Neurofibroma

A moderately firm, benign, encapsulated tumor resulting from proliferation of SCHWANN CELLS and FIBROBLASTS that includes portions of nerve fibers. The tumors usually develop along peripheral or cranial nerves and are a central feature of NEUROFIBROMATOSIS 1, where they may occur intracranially or involve spinal roots. Pathologic features include fusiform enlargement of the involved nerve. Microscopic examination reveals a disorganized and loose cellular pattern with elongated nuclei intermixed with fibrous strands. (From Adams et al., Principles of Neurology, 6th ed, p1016)

Also Known As:

Networked: 844 relevant articles (20 outcomes, 62 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neurilemmoma (Schwannoma)
2.	Neoplasms (Cancer)
3.	Neurofibromatosis 1 (Neurofibromatosis Type I)
4.	Neurofibroma
5.	Meningioma (Meningiomas)

Experts

1.	Ratner, Nancy: 28 articles (01/2022 - 12/2003)
2.	Wu, Jianqiang: 16 articles (01/2022 - 01/2005)
3.	Rizvi, Tilat A: 13 articles (01/2022 - 01/2005)
4.	Dombi, Eva: 13 articles (11/2020 - 08/2008)
5.	Wolkenstein, Pierre: 12 articles (10/2022 - 12/2003)
6.	Friedrich, Reinhard E: 10 articles (01/2019 - 08/2004)
7.	Kluwe, Lan: 10 articles (01/2019 - 08/2004)
8.	Choi, Kwangmin: 9 articles (11/2020 - 10/2012)
9.	Carroll, Steven L: 9 articles (09/2018 - 11/2004)
10.	Le, Lu Q: 8 articles (01/2022 - 07/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Neurofibroma:

