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Microcystic/Reticular Schwannoma: A Distinct Variant With Predilection for Visceral Locations
(Am J Surg Pathol 2008;32:1080–1087)
Abstract: Schwannomas are benign, generally nonrecurring
tumors most frequently arising in the subcutaneous tissue of
adults with no sex predilection. Herein we report 10 cases of a
distinctive morphologic variant of schwannoma with predominantly
microcystic-reticular morphology and characterize the
clinicopathologic spectrum. The age at presentation ranged
from 11 to 93 years (median age 63 y). The tumor size ranged
from 0.4 to 23 cm (median size 4.3 cm). Five tumors arose in the
gastrointestinal tract, most often in the submucosa. Two cases
arose in subcutaneous tissue and 1 case each in the upper
respiratory tract, the adrenal gland, and deep soft tissue. None
of the patients had features of neurofibromatosis type 1 or type
2 (NF1, NF2). Histologically 8 tumors were circumscribed but
unencapsulated and 2 cases located in the subcutaneous fat were
circumscribed and encapsulated. At visceral locations, focally
pushing margins and microscopic foci of infiltration into
surrounding parenchyma were seen. All cases showed a striking
microcystic and reticular lesional growth pattern with anastomosing
and intersecting strands of spindle cells with eosinophilic
cytoplasm distributed around islands of myxoid or collagenous/
hyalinized stroma. The nuclei were round, oval, and tapered and
showed inconspicuous nucleoli. Three cases had smaller areas
resembling conventional schwannoma. Mitotic activity did not
exceed more than 3 mitoses/50 high-power fields (HPF) (median
1/30 HPF). Pleomorphism and necrosis were absent. All tumors
showed strong nuclear and cytoplasmic positivity for S-100 and
variably strong glial fibrillary acidic protein staining. A
surrounding tumor capsule was highlighted with epithelial
membrane antigen in 2 out of 10 cases. Smooth muscle actin,
Desmin, Pan-CK, AE/AE3, Cam5.2, and p-63 were negative in
all cases evaluated. Neurofilament protein highlighted axons in
one out of 7 cases investigated. CD117 showed weak focal
positivity in 1 out of 4 cases. Follow-up data were available in
7 cases (median duration 15 mo). None has recurred to date.
Microcystic schwannoma represents a distinctive morphologic
variant of schwannoma with predilection for visceral locations.
Recognition of this distinct entity is essential to avoid confusion
with malignant tumors, especially in the gastrointestinal and
upper respiratory tracts.

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