Soft tissue
Other tumors
Clear cell sarcoma

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 15 September 2016, last major update November 2012

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Clear cell sarcoma [title] soft tissue

Cite this page: Clear cell sarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueclearcellsarcoma.html. Accessed December 14th, 2017.
Definition / general
  • Also known as melanoma of soft parts
  • Rare aggressive tumor of adolescents / young adults
  • Median age 30 years, range 13 - 73 years; 60% male
  • Deep soft tissues of extremities, trunk or limb girdles, tends to occur near tendon, fascia or aponeuroses
  • Slow progression, frequent local recurrences; eventually nodal and distant metastases
  • 5 year overall survival is 63% (Am J Surg Pathol 2008;32:452)
  • Size is most significant prognostic factor (Dermatol Res Pract 2012;2012:984096)
Case reports
Treatment
  • Complete surgical resection
Clinical images
Images hosted on other servers:

Arm

Gross description
  • Firm, well circumscribed, gray - white, gritty sensation when cutting
  • Median 4 cm, range 1 - 15 cm
  • Variable necrosis
Gross images
Images hosted on PathOut server:

Tendon, courtesy of Dr. Mark R. Wick

Courtesy of Dr. Mark R. Wick

Microscopic (histologic) description
  • Distinctly nested growth pattern with mixture of spindle, epithelioid and tumor giant cells
  • Melanin pigment in 2 / 3
  • May have floret-like multinucleated giant cells
  • Often rhabdoid cells, bizarre pleomorphic cells
  • Usually necrosis
  • Mean 4 MF / 10 HPF
Microscopic (histologic) images
Images hosted on PathOut server:

Tendon, courtesy of Dr. Mark R. Wick


S100, HMB45, Fontana Masson, courtesy of Dr. Mark R. Wick



Images hosted on other servers:

Arm

Lower leg tumor


Foot tumor

Spindled and epithelioid cells

Microphthalmia transcription factor

Cytology description
Cytology images
Images hosted on PathOut server:

Tendon FNA, courtesy of Dr. Mark R. Wick

Electron microscopy description
  • Melanosomes
Molecular / cytogenetics description
  • t(12;22)(q13;q12): ATF1 and EWS (not seen in melanoma)
  • Usually diploid or less aneuploidy than metastatic melanoma to soft tissue
  • Tumors in GI tract may have a variant fusion gene EWSR1 - CREB1
Molecular / cytogenetics images
Images hosted on other servers:

FISH

Differential diagnosis