Virchows Archiv

, Volume 445, Issue 4, pp 347–353 | Cite as

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

  • Michal Michal
  • Dmitry V. Kazakov
  • Irena Belousova
  • Michele Bisceglia
  • Michal Zamecnik
  • Petr Mukensnabl
Original Article


We present six cases of a distinctive soft tissue tumor which occurred in five women and one man. None of the patients had signs of neurofibromatosis. All tumors occurred on the fingers (n=5) or the thenar eminence of the hand (n=1). The mean age of the patients was 33 years. The tumors were 1–2.5 cm in diameter (mean size 1.6 cm). Three patients with follow-up were without signs of recurrence or metastasis. Microscopically the lesions were nonencapsulated and featured a multilobular architecture and both myxoid and pseudocystic change. The lobules varied in size and shape and were separated by variably thickened, dense, sclerotic/collagenous septae. The lobules were composed of two components: schwannomatous and perineuriomatous. The schwannomatous component was immunohistochemically S-100 protein positive and CD34 and EMA negative, and the perineuriomatous component had the appearance of retiform perineurioma. The perineurial parts were mostly S-100 protein and CD34 negative and EMA positive. These two components either formed separate nodules or the schwannomatous tissue surrounded the perineurial parts located in the centers of the lobules. We interpreted the lesions as hybrid tumors with features of schwannoma and retiform perineurioma.


Retiform perineurioma Reticular perineurioma Benign schwannoma-perineurioma Neurothekeoma 


