evans (1979). “Liposarcoma: a study of 55 cases with a reassessment of its classification”. The American journal of surgical pathology. 3 (6): 507–523. PMID 534388. Unknown parameter |month= ignored (help)
  57. ↑ A. P. Dei Tos, T. Mentzel, P. L. Newman & C. D. Fletcher (1994). “Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases”. The American journal of surgical pathology. 18 (9): 913–921. PMID 8067512. Unknown parameter |month= ignored (help)
  58. ↑ D. C. Dahlin, K. K. Unni & T. Matsuno (1977). “Malignant (fibrous) histiocytoma of bone–fact or fancy?”. Cancer. 39 (4): 1508–1516. PMID 192432. Unknown parameter |month= ignored (help)
  59. ↑ 59.0 59.1 Coffin CM, Watterson J, Priest JR, Dehner LP (1995). “Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases”. Am J Surg Pathol. 19 (8): 859–72. PMID 7611533.
  60. ↑ 60.0 60.1 60.2 60.3 Wenig BM, Devaney K, Bisceglia M (1995). “Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm”. Cancer. 76 (11): 2217–29. PMID 8635024.
  61. ↑ 61.0 61.1 Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD (1995). “Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases”. Histopathology. 27 (4): 313–23. PMID 8847061.
  62. ↑ 62.0 62.1 Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B (2003). “Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review”. Hum Pathol. 34 (3): 253–62. doi:10.1053/hupa.2003.35. PMID 12673560.
  63. ↑ 63.0 63.1 63.2 63.3 Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA (2005). “Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors”. Am J Surg Pathol. 29 (10): 1348–55. PMID 16160478.
  64. ↑ 64.0 64.1 Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R; et al. (2006). “Inflammatory myofibroblastic tumors”. J Surg Oncol. 94 (5): 385–91. doi:10.1002/jso.20516. PMID 16967468.
  65. ↑ 65.0 65.1 Coffin CM, Dehner LP, Meis-Kindblom JM (1998). “Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations”. Semin Diagn Pathol. 15 (2): 102–10. PMID 9606802.
  66. ↑ 66.0 66.1 Berardi RS, Lee SS, Chen HP, Stines GJ (1983). “Inflammatory pseudotumors of the lung”. Surg Gynecol Obstet. 156 (1): 89–96. PMID 6336632.
  67. ↑ 67.0 67.1 Coffin CM, Hornick JL, Fletcher CD (2007). “Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases”. Am J Surg Pathol. 31 (4): 509–20. doi:10.1097/01.pas.0000213393.57322.c7. PMID 17414097.
  68. ↑ Cukic O, Jovanovic MB (2019). “Large Fibroepithelial Polyp of the Palatine Tonsil”. Ear Nose Throat J: 145561319841203. doi:10.1177/0145561319841203. PMID 30997841.
  69. ↑ Vatansever M, Dinç E, Dursun Ö, Oktay ÖÖ, Arpaci R (2019). “Atypical presentation of fibroepithelial polyp: a report of two cases”. Arq Bras Oftalmol. doi:10.5935/0004-2749.20190050. PMID 30916216.
  70. ↑ Rexhepi M, Trajkovska E, Besimi F, Rufati N (2018). “Giant Fibroepithelial Polyp of Vulva: A Case Report and Review of Literature”. Pril (Makedon Akad Nauk Umet Odd Med Nauki). 39 (2–3): 127–130. doi:10.2478/prilozi-2018-0051. PMID 30864355.
  71. ↑ Jabbour J, Chappell JR, Busby M, McCubbery NW, Brown DF, Park SJK; et al. (2019). “Glottic Obstruction from Fibroepithelial Polyp”. Am J Case Rep. 20: 219–223. doi:10.12659/AJCR.914907. PMC 6388646. PMID 30778021.
  72. ↑ Hong P, Cai Y, Li Z, Fan S, Yang K, Hao H; et al. (2019). “Modified Laparoscopic Partial Ureterectomy for Adult Ureteral Fibroepithelial Polyp: Technique and Initial Experience”. Urol Int. 102 (1): 13–19. doi:10.1159/000494804. PMID 30448831.
