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Presented by Dr. Elizabeth Thompson and prepared by Dr. Tatianna Larman.
An intradural mass in a 40 year old woman.
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1. Question
An intradural mass in a 40 year old woman. Choose the correct diagnosis.
Correct
Answer: Schwannoma
Histology: Sections of the lesion show elongated spindled cells with a “wavy” shape and tapered ends with abundant intervening collagen. Cellularity varies throughout and there is prominent nuclear palisading. There are vessels of varying sizes, often with hyalinized walls, and focal areas of cystic change.
Discussion: This is a beautiful example of a schwannoma with classic histologic features of Antoni A ares (cellular, monomorphic spindled Schwann cells in a variably collagenous stroma) and Antoni B areas (hypocelluar, spindled cells in myxoid matrix). Antoni A areas often show nuclear palisading and parallel arrays with intervening eosinophilic cytoplasm known as Verocay bodies (very prominent in this example). Degenerative or so-called “ancient change” in schwannomas is common and includes hyalinization of blood vessels, stromal hemorrhage, cystic change and calcification. Variants include: ancient schwannoma, cellular schwannoma (up to 10 mitoses per 10 hpf, can see focal necrosis and, inconspicuous or focal Antoni B areas), plexiform schwannoma, melanotic schwannoma, epithelioid schwannoma, and glandular schwannoma. Schwannomas are strongly and diffusely S100 positive. Those in the GI tract are also often GFAP positive and those in the retroperitoneum and mediastinum can be keratin positive. The vast majority have a benign course with only rare recurrence. Malignant transformation is very rare. They are typically solitary lesions. Bilateral schwannomas of the acoustic nerve are an almost pathognomonic feature of NF2. Multiple peripheral schwannomas (schwannomatosis) can develop in patients with SMARCB1 (INI1) mutations as well as NF2 inactivation, and can be familial.
Incorrect
Answer: Schwannoma
Histology: Sections of the lesion show elongated spindled cells with a “wavy” shape and tapered ends with abundant intervening collagen. Cellularity varies throughout and there is prominent nuclear palisading. There are vessels of varying sizes, often with hyalinized walls, and focal areas of cystic change.
Discussion: This is a beautiful example of a schwannoma with classic histologic features of Antoni A ares (cellular, monomorphic spindled Schwann cells in a variably collagenous stroma) and Antoni B areas (hypocelluar, spindled cells in myxoid matrix). Antoni A areas often show nuclear palisading and parallel arrays with intervening eosinophilic cytoplasm known as Verocay bodies (very prominent in this example). Degenerative or so-called “ancient change” in schwannomas is common and includes hyalinization of blood vessels, stromal hemorrhage, cystic change and calcification. Variants include: ancient schwannoma, cellular schwannoma (up to 10 mitoses per 10 hpf, can see focal necrosis and, inconspicuous or focal Antoni B areas), plexiform schwannoma, melanotic schwannoma, epithelioid schwannoma, and glandular schwannoma. Schwannomas are strongly and diffusely S100 positive. Those in the GI tract are also often GFAP positive and those in the retroperitoneum and mediastinum can be keratin positive. The vast majority have a benign course with only rare recurrence. Malignant transformation is very rare. They are typically solitary lesions. Bilateral schwannomas of the acoustic nerve are an almost pathognomonic feature of NF2. Multiple peripheral schwannomas (schwannomatosis) can develop in patients with SMARCB1 (INI1) mutations as well as NF2 inactivation, and can be familial.
