Embryonal "Botryoid" rhabdomyosarcoma of the larynx: a clinicopathologic and immunohistochemical study of two cases

Ann Diagn Pathol. 1999 Dec;3(6):341-9. doi: 10.1016/s1092-9134(99)80012-4.

Abstract

Two cases of embryonal rhabdomyosarcoma of the larynx are reported. The tumors occurred in a 16-year-old boy and in a 66-year-old man. They manifested clinically with nonspecific symptoms, including voice hoarseness and sense of throat fullness. Treatment consisted of total and partial laryngectomy, respectively. Grossly, both lesions had an exophytic growth pattern and microscopically featured a proliferation of small round to oval cells. Cell cytoplasms were occasionally stainable and fibrillary. Quite often, tumor cellularity was denser beneath the covering mucosa, recalling a "cambium layer" pattern. Tumor cells immunoreacted for desmin, actins, myoglobin, and sarcomeric actin; no immunostaining was noted for epithelial markers. No further antitumoral treatment was administered after surgery. There has been no recurrence of tumor at 2 and 10 years, respectively. Based on our series and the available literature, it seems that rhabdomyosarcoma of the larynx pursues a less-aggressive course than that seen in the homonimic juvenile or adult soft tissue lesion. Surgery alone appears to be a valid treatment option, especially when a polypoid, or "botryoid" gross pattern, coupled with the embryonal small cell histotype is encountered. In light of these findings, it is suggested that botryoid rhabdomyosarcoma of the larynx may deserve a specific consideration among the various laryngeal mesenchymal malignancies.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Aged
  • Hoarseness
  • Humans
  • Immunohistochemistry / methods
  • Laryngeal Neoplasms / metabolism
  • Laryngeal Neoplasms / pathology*
  • Laryngeal Neoplasms / physiopathology
  • Male
  • Neoplasms, Germ Cell and Embryonal / metabolism
  • Neoplasms, Germ Cell and Embryonal / pathology*
  • Neoplasms, Germ Cell and Embryonal / physiopathology
  • Rhabdomyosarcoma / metabolism
  • Rhabdomyosarcoma / pathology*
  • Rhabdomyosarcoma / physiopathology
  • Staining and Labeling