Plexiform Neurofibroma Prognosis [Neurosurgery Wiki]

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===== Plexiform Neurofibroma Prognosis =====

{{rss>https://pubmed.ncbi.nlm.nih.gov/rss/search/18Yp8FTN9Jhx6NzaLUNZBwmsosCuQUZcaHY1cOwnQghoPwzAQn/?limit=15&utm_campaign=pubmed-2&fc=20250718014405}}

==== 📌 General Prognosis ====
  * Benign but potentially progressive tumor.
  * Commonly associated with **Neurofibromatosis Type 1 (NF1)**.
  * Growth behavior is unpredictable: some remain stable, others grow rapidly — especially during childhood and puberty.
  * Can be disfiguring and cause functional impairment depending on location and size.

==== ⚠️ Risk of Malignant Transformation ====
  * Lifetime risk of transformation into **Malignant Peripheral Nerve Sheath Tumor (MPNST)**: **5–15%**.
  * Independent risk factors for malignancy:
    * Involvement of cranial nerve ganglia
    * Rapid increase in tumor size
    * New onset of pain or neurologic deficit
    * Deep or visceral tumor location (e.g., paraspinal, retroperitoneal)

==== 💡 Prognostic Factors ====
^ Factor ^ Impact on Prognosis ^
| **Age of onset**              | Earlier onset → longer exposure, higher cumulative risk |
| **Tumor size and depth**      | Larger or deep-seated tumors → worse prognosis |
| **NF1 status**                | Associated with greater tumor burden and risk |
| **Surgical resectability**    | Incomplete resection → frequent recurrence |
| **MRI findings**              | Heterogeneity, necrosis, or loss of "target sign" may suggest aggressive behavior |

==== 🧠 Neurological and Functional Impact ====
  * Tumors may compress or infiltrate nerves, leading to:
    * Sensory or motor deficits
    * Pain or neuropathic symptoms
    * Disfigurement (especially in head and neck PNFs)
    * Loss of function (e.g., brachial plexus, spine involvement)


==== 🧾 Summary ====
> Plexiform neurofibromas are benign tumors with variable clinical behavior. Prognosis depends on tumor location, growth rate, NF1 association, and the risk of malignant transformation. Lifelong follow-up and individualized management are essential.

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Tonsgard et al. examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors.

Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown.

CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1.

Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas.

The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions
((Tonsgard JH, Kwak SM, Short MP, Dachman AH. CT imaging in adults with
neurofibromatosis-1: frequent asymptomatic plexiform lesions. Neurology. 1998
Jun;50(6):1755-60. PubMed PMID: 9633723.
)).
----
Cervical cord compression from cervical root neurofibromas represents an important clinical problem in patients with neurofibromatosis type 1 (NF1), but is rarely reported. The aim of this study was to describe the clinical presentation and follow-up of children and adults with NF1 and cervical cord compression. A retrospective review of clinical records and neuroimaging studies from two large tertiary care centres between 1996 and 2006 was performed. 13 patients with NF1 and cervical cord compression were identified. Age at presentation ranged from 9 to 61 years. The most common presentation was progressive quadriparesis. 11 of 13 patients underwent cervical decompression and subtotal resection of the associated neurofibroma. The majority of patients had recovery of neurological function and no further clinical progression. Progressive neurological deficit (typically quadriparesis), rather than neuroimaging appearances, should dictate the need for surgery
((Leonard JR, Ferner RE, Thomas N, Gutmann DH. Cervical cord compression from
plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry.
2007 Dec;78(12):1404-6. Epub 2007 Jul 17. PubMed PMID: 17635968; PubMed Central
PMCID: PMC2095617.
)).