  1. 1.
    Allen PW (2000) Myxoma is not a single entity: A reviewf of the concept of myxoma. Ann Diagn Pathol 4:99–123CrossRefPubMedGoogle Scholar
  2. 2.
    Alper M, Kavak A, Parlak AH, Demirci R, Belenli I, Yesildal N (2004) Measurement of epidermal thickness in a patient with psoriasis by computer-supported image analysis. Braz J Med Biol Res 37:111–117PubMedGoogle Scholar
  3. 3.
    Argenyi Z, LeBoits PE, Santa Cruz D, Swanson PE, Kutzner H (1993) Nerve sheath myxoma (neurothekeoma) of the skin: light microscopic and immunohistochemical reappraisal of the cellular variant. J Cutan Pathol 20:294–303PubMedGoogle Scholar
  4. 4.
    Ariza A, Bilbao JM, Rosai J (1988) Immunohistochemical detection of epithelial membrane antigen in normal perineurial cells and perineurioma Am J Surg Pathol 12:678–683Google Scholar
  5. 5.
    Blumberg AK, Adelaar RS (1989) Nerve sheath myxoma of digital nerve. Cancer 63:1215–1218PubMedGoogle Scholar
  6. 6.
    Braun-Falco M, Ring J (2003) Enhanced cytoplasmic expression of desmocollin 3 in epidermal rete ridges of Dowling-Degos syndrome. Br J Dermatol 149:1293–1296CrossRefPubMedGoogle Scholar
  7. 7.
    Burger PC, Scheithauer BW (1994) Atlas of tumor pathology. Tumors of the central nervous system, 3rd series, fascicle 10. AFIP, Washington, DCGoogle Scholar
  8. 8.
    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 103:696–703PubMedGoogle Scholar
  9. 9.
    Erlandson RA (1991) The enigmatic perineurial cell and its participation in tumors and in tumorlike entities. Ultrastruct Pathol 15:335–351PubMedGoogle Scholar
  10. 10.
    Fetsch JF, Miettinen M (1997) Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a predilection for the fingers and palms of young adults. Am J Surg Pathol 21:1433–1442CrossRefPubMedGoogle Scholar
  11. 11.
    Fletcher CDM (1989) Solitary circumscribed neuroma of the skin (so-called palisaded, encapsulated neuroma). A clinicopathologic and immunohistochemical study. Am J Surg Pathol 13:574–580PubMedGoogle Scholar
  12. 12.
    Hamada N, Ikuta Y, Ikeda A (1994) Arteriographic study of the arterial supply of the foot in one hundred cadaver feet. Acta Anat (Basel) 151:198–206Google Scholar
  13. 13.
    Henmi A, Sato H, Wataya T, Inaniwa Y, Mori Y (1986) Neurothekeoma. Report of a case with immunohistochemical and ultrastructural features. Acta Pathol Jpn 36:1911–1919PubMedGoogle Scholar
  14. 14.
    Hirokawa M, Takenaka R, Takahashi A, Sugihara K, Wada H, Tashiro T, Horiguchi H, Wakatsuki S, Sano T (2003) Esophageal xanthoma: report of two cases and a review of the literature. J Gastroenterol Hepatol 18:1105–1108CrossRefPubMedGoogle Scholar
  15. 15.
    Hirose T, Sano T, Hizawa K (1986) Ultrastructural localization of S100-protein in neurofibroma. Acta Neuropathol (Berl) 69:103–110Google Scholar
  16. 16.
    Holden CA, Wilson-Jones E, McDonald DM (1982) Cutaneous lobular neuromyxoma. Br J Dermatol 106:211–215Google Scholar
  17. 17.
    Husain S, Silvers DN, Halperin AJ, McNutt NS (1994) Histologic spectrum of neurothekeoma and the value of immunoperoxidase staining for S-100 protein in distinguishing it from melanoma. Am J Dermatol 16:496–503Google Scholar
  18. 18.
    Jurecka W (1987) Neurogenic tumors of the skin. Wien Klin Wochenschr 99 [Suppl 176]:3–16Google Scholar
  19. 19.
    Kahn DG, Duckett T, Bhuta SM (1993) Perineurioma of the kidney: report of a case with histologic, immunohistochemical and ultrastructural studies. Arch Pathol Lab Med 117:654–657PubMedGoogle Scholar
  20. 20.
    Karasawa J, Touho H, Ohnishi H, Kawaguchi M (1997) Rete mirabile in humans-case report. Neurol Med Chir (Tokyo) 37:188–192Google Scholar
  21. 21.
    Khalifa MA, Montgomery EA, Ismiil A, Azumi A (2000) What are the CD34+ cells in benign peripheral nerve sheath tumors? Double immunostaining study of CD34 and S100 protein. Am J Clin Pathol 114:123–126CrossRefPubMedGoogle Scholar
  22. 22.
    King DT, Barr RJ (1980) Bizarre cutaneous neurofibromas. J Cutan Pathol 7:21–31PubMedGoogle Scholar
  23. 23.
    Koppang EO, Bjerkas E, Bjerkas I, Sveier H, Hordvik I (2003) Vaccination induces major histocompatibility complex class II expression in the Atlantic salmon eye. Scand J Immunol 58:9–14PubMedGoogle Scholar
