(1)
Department of Pathology, University of Manitoba Max Rady College of Medicine, Winnipeg, MB, Canada
Keywords
Granular cell tumorMalignant peripheral nerve sheath tumorNeurofibromaPerineuriomaPeripheral nerveSchwannomaPeripheral 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).
Fig. 9.1
Hypercellular Antoni A area of a schwannoma
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
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).
Fig. 9.4
Perivascular hyalinization in a schwannoma with degenerative changes
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.
Schwannomas that lack less cellular areas can sometimes be confused for a malignant peripheral nerve sheath tumor.
- 2.
Although a portion of malignant peripheral nerve sheath tumors can stain for S100, the staining is typically weak or focal in nature.
- 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.
- 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].
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
Fig. 9.8
Well-circumscribed neurofibroma in the subcutaneous tissue
Fig. 9.9
Plexiform neurofibroma with multiple well-circumscribed bundles of tumor cells pushing through the surrounding soft tissue
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.
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.
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.
Schwannomas contain a more homogenous population of Schwann cells with associated hyalinized fibrosis.
- 4.
Perineuriomas contain a more homogenous population of cells that are positive for EMA but negative for S100.Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree