CCS is an inherited disorder that commonly involves the development of multiple but on occasion a single or few tumors that closely resemble, and may be confused with, the sporadic SA and SAC tumors described here. CCS spiradenoma and CCS spiradenocarcinoma tumors must be distinguished form the sporadic SA and SAC tumors reviewed here in order to afford genetic counseling to individuals with CCS as well as to the close family members of these individuals.Currently, SA and SAC are usually treated by complete excision making sure that no tumor cells are left behind at the surgical site. This is particularly important in SAC where the incomplete surgical removal of all tumor cells may result in recurrence of the tumor at the surgical site and/or its metastasis to the local lymph nodes draining the surgical site and/or to distant tissues. In addition to surgical resection, some cases of SAC tumors have been treated with adjuvant therapy that includes radiotherapy and/or chemotherapy. It is not clear that these adjuvant treatments improve patient prognoses. Further studies are needed to determine the best treatment(s) for localize SA, localized SAC, and, in particular, metastatic SAC tumors. Presentation
Spiradenoma tumors occur in individuals of various ages but tend to develop in middle-aged and elder adults (e.g. in a study of 27 patients, the median age at diagnosis was 62 years). However, cases have been reported in children as young as 2 years. These tumors usually present as a solitary, sometimes painful, slowly growing, 1–2 cm (i.e.
An intraneural perineurioma is a rare benign tumor within the sheath of a single nerve that grows but typically does not recur or metastasize. These lesions are only composed of perineurial cells, cloned from a single cell. They are distinct from schwannoma and neurofibroma. Intraneural perineurioma is a neoplastic proliferation of perineurial cells with unique immunohistochemistry and ultrastructural features, and it is distinct from other onion bulb Schwann cell-derived entities. Despite harboring molecular abnormalities of the long arm of chromosome 22, intraneural perineurioma has not been associated with neurofibromatosis.Due to the involvement of one or more nerve fascicles, intraneural perineuriomas are distinguished by a localized, solitary expansion of peripheral nerves. These tumors develop slowly or stay stable over time. References
Further reading
Macarenco RS, Ellinger F, Oliveira AM (2007). "Perineurioma: a distinctive and underrecognized peripheral nerve sheath neoplasm". Archives of Pathology & Laboratory Medicine. 131 (4): 625–36. doi:10.1043/1543-2165(2007)131[625:PADAUP]2.0.CO;2. PMID 17425397. Kum YS, Kim JK, Cho CH, Kim HK (2009). "Intraneural reticular perineurioma of the hypoglossal nerve". Head Neck. 31 (6): 833–7. doi:10.1002/hed.20965. PMID 18972430. Chung JH, Jeong SH, Dhong ES, Han SK (2014). "Surgical removal of intraneural perineurioma arising in the brachial plexus using an interfascicular dissection technique". Arch Plast Surg. 41 (3): 296–9. doi:10.5999/aps.2014.41.3.296. PMC 4037780. PMID 24883285. Emory TS, Scheithauer BW, Hirose T, Wood M, Onofrio BM, Jenkins RB (1995). "Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22". Am J Clin Pathol. 103 (6): 696–704. doi:10.1093/ajcp/103.6.696. PMID 7785653.