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Quick Review for Patients
Note: click on underlined words for more detail or photos. Schwannoma (neurilemoma) is
a benign neoplasm of the Schwann cells of the neural
sheath. At least a fourth, and perhaps as much as half
of all cases of neurilemoma and neurofibroma occur in the head and
neck region. In some series, one-fourth of the head and neck
cases were found in patients with von Recklinghausen neurofibromatosis
(neurofibromatosis I). Clinical Features The neurilemoma
of the oral region is a slowly enlarging, painless submucosal nodule which
is somewhat movable beneath the surface and rarely becomes larger than 2
cm. in greatest diameter. It is most frequently diagnosed
in the 25-55 year age group, but can occur at any age; there is no gender
predilection. The tongue is the most frequent site of occurrence, perhaps
resulting in macroglossia, but any oral site is susceptible. Most lesions
are sessile with a normal surface coloration, but occasional lesions will be
pedunculated (Figure
1) and larger ones may be lobulated. Dorsal tongue lesions often show a loss of papillae of the overlying
epithelium. Pathology and Differential Diagnosis The neurilemoma is characterized by two basic tissue types. Antoni type A tissue shows fascicles of spindle-shaped Schwann cells streaming around numerous acellular, eosinophilic areas surrounded by paralleled or palisaded spindled cells with blunt, elongated nuclei (Figures 2 & 3). The cells of these Verocay bodies all orient their long axes toward the acellular area, and the areas themselves are oval, linear or serpiginous in shape. A low power microscopic view of this tissue is reminiscent of an aerial view of soldiers aligned against each other across multiple battlefields. The second tissue type, Antoni type B, lacks the organoid, homogeneous Verocay bodies and consists entirely of less cellular and more randomly arranged spindle cells in a loose, myxomatous stroma (Figures 4 & 5). Neurites usually cannot be demonstrated in either tissue type.
The tumor is typically encapsulated and the
associated peripheral nerve may be seen in the microscopic section. Occasional
older lesions show degenerative changes consisting of hemorrhage, hemosiderin
deposition, mild chronic inflammatory cell infiltration, dense fibrosis,
and nuclear pleomorphism. This ancient neurilemoma is benign but
must be differentiated from neurofibrosarcoma and malignant
neurilemoma. Treatment and Prognosis The
neurilemoma is treated by conservative surgical excision, with minimal risk
of recurrence. Malignant transformation in untreated lesions
has been reported but is uncommon. Patients with multiple neural tumors should
be evaluated for von Recklinghausen neurofibromatosis or
MEN III.
References (Chronologic Order) Note: General references can be found by clicking on that topic to the left. Hatziotis JC, Asprides H. Neurilemoma (schwannoma) of the oral cavity. Oral Surg Oral Med Oral Pathol 1967; 24:510-526. Wright BA, Jackson D. Neural tumors of the oral cavity. Oral Surg Oral Med Oral Pathol 1980; 49:509-522. Sharma S, Sarkar C, Mathur M, et al. Benign nerve sheath tumors: a light microscopic, electron microscopic and immunohistochemical study of 102 cases. Pathology 1990; 22;191-195. White W, Shui MH, Rosenblum MK, et al. Cellular schwannoma. A clinicopathologic study of 57 patients and 58 tumors. Cancer 1990; 66:1266-1275. Colmenero C, Rivers T, Patron M, et al. Maxillofacial malignant peripheral nerve sheath tumours. J Craniomaxillofac Surg 1991; 19:40-46. Williams HK, et al. Neurilemmoma of the head and neck. Br J Oral Maxillofac Surg 1993; 31:32-35.
Woodruff JM. The pathology and treatment
of peripheral nerve tumors and tumor-like conditions. CA Cancer J Clin 1993;
43:290-308. Note: To see enlarged photo, click on
the left-hand picture;
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Figure 1: Cut surface of a schwannoma shows a thick stalk and a central core of pure white neural tissue, surrounded by more routine fibrovascular tissue. Note the traumatic ulcer of the superior surface. [return to text] |
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Figure 2: [return to text] |
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Figure 3: Upper left shows hyaline areas lined by nuclei in a pattern reminiscent of the aerial view of a battlefield. Lower right area shows streaming lesional cells. [return to text] |
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