Skip to main content
Log in

Microcystic/Reticular Schwannoma Arising in the Submandibular Gland: A Rare Benign Entity that Mimics More Common Salivary Gland Carcinomas

  • Case Report
  • Published:
Head and Neck Pathology Aims and scope Submit manuscript

Abstract

Microcystic/reticular schwannoma is a recently described variant of schwannoma with a predilection for the gastrointestinal tract, rarely involving the head/neck region. This is the first reported case involving the submandibular gland. We present a case in a 34 year old man with 4.5 cm submandibular mass. Fine needle aspiration suggested a spindle cell lesion. Frozen section evaluation raised the possibility of mucoepidermoid carcinoma. Resection showed a well circumscribed mass with a mucoid appearance. Histologic findings include a lobular architecture with fibrous septa, a lympho-plasmacytic infiltrate, and scattered lymphoid aggregates at the periphery. There are two distinct histologic patterns with solid areas of spindle cells and areas of spindle/ovoid cells with a microcystic pattern in a myxoid background. The tumor has a pushing border, with extension into adipose and adjacent parenchyma, without cytologic atypia or necrosis. Immunohistochemical stains are positive for S-100 and CD34, and negative for calponin, mammoglobin, ALK1, p63, ER, GFAP, SMA, desmin, cytokeratin 7, cytokeratin AE1/AE3, and C-Kit. Mucicarmine stain is negative. Recognition of this benign unusual variant of schwannoma is paramount for appropriate conservative treatment due to the morphologic and immunohistochemical overlap with primary salivary gland carcinomas.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Antinheimo J, Sankila R, Carpén O, Pukkala E, Sainio M, Jääskeläinen J. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology. 2000;54(1):71–6.

    Article  CAS  PubMed  Google Scholar 

  2. Weiss, SW. Enzinger and Weiss’s soft tissue tumors. 4th ed. St. Louis, MO: Mosby, Inc.; 2001. p. 1146–60.

    Google Scholar 

  3. Ackerman LV, Taylor FH. Neurogenous tumors within the thorax; a clinicopathological evaluation of forty-eight cases. Cancer. 1951;4(4):669–91.

    Article  CAS  PubMed  Google Scholar 

  4. Harkin JC, Reed RJ. Tumors of the peripheral nervous system. In: Scheithauer BW, Woodruff JM, Erlandson RA, editors. Atlas of tumor pathology, 2nd series, Fascicle 3. Washington: Armed Forces Institute of Pathology; 1969.

  5. Woodruff JM, Godwin TA, Erlandson RA, et al. Cellular schwannoma: a variety of schwannoma sometimes mistaken for a malignant tumor. Am J Surg Pathol. 1981;5:733.

    Article  CAS  PubMed  Google Scholar 

  6. Kindblom LG, Meis-Kindblom JM, Havel G, Busch C. Benign epithelioid schwannoma. Am J Surg Pathol. 1998;22:762–70.

    Article  CAS  PubMed  Google Scholar 

  7. Michal M, Kazakov DV, Belousova I, et al. A benign neoplasm with histopathological features of both schwannoma and retiform perineurioma (benign schwannoma-perineurioma): a report of six cases of a distinctive soft tissue tumor with a predilection for the fingers. Virchows Arch. 2004;445:347–53.

    Article  PubMed  Google Scholar 

  8. Feany MB, Anthony DC, Fletcher CD. Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge. Histopathology. 1998;32:405–10.

    Article  CAS  PubMed  Google Scholar 

  9. Font RL, Truong LD. Melanotic schwannoma of soft tissues. Electron-microscopic observations and review of literature. Am J Surg Pathol. 1984;8:129–38.

    Article  CAS  PubMed  Google Scholar 

  10. Woodruff JM, Marshall NL, Godwin TA, Funkhouser JW, Thompson NJ, Erlandson RA. Plexiform (multinodular) schwannoma. A tumor simulating the plexiform neurofibroma. Am J Surg Pathol. 1983;7:691–7.

