plexiform neurofibroma

Summary

Summary: A type of neurofibroma manifesting as a diffuse overgrowth of subcutaneous tissue, usually involving the face, scalp, neck, and chest but occasionally occurring in the abdomen or pelvis. The tumors tend to progress, and may extend along nerve roots to eventually involve the spinal roots and spinal cord. This process is almost always a manifestation of NEUROFIBROMATOSIS 1. (From Adams et al., Principles of Neurology, 6th ed, p1016; J Pediatr 1997 Nov;131(5):678-82)

Top Publications

  1. Bourgeois J, Radhi J, Elden L, Gill G. Plexiform neurofibroma of the submandibular salivary gland in a child. Can J Gastroenterol. 2001;15:835-7 pubmed
    ..Resection of the lesion revealed a plexiform neurofibroma involving the submandibular gland...
  2. Cebesoy O, Tutar E, Isik M, Arpacioglu O. A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve. Arch Orthop Trauma Surg. 2007;127:709-12 pubmed
    b>Plexiform neurofibroma is generally considered as a component of neurofibromatosis 1, and a great majority of the cases with plexiform neurofibroma display other symptoms related to neurofibromatosis...
  3. Sehgal V, Sharma S, Oberai R. Evaluation of plexiform neurofibroma in neurofibromatosis type 1 in 18 family members of 3 generations: ultrasonography and magnetic resonance imaging a diagnostic supplement. Int J Dermatol. 2009;48:275-9 pubmed publisher
    Abstract Plexiform neurofibroma in neurofibromatosis type 1, an autosomal-dominant genetic disorder, is characterized by a combination of interlacing components or a network...
  4. Lapid Gortzak R, Lapid O, Monos T, Lifshitz T. CO2-laser in the removal of a plexiform neurofibroma from the eyelid. Ophthalmic Surg Lasers. 2000;31:432-4 pubmed
    ..Using the CO2-laser achieves better cosmesis, while reducing operation time and limiting complications...
  5. Dombi E, Solomon J, Gillespie A, Fox E, Balis F, Patronas N, et al. NF1 plexiform neurofibroma growth rate by volumetric MRI: relationship to age and body weight. Neurology. 2007;68:643-7 pubmed
    To longitudinally analyze changes in plexiform neurofibroma (PN) volume in relation to age and body growth in children and young adults with neurofibromatosis type 1 and inoperable, symptomatic, or progressive PNs, using a sensitive, ..
  6. Wilkinson L, Manson D, Smith C. Best cases from the AFIP: plexiform neurofibroma of the bladder. Radiographics. 2004;24 Suppl 1:S237-42 pubmed
  7. Korf B. Plexiform neurofibromas. Am J Med Genet. 1999;89:31-7 pubmed
    ..but research into the molecular pathogenesis of NF1, as well as advances in tumor therapy in general, are opening the way towards clinical trials for plexiform neurofibroma. Am. J. Med. Genet. (Semin. Med. Genet.) 89:31-37, 1999.
  8. Demestre M, Herzberg J, Holtkamp N, Hagel C, Reuss D, Friedrich R, et al. Imatinib mesylate (Glivec) inhibits Schwann cell viability and reduces the size of human plexiform neurofibroma in a xenograft model. J Neurooncol. 2010;98:11-9 pubmed publisher
    ..001). We demonstrated that grafting human PNF tumour fragments into nude mice provides an adequate in vivo model for drug testing. Our results provide in vivo and in vitro evidence for efficacy of imatinib mesylate for PNF...
  9. Dave S, Farooq U, Civantos F. Management of advanced laryngeal and hypopharyngeal plexiform neurofibroma in adults. Am J Otolaryngol. 2008;29:279-83 pubmed publisher
    ..Because it is unencapsulated and more aggressive, plexiform neurofibroma presents a management dilemma...

More Information

Publications91

  1. Fisher M, Basu S, Dombi E, Yu J, Widemann B, Pollock A, et al. The role of [18F]-fluorodeoxyglucose positron emission tomography in predicting plexiform neurofibroma progression. J Neurooncol. 2008;87:165-71 pubmed
    ..We hypothesized that PN tumors with high metabolic activity as demonstrated by FDG-PET are more likely to progress in the following year...