1.	ProgesteroneFDA LinkGeneric 07/01/2008 - "These data suggest that progesterone plays an important role in the development of neurofibromas in NF1." 07/01/2008 - "Schwann cells from human neurofibromas show increased proliferation rates under the influence of progesterone." 02/09/2005 - "High doses of progesterone might stimulate the growth of neurofibromas and deserve more caution." 02/15/2003 - "We hypothesize that progesterone may play an important role in neurofibroma growth and suggest that antiprogestins may be useful in the treatment of this tumor." 07/01/2012 - "The rapid growth of the neurofibroma may have been the result of hormonal influence of estrogen or progesterone or both. "
2.	Somatostatin Receptors (Somatostatin Receptor)IBA 11/01/1987 - "Tumors originating from neuroblasts, i.e., ganglioneuroblastoma and medulloblastoma, contained a high density of somatostatin receptors, whereas neurinomas and neurofibromas as well as the ependymomas, one teratoma, and one plexus papilloma were lacking such receptors. " 11/01/1987 - "Fifty-two brain tumors, consisting of 17 astrocytomas, 4 oligodendrogliomas, 20 glioblastomas, 3 neurinomas, 2 ependymomas, 1 neurofibroma, 1 ganglioneuroblastoma, 1 medulloblastoma, 1 plexus papilloma, 1 teratoma, and 1 germinoma, were tested for their content of specific somatostatin receptors using autoradiographic techniques or in vitro binding assays with membrane homogenates. " 12/01/2005 - "In the present study, we examined the expression of all 5 somatostatin receptor subtypes (sst1-5) in 3 normal peripheral nerves, 3 traumatic neuromas, 27 schwannomas, 18 neurofibromas, and 177 malignant peripheral nerve sheath tumors (MPNSTs) by immunohistochemistry as well as by Western blot and reverse transcriptase-polymerase chain reaction investigations in 2 normal peripheral nerves, one neurofibroma, 5 schwannomas, and 5 MPNSTs. " 01/15/1987 - "The following tumors did not contain somatostatin receptors: prostate carcinomas (n = 17); prostate hyperplasia (n = 2); ovarian carcinomas (n = 6); endometrial carcinomas (n = 4); primary liver cell carcinomas (n = 3); pheochromocytomas (n = 3); aldosteronomas (n = 2); medullary thyroid carcinomas (n = 2); one adrenocorticotropic hormone-secreting pulmonary carcinoid; one astrocytoma; one neurofibroma; one lung tumor; and one bladder tumor. " 07/01/2001 - "Imaging of somatostatin receptors (SSTRs) using [111In]diethylenetriaminepentaacetic-acid-octreotide (DTPAOC) has proven to be helpful in the differentiation of meningiomas, neurinomas or neurofibromas, and metastases as well as in the follow-up of meningiomas. "
3.	AcidsIBA 11/01/1987 - "Single photon emission computed tomography (SPECT) was used to study uptake of technetium-99m diethylenetriaminepentaacetic acid ([99mTc]DTPA) by para-axial neurofibromas in 13 patients. " 01/01/1969 - "[On the occurrence of acid mucopolysaccharides in neurofibromas and neurofibrosarcomas]." 07/01/2001 - "Imaging of somatostatin receptors (SSTRs) using [111In]diethylenetriaminepentaacetic-acid-octreotide (DTPAOC) has proven to be helpful in the differentiation of meningiomas, neurinomas or neurofibromas, and metastases as well as in the follow-up of meningiomas. "
4.	Monoclonal AntibodiesIBA 01/01/1986 - "The Leu-7 (HNK-1) monoclonal antibody, a hematological marker, has been found to cross-react with a number of cells of schwannian origin and may be helpful in the differential diagnosis of schwannomas and neurofibromas from other soft tissue neoplasms. " 02/01/1988 - "Leu-7 immunoreactivity was detected by a monoclonal antibody in 12 of 16 schwannomas, 12 of 20 neurofibromas, and 17 of 17 traumatic neuromas. " 01/01/2015 - "We investigated transducer-like enhancer of split 1 (TLE1), monoclonal antibody, expression by immunohistochemical analysis in a group of 74 synovial sarcoma cases, 20 cases of MPNST, 12 cases of neurofibroma, 15 cases of schwannoma, 5 cases of MFH, 10 cases of lieomyosarcoma and 10 cases of solitary fibrous tumor. " 02/01/2012 - "We retrospectively analyzed surgically resected primary tumors, including 20 dermal neurofibromas, 23 plexiform neurofibromas, and 17 MPNSTs, by immunohistochemistry in paraffin sections of NF-1 tumors with the use of the L1-specific monoclonal antibody UJ127, which does not cross-react with other members of the L1 family. " 07/01/1988 - "Seventeen surgically resected meningiomas and six neurinomas including one neurofibroma were labelled with frozen sections using a monoclonal antibody Ki-67, which reacts with a nuclear antigen expressed by proliferating cells. "
5.	Capsules (Microcapsules)IBA 01/01/2018 - "Neurofibromas infiltrate between nerve fascicles and do not have a discrete capsule. " 01/01/1997 - "A solitary neurofibroma arising from the thyroid capsule in a 64 year-old woman is reported. " 01/01/1997 - "An Unusual Case of Neurofibroma of the Thyroid Capsule." 11/01/2006 - "Microdebrider decompression of the neck neurofibroma allowed for preservation of the capsule without injuring vital structures in the neck. " 07/01/1991 - "It can also be helpful in distinguishing between schwannomas and neurofibromas by the location of the nerve of origin and the presence of a capsule."
6.	MART-1 AntigenIBA 01/01/2023 - "La biopsia de piel presentó cambios característicos de neurofibroma; sin embargo, en la porción profunda de la lesión se observaron depósitos de melanina positivos para S100, Melan-A y HMB45, con lo que se estableció el diagnóstico de neurofibroma pigmentado. " 07/01/2012 - "Our data indicate that Melan-A immunohistochemical staining is helpful in differentiating neurotized melanocytic nevi from neurofibromas when distinction on histomorphology alone is difficult." 01/01/2000 - "To evaluate the role of Melan-A in differentiating melanocytic and nonmelanocytic lesions we assessed a number of benign nonmelanocytic skin lesions including neurofibromas, granular cell tumors, and dermatofibromas. " 01/01/2023 - "The skin biopsy showed characteristics of neurofibroma; however, in the deep portion of the lesion, melanin deposits positive for S100, Melan-A, and HMB45 were observed, thus establishing the diagnosis of pigmented neurofibroma. " 08/01/2010 - "All 5 cases of neurofibroma that were MART-1/MelanA positive also demonstrated positive staining with nestin. "
7.	Sirolimus (Rapamycin)FDA Link 05/01/2019 - "This case demonstrates the efficacy of topical sirolimus in the management of neurofibromas in NF1." 06/01/2014 - "We present 3 cases of patients with NF1, aged 8, 16, and 17 years, followed for inoperable and symptomatic PNs; patients received sirolimus for life-threatening and painful neurofibromas after multidisciplinary consultation. " 04/30/2019 - "Surgical removal of the oval pelvic formation, identified as neurofibroma was planned, as well as the initiation of systemic therapy with Sirolimus for the plexiform sciatic formation, infiltrating the bladder. " 05/01/2019 - "Topical sirolimus as an effective treatment for a deep neurofibroma in a patient with neurofibromatosis type I." 01/01/2023 - "Pilot study for the treatment of cutaneous neurofibromas in neurofibromatosis type 1 patients using topical sirolimus gel."
8.	AZD 6244IBA 12/29/2016 - "Treatment with selumetinib resulted in confirmed partial responses (tumor volume decreases from baseline of ≥20%) in 17 of the 24 children (71%) and decreases from baseline in neurofibroma volume in 12 of 18 mice (67%). " 12/29/2016 - "We also tested selumetinib using a mouse model of neurofibromatosis type 1-related neurofibroma. " 06/01/2023 - "The efficacy of selumetinib in various solid tumors such as colorectal cancer, lung cancer, neurofibroma, and melanoma is investigated. " 01/01/2020 - "The MEK inhibitor selumetinib reduces spinal neurofibroma burden in patients with NF1 and plexiform neurofibromas."
9.	tranilastIBA 03/01/2009 - "Thus, tranilast can be a potent therapeutic agent to inhibit the growth of neurofibromas." 01/01/2018 - "With the use of a cell-based drug screening assay, we have now identified the antiallergy drug tranilast (N-(3,4-dimethoxycinnamoyl) anthranilic acid) as an inhibitor of EMT and found that it attenuated the expression of mesenchymal markers and angiogenesis-related genes in NF1-mutated sNF96.2 cells and in neurofibroma cells from NF1 patients. " 06/01/2009 - "Tranilast: a novel preventor of neurofibroma growth?" 03/01/2009 - "We assumed that tranilast would suppress neurofibroma cell growth. " 03/01/2009 - "In order to prove this hypothesis, we investigated the effectiveness of tranilast in inhibiting the tumor growth using a new cell culture system obtained from patients with neurofibromas. "
10.	Octreotide (Sandostatin)FDA LinkGeneric 07/01/1998 - "[111In-DTPA-d-Phe1]-octreotide scintigraphy allows differentiation between meningiomas and neurinomas or neurofibromas and therefore provides complementary information to computed tomography or magnetic resonance imaging. " 07/01/2001 - "Imaging of somatostatin receptors (SSTRs) using [111In]diethylenetriaminepentaacetic-acid-octreotide (DTPAOC) has proven to be helpful in the differentiation of meningiomas, neurinomas or neurofibromas, and metastases as well as in the follow-up of meningiomas. "

