Alveolar Rhabdomyosarcoma: A Prognostically Unfavorable Rhabdomyosarcoma Type and Its Necessary Distinction from Embryonal Rhabdomyosarcoma

  • D. Harms
Part of the Current Topics in Pathology book series (CT PATHOLOGY, volume 89)


Since publication of the classic descriptions by Riopelle and ThÉRiault (1956) and Enzinger and Shiraki (1969) the morphology of alveolar rhabdomyosarcoma (aRMS) has been well known. RMS presenting with clear and unequivocal (pseudo)alveolar spaces can be recognized at first sight as the alveolar (sub-)type of RMS. On the other hand, a diagnosis of aRMS may be difficult in cases which display neither significant evidence of rhabdomyogenesis in conventionally stained slides nor unambiguous alveolar spaces. In such cases the diagnosis of RMS must be confirmed by special staining including immunohistochemistry.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Almannsberger M, Osborn M, Treuner J, Hölscher A, Weber K, Schauer A (1982) Diagnosis of human childhood rhabdomyosarcoma by antibodies to desmin, the structural protein of muscle specific intermediate filaments. Virchows Arch [B] 39: 203–215CrossRefGoogle Scholar
  2. Bale PM, Parsons RE, Stevens MM (1983) Diagnosis and behavior of juvenile rhabdomyosarcoma. Hum Pathol 14: 596–611PubMedCrossRefGoogle Scholar
  3. Brooks J J (1982) Immunohistochemistry of soft tissue tumors. Myoglobin as a tumor marker for rhabdomyosarcoma. Cancer 50: 1757–1763PubMedCrossRefGoogle Scholar
  4. Coindre JM, de Mascarel A, Trojani M, de Mascarel J, Pages A (1988) Immunohistochemical study of rhabdomyosarcoma. Unexpected staining with S 100 protein and cytokeratin. J Pathol 155: 127–132PubMedCrossRefGoogle Scholar
  5. Cordell JL, Falini B, Erber WN, Ghosh AK, Zainalabideen A, MacDonald S, Pulford KAF, Stein H, Mason DY (1984) Immunoenzymatic labeling of monoclonal antibodies using immune complexes of alkaline phosphatase and monoclonal anti-alkaline phosphatase ( APAAP complexes ). J Histochem Cytochem 32: 219–229PubMedCrossRefGoogle Scholar
  6. Crist WM, Garasey L, Beltangady MS, Gehan E, Ruymann F, Webber B, Hays DM, Wharam M, Maurer HM (1990) Prognosis in children with rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Studies I and II. J Clin Oncol 8: 443–452PubMedGoogle Scholar
  7. de Jong ASH, van Vark M, Albus-Lutter CE, van Raamsdonk W, Voûte PA (1984) Myosin and myoglobin as tumor markers in the diagnosis of rhabdomyosarcoma. A comparative study. Am J Surg Pathol 8: 521–528PubMedCrossRefGoogle Scholar
  8. Dodd S, Malone M, McCulloch W (1989) Rhabdomyosarcoma in children: a histological and immunohistochemical study of 59 cases. J Pathol 158: 13–18PubMedCrossRefGoogle Scholar
  9. Donglass EC, Valentine M, Etcubanas E et al. (1987) A specific chromosomal abnormality in rhabdomyosarcoma. Cytogenet Cell Genet 45: 148–155CrossRefGoogle Scholar
  10. Engel R, Ritterbach J, Schwabe D, Lampert F (1988) Chromosome translocation (2;13)(q37;q14) in a disseminated alveolar rhabdomyosarcoma. Eur J Pediatr 148: 69–71PubMedCrossRefGoogle Scholar
  11. Enzinger FM, Shiraki M (1969) Alveolar rhabdomyosarcoma. An analysis of 110 cases. Cancer 24: 18–31PubMedCrossRefGoogle Scholar
  12. Erlandson RA (1987) The ultrastructural distinction between rhabdomyosarcoma and other undifferentiated “sarcomas”. Ultrastruct Pathol 11: 83–101PubMedCrossRefGoogle Scholar
  13. Gehan EA, Glover FN, Maurer HM, Sutow WW, Hays DM, Lawrence W Jr, Newton WA Jr, Soule EH (1981) Prognostic factors in children with rhabdomyosarcoma. Natl Cancer Inst Monogr 56: 83–92PubMedGoogle Scholar
  14. Haas JE, Palmer NF, Weinberg AG, Beckwith JB (1981) Ultrastructure of malignant rhabdoid tumor of the kidney. A distinctive renal tumor of children. Hum Pathol 12: 646–657PubMedCrossRefGoogle Scholar