  2. Mautner V, Brenner W, Fünsterer C, Hagel C, Gawad K, Friedrich R. Clinical relevance of positron emission tomography and magnetic resonance imaging in the progression of internal plexiform neurofibroma in NF1. Anticancer Res. 2007;27:1819-22 pubmed
    ..MPNST frequently arise from a previously undetected plexiform neurofibroma (PNF)...
  3. Park J. Mesenteric plexiform neurofibroma in an 11-year-old boy with von Recklinghausen disease. J Pediatr Surg. 2007;42:E15-8 pubmed
    ..Histopathologic study revealed a plexiform neurofibroma involving the mesentery and intestine without evidence of malignant transformation...
  4. Sehgal V, Srivastava G, Aggarwal A, Oberoi R, Sharma S. Solitary plexiform neurofibroma(s): role of magnetic resonance imaging. Skinmed. 2007;6:99-100 pubmed
    ..Based on the MRI findings, a diagnosis of superficial plexiform neurofibroma was considered...
  5. Marocchio L, Pereira M, Soares C, Oliveira D. Oral plexiform neurofibroma not associated with neurofibromatosis type I: case report. J Oral Sci. 2006;48:157-60 pubmed
    An unusual case of isolated plexiform neurofibroma arising in the oral cavity without other clinical manifestations or family history of neuro-fibromatosis-1 (NF-1) is described...
  6. Wei E, Albores Saavedra J, Fowler M. Plexiform neurofibroma of the uterine cervix: a case report and review of the literature. Arch Pathol Lab Med. 2005;129:783-6 pubmed
    ..We describe a case of plexiform neurofibroma affecting the uterine cervix in a patient with chronic pelvic pain and menorrhagia who had multiple ..
  7. Wise J, Patel S, Shah J. Management issues in massive pediatric facial plexiform neurofibroma with neurofibromatosis type 1. Head Neck. 2002;24:207-11 pubmed
    b>Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF1)...
  8. Gupta A, Cohen B, Ruggieri P, Packer R, Phillips P. Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1. Neurology. 2003;60:130-2 pubmed
    ..phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF). TL was well tolerated in doses up to 200 mg/d...
  9. Renshaw A, Borsetti M, Nelson R, Orlando A. Massive plexiform neurofibroma with associated meningo-encephalocoele and occipital bone defect presenting as a cervical mass. Br J Plast Surg. 2003;56:514-7 pubmed
    ..We report a rare case of a 54-year-old lady with a massive plexiform neurofibroma extending from the auricular region down her left neck and into her shoulder, with an associated large ..
  10. Guneri E, Akoglu E, Sutay S, Ceryan K, Sagol O, Pabuccuoglu U. Plexiform neurofibroma of the tongue: a case report of a child. Turk J Pediatr. 2006;48:155-8 pubmed
    A three-year-old girl with a lingual plexiform neurofibroma treated by total excision is presented. Despite their occurrence in the head and neck region, neural sheath tumors are rarely encountered in the oral cavity...
  11. Darrigo L, Geller M, Bonalumi Filho A, Azulay D. Prevalence of plexiform neurofibroma in children and adolescents with type I neurofibromatosis. J Pediatr (Rio J). 2007;83:571-3 pubmed
    To assess prevalence of plexiform neurofibroma in children and adolescents with type I neurofibromatosis and its malignant potential.
  12. Fenton L, Foreman N, Wyatt Ashmead J. Diffuse, retroperitoneal mesenteric and intrahepatic periportal plexiform neurofibroma in a 5-year-old boy. Pediatr Radiol. 2001;31:637-9 pubmed
    We present a case of plexiform neurofibroma involving the retroperitoneum, mesentery, and liver in a 5-year-old boy who underwent evaluation for extent of a palpable left neck mass...
  13. Lantieri L, Meningaud J, Grimbert P, Bellivier F, Lefaucheur J, Ortonne N, et al. Repair of the lower and middle parts of the face by composite tissue allotransplantation in a patient with massive plexiform neurofibroma: a 1-year follow-up study. Lancet. 2008;372:639-45 pubmed publisher
    ..We report here a 1-year follow-up of a patient who underwent face transplantation with a composite tissue allograft...
