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الورم الليفي العصبي Neurofibroma أسباب، أعراض، و طرق علاج - مجلة اسال طبيبك انتقل إلى المحتوى الورم الليفي العصبي Neurofibroma أسباب، أعراض، و طرق علاج فريق التحرير | مارس 27, 2020 ديسمبر 28, 2017 | السرطان
الورم الليفي العصبي Neurofibroma, و هو ورم حميد ينتج عن اضطراب وراثي يقوم بتعطيل نمو الخلايا في الجهاز العصبي, ما يؤدي الى تكون اورام حميدة في الأنسجة العصبية.
يعتبر من الامراض العصبية الجلدية التي تصيب الاطفال, يتسبب بظهور آفات في الجهاز العصبي و الجلد و العين, له العديد من الأشكال, منها النمط الاول داء فون ريكلينغ هاوزن, و النمط الثاني هو الورم الليفي العصبي الجانبي, و هما اكثر شيوعاً عند الأطفال, كما قد تظهر هذه الاورام في أي جزء من الجهاز العصبي بما في ذلك الدماغ و الحبل الشوكي و الأعصاب, و عادةً ما يصيب هذا النوع من الاورام الاطفال حتى بداية سن البلوغ, و تكون هذه اللأورام غير سرطانية الا في حالات قليلة جداً. تحدث الاورام الليفية العصبية نتيجة لطفرات وراثية تنتقل من احد الابوين الى الطفل اثناء الحمل, و تصيب هذه الطفرات الجينات المختلفة في الجسم.
النمط الأول من الورم الليفي العصبي :
النمط الأول من الورم الليفي العصبي عادة ما يظهر هذا النوع من الورم في سن الطفولة و يتسبب في ظهور عدة أعراض منها:
بقع مسطحة على الجلد بنية اللون, تكون هذه البقع غير ضارة و تسمى ببقع القهوة باللبن, و تعد من العلامات الأساسية للإصابة بالورم الليفي من النمط 1, NF1. تظهر هذه البقع عادة عند الولادة او في السنوات الاولى للطفل, لا تشكل أي خطورة على الطفل من الناحية الصحية الا انها مسألة تجميلية. نمش في الابطين او منطقة الابط, يظهر هذا النمط في بداية سن الرابعة او الخامسة. بثور رخوة على الجلد او تحته. قد تنمو الاورام الليفية العصبية في عدة اعصاب لتكون ورم ليفي عصبي ضفيري. بثور دقيقة في منطقة القزحية من العين, و هي بثور غير مضرة تسمى عقيدات ليش, لا تؤثر على حدة البصر, لا يمكن رؤيتها بالعين المجردة, لكنها تظهر اثناء الفحص الطبي باستخدام آلة خاصة. خلل في نمو العظام و نقص في كثافة المعادن داخل العظام, ما يتسبب بتشوهات في العمود الفقري و انحرافه او تقوسه الى الاسفل. قد يعاني الطفل المصاب بالورم الليفي العصبي من صعوبة في الإدراك و التشوش و فرط الحركة و تشتت الانتباه. زيادة في حجم الرأس عند الطفل عن المعدل الطبيعي. قصر القامة, فالاطفال المصابون بالورم الليفي العصبي من النمط1 يكونون قصار القامة مقارنة مع اقرانهم. الورم الليفي العصبي من النمط الثاني : الورم الليفي العصبي من النمط الثاني يكون الورم الليفي العصبي من النوع الثاني NF2 اقل شيوعاً من النمط الاول, عادة ماتظهر اعراضه عند ظهور اورام العصب السمعي (الاورام الشفانية الدهليزية), و ينمو الورم الليفي من هذا النوع على العصب القحفي الثامن, وظيفته هي القيام بعمليات السمع و الاتزان في الاذن الداخلية و نقلها الى المخ, عادة ما يصيب الورم الليفي العصبي من النمط 2 الاشخاص في نهاية سن المراهقة, و بداية سن الرشد, و يؤدي الى ظهور عدة اعراض منها :
ضعف في القدرة على التوازن. فقدان حاسة السمع بشكل تدريجي. الشعور بطنين في الإذن. يؤدي هذا الورم الى نمو الاورام الشفانية في الأعصاب الاخرى من الجسم مثل الأعصاب القحفية في الدماغ و الاعصاب البصرية, و الاعصاب الطرفية و الشوكية. تدلي الوجه. تنميل و ضعف في الذراعين و الساقين. اضطرابات في الرؤية و حدوث خلل في نمو الشبكية عند الاطفال. أنواع الورم الليفي العصبي : تنقسم الأورام الليفية العصبية الى نوعين رئيسيين هما:
الورم الليفي الجلدي :
تظهر الاورام الليفية العصبية الجلدية في الأعصاب الموجودة في الجلد, و تنقسم الى ثلاثة انواع :
ورم ليفي جلدي عصبي منفصل, عبارة عن كتل عنقية او لاعنقية متواجدة على سطح الجلد و تكون لحمية غير مؤلمة. ورم ليفي عصبي تحت الجلد منفصل, يتكون تحت الجلد على شكل تحدبات فوق الجلد مؤلمة في بعض الاحيان. ورم ليفي عصبي عقيدي عميق, يشمل الانسجة و الاعضاء الموجودة تحت طبقة الأدمة في الجلد يشبه النوعين السابقين. تنشأ هذه الاورام في سن البلوغ, تزداد في الحجم و العدد مع مرور الوقت, تقف عند حد معين, تؤدي الى الشعور بالوخز او الحكة, و تشوهات و آلام في منطقة الجلد المصابة.