Therapies and Procedures

1.	Radiotherapy 06/01/2005 - "The purpose of this study was to evaluate the efficacy of radiotherapy (RT) and stereotactic radiosurgery (SRS) for neurofibromas. " 03/31/2023 - "Radiotherapy should be avoided because of potential carcinogenicity to the neurofibromas. " 01/01/2007 - "Occurrence of clinically symptomatic benign neurofibromas of peripheral nerves after radiotherapy is a rarity. " 06/01/2005 - "Radiotherapy and radiosurgery for benign neurofibromas." 11/01/1981 - "Radiation therapy's association with malignant transformation of neurofibroma is discussed."
2.	Therapeutics 02/01/2012 - "So preclinical models for testing potential therapies are urgently needed to prioritize drugs for clinical trials of neurofibromas. " 09/15/2022 - "To summarize current widely-used therapies for cutaneous neurofibroma (cNF) and related research progress. " 09/01/2021 - "After 18 months of therapy, no new neurofibromas were detected and preexistent lesions showed no increase in size. " 07/01/2020 - "Management of cutaneous neurofibroma: current therapy and future directions." 02/07/2019 - "Antiinflammatory therapy targeting these cell populations has been suggested as a means to prevent neurofibroma development. "
3.	Lasers (Laser) 01/01/1996 - "Our follow-up examinations have shown there to be no recurrence of neurofibromas eradicated by laser-surgical treatment provided they are removed completely and radically. " 02/27/2020 - "LASER Assisted Excision of Solitary Neurofibroma in the Gingiva." 12/04/2019 - "The objectives of this patient survey were to better understand the real-world experiences of patients living with cutaneous neurofibromas (cNF), to perceive their satisfaction and feelings about cNF current management (only laser and surgery are currently available), and to highlight their expectations of new therapeutic modalities. " 11/01/2018 - "This case report describes the novel use of transoral robotic surgery (TORS) with delayed transoral laser microsurgery (TLM) for residual disease in the resection of a pediatric neurofibroma with supraglottic and parapharyngeal space extension. " 01/01/2018 - "Case Presentation: In the present article, we present the use of a high-power surgical laser to excise a neurofibroma in a patient with several intraoral manifestations associated with NF1. "
4.	Semiconductor Lasers 02/01/2016 - "This study aimed to evaluate the efficacy of diode laser for the surgical treatment of neurofibromas (NF), in terms of clinical outcome and therapeutic success. " 01/01/2018 - "Conclusion: The use of diode laser (808 nm) for excision biopsy of tongue nodules showed no thermal damage to the tissue, allowing an adequate histopathological analysis of the neurofibroma." 01/01/2017 - "Application of the laser diode with central wavelength 975 nm for the therapy of neurofibroma and hemangiomas."
5.	Tissue Expansion 07/01/2012 - "The role of tissue expansion for coverage of soft-tissue defects of the face following neurofibroma resection has been briefly described previously with good results but limited follow-up. " 07/01/2012 - "In both patients, tissue expansion was used to recruit tissue for fascio-cutaneous flaps following radical excision of adjacent neurofibromas. " 09/01/2011 - "Use of tissue expansion to allow reconstruction of the back after wide resection of a diffuse neurofibroma."