Soft tissue
Uncertain differentiation
Clear cell sarcoma


Topic Completed: 1 November 2012

Revised: 5 September 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Clear cell sarcoma [title] soft tissue

Vijay Shankar, M.D.
Page views in 2018: 10,424
Page views in 2019 to date: 8,946
Cite this page: Shankar V. Clear cell sarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissueclearcellsarcoma.html. Accessed September 18th, 2019.
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:

Contributed by Mark R. Wick, M.D.

Tendon

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:

Contributed by Mark R. Wick, M.D.

Tendon


S100, HMB45, Fontana Masson



Images hosted on other servers:

Arm

Lower leg tumor

Foot tumor

Cytology description
Cytology images

Images hosted on PathOut server:

Contributed by Mark R. Wick, M.D.

Tendon FNA

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

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FISH

Differential diagnosis
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