Primary Cutaneous PEComa...Explained by a Dermatopathologist

A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: kikoxp.com/pos... (dermpath) & kikoxp.com/pos... (bone/soft tissue sarcoma pathology).
PEComas are an odd family of tumors with melanocytic and myogenic immunophenotype. They can arise in the visceral organs, soft tissue, or, very rarely, skin. Primary cutaneous PEComas have some features that differ from many of their deep counterparts, including a distinct clear cell nest-like growth pattern and a tendency to lack desmin or actin staining in around half of cases. Watch the video to learn more including how to work up these case and exclude other entities in the differential diagnosis.
Additional reading:
- Our series of 5 primary cutaneous PEComas in Journal of Cutaneous Pathology: www.ncbi.nlm.n...
- Excellent paper by Phil LeBoit's group: www.ncbi.nlm.n...
- Early paper by Mentzel's group: www.ncbi.nlm.n...
- Great paper on deep soft tissue & gynecologic PEComas by Folpe et al: www.ncbi.nlm.n...
Please check out my Soft Tissue Pathology & Dermatopathology survival guide textbooks: bit.ly/2Te2haB ‬
This video is geared towards medical students, pathology or dermatology residents, or practicing pathologists or dermatologists. Of course, this video is for educational purposes only and is not formal medical advice or consultation.
Presented by Jerad M. Gardner, MD. Please subscribe to my channel to be notified of new pathology teaching videos.
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[@JMGardnerMD]

A complete organized library of all my videos, digital slides, pics, & sample pathology reports is available here: kikoxp.com/posts/5084 (dermpath) & kikoxp.com/posts/5083 (bone/soft tissue sarcoma pathology).

[@tinocasadeitherezo6063]

How rich in information a presentation can be! Thank you, Dr Gardner, for your generosity and professionalism!

[@madhudewan8207]

Please give features of uterine pecoma also. In cutaneous PECOMA, have you seen ALK negativity

[@JoaoHenrique-rl8pm]

How about leprosy in the differential diagnosis?

[@MTFeng0105]

My friend passed away because of Pecoma ....so sad...

[@amaliyadipita6906]

👍🏾👍🏾👍🏾👍🏾👍🏾👍🏾👍🏾👍🏾👍🏾

[@carlosnousari129]

Pristine lecture. Great job Jerad! Carlos Nousari, MD

[@umarfarooqkhan8208]

Dear dr jeerad your presentation is really exec Elle this

[@jadenisntonfire4445]

How would you interpret a possible cutaneous PEComa with
HMB45: patchy focal staining in clear cells, s100 highlighting the dendritic and melanocytes
Mart-1 negative,SMA negative, CD163 neg in clear cells, CD34 neg in clear cells, PAX 8 neg, CD117 neg, pancytokeratin neg?
Lesion extends to deep margin after two reexcision

[@dr.remishazahid]

Hey Dr. Jerad
Is cathepsin K helpful in cutaneous pecomas?

[@JMGardnerMD]

Supposedly per literature. But I’ve never used it myself.

[@dr.remishazahid]

@@JMGardnerMD Many thanks sir

[@LisaCulton]

I think that I may have a case now and that's why I'm watching!

[@joanchen8972]

Thank you, for· your· wonderful presentation, Dr Gardner !

[@virenvaghasiya2303]

Do they recur?

[@lubnasuaiti1334]

Can PECOMA express AE1/3?

[@JMGardnerMD]

Rarely can express keratin. Usually negative.

[@lubnasuaiti1334]

@@JMGardnerMD Thank you so much!

[@michaelchristopher4808]

what's the clinical presentation?

[@JMGardnerMD]

Non-descript usually. We have nice clinical and gross images in our paper on this entity from 2017: www.ncbi.nlm.nih.gov/m/pubmed/28556973/