  24. 24.
    Laskin WB, Fetsch JF, Miettinen M (2000) The “neurothekeoma”: immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics. Hum Pathol 31:1230–1241CrossRefPubMedGoogle Scholar
  25. 25.
    Lassmann H, Jurecka W, Lassmann G, Gebhart W, Matras H, Watzek G (1997) Different types of nerve sheath tumors. Light microscopy, electron microscopy and autoradiography. Virchows Arch A Pathol Anat Histopathol 375:197–210Google Scholar
  26. 26.
    Lazarus SS, Trombetta LD (1978) Ultrastructural identification of benign perineurial cell tumor. Cancer 41:1823–1829PubMedGoogle Scholar
  27. 27.
    McDonnald DM, Wilson-Jones E (1977) Pacinian neurofibroma. Histopathology 1:247–255PubMedGoogle Scholar
  28. 28.
    Michal M (1999) Extraneural retiform perineuriomas. A report of four cases. Pathol Res Pract 195:759–763PubMedGoogle Scholar
  29. 29.
    Perentes E, Nakagawa Y, Ross GW, Stanton C, Rubinstein LJ (1987) Expression of epithelial membrane antigen in perineurial cells and their derivatives. An immunohistochemical study with multiple markers. Acta Neuropathol (Berl) 75:160–165Google Scholar
  30. 30.
    Roggen JFG van, McMenamin ME, Belchis D, Nielsen GP, Rosenberg AE, Fletcher CDM (2001) Reticular perineurioma. A distinctive variant of soft tissue perineurioma. Am J Surg Pathol 25:485–493CrossRefPubMedGoogle Scholar
  31. 31.
    Scheithauer BW, Woodruff JM, Erlandson RA (1999) In: Atlas of tumor pathology. Tumors of the peripheral nervous system, 3rd series, fascicle 24. AFIP, WashingtonGoogle Scholar
  32. 32.
    Smith TW, Bhawan J (1980) Tactile-like structures in neurofibromas. An ultrastructural study. Acta Neuropathol (Berl) 50:233–236Google Scholar
  33. 33.
    Theaker JM, Gatter KC, Puddle J (1988) Epithelial membrane antigen expression by the perineurium of peripheral nerve and in peripheral nerve tumors. Histopathology 13:171–179PubMedGoogle Scholar
  34. 34.
    Tsang WYW (1996) Perineuriomas: perineurial cell neoplasms with distinctive extra- and intra-neural forms. Adv Anat Pathol 3:212–222Google Scholar
  35. 35.
    Ushigome S, TakakuwaT, Hyuga M, Tadokoro M, Shinagawa T (1986) Perineurial cell tumor and the significance of the perineurial cells in neurofibroma. Acta Pathol Jpn 36:973–987PubMedGoogle Scholar
  36. 36.
    Weiser G (1975) An electron microscopic study of “pacinian neurofibroma.” Virchows Arch A Pathol Anat Histopathol 366:331–340Google Scholar
  37. 37.
    Weiser G (1978) Neurofibroma and perineurial cell. Electron optical examination of 9 neurofibromas. Virchows Arch A Pathol Anat Histopathol 379:73–83Google Scholar
  38. 38.
    Weiss SW, Nickoloff BJ (1993) CD-34 is expressed by a distinctive cell population in peripheral nerve, nerve sheath tumors, and related lesions Am J Surg Pathol 17:1039–1045Google Scholar
  39. 39.
    Yilmaz S, Aydin A, Dinc G (2001) The anatomy of the arterial supply of the thoracic limb of the porcupine (Hystrix cristata). Anat Histol Embryol 30:273–275CrossRefPubMedGoogle Scholar
  40. 40.
    Zamecnik M, Gomolcak P (1997) A case of perineurioma. Gen Diagn Pathol 143:261PubMedGoogle Scholar
  41. 41.
    Zamecnik M, Gomolcak P (1997) Perineurioma (in Slovak). CS Patol 33:28–30Google Scholar
  42. 42.
    Zamecnik M, Michal M (2001) Perineurial cell differentiation in neurofibromas. Report of eight cases including a case with composite perineurioma-neurofibroma features. Pathol Res Pract 197:537–544PubMedGoogle Scholar
  43. 43.
    Zelger B, Weinlich G, Zelger B (2000) Perineuroma. A frequently unrecognized entity with emphasis on a plexiform variant. Adv Clin Pathol 4:25–33Google Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Michal Michal
    • 1
  • Dmitry V. Kazakov
    • 1
  • Irena Belousova
    • 2
  • Michele Bisceglia
    • 3
  • Michal Zamecnik
    • 1
  • Petr Mukensnabl
    • 1
  1. 1.Sikl’s Department of PathologyCharles University, Medical Faculty HospitalPilsenCzech Republic
  2. 2.Department of DermatologyMedical Military AcademySaint PetersburgRussia
  3. 3.Department of PathologyIRCCS-Ospedale Casa Sollievo della SofferenzaSan Giovanni RotondoItaly

Personalised recommendations