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In this section we have spot diagnoses posted on a daily basis since June 2010, now over 1700! You can review the archived cases and read the suggested diagnoses by users and the final comment by Dr Uma Sundram, the Editor-in-Chief and main spot diagnosis host. Case are uploaded each week day by 10 a.m. UK time with the correct diagnosis will generally be posted at 8 p.m. UK time. Why not view the most recent spot diagnosis and proffer a diagnosis?

Case Number : Case 3041 - 03 March 2022 Posted By: Saleem Taibjee

Please read the clinical history and view the images by clicking on them before you proffer your diagnosis.
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14-year-old girl, pigmented lesion right cheek


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Shawn

Posted

Epithelioid blue nevus VS PEM. Those large "RS-like" cells with prominent nucleoli are expected in PEM. PRKAR1A status for confirmation.

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Richard Logan

Posted

Cellular/epithelioid blue naevus.

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Meenakshi Batrani

Posted

On 03/03/2022 at 02:20, Shawn said:

Epithelioid blue nevus VS PEM. Those large "RS-like" cells with prominent nucleoli are expected in PEM. PRKAR1A status for confirmation.

Agree

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vincenzo

Posted

Favor PEM. Thought of DPN also, but, as pointed out by Shawn, RS-like cells are definitely in keeping with PEM. 

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Meenakshi Batrani

Posted

Challenging. The entire gamut of epithelioid blue nevus, PEM and BLITZ (Blue+Sptiz) or called as superficial variant of DPN by some, are overlapping. PRAKAR1 and Beta-Catenin will be helpful. 

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Saleem Taibjee

Posted

Yes, very well done. This is indeed pigmented epithelioid melanocytoma.

This case had already been signed out by another pathologist as blue naevus. I reviewed this case prior to MDT, when the small focus of epithelioid cells which show prominent nucleoi - 'fried eggs' - caught my attention, and which are highlighted above.

You can appreciate my excitement when I learned at the MDT that the patient has facial freckling and other pigmented lesions, and a history of angiomyxoma of the vulva previously, and the clinician was suspecting Carney complex. This information had not appeared on the original clinical request form. But, of course, on discovering this information, this puts this lesion more firmly into the PEM category. These are lesions which historically were called epithelioid blue naevus in the context of Carney, but this is no longer the appropriate term.

I show the Melan-A and S100 below. The Melan-A makes the fried egg cells really 'pop out'. The S100 also stains quite strongly. As you know this is indirect evidence that this is not a blue naevus (in which S100 is often diminished).

I have since heard that the patient now has genetically confirmed Carney complex.

I don't have access to IHC / tissue testing for PRKAR1A, and so have sent this case on to Arnaud de la Fouchardiere in Lyon; he has also confirmed loss of PRKAR1A on IHC.

BW
Saleem

58717_40.0xb Melan-A.jpg

58717_63.0x Melan-A.jpg

58717_40.0xb S100 labelled.jpg

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vincenzo

Posted

Great case, Saleem. And many learning points. Thanks.

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