  14. Kretschmer T, Nguyen D, Beuerman R, Tiel R, Kline D. Elevated ankyrin G in a plexiform neurofibroma and neuromas associated with pain. J Clin Neurosci. 2004;11:886-9 pubmed
    ..Ankyrin G, a transmembrane, structural protein of the axon, was examined in four conditions: (a) painful plexiform neurofibroma; (b) painful neuroma; (c) non-painful neuromas; (d) normal nerve...
  15. Staser K, Yang F, CLAPP D. Plexiform neurofibroma genesis: questions of Nf1 gene dose and hyperactive mast cells. Curr Opin Hematol. 2010;17:287-93 pubmed publisher
    ..Recent mouse and human studies indicate that mast cells underpin inflammation in the plexiform neurofibroma microenvironment of neurofibromatosis type 1...
  16. Lévy P, Bieche I, Leroy K, Parfait B, Wechsler J, Laurendeau I, et al. Molecular profiles of neurofibromatosis type 1-associated plexiform neurofibromas: identification of a gene expression signature of poor prognosis. Clin Cancer Res. 2004;10:3763-71 pubmed
    ..NF1 patients with plexiform neurofibromas are at risk of developing malignant peripheral nerve sheath tumors...
  17. Lin V, Daniel S, Forte V. Is a plexiform neurofibroma pathognomonic of neurofibromatosis type I?. Laryngoscope. 2004;114:1410-4 pubmed
    Several prominent textbooks have claimed that a plexiform neurofibroma is pathognomonic for neurofibromatosis type I...
  18. Gómez Oliveira G, Fernández Alba Luengo J, Martín Sastre R, Patiño Seijas B, Lopez Cedrún Cembranos J. Plexiform neurofibroma of the cheek mucosa. A case report. Med Oral. 2004;9:263-7 pubmed
    The case reported deals with a solitary plexiform neurofibroma affecting the cheek submucosa. Neurofibroma is an uncommon tumor which rarely appears in oral cavity but it represents the most common neurogenic tumor...
  19. Aribandi M, Wood W, Elston D, Weiss D. CT features of plexiform neurofibroma of the submandibular gland. AJNR Am J Neuroradiol. 2006;27:126-8 pubmed
    b>Plexiform neurofibroma of the submandibular gland is an extremely rare tumor. We report the CT findings in a 6-year-old girl with type 1 neurofibromatosis who had a histopathologically proven submandibular gland plexiform neurofibroma...
  20. Weber A, Montandon C, Robson C. Neurogenic tumors of the neck. Radiol Clin North Am. 2000;38:1077-90 pubmed
    ..The plexiform neurofibroma and multiple localized neurofibromas are characteristic of NF1...
  21. Lavell A, Jones C, Wong D, Counsel P, Carey Smith R. Plexiform neurofibroma causing an ossifying subperiosteal haematoma: a rare case in the tibia of an 11-year-old girl. Skeletal Radiol. 2017;46:1405-1413 pubmed publisher
    ..We report an interesting case of plexiform neurofibroma causing a rapidly growing tibial mass as a result of subperiosteal haemorrhage, in an 11-year-old girl ..
  22. Roldan Marin R, Dominguez Cherit J, Vega Memije M, Toussaint Caire S, Hojyo Tomoka M, Dominguez Soto L. Solitary subungual neurofibroma: an uncommon finding and a review of the literature. J Drugs Dermatol. 2006;5:672-4 pubmed
    ..Surgical excision was used for the treatment. Common clinical findings, treatment modalities, and reported recurrence rates in previous studies are reviewed. ..
  23. Ramanjam V, Adnams C, Ndondo A, Fieggen G, Fieggen K, Wilmshurst J. Clinical phenotype of South African children with neurofibromatosis 1. J Child Neurol. 2006;21:63-70 pubmed
    ..The combined clinic has resulted in a holistic approach to patient care, early detection of pathology, consistent therapies across the specialties, and better patient attendance and compliance. (J Child Neurol 2006;21:63-70). ..
