Neural and Nerve Sheath Lesions




(1)
Department of Pathology, University of Manitoba Max Rady College of Medicine, Winnipeg, MB, Canada

 



Keywords
Granular cell tumorMalignant peripheral nerve sheath tumorNeurofibromaPerineuriomaPeripheral nerveSchwannoma


Peripheral nerves are composed of axons that are supported and insulated by a myelin substance produced by surrounding Schwann cells . These axons are further organized into bundles that are supported and surrounded by connective tissue and covered by perineurium . In neural and nerve sheath neoplasms, one or a combination of these anatomic structures proliferate to form a soft tissue mass.


9.1 Schwannoma


Schwannomas arise from the myelin-producing Schwann cells that surround the nerve and often occur in adults between 20 and 50 years of age. Frequent sites of involvement include the head and neck or flexor surfaces of the extremities [1]. Although benign, these tumors can cause clinical concern when they arise in the deep portions in the extremities, retroperitoneum, or posterior mediastinum [2]. Although schwannomas can be suspected on radiologic examination, needle core biopsies are often performed to exclude other malignant neoplasms, such as malignant peripheral nerve sheath tumor.


Pathology

Microscopically, schwannomas show spindle cells with dense homogenous eosinophilic cytoplasm (Antoni A areas ) admixed with less cellular areas of cells intermixed with looser background collagen (Antoni B areas ) (Figs. 9.1 and 9.2). Sometimes, the nuclei of the spindle cells can form parallel arrangements, termed Verocay bodies (Fig. 9.3).

A416307_1_En_9_Fig1_HTML.jpg


Fig. 9.1
Hypercellular Antoni A area of a schwannoma


A416307_1_En_9_Fig2_HTML.jpg


Fig. 9.2
Hypocellular Antoni B area of a schwannoma. The cells have nuclei that are less plump and the background stroma has a vaguely myxoid appearance


A416307_1_En_9_Fig3_HTML.jpg


Fig. 9.3
In this Verocay body found in a schwannoma, the nuclei are arranged in a parallel formation

Schwannomas can vary in appearance. A cellular schwannoma is predominantly composed of dense Antoni A areas. A schwannoma with degenerative changes (ancient schwannoma) can show perivascular hyalinization (Fig. 9.4) or cells with degenerative atypia (Fig. 9.5).

A416307_1_En_9_Fig4_HTML.jpg


Fig. 9.4
Perivascular hyalinization in a schwannoma with degenerative changes


A416307_1_En_9_Fig5_HTML.jpg


Fig. 9.5
Focal nuclear atypia (arrow) in a schwannoma with degenerative changes


Ancillary Studies





  • The spindle cells of a schwannoma are diffusely positive for S100 and SOX-10 immunohistochemical stains.


Differential Diagnosis





  • Malignant peripheral nerve sheath tumor


  • Neurofibroma


Comments




  1. 1.


    Schwannomas that lack less cellular areas can sometimes be confused for a malignant peripheral nerve sheath tumor.

     

  2. 2.


    Although a portion of malignant peripheral nerve sheath tumors can stain for S100, the staining is typically weak or focal in nature.

     

  3. 3.


    The less cellular areas of a schwannoma can sometimes mimic a neurofibroma. However, neurofibromas are composed of multiple nerve elements, including perineurial cells, fibroblasts, and axons that can be detected by immunohistochemistry.

     

  4. 4.


    Schwannomas can rarely arise as multiple lesions in certain syndromes, such as schwannomatosis or neurofibromatosis type 2. Patients with multiple schwannomas should be assessed for these conditions [3, 4].

     

  5. 5.


    Schwannomas are benign and only rarely recur after conservative excision [5].

     


9.2 Neurofibroma


Neurofibroma is a benign nerve tumor that frequently arises in the skin as a painless circumscribed nodule. It can also manifest as a large mass in association with larger nerves in deeper sites such as the abdomen [6]. Multiple and large neurofibromas occur in patients with neurofibromatosis type 1 (NF1 ) or neurofibromatosis type 2 (NF2 ). Additional findings of NF1 include café-au-lait spots of the skin , Lisch nodules , and optic gliomas . Additional features of NF2 include bilateral vestibular schwannomas , meningiomas, and gliomas [7].


Pathology

These tumors contain a mixture of perineurial cells, fibroblasts, and Schwann cells. Microscopically, the sections show a haphazard arrangement of spindle cells with wavy nuclei and disorganized collagen fibers (Figs. 9.6 and 9.7). These tumors can be well circumscribed (Fig. 9.8), plexiform (Fig. 9.9), or diffusely infiltrative into surrounding soft tissue (Figs. 9.10 and 9.11) [7].

A416307_1_En_9_Fig6_HTML.jpg


Figs. 9.6 and 9.7
Neurofibroma with disorganized collagen and wavy nuclei. Some refer to the haphazard collagen arrangement as a “shredded carrot” appearance


A416307_1_En_9_Fig7_HTML.jpg


Fig. 9.8
Well-circumscribed neurofibroma in the subcutaneous tissue


A416307_1_En_9_Fig8_HTML.jpg


Fig. 9.9
Plexiform neurofibroma with multiple well-circumscribed bundles of tumor cells pushing through the surrounding soft tissue


A416307_1_En_9_Fig9_HTML.jpg


Figs. 9.10 and 9.11
Diffuse neurofibroma with extensive infiltration into the adjacent adipose tissue


Ancillary Studies





  • The Schwann cells in neurofibroma are positive for S100. Additionally, an EMA stain highlights scattered perineurial cells and a neurofilament stain identifies scattered axons.


Differential Diagnosis





  • Malignant peripheral nerve sheath tumor


  • Schwannoma


  • Perineurioma


Comment




  1. 1.


    Because neurofibromas are composed of multiple cell types, these can be confused for other nerve sheath tumors, such as schwannoma, perineurioma, or malignant peripheral nerve sheath tumor.

     

  2. 2.


    Malignant peripheral nerve sheath tumor can sometimes arise from a neurofibroma, but the sections show more nuclear atypia, mitosis, and organized arrangement of spindle cells.

     

  3. 3.


    Schwannomas contain a more homogenous population of Schwann cells with associated hyalinized fibrosis.

     

  4. 4.
Jan 30, 2018 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Neural and Nerve Sheath Lesions

Full access? Get Clinical Tree

Get Clinical Tree app for offline access