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Mucosal benign epithelioid nerve sheath tumors
Marc R Lewin1, H Parry Dilworth, Amer K Abu Alfa
1Johns Hopkins Medical Institutions, Weinberg 2242, 401 N. Broadway, Baltimore, MD 21231, USA.
The American Journal of Surgical Pathology
|September 15, 2005
Summary
This study identifies a rare type of mucosal epithelioid nerve sheath tumor, characterized by small, benign epithelioid cells found in colon and bladder biopsies. These tumors show an infiltrative pattern but have a favorable prognosis with no recurrence observed.
Area of Science:
- Gastrointestinal Pathology
- Surgical Pathology
- Oncology
Background:
- Mucosal nerve sheath tumors are documented in various sites.
- A distinct subset of epithelioid nerve sheath tumors in mucosal biopsies presents with unique characteristics.
- These epithelioid tumors have been noted as a component of colorectal schwannomas.
Purpose of the Study:
- To detail the histologic and clinicopathologic features of a rare subset of mucosal peripheral nerve sheath tumors.
- To characterize these epithelioid nerve sheath tumors found in mucosal biopsies.
- To assess the clinical behavior and prognosis of these distinct lesions.
Main Methods:
- Review of 7 mucosal biopsy cases (6 colonic, 1 bladder) with peripheral nerve sheath tumors.
- Histologic examination focusing on cellular morphology, growth patterns, and immunohistochemical markers (S-100, CD34, CD117, calretinin, EMA).
- Clinicopathologic review including patient demographics, lesion location, discovery method, and follow-up data.
Main Results:
- The study identified 7 cases of mucosal epithelioid nerve sheath tumors (mean age 58.6 years, slight female predominance).
- Histology revealed infiltrative lesions composed of epithelioid cells with uniform nuclei and eosinophilic cytoplasm; no mitoses were observed.
- Immunohistochemistry showed diffuse S-100 positivity, variable CD34, and negativity for CD117 and calretinin. Follow-up confirmed a benign clinical course with no recurrence.
Conclusions:
- Mucosal epithelioid nerve sheath tumors are a rare entity.
- These tumors are characterized by prominent epithelioid cells, an infiltrative growth pattern, and a benign clinical course.
- They are often discovered incidentally during endoscopic procedures or imaging.