22 October 2008 – Case of the Week #132


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We thank Dr. Daniel Ostler, MD Anderson Cancer Center, Houston, Texas (USA) for contributing this case.  To contribute a Case of the Week, email NatPernick@Hotmail.com with the clinical history, your diagnosis and diagnostic microscopic images in JPG, GIF or TIFF format (send as attachments, we will shrink if necessary).  Please include any other images (gross, immunostains, etc.) that may be helpful or interesting.  We will write the discussion (unless you want to), list you as the contributor, and send you $35 (US dollars) by check or PayPal for your time after we send out the case.  Please only send cases with high quality images and a diagnosis that is somewhat unusual (or a case with unusual features).


Case of the Week #132


Clinical History


A 54 year old woman had a right supraclavicular mass, which clinically resembled a lymph node.


Micro images: #1#2#3#4#5


What is your diagnosis?


































Dermatofibrosarcoma protuberans (DFSP), myxoid variant




The microscopic images show a sheet like proliferation of relatively bland spindle cells that infiltrate the adjacent fat, in a myxoid stroma.  Numerous thin walled vessels are present.  The spindle cells have pale eosinophilic cytoplasm and stellate nuclei without pleomorphism.  The tumor cells were immunoreactive for CD34 (images not provided).


The myxoid variant of DFSP is defined as having at least 50% myxoid stroma.  It is an uncommon variant, but has the same prognosis as classic DFSP (AJSP 2007;31:1371).  The tumor cells are immunoreactive for CD34, and negative for S100 and muscle markers.


The differential diagnosis includes benign and malignant tumors.  Myxoid neurofibroma has wavy nuclei, and often intratumoral axons.  It is strongly S100 positive.  Superficial angiomyxoma also has a myxoid stroma with numerous small vessels and may be CD34 positive.  However, it does not infiltrate fat and tends to be less cellular.  Myxoid liposarcoma has vessels that are more abundant, delicate and branching than the vessels of myxoid DFSP.  In addition, lipoblasts are prominent. 


Treatment of myxoid DFSP, like classic DFSP, consists of complete excision.  The prognosis is good, with only occasional recurrences (Am J Dermatopathol 2007;29:443).




Nat Pernick, M.D., President
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