  73. ↑ Uçar M, Baş E, Akkoç A, Topçuoğlu M (2018). “Fibroepithelial Polyp of the Ureter: A Rare Cause of Hydronephrosis”. J Endourol Case Rep. 4 (1): 166–168. doi:10.1089/cren.2018.0031. PMC 6225073. PMID 30426076.
  74. ↑ Chaker K, Rhouma SB, Daly KM, Zehani A, Bibi M, Chehida MAB; et al. (2019). “Benign fibroepithelial polyp of the ureter: A case report”. Urol Case Rep. 22: 52–53. doi:10.1016/j.eucr.2018.10.019. PMC 6226574. PMID 30425926.
  75. ↑ Hajji F, Moufid K, Ghoundale O, Touiti D (2019). “A rare case of successful endoscopic management of a fibroepithelial polyp with intussusception of the ureter and periodic prolapse into bladder”. Ann R Coll Surg Engl. 101 (2): e66–e70. doi:10.1308/rcsann.2018.0198. PMC 6351868. PMID 30421620.
  76. ↑ Lee H, Sade I, Gilani S, Zhong M, Lombardo G (2018). “A Giant Fibroepithelial Polyp of the Small Bowel Associated with High-Grade Obstruction”. Am Surg. 84 (7): e210–e211. PMID 30401014.
  77. ↑ Chaker K (2019). “Benign fibroepithelial polyp of the ureter: A case report”. Urol Case Rep. 22: 15–16. doi:10.1016/j.eucr.2018.09.021. PMC 6180234. PMID 30319938.
  78. ↑ Lozano-Peña AK, Lamadrid-Zertuche AC, Ocampo-Candiani J (2019). “Giant fibroepithelial polyp of the vulva”. Australas J Dermatol. 60 (1): 70–71. doi:10.1111/ajd.12886. PMID 30009441.
  79. ↑ Eckstein M, Agaimy A, Woenckhaus J, Winter A, Bittmann I, Janzen J; et al. (2019). “DICER1 mutation-positive giant botryoid fibroepithelial polyp of the urinary bladder mimicking embryonal rhabdomyosarcoma”. Hum Pathol. 84: 1–7. doi:10.1016/j.humpath.2018.05.015. PMID 29883781.
  80. ↑ Akdere H, Çevik G (2018). “Rare Fibroepithelial Polyp Extending Along the Ureter: A Case Report”. Balkan Med J. 35 (3): 275–277. doi:10.4274/balkanmedj.2017.1537. PMC 5981127. PMID 29843497.
  81. ↑ Ballard DH, Rove KO, Coplen DE, Chen TY, Hulett Bowling RL (2018). “Fibroepithelial polyp causing urethral obstruction: Diagnosis by cystourethrogram”. Clin Imaging. 51: 164–167. doi:10.1016/j.clinimag.2018.05.009. PMC 6404776. PMID 29800931.
  82. ↑ Amin A, Amin Z, Al Farsi AR (2018). “Septic presentation of a giant fibroepithelial polyp of the vulva”. BMJ Case Rep. 2018. doi:10.1136/bcr-2017-222789. PMID 29574427.
  83. ↑ Gupta R, Smita S, Sinha R, Sinha N, Sinha L (2018). “Giant fibroepithelial polyp of the thigh and retroperitoneal fibromatosis in a young woman: a rare case”. Skeletal Radiol. 47 (9): 1299–1304. doi:10.1007/s00256-018-2904-x. PMID 29487969.
  84. ↑ Rajeesh Mohammed PK, Choudhury BK, Dalai RP, Rana V (2017). “Fibroepithelial Polyp with Sebaceous Hyperplasia: A Case Report”. Indian J Med Paediatr Oncol. 38 (3): 404–406. doi:10.4103/ijmpo.ijmpo_124_17. PMC 5686997. PMID 29200704.
  85. ↑ Lee MH, Hwang JY, Lee JH, Kim DH, Song SH (2017). “Fibroepithelial polyp of the vulva accompanied by lymphangioma circumscriptum”. Obstet Gynecol Sci. 60 (4): 401–404. doi:10.5468/ogs.2017.60.4.401. PMC 5547092. PMID 28791276.
  86. ↑ Ten Donkelaar CS, Houwert AC, Ten Kate FJW, Lock MTWT (2017). “Polypoid arteriovenous malformation of the ureter mimicking a fibroepithelial polyp, a case report”. BMC Urol. 17 (1): 55. doi:10.1186/s12894-017-0237-z. PMC 5504856. PMID 28693464.
  87. ↑ Saito N, Yamasaki M, Daido W, Ishiyama S, Deguchi N, Taniwaki M (2017). “A bronchial fibroepithelial polyp with abnormal findings on auto-fluorescence imaging”. Respirol Case Rep. 5 (5): e00244. doi:10.1002/rcr2.244. PMC 5465754. PMID 28603622.


