Neurofibroma medical therapy: Difference between revisions
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==Treatment== | ==Treatment== | ||
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===Dermal neurofibroma=== | |||
Dermal neurofibromas are not usually surgically removed unless they are painful or disfiguring, because there are generally so many of them and they are not dangerous. | |||
CO<sub>2</sub> lasers have been used to remove dermal neurofibromas. In a paper titled ''Hypertrophic Scars After Therapy with CO<sub>2</sub> Laser for Treatment of Multiple Cutaneous Neurofibromas'' Ostertag et al. said this about treatment by laser: “The cosmetic disfigurement is the most important issue in the decision to treat cutaneous symptoms of neurofibromatosis. Treating patients with extensive neurofibromas with [a] CO<sub>2</sub> laser is still the best choice. However, it is strongly advised that a test treatment be performed to judge the effectiveness of the procedure and whether the developed scar is an acceptable trade-off.” <ref name=" pmid11896787">{{cite journal |author=Ostertag JU, Theunissen CC, Neumann HA. |title=Hypertrophic scars after therapy with CO<sub>2</sub> laser for treatment of multiple cutaneous neurofibromas |journal=Clinical Orthopaedics and Related Research |volume=28 |issue=3 |pages=296–8 |year=2002 |pmid=11896787|doi=10.1046/j.1524-4725.2002.01145.x}}</ref> | |||
===Plexiform neurofibroma=== | |||
====Surgery==== | |||
As of 2002, the primary treatment option for plexiform neurofibroma was [[surgery]].<ref>{{cite journal |vauthors=Packer RJ, Gutmann DH, Rubenstein A, etal |title=Plexiform neurofibromas in NF1: toward biologic-based therapy |journal=Neurology |volume=58 |issue=10 |pages=1461–70 |date=May 2002 |pmid=12041525 |url=http://www.neurology.org/cgi/pmidlookup?view=long&pmid=12041525 |doi=10.1212/wnl.58.10.1461}}</ref> | |||
Removal of plexiform neurofibromas is difficult because they can be large and cross tissue boundaries. However, besides pain, plexiform neurofibromas are sometimes removed due to the possibility of [[malignant transformation]]. | |||
The following examples show that plexiform neurofibromas can form anywhere and can make surgical resection difficult: | |||
* A large plexiform neurofibroma in the leg of a 6-year-old male. The authors state: “Our case was operated, as both the cutaneous and deep branches of the peroneal nerve were involved causing pain and numbness in the leg, and because there was a possibility for malignant transformation, as growth in the mass was realized by the family members of the patient.” In [[Wiktionary:in layman's terms|layman’s terms]], they decided to remove the neurofibroma because it was causing the boy pain and numbness in his leg, and because there was a possibility that it was undergoing a malignant transformation as his family noticed an increase in the tumor's size. The authors also note, “However, complete resection is quite difficult due to invasion of the tumor into the surrounding soft tissues.” <ref name=" pmid17377797">{{cite journal |author=Cebesoy O, Tutar E, Isik M, Arpacioglu O. |title=A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve |journal=Archives of Orthopaedic and Trauma Surgery |volume=127 |issue=8 |pages=709–12 |year=2007 |pmid=17377797|doi=10.1007/s00402-007-0303-1}}</ref> | |||
* A neurofibroma on the [[left ventricle]]. The neurofibroma was removed and the patient’s [[mitral valve]] had to be replaced.<ref>{{cite journal |vauthors=Iino K, Matsumoto Y, Endo M, etal |title=Surgical treatment of a left ventricular neurofibroma |journal=Journal of Cardiac Surgery |volume=21 |issue=3 |pages=278–80 |year=2006 |pmid=16684061 |doi=10.1111/j.1540-8191.2005.00135.x}}</ref> | |||
* A 14-year-old girl with NF1 was diagnosed with a neurofibroma involving her bladder, a rare location.<ref name=" pmid18458838">{{cite journal |author=Meesa IR, Junewick JJ |title=Pelvic plexiform neurofibroma involving the urinary bladder |journal=Pediatric Radiology |volume=38 |issue=8 |pages=916 |date=August 2008 |pmid=18458838 |doi=10.1007/s00247-008-0865-2}}</ref> | |||
====Radiation==== | |||
Once a plexiform neurofibroma has undergone malignant transformation, radiation and [[chemotherapy]] can be used as treatment. However, radiation is generally not used as a treatment for plexiform neurofibromas because of concerns that this could actually promote malignant transformation. There has even been a documented case of a Schwannoma being induced from a neurofibroma due to radiation therapy.<ref name=" pmid8998885">{{cite journal |author=Isler MH, Fogaça MF, Mankin HJ. |title=Radiation induced malignant schwannoma arising in a neurofibroma |journal=Clinical Orthopaedics and Related Research |issue=325 |pages=251–5 |year=1996 |pmid=8998885|doi=10.1097/00003086-199604000-00031 |volume=325}}</ref> | |||
===Medications=== | |||
[[ACE inhibitor]]s have been proposed as a novel treatment of neurofibromas. ACE inhibitors are currently used to treat hypertension and congestive heart failure, to avert remodeling and reinfarction after myocardial infarction, and to ameliorate diabetic nephropathy and other renal diseases. ACE inhibitors work by indirectly down regulating [[TGF beta|TGF-beta]], which is a growth factor that has been shown to influence the development of tumors.<ref name=" pmid18157575">{{cite journal |author=Namazi H. |title=ACE inhibitors: a novel treatment for neurofibroma |journal=Clinical Orthopaedics and Related Research |volume=15 |issue=5 |pages=1538–9 |year=2008 |pmid=18157575|doi=10.1245/s10434-007-9737-5}}</ref> | |||
==References== | ==References== | ||
Revision as of 20:04, 12 November 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Treatment
Dermal neurofibroma
Dermal neurofibromas are not usually surgically removed unless they are painful or disfiguring, because there are generally so many of them and they are not dangerous.