  24. Akiba T, Ishiyama M, Marushima H, Nojima K, Kobayashi S, Morikawa T. Temporary claviculectomy approach for plexiform neurofibroma of the first intercostal nerve. Surg Today. 2009;39:544-7 pubmed publisher
    b>Plexiform neurofibroma at the thoracic inlet has rarely been reported and to our knowledge, the use of a temporary middle claviculectomy approach for thoracic inlet tumors has never been reported...
  25. Calligaris L, Marchetti F, Ventura A. Intestinal plexiform neurofibromas. Dig Liver Dis. 2008;40:578 pubmed publisher
  26. Reilly K, Kim A, Blakely J, Ferner R, Gutmann D, Legius E, et al. Neurofibromatosis Type 1-Associated MPNST State of the Science: Outlining a Research Agenda for the Future. J Natl Cancer Inst. 2017;109: pubmed publisher
    ..for MPNST; 2) development of better understanding and histologic criteria for the transformation of a plexiform neurofibroma to MPNST; 3) establishment of a centralized database to collect genetic, genomic, histologic, ..
  27. Karaosmanoğlu B, Kocaefe Ã, SöylemezoÄŸlu F, Anlar B, Varan A, Vargel Ä, et al. Heightened CXCR4 and CXCL12 expression in NF1-associated neurofibromas. Childs Nerv Syst. 2018;34:877-882 pubmed publisher
    ..The patient group comprised 1 plexiform neurofibroma, 1 pheochromocytoma, and 6 dermal neurofibromas...
  28. Friedrich R, Reul A. Periapical Cemento-osseous Dysplasia Is Rarely Diagnosed on Orthopantomograms of Patients with Neurofibromatosis Type 1 and Is Not a Gender-specific Feature of the Disease. Anticancer Res. 2018;38:2277-2284 pubmed
    ..The NF1 patient group was further differentiated according to the evidence of facial plexiform neurofibroma. COD was a very rare finding in both groups...
  29. Fadda M, Verdino G, Mustazza M, Bartoli D, Iannetti G. Intra-parotid facial nerve multiple plexiform neurofibroma in patient with NF1. Int J Pediatr Otorhinolaryngol. 2008;72:553-7 pubmed publisher
    ..At the age of 3 years, a plexiform neurofibroma was diagnosed by biopsy of the lesion...
  30. Yang K, Rhim H, Cho O, Ko B, Kim Y, Lee H, et al. Segmental colonic involvement of plexiform neurofibroma in neurofibromatosis type 1. J Comput Assist Tomogr. 2002;26:129-31 pubmed
    In a 36-year-old man with neurofibromatosis type 1, rare colonic involvement of plexiform neurofibroma is presented. The diagnosis was confirmed by operation...
  31. Baujat B, Krastinova Lolov D, Blumen M, Baglin A, Coquille F, Chabolle F. Radiofrequency in the treatment of craniofacial plexiform neurofibromatosis: a pilot study. Plast Reconstr Surg. 2006;117:1261-8 pubmed
    ..This treatment is well tolerated. The best effect can be observed in the early stages of the disease. The optimal dose and frequency of the procedure require further study. ..
  32. Michal M, Kazakov D, Hadravsky L, Michalova K, Rychly B, Michal M. Multivacuolated mucin-filled cells: a unique cell characteristic of plexiform neurofibroma. A report of 11 cases. Hum Pathol. 2017;60:167-173 pubmed publisher
    The authors present 11 cases of plexiform neurofibroma (PN) that featured a very characteristic type of cell appearing as multivacuolated mucin-filled cells (MMFC). The 11 cases were obtained after reviewing 109 cases of PN...
  33. Serletis D, Parkin P, Bouffet E, Shroff M, Drake J, Rutka J. Massive plexiform neurofibromas in childhood: natural history and management issues. J Neurosurg. 2007;106:363-7 pubmed
    ..Nevertheless, patients with massive PNs will benefit from close surveillance by a team of specialists to monitor for ongoing disease progression. ..
  34. Nonaka D, Chiriboga L, Rubin B. Differential expression of S100 protein subtypes in malignant melanoma, and benign and malignant peripheral nerve sheath tumors. J Cutan Pathol. 2008;35:1014-9 pubmed publisher
    ..S100A1 expression was diffuse in most of MMs but absent or focal in BPNSTs and MPNSTs. This may be helpful to distinguish between the two entities. ..