    Article  CAS  PubMed  Google Scholar 

  11. Carney JA. Psammomatous melanotic schwannoma. A distinctive heritable tumor with special associations, including cardiac myxoma and the Cushing syndrome. Am J Surg Pathol. 1990;14:206–22.

    Article  CAS  PubMed  Google Scholar 

  12. Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM. The pathobiologic spectrum of schwannomas. Histol Histopathol. 2003;18:925–34.

    CAS  PubMed  Google Scholar 

  13. Liegl B, Bennett MW, Fletcher CD. Microcystic/reticular schwannoma: a distinct variant with predilection for visceral locations. Am J Surg Pathol. 2008;32(7):1080–7.

    Article  PubMed  Google Scholar 

  14. Gu MJ, Choi JH. Microcystic/reticular schwannoma of the esophagus: the first case report and a diagnostic pitfall. BMC Gastroenterol. 2014;18(14):193.

    Article  Google Scholar 

  15. Trivedi A, Ligato S. Microcystic/reticular schwannoma of the proximal sigmoid colon: case report with review of literature. Arch Pathol Lab Med. 2013;137(2):284–8.

    Article  PubMed  Google Scholar 

  16. Lau PP, Yau DT, Lau WH, Mak LS, Chan JK. Multinodular reticular schwannoma in the head and neck region: a potential diagnostic pitfall. Int J Surg Pathol. 2013;21(1):54–8.

    Article  PubMed  Google Scholar 

  17. Lee SM, Goldblum J, Kim KM. Microcystic/reticular schwannoma in the colon. Pathology. 2009;41(6):595–6.

    Article  PubMed  Google Scholar 

  18. Liegl B, Bodo K, Martin D, Tsybrovskyy O, Lackner K, Beham A. Microcystic/reticular schwannoma of the pancreas: a potential diagnostic pitfall. Pathol Int. 2011;61(2):88–92.

    Article  PubMed  Google Scholar 

  19. Kienemund J, Liegl B, Siebert F, Jagoditsch M, Spuller E, Langner C. Microcystic reticular schwannoma of the colon. Endoscopy. 2010;42(Suppl 2):E247.

    Article  PubMed  Google Scholar 

  20. Agaimy A, Märkl B, Kitz J, Wünsch PH, Arnholdt H, Füzesi L, Hartmann A, Chetty R. Peripheral nerve sheath tumors of the gastrointestinal tract: a multicenter study of 58 patients including NF1-associated gastric schwannoma and unusual morphologic variants. Virchows Arch. 2010;456(4):411–22.

    Article  PubMed  Google Scholar 

  21. Pang JM, Mahar A, Shannon K, Kench J, Chan C, Gupta R. Reticular and microcystic schwannoma of the parotid gland. Pathology. 2013;45(1):96–8.

    Article  PubMed  Google Scholar 

  22. Chaurasia JK, Afroz N, Sahoo B, et al. Reticular schwannoma mimicking myxoid sarcoma. BMJ Case Rep. 2014. doi:10.1136/bcr-2013-202963.

  23. Tang SX, Sun YH, Zhou XR, Wang J. Bowel mesentery (meso-appendix) microcystic/reticular schwannoma: case report and literature review. World J Gastroenterol. 2014;20(5):1371–6.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Chetty R. Reticular and microcystic schwannoma: a distinctive tumor of the gastrointestinal tract. Ann Diagn Pathol. 2011;15(3):198–201.

    Article  PubMed  Google Scholar 

  25. Tozbikian G, Shen R, Suster S. Signet ring cell gastric schwannoma: report of a new distinctive morphological variant. Ann Diagn Pathol. 2008;12(2):146–52.

    Article  PubMed  Google Scholar 

  26. Li BZ, Wang JW, Wei HQ. Microcystic/reticular schwannoma occurring in cervical spine: report of a case with literature review. Zhonghua Bing Li Xue Za Zhi. 2010;39(6):396–9.

