Facial Plexiform Neurofibromatosis Type I
Introduction Plexiform neurofibroma is a benign tumor of peripheral nerves arising from a proliferation of all neural elements. Clinically, it presents as a subcutaneous mass which feels like a "bag of worms". Case Report A 23-years-old male with a swelling over left side of the face...
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| Format: | Article |
| Language: | English |
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The Association of Otolaryngologists of India, West Bengal
2018-08-01
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| Series: | Bengal Journal of Otolaryngology and Head Neck Surgery |
| Subjects: | |
| Online Access: | https://bjohns.in/journal/index.php/bjohns/article/view/297 |
| _version_ | 1827295137253818368 |
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| author | Bapan Devnath Debraj Dey Avinava Ghosh |
| author_facet | Bapan Devnath Debraj Dey Avinava Ghosh |
| author_sort | Bapan Devnath |
| collection | DOAJ |
| description | Introduction
Plexiform neurofibroma is a benign tumor of peripheral nerves arising from a proliferation of all neural elements. Clinically, it presents as a subcutaneous mass which feels like a "bag of worms".
Case Report
A 23-years-old male with a swelling over left side of the face large enough to involve whole one side of the face. There was tenderness and the swelling had a very peculiar consistency, soft in most of the areas with few firm nodular areas.
Operative procedure: surgery was done in two stages. Initially subtotal excision was done and 3 months later re-explored to excise the recurrent disease. Complete excision was not possible.
Discussion
Plexiform neurofibroma (PNF) occurs due to overgrowth of neural tissue in the subcutaneous region. Surgical management remains the mainstay of treatment but functional disturbances are almost inevitable while resecting tumors involving the head and neck region. Resection and de bulking of invasive PNF is however associated with a high rate of recurrence. One of the limiting factors is vascularity of these lesions and their abnormal propensity to bleed.
Conclusion
Although benign, plexiform neurofibromas can cause pain, disfigurement and functional changes and more importantly, may turn malignant. Surgery should be attempted considering psychological benefits. |
| first_indexed | 2024-03-09T06:07:55Z |
| format | Article |
| id | doaj.art-0cbe116882db4337b2e31f47cec5e81e |
| institution | Directory Open Access Journal |
| issn | 2395-2407 2395-2407 |
| language | English |
| last_indexed | 2024-04-24T14:14:45Z |
| publishDate | 2018-08-01 |
| publisher | The Association of Otolaryngologists of India, West Bengal |
| record_format | Article |
| series | Bengal Journal of Otolaryngology and Head Neck Surgery |
| spelling | doaj.art-0cbe116882db4337b2e31f47cec5e81e2024-04-03T08:48:00ZengThe Association of Otolaryngologists of India, West BengalBengal Journal of Otolaryngology and Head Neck Surgery2395-24072395-24072018-08-01262117121157Facial Plexiform Neurofibromatosis Type IBapan Devnath0Debraj Dey1Avinava Ghosh2Silchar Medical College, AssamTezpur Medical College & Hospital, AssamJain ENT Hospital, Jaipur, RajasthanIntroduction Plexiform neurofibroma is a benign tumor of peripheral nerves arising from a proliferation of all neural elements. Clinically, it presents as a subcutaneous mass which feels like a "bag of worms". Case Report A 23-years-old male with a swelling over left side of the face large enough to involve whole one side of the face. There was tenderness and the swelling had a very peculiar consistency, soft in most of the areas with few firm nodular areas. Operative procedure: surgery was done in two stages. Initially subtotal excision was done and 3 months later re-explored to excise the recurrent disease. Complete excision was not possible. Discussion Plexiform neurofibroma (PNF) occurs due to overgrowth of neural tissue in the subcutaneous region. Surgical management remains the mainstay of treatment but functional disturbances are almost inevitable while resecting tumors involving the head and neck region. Resection and de bulking of invasive PNF is however associated with a high rate of recurrence. One of the limiting factors is vascularity of these lesions and their abnormal propensity to bleed. Conclusion Although benign, plexiform neurofibromas can cause pain, disfigurement and functional changes and more importantly, may turn malignant. Surgery should be attempted considering psychological benefits.https://bjohns.in/journal/index.php/bjohns/article/view/297Neurofibroma, PlexiformFacial AsymmetryMale |
| spellingShingle | Bapan Devnath Debraj Dey Avinava Ghosh Facial Plexiform Neurofibromatosis Type I Bengal Journal of Otolaryngology and Head Neck Surgery Neurofibroma, Plexiform Facial Asymmetry Male |
| title | Facial Plexiform Neurofibromatosis Type I |
| title_full | Facial Plexiform Neurofibromatosis Type I |
| title_fullStr | Facial Plexiform Neurofibromatosis Type I |
| title_full_unstemmed | Facial Plexiform Neurofibromatosis Type I |
| title_short | Facial Plexiform Neurofibromatosis Type I |
| title_sort | facial plexiform neurofibromatosis type i |
| topic | Neurofibroma, Plexiform Facial Asymmetry Male |
| url | https://bjohns.in/journal/index.php/bjohns/article/view/297 |
| work_keys_str_mv | AT bapandevnath facialplexiformneurofibromatosistypei AT debrajdey facialplexiformneurofibromatosistypei AT avinavaghosh facialplexiformneurofibromatosistypei |