  35. Tanaka J, Kuramochi A, Nishi N, Yuasa M, Heshiki A. Preoperative transarterial embolization enhances the surgical management of diffuse plexiform neurofibroma: a case report. Cardiovasc Intervent Radiol. 2005;28:686-8 pubmed
  36. Yan J, Li Y, Wu Z. Orbital neurofibroma presenting with a negative Hounsfield unit on computerized tomography. Orbit. 2006;25:239-41 pubmed
    ..An anterior orbitotomy was performed, and the surgical specimen demonstrated a typical plexiform neurofibroma with routine hematoxylin and eosin staining and immunohistochemical studies...
  37. Tanna N, Joshi A, Sidell D, Misra S, Santi M, Collins W. Pathology quiz case 1. Plexiform neurofibroma. Arch Otolaryngol Head Neck Surg. 2007;133:302, 304-5 pubmed
  38. Holtkamp N, Reuss D, Atallah I, Kuban R, Hartmann C, Mautner V, et al. Subclassification of nerve sheath tumors by gene expression profiling. Brain Pathol. 2004;14:258-64 pubmed
    ..NF1-associated and sporadic MPNST could not be distinguished by their gene expression patterns. We present a panel of discriminating genes that may assist subclassification of nerve sheath tumors. ..
  39. Fu C, Lin C, Peng Y, Yu T, Lu T, Chen T. Acute abdominal pain caused by spontaneous hemorrhagic infarction of a solitary plexiform neurofibroma of lesser omentum. Z Gastroenterol. 2008;46:344-7 pubmed publisher
    ..because of sudden onset of abdominal pain, which was found to be induced by hemorrhagic infarction of a plexiform neurofibroma arising from the lesser omentum...
  40. Guarneri C, Vaccaro M. What is your call?: cobblestone-like skin. Elephantiasis nostras verrucosa. CMAJ. 2008;179:673-4 pubmed publisher
  41. Khajavi M, Khoshsirat S, Ahangarnazari L, Majdinasab N. A brief report of plexiform neurofibroma. Curr Probl Cancer. 2018;42:256-260 pubmed publisher
    b>Plexiform neurofibroma (PNF) is a rare variant of neurofibromatosis type1 (NF-1), which histopathologically, is a subtype of benign nerve sheath tumors, neurofibromas (NF)...
  42. Azizi A, Slavc I, Theisen B, Rausch I, Weber M, Happak W, et al. Monitoring of plexiform neurofibroma in children and adolescents with neurofibromatosis type 1 by [18 F]FDG-PET imaging. Is it of value in asymptomatic patients?. Pediatr Blood Cancer. 2018;65: pubmed publisher
    ..type 1 (NF-1) develop malignant peripheral nerve sheath tumours (MPNST) mostly arising in plexiform neurofibroma (PN); 15% of MPNST arise in children and adolescents...
  43. Cooper J. Plexiform neurofibromatosis involving the colon. Radiographics. 2005;25:454 pubmed
  44. Savas H, Karabacakoglu A, Karakose S, Durmus E. [Plexiform neurofibroma in the gingiva]. Tani Girisim Radyol. 2004;10:39-43 pubmed
    ..Gingival involvement is very rare. We report radiological findings of three patients with plexiform neurofibroma who had diffuse, unilateral gingival involvement.
  45. Sánchez Salas M, Lázaro A, Martin J, Grasa M, Carapeto F. [Atypical pachydermatous cutaneous course of mastocytosis]. Actas Dermosifiliogr. 2007;98:707-10 pubmed
    ..This represents an extremely rare form of cutaneous involvement in mastocytosis and only 1 case has been described in the literature. ..
  46. Citak E, Oguz A, Karadeniz C, Okur A, Memis L, Boyunaga O. Management of plexiform neurofibroma with interferon alpha. Pediatr Hematol Oncol. 2008;25:673-8 pubmed publisher
    b>Plexiform neurofibroma is a relatively common but potentially devastating manifestation of neurofibromatosis type 1 (NF 1). A substantial number of plexiform neurofibroma causes morbidity...
