Soft tissue chordomas: A clinicopathologic analysis of 11 cases

Scott R. Lauer, Mark A. Edgar, Jerad M. Gardner, Anita Sebastian, Sharon W. Weiss

Research output: Contribution to journalArticle

27 Citations (Scopus)

Abstract

Soft tissue chordomas (STCs) have never been systematically studied because of their rarity and the difficulty in separating them from similar-appearing lesions. Using brachyury to confirm the diagnosis, we have analyzed our experience with 11 cases. Cases coded as "chordoma" or "parachordoma" were retrieved from institutional and consultation files (1989 to 2011) and were excluded from further analysis if they arose from the bone or in a patient with previous axial chordoma. Eleven of 27 cases met inclusion criteria. Patients (8 male; 3 female) ranged in age from 13 to 71 years (mean 44 y). Tumors were located on the buttock (n=2), wrist (n=2), leg (n=2), toe (n=1), thumb (n=1), ankle (n=1), shoulder (n=1), and chest wall (n=1), ranged in size from 0.5 to 10.9 cm (mean 5.3 cm), and consisted of cords and syncytia of spindled/epithelioid cells with vacuolated eosinophilic cytoplasm and a partially myxoid background. Tumors expressed brachyury (10/10), 1 or more cytokeratins (11/11), and S100 protein (10/11). Follow-up information was available for 10 patients (69 mo; range, 2 to 212 mo). Most (n=6) were alive without disease, 2 developed local recurrence and lung metastases, and 1 developed lung metastasis only. One died with unknown disease status. STCs are histologically identical to osseous ones, but differ in their greater tendency to occur in distal locations where small size and surgical resectability result in better disease control. The existence of STC implies that notochordal remnants are not a prerequisite for chordoma development.

Original languageEnglish (US)
Pages (from-to)719-726
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume37
Issue number5
DOIs
StatePublished - May 1 2013

Fingerprint

Chordoma
Neoplasm Metastasis
Epithelioid Cells
Lung
Buttocks
S100 Proteins
Thumb
Toes
Thoracic Wall
Giant Cells
Keratins
Wrist
Ankle
Neoplasms
Leg
Cytoplasm
Referral and Consultation
Bone and Bones
Recurrence

Keywords

  • brachyury
  • extra-axial chordoma
  • parachordoma
  • soft tissue chordoma

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Soft tissue chordomas : A clinicopathologic analysis of 11 cases. / Lauer, Scott R.; Edgar, Mark A.; Gardner, Jerad M.; Sebastian, Anita; Weiss, Sharon W.

In: American Journal of Surgical Pathology, Vol. 37, No. 5, 01.05.2013, p. 719-726.

Research output: Contribution to journalArticle

Lauer, Scott R. ; Edgar, Mark A. ; Gardner, Jerad M. ; Sebastian, Anita ; Weiss, Sharon W. / Soft tissue chordomas : A clinicopathologic analysis of 11 cases. In: American Journal of Surgical Pathology. 2013 ; Vol. 37, No. 5. pp. 719-726.
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