Malignant Peripheral Nerve Sheath Tumor (MPNST / मैलिग्नेंट पेरिफेरल नर्व शीथ ट्यूमर) एक rare aggressive soft tissue sarcoma है जो peripheral nerves (जिन्हें body में motor और sensory signals भेजते हैं) की sheath (covering) से उत्पन्न होता है।
- यह tumor neurofibromatosis type 1 (NF1) patients में अधिक common है।
- Common sites: arms, legs, trunk, and plexus regions।
- Aggressive nature के कारण early detection और treatment जरूरी है।
Malignant Peripheral Nerve Sheath Tumor क्या है (What is MPNST)
- Sarcoma arising from Schwann cells, perineural cells, or fibroblasts of nerve sheath
- Tumor often rapidly enlarging, firm, and painful
- High recurrence rate और metastasis risk (lungs, liver, bones)
- Usually NF1 patients में develop होता है, लेकिन sporadic cases भी होते हैं
Malignant Peripheral Nerve Sheath Tumor कारण (Causes of MPNST)
1. Genetic Factors
- Neurofibromatosis type 1 (NF1) – major risk factor
- Mutation in NF1 gene leads to abnormal nerve growth
2. Radiation Exposure
- Previous radiation therapy increases risk
- Often after decades of radiation
3. Sporadic / Idiopathic
- Some tumors occur without known risk factors
4. Malignant Transformation
- Pre-existing plexiform neurofibroma in NF1 patients may transform into MPNST
Malignant Peripheral Nerve Sheath Tumor लक्षण (Symptoms of MPNST)
- Painless or painful mass in arms, legs, or trunk
- Rapid growth of existing lump
- Numbness, tingling, or weakness in affected limb
- Muscle atrophy in severe cases
- Rare systemic symptoms: weight loss, fatigue
Symptoms often subtle initially, leading to delayed diagnosis।
Malignant Peripheral Nerve Sheath Tumor कैसे पहचाने (Diagnosis of MPNST)
- Physical Examination – firm, deep-seated mass, sometimes tender
- Imaging
- MRI: defines tumor size, nerve involvement, and surrounding tissue
- CT: staging and metastasis evaluation
- Biopsy – core needle or excisional biopsy for histopathology
- Histopathology Features
- Spindle cells, high mitotic activity, necrosis
- Immunohistochemistry: S100 protein may be positive
- Staging Workup – chest CT to check lung metastasis
Early biopsy critical for accurate diagnosis and treatment planning।
Malignant Peripheral Nerve Sheath Tumor इलाज (Treatment of MPNST)
1. Surgical Excision
- Wide local excision with clear margins
- Limb-sparing surgery preferred if feasible
- Amputation rarely required for large tumors
2. Radiotherapy
- Postoperative radiation to reduce recurrence risk
- Preoperative radiation in some high-risk cases
3. Chemotherapy
- Doxorubicin and Ifosfamide based regimens
- Limited efficacy, mostly for high-grade or metastatic tumors
4. Targeted / Experimental Therapy
- Clinical trials for NF1-related MPNST
- Research ongoing for molecular targeted agents
5. Follow-up & Monitoring
- Regular imaging for local recurrence and metastasis
- Lifelong follow-up recommended, especially in NF1 patients
रोकथाम (Prevention)
- NF1 patients को regular screening
- Early evaluation of rapidly enlarging neurofibromas
- Avoid unnecessary radiation exposure
- Awareness of symptoms like rapid growth, pain, or neurological deficits
सावधानियाँ (Precautions)
- Do not ignore new or enlarging lumps
- Early consultation with oncologist / surgical specialist
- Complete post-surgical therapy and follow-up
- Monitor for neurological deficits
FAQs (अक्सर पूछे जाने वाले प्रश्न)
Q1. MPNST कितनी aggressive होती है?
यह high-grade sarcoma है, जल्दी grow और metastasize कर सकती है।
Q2. Surgery के बाद recurrence rate क्या है?
Recurrence high है (40–60%), इसलिए wide excision और radiation therapy जरूरी है।
Q3. NF1 patients में risk कितना है?
NF1 patients में 10% lifetime risk of MPNST होती है।
Q4. Prognosis कैसा है?
Early detection और complete excision से 5-year survival 50–60%, late diagnosis में poor prognosis।
निष्कर्ष (Conclusion)
Malignant Peripheral Nerve Sheath Tumor (MPNST / मैलिग्नेंट पेरिफेरल नर्व शीथ ट्यूमर) एक aggressive soft tissue sarcoma है।
Early detection, wide surgical excision, adjuvant radiotherapy और regular follow-up से survival और quality of life improve की जा सकती है।
अगर किसी को rapidly growing mass, pain, numbness या weakness दिखे, तो तुरंत oncologist या surgical specialist से consultation जरूरी है।