  47. Kloos R, Rufini V, Gross M, Shapiro B. Bone scans in neurofibromatosis: neurofibroma, plexiform neuroma and neurofibrosarcoma. J Nucl Med. 1996;37:1778-83 pubmed
  48. Ganeshan A, Hon L, Soonawalla Z, De Costa H. Plexiform neurofibroma of the oesophagus: a mimicker of malignancy. Br J Radiol. 2005;78:1095-7 pubmed
    We report the first case of a plexiform neurofibroma of the oesophagus, presenting with dysphagia in a 67-year-old man known to suffer from von Recklinghausen's neurofibromatosis...
  49. Kurimoto M, Mizumaki Y, Fukuda O, Hayashi N, Kuwayama N, Endo S. Giant plexiform neurofibroma and suboccipital meningocele manifesting as segmental neurofibromatosis. Neurol Med Chir (Tokyo). 2008;48:271-4 pubmed
    ..Biopsy lead to a histological diagnosis of giant plexiform neurofibroma. During biopsy, massive local bleeding occurred and hemostasis was achieved by electrocautery and ..
  50. Friedrich R, Schmelzle R, Hartmann M, Mautner V. Subtotal and total resection of superficial plexiform neurofibromas of face and neck: four case reports. J Craniomaxillofac Surg. 2005;33:55-60 pubmed
    ..It has been previously shown that magnetic resonance tomography can distinguish the growth patterns of plexiform neurofibromas into three different categories: superficial, displacing and invasive...
  51. Valeyrie Allanore L, Ortonne N, Lantieri L, Ferkal S, Wechsler J, Bagot M, et al. Histopathologically dysplastic neurofibromas in neurofibromatosis 1: diagnostic criteria, prevalence and clinical significance. Br J Dermatol. 2008;158:1008-12 pubmed publisher
    ..Malignant peripheral nerve sheath tumours (MPNSTs) correspond to the most frequent and aggressive neoplasic complications associated with poor prognosis in neurofibromatosis 1...
  52. Piazza E, Donnellan K, Goodier A, Carron J. Massive retropharyngeal neurofibroma in a pediatric patient. Int J Pediatr Otorhinolaryngol. 2010;74:939-41 pubmed
    ..We present the case of an 11-year-old patient with a huge retropharyngeal plexiform neurofibroma which was successfully removed without sequelae...
  53. Montard R, Putz C, Barrali M, Kantelip B, Montard M. [Orbitotemporal segmental neurofibromatosis]. J Fr Ophtalmol. 2007;30:938-41 pubmed
    ..Neurofibromatosis is a rare pathology with heterogeneous clinical presentation...
  54. Guclu E, Tokmak A, Oghan F, Ozturk O, Egeli E. Hemimacroglossia caused by isolated plexiform neurofibroma: a case report. Laryngoscope. 2006;116:151-3 pubmed
    The present case is a diffuse isolated plexiform neurofibroma of the tongue that was not associated with neurofibromatosis that we treated with intraoral surgery.
  55. Ferner R, Golding J, Smith M, Calonje E, Jan W, Sanjayanathan V, et al. [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 2008;19:390-4 pubmed
  56. Madill K, Brammar R, Leatherbarrow B. A novel approach to the management of severe facial disfigurement in neurofibromatosis type 1. Ophthalmic Plast Reconstr Surg. 2007;23:227-8 pubmed
    ..type 1 underwent subtotal anterior exenteration of a blind, unsightly, buphthalmic eye and orbital/eyelid plexiform neurofibroma. Simultaneously, a channeled Medpor orbital implant was positioned to reduce a meningoencephalocele ..
  57. Packer R, Gutmann D, Rubenstein A, Viskochil D, Zimmerman R, Vezina G, et al. Plexiform neurofibromas in NF1: toward biologic-based therapy. Neurology. 2002;58:1461-70 pubmed
    ..The ability to design more rational therapies for NF1-associated neurofibromas is heavily predicated on an improved understanding of the molecular and cellular biology of the cells involved in neurofibroma formation and growth...
  58. Patil K, Mahima V, Shetty S, Lahari K. Facial plexiform neurofibroma in a child with neurofibromatosis type I: a case report. J Indian Soc Pedod Prev Dent. 2007;25:30-5 pubmed
    b>Plexiform neurofibroma is a non-circumscribed, thick and irregular benign tumor of the peripheral nerve sheath. It is a virtually pathognomonic and often disabling feature of neurofibromatosis type I...
