plexiform schwannoma

plexiform schwannoma

An uncommon schwannoma (4% of all schwannomas) which arises in superficial soft tissues, most often of the head and neck with a broad age range.

Clinical findings
Can be very large and cause pain, disfigurement, and neurologic and other deficits.

Location
Extremities, retroperitoneum/pelvis, trunk, parotid, vulva, thoracic oesophagus.

Prognosis
Local recurrence, 50%.

Worrisome histologic features
Increased cellularity, 68%; pleomorphism, 50%.

Plexiform schwannoma variants
• Superficial plexiform schwannoma.
• Extensive plexiform schwannoma.
• Deep plexiform schwannoma.
References in periodicals archive ?
Plexiform schwannoma is an uncommon variant of schwannoma and is characterized by its distinctive plexiform growth pattern.
Histologically, plexiform schwannoma is composed of Schwann cells with a multinodular or plexiform architecture (Figure 13, A and B) with a rim of perineurial cells and a thin fibrous capsule around each nodule.
Highly cellular examples of plexiform schwannoma have been reported in infants and children.
The differential diagnosis of plexiform schwannoma includes plexiform neurofibroma and malignant peripheral nerve sheath tumor (MPNST).
Plexiform schwannoma is a rare variant of schwannoma, characterized by a multinodular (plexiform) intraneural growth pattern [3].
Plexiform schwannoma usually presents as an isolated finding, and although it is unassociated with NF1, it may occur in the setting of NF2 and schwannomatosis [3, 6-10].
These morphologic features, along with the immunoprofile, are diagnostic of plexiform schwannoma.
Plexiform schwannoma usually presents as a single lesion in the skin and less commonly in the deep tissues [9, 12].
Psammomatous melanotic schwannoma, plexiform schwannoma, microcystic/reticular schwannoma are further variants of schwannoma8,9.
Plexiform schwannoma has been rarely reported in association with NF2.
Tumours from the small nerve branches had histopathological pattern of classical schwannomas (plexiform schwannomas).