  15. Harms D (1990) Cancer in the first year of life — pathology. Report from the Kiel Pediatric Tumor Registry. Gaslini 22: 115–121Google Scholar
  16. Harms D, Schmidt D, Treuner J (1985) Soft-tissue sarcomas in childhood. A study of 262 cases including 169 cases of rhabdomyosarcoma. Z Kinderchir 40: 140–145 HaysPubMedGoogle Scholar
  17. Harms DM, Soule EH, Lawrence W Jr, Gehan EA, Maurer HM, Donaldson M, Raney RB, Tefft M (1982) Extremity lesions in the Intergroup Rhabdomyosarcoma Study (IRS-I): a preliminary report. Cancer 48: 1–8Google Scholar
  18. Hays DM, Newton W Jr, Soule EH, Foulkes MA, Raney RB, Tefft M, Ragab A, Maurer HM (1983) Mortality among children with rhabdomyosarcomas of the alveolar histologic subtype. J Pediatr Surg 18: 412–417PubMedCrossRefGoogle Scholar
  19. Kindblom LG, Seidal T, Karlsson K (1982) Immuno-histochemical localization of myoglobin in human muscle tissue and embryonal and alveolar rhabdomyosarcoma. Acta Pathol Microbiol Immunol Scand [A] 90: 167–174Google Scholar
  20. Kodet R (1985) Rabdomyosarkom v detskem veku. II. Ultrastruktura (Rhabdomyosarcoma in childhood. II. Ultrastructure). Cesk Patol 21: 201–208PubMedGoogle Scholar
  21. Kodet R (1989) Rhabdomyosarcoma in childhood. An immunohistochemical analysis with myoglobin, desmin and vimentin. Pathol Res Pract 185: 207–213PubMedCrossRefGoogle Scholar
  22. Kodet R, Newton WA Jr, Hamoudi AB, Asmar L (1991) Rhabdomyosarcomas with intermediate-filament inclusions and features of rhabdoid tumors. Light microscopic and immunohistochemical study. Am J Surg Pathol 15: 257–267PubMedCrossRefGoogle Scholar
  23. Kodet R, Newton WA Jr, Hamoudi AB, Asmar L, Jacobs DL, Maurer HM (1993) Childhood rhabdomyosarcoma with anaplastic (pleomorphic) features. A report of the Intergroup Rhabdomyosarcoma Study. Am J Surg Pathol 17: 443–453PubMedCrossRefGoogle Scholar
  24. Koscielniak E, Harms D, Schmidt D, Ritter J, Keim M, Riehm H, Treuner J (1989) Soft tissue sarcomas in infants younger than 1 year of age: a report of the German Soft Tissue Sarcoma Study Group (CWS–81). Med Pediatr Oncol 17: 105–110PubMedCrossRefGoogle Scholar
  25. Koscielniak E, Rodary C, Flamant F, Carli M, Treuner J, Pinkerton R, Grotto P (1992) Metastatic rhabdomyosarcoma and histologically similar tumors in childhood: a retrospective European multi-center analysis. Med Pediatr Oncol 20: 209–214PubMedCrossRefGoogle Scholar
  26. Kowal-Vern A, Gonzalez-Crussi F, Turner J, Trujillo Y, Chou P, Herman C, Castelli M, Walloch J (1990) Flow and image cytometric DNA analysis in rhabdomyosarcoma. Cancer Res 50: 6023–6027PubMedGoogle Scholar
  27. Leuschner I, Schmidt D, Möller R, Harms D (1990) DNA-Ploidie und “nuclear organizer regions” bei Rhabdomyosarkomen. Ein Vergleich mit den klinischen Stadien und dem Verlauf. Verh Dtsch Ges Pathol 74: 589Google Scholar
  28. Mackay B (1990) Electron microscopy of soft tissue tumours. In: Fletcher CDM, McKee PH (eds) Pathobiology of soft tissue tumours. Churchill Livingstone, Edinburgh, pp 199–220Google Scholar
  29. Mechtersheimer G, Haas R, Katus HA, Möller P (1989) Leukämieverdächtige Panzytopenie als Initialsymptom eines alveolären Rhabdomyosarkoms des Hodens. Pathologe 10: 252–256PubMedGoogle Scholar
  30. Mierau GM, Favara BE (1980) Rhabdomyosarcoma in children: Ultrastructural study of 31 cases. Cancer 46: 2035–2040PubMedCrossRefGoogle Scholar
  31. Miettinen M, Rapola J (1989) Immunohistochemical spectrum of rhabdomyosarcoma and rhabdomyosarcoma-like tumors. Expression of cytokeratin and the 68-kD neurofilament protein. Am J Surg Pathol 13: 120–132PubMedCrossRefGoogle Scholar
  32. Miettinen M, Lehto VP, Badley RA, Virtanen I (1982) Alveolar rhabdomyosarcoma. Demonstration of the muscle type of intermediate filament protein, desmin, as a diagnostic aid. Am J Pathol 108: 246–251PubMedGoogle Scholar