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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

The incidence of ganglioneuroma is approximately 1 per 100,000 children in the United States.[1] Ganglioneuroma is a rare disease that tends to affect children and young adults.[2] Females are more commonly affected with ganglioneuroma than males.[1] There is no racial predilection to ganglioneuroma.

Epidemiology and Demographics

Incidence

The incidence of ganglioneuroma is approximately 1 per 100,000 children in the United States.[1]

Age

Ganglioneuroma is a rare disease that tends to affect children and young adults. The median age at diagnosis is 7 years.[2]

Gender

Females are more commonly affected with ganglioneuroma than males. The female to male ratio is approximately 1.5 to 1.[1]

Race

There is no racial predilection to ganglioneuroma.

References

  1. ↑ 1.0 1.1 1.2 1.3 Adam, O; Boia, ES (2007). “ganglioneuroma” (PDF). jurnalul pediatrului. 10 (39–40). Retrieved 10 September 2015.
  2. ↑ 2.0 2.1 Epidemiology of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma


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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

There are no established risk factors for ganglioneuroma.[1]

Risk Factors

There are no established risk factors for ganglioneuroma.[1]

References

  1. ↑ 1.0 1.1 Risk factors for ganglioneuroma. National Library of Meidicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm


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Screening

Screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Screening for ganglioneuroma is not recommended.[1]

Screening

Screening for ganglioneuroma is not recommended.[1]

References

  1. ↑ 1.0 1.1 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.


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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Common complications of ganglioneuroma include paralysis, metastases, and local recurrence.[1] The prognosis of ganglioneuroma is excellent with treatment.[2]

Complications

Common complications of ganglioneuroma include:[3]

Common complications that can develop as a result of the treatment of ganglioneuroma include:[4][5]

Prognosis

The prognosis of ganglioneuroma is excellent with treatment.[2]

References

  1. ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
  2. ↑ 2.0 2.1 Prognosis of ganglioneuroma. Dr Bruno Di Muzio and Dr Yuranga Weerakkody et al. Radiopaedia 2015. http://radiopaedia.org/articles/ganglioneuroma
  3. ↑ Complications of ganglioneuroma. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/001437.htm
  4. ↑ Sánchez-Galán A, Barrena S, Vilanova-Sánchez A, Martín SH, Lopez-Fernandez S, García P; et al. (2014). “Ganglioneuroma: to operate or not to operate”. Eur J Pediatr Surg. 24 (1): 25–30. doi:10.1055/s-0033-1358790. PMID 24327216.
  5. ↑ Leuthardt R, Petralli C, Lütschg J, von Schweinitz D, Kaiser G (2001). “Cortical blindness: an unusual complication after removal of a ganglioneuroma of the neck”. Childs Nerv Syst. 17 (6): 356–8. PMID 11417417.


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Diagnosis

Diagnosis

Staging | History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case#1


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