  59. Fujiwara M, Isaka F, Honjo G, Ozawa T, Tachibana T. Solitary plexiform neurofibroma: recurrence 32 years after excision. J Eur Acad Dermatol Venereol. 2006;20:756-7 pubmed
  60. Bourne J, Mangaleshkar S, Meis Kindblom J, Bradish C. Recurrent plexiform neurofibromatosis of the foot with extensive bone destruction. J Pediatr Orthop B. 2009;18:176-8 pubmed publisher
    ..Plexiform neurofibromatosis is uncommon in the foot. No case of neurofibromatous involvement of the bone in the foot has been reported. We report the first case of plexiform neurofibromatosis with destruction of bones in the foot...
  61. Wolkenstein P, Rodriguez D, Ferkal S, Gravier H, Buret V, Algans N, et al. Impact of neurofibromatosis 1 upon quality of life in childhood: a cross-sectional study of 79 cases. Br J Dermatol. 2009;160:844-8 pubmed publisher
    ..Neurofibromatosis 1 (NF1) has a significant impact on quality of life (QoL)...
  62. Oguzkan S, Cinbis M, Ayter S, Anlar B, Aysun S. Familial segmental neurofibromatosis. J Child Neurol. 2004;19:392-4 pubmed
    ..The genetic mechanisms underlying this association are discussed. This family can be further investigated by examination of tissue samples from affected and unaffected sites for mutations...
  63. Keehn C, Myers P, Paidas C, Gilbert Barness E. Plexiform neurofibroma in type 1 neurofibromatosis. Fetal Pediatr Pathol. 2006;25:87-93 pubmed
    ..Family history was negative for neurofibromatosis...
  64. Nichol J, Amato J, Chung S. Lisch nodule asymmetry in a patient with neurofibromatosis type 1. J Pediatr Ophthalmol Strabismus. 2003;40:243-4 pubmed
  65. Martinez Garcia S, Vera Casano A, Eloy Garcia Carrasco C, del Boz González J, Martínez Pilar L, Crespo Erchiga V. Elephantiasis neuromatosa in a patient with neurofibromatosis type 1. J Eur Acad Dermatol Venereol. 2008;22:103-5 pubmed publisher
  66. Babovic Vuksanovic D, Widemann B, Dombi E, Gillespie A, Wolters P, Toledo Tamula M, et al. Phase I trial of pirfenidone in children with neurofibromatosis 1 and plexiform neurofibromas. Pediatr Neurol. 2007;36:293-300 pubmed
    ..The second dose level was the pharmacokinetically comparable dose and is being used in an ongoing phase II trial of pirfenidone for children with neurofibromatosis 1 and progressive plexiform neurofibroma.
  67. Stambouli O, Belbachir A. [KID syndrome: a cause of pachydermatoglyphia]. Ann Dermatol Venereol. 2003;130:1143-5 pubmed
    ..Other disorders such as sensitivity to infections or hypohidrosis may be associated. Pachydermatoglyphia is characterized by diffuse hyperkeratosis in which the dermatoglyphe crests are accentuated in thickness and height...
  68. Gesundheit B, Parkin P, Greenberg M, Baruchel S, Senger C, Kapelushnik J, et al. The role of angiogenesis in the transformation of plexiform neurofibroma into malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. J Pediatr Hematol Oncol. 2010;32:548-53 pubmed publisher
    ..The role of angiogenesis in the transformation of peripheral neurofibroma (PNF) to malignant peripheral nerve sheath tumor (MPNST) in neurofibromatosis type 1 (NF1) remains elusive and forms the objective of this study...
  69. Friedrich R, Giese M, Mautner V, Schmelzle R, Scheuer H. [Abnormalities of the maxillary sinus in type 1 neurofibromatosis]. Mund Kiefer Gesichtschir. 2002;6:363-7 pubmed
    ..The aim of this study was to determine the malformations of the maxillary sinus in neurofibromatosis type 1 patients (NF1)...