CO2 lasers have been used to remove dermal neurofibromas. In a paper titled Hypertrophic Scars After Therapy with CO2 Laser for Treatment of Multiple Cutaneous Neurofibromas Ostertag et al. said this about treatment by laser: “The cosmetic disfigurement is the most important issue in the decision to treat cutaneous symptoms of neurofibromatosis. Treating patients with extensive neurofibromas with [a] CO2 laser is still the best choice. However, it is strongly advised that a test treatment be performed to judge the effectiveness of the procedure and whether the developed scar is an acceptable trade-off.” [1]
Plexiform neurofibroma
Surgery
As of 2002, the primary treatment option for plexiform neurofibroma was surgery.[2]
Removal of plexiform neurofibromas is difficult because they can be large and cross tissue boundaries. However, besides pain, plexiform neurofibromas are sometimes removed due to the possibility of malignant transformation.
The following examples show that plexiform neurofibromas can form anywhere and can make surgical resection difficult:
- A large plexiform neurofibroma in the leg of a 6-year-old male. The authors state: “Our case was operated, as both the cutaneous and deep branches of the peroneal nerve were involved causing pain and numbness in the leg, and because there was a possibility for malignant transformation, as growth in the mass was realized by the family members of the patient.” In layman’s terms, they decided to remove the neurofibroma because it was causing the boy pain and numbness in his leg, and because there was a possibility that it was undergoing a malignant transformation as his family noticed an increase in the tumor's size. The authors also note, “However, complete resection is quite difficult due to invasion of the tumor into the surrounding soft tissues.” [3]
- A neurofibroma on the left ventricle. The neurofibroma was removed and the patient’s mitral valve had to be replaced.[4]
- A 14-year-old girl with NF1 was diagnosed with a neurofibroma involving her bladder, a rare location.[5]
Radiation
Once a plexiform neurofibroma has undergone malignant transformation, radiation and chemotherapy can be used as treatment. However, radiation is generally not used as a treatment for plexiform neurofibromas because of concerns that this could actually promote malignant transformation. There has even been a documented case of a Schwannoma being induced from a neurofibroma due to radiation therapy.[6]
Medications
ACE inhibitors have been proposed as a novel treatment of neurofibromas. ACE inhibitors are currently used to treat hypertension and congestive heart failure, to avert remodeling and reinfarction after myocardial infarction, and to ameliorate diabetic nephropathy and other renal diseases. ACE inhibitors work by indirectly down regulating TGF-beta, which is a growth factor that has been shown to influence the development of tumors.[7]
References
- ↑ Ostertag JU, Theunissen CC, Neumann HA. (2002). "Hypertrophic scars after therapy with CO2 laser for treatment of multiple cutaneous neurofibromas". Clinical Orthopaedics and Related Research. 28 (3): 296–8. doi:10.1046/j.1524-4725.2002.01145.x. PMID 11896787.
- ↑ Packer RJ, Gutmann DH, Rubenstein A, et al. (May 2002). "Plexiform neurofibromas in NF1: toward biologic-based therapy". Neurology. 58 (10): 1461–70. doi:10.1212/wnl.58.10.1461. PMID 12041525.
- ↑ Cebesoy O, Tutar E, Isik M, Arpacioglu O. (2007). "A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve". Archives of Orthopaedic and Trauma Surgery. 127 (8): 709–12. doi:10.1007/s00402-007-0303-1. PMID 17377797.
- ↑ Iino K, Matsumoto Y, Endo M, et al. (2006). "Surgical treatment of a left ventricular neurofibroma". Journal of Cardiac Surgery. 21 (3): 278–80. doi:10.1111/j.1540-8191.2005.00135.x. PMID 16684061.
- ↑ Meesa IR, Junewick JJ (August 2008). "Pelvic plexiform neurofibroma involving the urinary bladder". Pediatric Radiology. 38 (8): 916. doi:10.1007/s00247-008-0865-2. PMID 18458838.
- ↑ Isler MH, Fogaça MF, Mankin HJ. (1996). "Radiation induced malignant schwannoma arising in a neurofibroma". Clinical Orthopaedics and Related Research. 325 (325): 251–5. doi:10.1097/00003086-199604000-00031. PMID 8998885.
- ↑ Namazi H. (2008). "ACE inhibitors: a novel treatment for neurofibroma". Clinical Orthopaedics and Related Research. 15 (5): 1538–9. doi:10.1245/s10434-007-9737-5. PMID 18157575.