Soft tissue tumors

Peripheral nerve tumors

Schwannoma
Reviewer: Vijay Shankar, M.D. (see Reviewers page) Revised: 7 March 2013, last major update November 2012 Copyright: (c) 2003-2013, PathologyOutlines.com, Inc. See subtypes below: ancient, angiosarcoma-arising in, cellular, epithelioid malignant change, microcystic-reticular variant, pigmented, plexiform, psammomatous melanotic General ===================================================================== ==== Also known as neurilemoma Encapsulated biphasic nerve sheath tumor derived from Schwann cells with highly ordered cellular component (Antoni A) that palisades (Verocay bodies), plus myxoid component (Antoni B) Small tumors may be all Antoni A Clinical features ===================================================================== ==== Ages 20-50; M=F Head, neck, flexor upper and lower extremities, retroperitoneum, posterior spinal roots, cerebellopontine angle Slow growing; no symptoms until becomes large; may wax and wane in size Pain or rapid enlargement of preexisting lesion are suggestive of malignant change May be due to alteration / loss of NF2 gene product Malignant transformation ===================================================================== ==== Occurs even without neurofibromatosis, tumors usually have epithelioid features and often areas of benign schwannoma (Am J Surg Pathol 2001;25:13) Transforms to MPNST, angiosarcoma or epithelioid malignant change (EMC) Most common sites are limb, limb girdles, head/neck Schwannoma with MPNST: benign schwannoma with no other primary tumor that may have metastasized to schwannoma, histologically malignant cells resembling epithelioid MPNST; 5 year survival < 20%

Treatment ===================================================================== ==== Excision; recurrence rare, so attempt to preserve nerve if clinically significant Gross description ===================================================================== ==== Usually solitary Large tumors may be cystic Nerve of origin present in periphery - does not penetrate substance of tumor Dumbbell tumor: in posterior mediastinum, originates from or extends into vertebral canal Gross images ===================================================================== ====

Well circumscribed Micro description ===================================================================== ==== Biphasic: compact hypercellular Antoni A areas and myxoid hypocellular Antoni B areas Nuclear palisading around fibrillary process (Verocay bodies are often seen in cellular areas) Large irregularly spaced vessels are most prominent in Antoni B areas Gaping tortuous lumina have thickened hyalinized walls and may have thrombi Tumor cells have ill defined cytoplasm, dense chromatin May have foamy macrophages Often displays degenerative nuclear atypia (ancient change) Amianthoid fibers or collagenous spherules: large nodular masses of collagen with radiating edges Rarely epithelioid, glandular (may be entrapped sweat glands), pigmented, plexiform, rosettes

Rare mitotic figures, no axons except where nerve is attached Micro images ===================================================================== ====

Various images Positive stains ===================================================================== ==== EMA (capsule), S100 (Schwann cells), calcinurin, laminin, type 4 collagen, vimentin, CD68, GFAP Negative stains ===================================================================== ==== Keratin, neurofilament, desmin Electron microscopy description ===================================================================== ==== Elongated cells with continuous basal lamina, thin cytoplasmic processes, aggregates of intracytoplasmic microfibrils, peculiar intracytoplasmic lamellar bodies, extracellular long-spacing collagen Contains lipid Has characteristic luse bodies (long spaced collagen) Differential diagnosis (palisading patterns) ===================================================================== ==== Appendiceal smooth muscle Calcifying aponeurotic fibroma Fibrous histiocytoma Leiomyoma Leiomyosarcoma