Cutaneous metastatic chordoma with concomitant tuberous sclerosis.
Publication/Presentation Date
8-1-2006
Abstract
BACKGROUND: Chordomas are rare malignant tumors of notochord origin and are locally aggressive with a metastatic potential. Of noted interest, the skin rarely is seen as a metastatic site.
METHODS AND OBSERVATIONS: We describe a 20-month-old infant whose primary presentation of a clivus-based chordoma was multiple asymptomatic cutaneous nodules. A skin biopsy showed large vacuolated cells embedded in a myxoid stroma. Immunohistochemical staining was positive for S-100, keratin, and epithelial membrane antigen markers consistent with malignant chordoma. Magnetic resonance imaging revealed a clivus-based chordoma with scattered metastases within the neuraxis and multiple visceral sites. Further examination also revealed a diagnosis of tuberous sclerosis.
LIMITATIONS: An observed association between chordoma and tuberous sclerosis cannot be established firmly on the basis of a case report.
CONCLUSIONS: This case illustrates a diagnostic challenge because of the unusual presentation of an already rare tumor. By reporting our case, we hope to assist in the recognition of this rare dermal condition and highlight the speculated coexistence of tuberous sclerosis with chordoma tumors.
Volume
55
Issue
2 Suppl
First Page
6
Last Page
10
ISSN
1097-6787
Published In/Presented At
Lountzis, N. I., Hogarty, M. D., Kim, H. J., & Junkins-Hopkins, J. M. (2006). Cutaneous metastatic chordoma with concomitant tuberous sclerosis. Journal of the American Academy of Dermatology, 55(2 Suppl), S6–S10. https://doi.org/10.1016/j.jaad.2005.08.061
Disciplines
Medicine and Health Sciences
PubMedID
16843131
Department(s)
Department of Medicine
Document Type
Article