  33. Newton WA Jr, Soule EH, Hamoudi AB, Reiman HM, Shimada H, Beltangady M, Maurer H (1988) Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation. J Clin Oncol 6: 67–75PubMedGoogle Scholar
  34. Newton W, Triche T, Marsden H, Ninfo V, Harms D, Gehan E, Maurer H (1989) International Childhood Soft Tissue Sarcoma Pathology Classification Study: design and implementation utilizing Intergroup Rhabdomyosarcoma Study II clinical and pathology data. Med Pediatr Oncol 17: 308 (abstract)Google Scholar
  35. Ninfo V, Cavazzana AO (1991) Tumors of muscle tissue. In: Ninfo V, Chung EB, Cavazzana AD (eds) Tumors and tumorlike lesions of soft tissue. Churchill Livingstone, New York, pp 91–121Google Scholar
  36. Nojima T, Abe S (1990) A case of alveolar rhabdomyosarcoma with a chromosomal translocation, t(2;13) (q37;q14). Virchows Arch [A] 417: 357–359CrossRefGoogle Scholar
  37. Nunez C, Abboud SL, Lemon NC, Kemp JA (1983) Ovarian rhabdomyosarcoma presenting as leukemia. Case report. Cancer 52: 297–300PubMedCrossRefGoogle Scholar
  38. Pais RC, Ragab AH (1991) Rhabdomyosarcoma in infancy. In: Maurer HM, Ruymann FB, Pochedly C (eds) Rhabdomyosarcoma and related tumors in children and adolescents. CRC Press, Boca Raton, pp 373–384Google Scholar
  39. Palmer NF, Foulkes MA, Sachs N, Newton WA (1983) Rhabdomyosarcoma: a cytological classification of prognostic significance. Proc Am Soc Clin Oncol 2: 229Google Scholar
  40. Ragab AH, Heyn R, Tefft M, Hays DN, Newton WA Jr, Beltangady M (1986) Infants younger than 1 year of age with rhabdomyosarcoma. Cancer 58: 2606–2610PubMedCrossRefGoogle Scholar
  41. Raney RB Jr, Tefft M, Maurer HM, Ragab AH, Hays DM, Soule EH, Foulkes MA, Gehan EA (1988) Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study ( IRS)-I. Cancer 62: 1257–1266PubMedCrossRefGoogle Scholar
  42. Riopelle JL, Thériault JP (1956) Sur une forme méconnue de sarcome des parties molles: Le rhabdomyosarcome alvéolaire. Ann Anat Pathol 1: 88–111Google Scholar
  43. Ruymann FB, Grufferman S (1991) Introduction and epidemiology of soft tissue sarcomas. In: Maurer HM, Ruymann FB, Pochedly C (eds) Rhabdomyosarcoma and related tumors in children and adolescents. CRC Press, Boca Raton, pp 3–18Google Scholar
  44. Ruymann FB, Newton WA Jr, Ragab AH, Donaldson MH, Foulkes M (1984) Bone marrow metastases at diagnosis in children and adolescents with rhabdomyosarcoma. Cancer 53: 368–373PubMedCrossRefGoogle Scholar
  45. Schmidt D, Harms D, Pilon VA (1987) Small-cell pediatric tumors: histology, immunohistochemistry, and electron microscopy. Clin Lab Med 7: 63–89PubMedGoogle Scholar
  46. Schmidt D, Leuschner I, Moeller R, Harms D (1990) Immunhistochemische Befunde bei Rhabdomyosarkomen. Pathologe 11: 283–289PubMedGoogle Scholar
  47. Seidal T, Kindblom LG (1984) The ultrastructure of alveolar and embryonal rhabdomyosarcoma. A correlative light and electron microscopic study of 17 cases. Acta Pathol Microbiol Immunol Scand [A] 92: 231–248Google Scholar
  48. Seidal T, Mark J, Haymar B, Angervall L (1982) Alveolar rhabdomyosarcoma: a cytogenetic and correlated cytological and histological study. Acta Pathol Microbiol Immunol Scand [A] 90: 345–354Google Scholar
  49. Seidal T, Kindblom LG, Angervall L (1987) Myoglobin, desmin and vimentin in ultrastructurally proven rhabdomyomas and rhabdomyosarcomas. An immunohistochemical study utilizing a series of monoclonal and polyclonal antibodies. Appl Pathol 5: 201–219PubMedGoogle Scholar
  50. Seidal T, Kindblom LG, Angervall L (1988) Alveolar and poorly differentiated rhabdomyosarcoma. A clinicopathologic, light-microscopic, ultrastructural and immunohistochemical analysis. Acta Pathol Microbiol Immunol Scand [A] 96: 825–838Google Scholar