    PubMed  Google Scholar 

  27. Agaimy A, Stachel KD, Jüngert J, Radkow T, Carbon R, Metzler M, Holter W. Malignant epithelioid peripheral nerve sheath tumor with prominent reticular/microcystic pattern in a child: a low-grade neoplasm with 18-years follow-up. Appl Immunohistochem Mol Morphol. 2014;22(8):627–33.

    Article  PubMed  Google Scholar 

  28. Cho KJ, Ro JY, Choi J, Choi SH, Nam SY, Kim SY. Mesencymal neoplasms of the major salivary glands: clinicopathological features of 18 cases. Eur Arch Oto Rhino Laryngol. 2008;25(1):47–56.

    Article  Google Scholar 

  29. Gnepp DR. Soft tissue tumors. In: Barnes L, Eveson JW, Reichart P, Sidransky D, editors. World Health Organization classification of tumours. Pathology and genetics of head and neck tumours. Lyon: IARC Press; 2005. p. 275.

  30. Val-Bernal JF, Mayorga M, Sedano-Tous MJ. Schwannomatosis presenting as pancreatic and submandibular gland schwannoma. Pathol Res Pract. 2013;209(12):817–22.

    Article  CAS  PubMed  Google Scholar 

  31. Ascani G, Junquera L, Rodríguez-Recio O, González F. Asymptomatic submandibular mass. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95:529–32.

    Article  PubMed  Google Scholar 

  32. Bondy PC, Block RM, Green J. Ancient schwannoma of the submandibular gland: a case report. Ear Nose Throat J. 1996;75:781–3.

    CAS  PubMed  Google Scholar 

  33. Chan MK, McGuire LJ. Cytodiagnosis of lesions presenting as salivary gland swellings: a report of seven cases. Diagn Cytopathol. 1992;8:439–43.

    Article  CAS  PubMed  Google Scholar 

  34. Diaz DD, Kennedy KS, Parker GS, White VJ. Schwannoma of the submandibular gland. Head Neck. 1991;13:239–42.

    Article  CAS  PubMed  Google Scholar 

  35. Sato J, Himi T, Matsui T. Parasympathetic schwannoma of the submandibular gland. Auris Nasus Larynx. 2001;28:283–5.

    Article  CAS  PubMed  Google Scholar 

  36. Sodagar R, Daneshbod K. Solitary neurilemmoma of salivary glands. Eye Ear Nose Throat Mon. 1971;50:306–8.

    CAS  PubMed  Google Scholar 

  37. Mair S, Leiman G. Benign neurilemmoma (schwannoma) masquerading as a pleomorphic adenoma of the submandibular salivary gland. Acta Cytol. 1989;33(6):907–10.

    CAS  PubMed  Google Scholar 

  38. Attie JN, Friedman E, Rotbherg M. Submandibular and axillary schwannomas not associated with von Recklinghausen’s disease. Journal of Oral and Maxillofacial Surgery. 1984;42:391–4.

    Article  CAS  PubMed  Google Scholar 

  39. Almeyda R, Kothari P, Chau H, Cumberworth V. Submandibular neurilemmoma; a diagnostic dilemma. J Laryngol Otol. 2004;118:156–8.

    Article  PubMed  Google Scholar 

  40. Gnepp DR. Mucinous myoepithelioma, a recently described new myoepithelioma variant. Head Neck Pathol. 2013;7(Suppl 1):S85–9.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Ryan P. Lau.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Lau, R.P., Melamed, J., Yee-Chang, M. et al. Microcystic/Reticular Schwannoma Arising in the Submandibular Gland: A Rare Benign Entity that Mimics More Common Salivary Gland Carcinomas. Head and Neck Pathol 10, 374–378 (2016). https://doi.org/10.1007/s12105-015-0674-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12105-015-0674-5

Keywords

Navigation