  70. Ars E, Kruyer H, Morell M, Pros E, Serra E, Ravella A, et al. Recurrent mutations in the NF1 gene are common among neurofibromatosis type 1 patients. J Med Genet. 2003;40:e82 pubmed
  71. Tucker T, Friedman J, Friedrich R, Wenzel R, Fünsterer C, Mautner V. Longitudinal study of neurofibromatosis 1 associated plexiform neurofibromas. J Med Genet. 2009;46:81-5 pubmed publisher
    ..In order to characterise the growth of plexiform neurofibromas better, we performed serial magnetic resonance imaging (MRI) in NF1 patients with such tumours...
  72. Samonakis D, Quaglia A, Joshi N, Tibballs J, Nagree A, Triantos C, et al. Obstructive jaundice secondary to neuroendocrine tumour in a patient with von Recklinghausen's disease. Eur J Gastroenterol Hepatol. 2005;17:1229-32 pubmed
    ..We present the case of a young adult with NF1, having a unique concurrence of plexiform neurofibroma involving the liver with an ampullary NET, and we discuss step by step the management in a specialist ..
  73. Abouchadi A, Nassih M, Rzin A, Elgbouri H, Jidal B. [Orbito-temporal plexiform neurofibroma: 6 cases]. Rev Stomatol Chir Maxillofac. 2005;106:272-5 pubmed
    Orbito-temporal plexiform neurofibroma is characteristic of Von Recklinghausen neurofibromatosis. We report 6 cases of orbito-temporal plexiform neurofibromas and review different aspects of diagnosis, course and treatment.
  74. Blitman N, Levsky J, Villanueva Siles E, Thornhill B. Spontaneous hemorrhage simulating rapid growth of a benign subperiosteal plexiform neurofibroma. Pediatr Radiol. 2007;37:925-8 pubmed
    ..Our case is unique among these as an underlying plexiform neurofibroma was visualized by MR imaging...
  75. Blitz N, Hutchinson B, Grabowski M. Pedal plexiform neurofibroma: review of the literature and case report. J Foot Ankle Surg. 2002;41:117-24 pubmed
    b>Plexiform neurofibroma typically occurs in the setting of neurofibromatosis type 1, and is included in the diagnostic criteria for neurofibromatosis...
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  77. Dailey R, Sullivan S, Wobig J. Surgical debulking of eyelid and anterior orbital plexiform neurofibromas by means of the carbon dioxide laser. Am J Ophthalmol. 2000;130:117-9 pubmed
    ..To present the carbon dioxide laser as an effective tool for surgical debulking of eyelid and orbital neurofibromas...
  78. Guarneri C, Guarneri F. An unusual haversack on the shoulder. QJM. 2008;101:415-6 pubmed publisher
  79. Mantripragada K, Caley M, Stephens P, Jones C, Kluwe L, Guha A, et al. Telomerase activity is a biomarker for high grade malignant peripheral nerve sheath tumors in neurofibromatosis type 1 individuals. Genes Chromosomes Cancer. 2008;47:238-46 pubmed
    ..042), PNF (P = 0.001) or DNF tumors (P = 0.010). These findings indicate that TA and expression level of TERT are potential markers for high grade malignancy in NF1 patients...
  80. Washington E, Placket T, Gagliano R, Kavolius J, Person D. Diffuse plexiform neurofibroma of the back: report of a case. Hawaii Med J. 2010;69:191-3 pubmed
    ..A review of the literature concerning the diagnosis and treatment of neurofibromatosis is included...
  81. Spinner R, Scheithauer B, Perry A, Amrami K, Emnett R, Gutmann D. Colocalized cellular schwannoma and plexiform neurofibroma in the absence of neurofibromatosis. Case report. J Neurosurg. 2007;107:435-9 pubmed
    ..combination of nerve sheath tumors-a large cellular schwannoma and multifascicular involvement of a plexiform neurofibroma arising from the same site within the radial nerve and posterior cord of the infraclavicular brachial ..
  82. Overdiek A, Feifel H, Schaper J, Mayatepek E, Rosenbaum T. Diagnostic delay of NF1 in hemifacial hypertrophy due to plexiform neurofibromas. Brain Dev. 2006;28:275-80 pubmed
    ..However, despite other typical NF1-associated features (e.g. multiple café-au-lait spots) diagnosis of a plexiform neurofibroma as underlying cause for the hemifacial hypertrophy was significantly delayed in all patients...