الورم الليفي العصبي الضفيري :
يحدث الورم الليفي العصبي عندما ينمو هذا الورم من الاعصاب في الجلد او الحزم العصبية الأكثر عمقاً, و من الصعب ازالة هذه الاورام بالكامل, حيث تمتد خلال طبقات عديدة من الجلد, و بالتالي عند محاولة ازالتها يمكن ان يتسبب ذلك في تلف الانسجة و الأعضاء السليمة. تظهر الاورام الليفية العصبية الضفيرية في سن مبكرة مرحلة الطفولة و في الغالب تنتج هذه الأورام عن عيوب خلقية في الجسم, و تؤدي الى ظهور مضاعفات خطيرة عند ظهورها في اماكن معينة من الجسم, كما ان 10% منها قد يتحول الى سرطان الغمد العصبي الطرفي.
علاج الورم الليفي العصبي : علاج الورم الليفي العصبي يقوم الطبيب بتشخيص الورم الليفي العصبي من خلال القيام بعدة اجراءات:
اجراء فحص جسدي شامل و تاريخ طبي للمريض, و يتم تشخيص النمط الول عبر ملاحظة عدة اعراض تشمل :
ظهور 6 بقع او اكثر من القهوة بالحليب تكون قطر الواحدة اصغر من 5ملم عند الأطفال و اكبر من 15 ملم عند المراهقين او البالغين. ظهور بقعتين او اكثر من عقد ليش او ورم واحد ليفي ضفيري الشكل او اورام بصرية دبقية. نمش ابطي و آفات مميزة مثل خلل التنسج الولادي. اجراء تحليل دم للشكف عن بروتين Melanoma inhibitory activity, و الذي يتم استخدامه للكشف عن اورام ليفية عصبية, و يتم تقييم الجلد للبحث عن الاورام الليفية العصبية و متابعة التغيرات الجديدة التي تحدث لهذه الاورام, و اجراء فحص لقياس ضغط الدم و تقييم نمو و تطور الطفل, بما في ذلك معرفة طول ووزن و محيط رأس الطفل, و اجراء فحص كامل للعين, و تقييم مهارات التعلم عند الطفل, و يعتمد العلاج بشكل اساسي على مراقبة المضاعفات التي تحدث نتيجة للإصابة بأورام ليفية عصبية, و البدء في العلاج المناسب في اقرب وقت ممكن, و يتم علاج الاورام الليفية العصبية الجلدي عبر اجراء جراحة و ذلك في حال اصبحت هذه الاورام مؤلمة او مشوهة, اما في الحالات العادية لا يتم اجراء ذلك لانها لا تشكل ضرر على حياة الإنسان, و يتم استخدام الليزر لإزالة الاورام التي تعتبر الخيار الامثل للعلاج. و في حالة الإصابة بالنمط الثاني من الاورام الليفية العصبية, يتم استخدام جراحة شعاعية بالتوضيع التجسيمي, و يمكن ان يقلل ذلك من خطر فقدان السمع على عكس الجراحات الأخرى التي قد تتسبب بفقدان السمع, و لكنها قد تشكل خطراً و يمكن ان يزيد الاشعاع من احتمالية الاصابة بالسرطان, و يمكن استخدام سناد الظهر الجنف, و في الحالات الشديدة يتم اللجوء الى الجراحة. و في حالة الاصابة بالاورام الخبيثة او انواع السرطان المرتبطة بالاورام العصبية الليفية, و التي غالباً ما ترتبط بالنمط الاول, و يتم علاجها عبر الجراحة, العلاج الكيميائي, العلاج الشعاعي, و ذلك لتدمير الخلايا السرطانية و القضاء عليها.
الورم العصبي في القدم : الورم العصبي في القدم يعد ورم مورتون من الاورام العصبية الحميدة, التي تظهر بين مشط العصب الأخمصي, تظهر عدة اعراض تدل على الإصابة بهذا المرض, و تشمل :
آلام و تنميل في منطقة القدم و الاصابع يقل عند خلع الحذاء. حرق او خدر في القدم و كأن المريض يمشي على شفرة او تواجد حصوات في الحذاء. الاصابة بآلام في الركبة و اسفل الظهر. و يتم تشخيص هذا المرض من خلال الأعراض التي تظهر على المريض, و تشمل ظهور اشارات لتشوهات واضحة, و وجود ضغط مبار بين رؤوس مشط القدم, و حدوث التهاب في الاربطة التي تحيط بين اثنين من العظام على مستوى المفصل الواحد.
العلاج :
يتم علاج ورم مورتون للأعصاب من خلال الراحة لفترة مناسبة, و استخدم احذية مريحة مناسبة لحجم القدم, و الابتعاد عن الاحذية الضيقة, و يمكن استخدام اكياس ثلج لتخفيف الألم و الالتهاب الموجود في القدم. و في الحالات الشديدة يمكن استخدام حقن موضعي بالستيرويدات و في حالة فشل هذه العلاجات يتم اللجوء الى الجراحة و للوقاية من ورم مورتون يجب علاج اي التهاب موجود في القدم, و ممارسة الرياضة بشكل مستمر لفقدان الوزن و عدم القيام بمجهود شاق من شأنه ان يحدث ضرر في القدم.