  51. Seidal T, Angervall L, Kindblom LG (1990) Expression of muscle-specific actins and myosin in light microscopically undifferentiated small and dark cell malignancies of soft tissues. Acta Pathol Microbiol Immunol Scand [A] 98: 1105–1112Google Scholar
  52. Shapiro DN, Parham DM, Douglass EC, Ashmun R, Webber BL, Newton WA Jr, Hancock ML, Maurer HM, Look AT (1991) Relationship of tumor-cell ploidy to histologic subtype and treatment outcome in children and adolescents with unresectable rhabdomyosarcoma. J Clin Oncol 9: 159–166PubMedGoogle Scholar
  53. Shimada H, Newton WA Jr, Soule EH, Beltangady MS, Maurer HM (1987) Pathology of fatal rhabdomyosarcoma. Report from Intergroup Rhabdomyosarcoma Study ( IRS–I and IRS–II ). Cancer 59: 459–465PubMedCrossRefGoogle Scholar
  54. Sternberger LA, Hardy PH Jr, Cuculis JJ, Meyer HG (1970) The unlabeled antibody enzyme method of immunohistochemistry. Preparation and properties of soluble antigen-antibody complex (horseradish peroxidase-antihorseradish peroxidase) and its use in identification of spirochetes. J Histochem Cytochem 18: 315–333PubMedCrossRefGoogle Scholar
  55. Swanson PE, Dehner LP (1991) Pathology of soft tissue sarcomas in children and adolescents. In: Maurer HM, Ruymann FB, Pochedly C (eds) Rhabdomyosarcoma and related tumors in children and adolescents. CRC Press, Boca Raton, pp 385–420Google Scholar
  56. Toch R (1972) Case records of the Massachusetts General Hospital. Case 4 — 1972. N Engl J Med 286: 205–212CrossRefGoogle Scholar
  57. Tsokos M, Miser A, Pizzo P, Triche T (1984) Histologic and cytologic characteristics of poor prognosis childhood rhabdomyosarcoma. Lab Invest 50: 61AGoogle Scholar
  58. Tsokos M, Miser A, Wesley R, Miser JS, Kinsella TJ, Grayson J, Pizzo PA, Glatstein E, Triche TJ (1985) Solid variant alveolar rhabdomyosarcoma: a primitive rhabdomyosarcoma with poor prognosis and distinct histology. Interational Society of Paediatric Oncology, XVIIth meeting, Venice (abstract-volume), pp 71–74Google Scholar
  59. Tsokos M, Webber BL, Parham DM, Wesley RA, Miser A, Miser JS, Etcubanas E, Kinsella T, Grayson J, Glatstein E, Pizzo PA, Triche TJ (1992) Rhabdomyosarcoma. A new classification scheme related to prognosis. Arch Pathol Lab Med 116: 847–855PubMedGoogle Scholar
  60. Ture-Carel C, Lizard-Nacol S, Justrabo E, Favrot M, Philip T, Tabone E (1986) Consistent chromosomal translocation in alveolar rhabdomyosarcoma. Cancer Genet Cytogenet 19: 361–362CrossRefGoogle Scholar
  61. Valentine M, Douglass EC, Look AT (1989) Closely linked loci on the long arm of chromosome 13 flank a specific 2; 13 translocation breakpoint in childhood rhabdomyosarcoma. Cytogenet Cell Genet 52: 128–132PubMedCrossRefGoogle Scholar
  62. Wang-Wuu S, Soukup S, Ballard E, Gotwals B, Lampkin B (1988) Chromosomal analysis of sixteen human rhabdomyosarcomas. Cancer Res 48: 983–987PubMedGoogle Scholar
  63. Weeks DA, Beckwith JB, Mierau GW (1986) “Pseudo-rhabdoid” tumors of the kidney. Lab Invest 54: 10 PGoogle Scholar
  64. Weeks DA, Beckwith JB, Mierau GW, Luckey DW (1989) Rhabdoid tumor of kidney. A report of 111 cases from the National Wilms’ Tumor Study Pathology Center. Am J Surg Pathol 13: 439–458PubMedCrossRefGoogle Scholar
  65. Weeks DA, Beckwith JB, Mierau GW, Zuppan CW (1991) Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms’ Tumor Study Pathology Center. Am J Surg Pathol 15: 1042–1054PubMedCrossRefGoogle Scholar
  66. Wiener ES, Hays DM (1991) Rhabdomyosarcoma in extremity and trunk sites. In: Maurer HM, Ruymann FB, Pochedly C (eds) Rhabdomyosarcoma and related tumors in children and adolescents. CRC Press, Boca Raton, pp 363–372Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1995

Authors and Affiliations

  • D. Harms

There are no affiliations available

Personalised recommendations