c. Presentation clinical of pigmented epithelioid melanocytoma

 
The diagnoses clinical of the 11 (out of 41) patients with metastases in the series of Zembowicz et al. were as follows:
 
Blue nodule, probably combined nevus (scalp)
Pigmented nevus (back)
Atypical blue nevus (toe)
Not stated (thigh)
Changing congenital nevus (scalp)
Black nevus changing (upper arm)
Seborrheic keratosis, poroma, pilomatrixoma, Spitz nevus (scalp)
Melanoma (scalp)
Tumor (calf)
Not stated (face)
Not stated (shoulder)
 
Of the lesions clinical that did not metastasize, two occurred in the genitalia, one, "a darkly pigmented ulcerated tumor on the shaft of the penis in an 18-year-old man, and the other, a vaginal vestibule lesion in a 44-year-old patient." Surely those were not the sites anatomic that Carney and Ferreiro, in 1996, found to be favored by epithelioid blue nevus. Strangely, no reference was made by Zembowicz and his collaborators to "equine melanocytic disease" although the anogenital region is a site of predilection for the lesion in horses. In the service of consistency, the coworkers should have created the term "animal-type" epithelioid blue nevus!
 
Zembowicz, Carney, and Mihm also made these comments about aspects clinical of pigmented epithelioid melanocytoma: "Our data show that pigmented epithelioid melanocytoma has a predilection for children, adolescents, and young adults, but it occurs over a broad age range. It has no ethnic predilection. Its frequent occurrence in races less susceptible to common melanoma, and wide site distribution, including involvement of genital and mucosal sites, suggest that sun exposure is unlikely to be a major factor in its pathogenesis. The majority of tumors apparently arose de novo, but occasional lesions were associated with a dermal or congenital nevus. None of the patients in our series had clinical features of Carney complex." Carney and Ferreiro, in 1996, recorded that epithelioid blue nevi did occur mostly on sites exposed to sunlight, namely, the extremities, trunk, and head and neck; none of the lesions involved "genital and mucosal sites."
 
Only one of the 11 examples of pigmented epithelioid melanocytoma that metastasized was diagnosed as "melanoma" prior to biopsy. Moreover, except for one lesion on the penis that had not metastasized, to wit, "a darkly pigmented ulcerated tumor," no description clinical was provided for any of the other 40 lesions said to be those of pigmented epithelioid melanocytoma. In brief, it is impossible on the basis of what is presented in the article by Zembowicz, Carney, and Mihm to have any idea of what a pigmented epithelioid melanocytoma looks like on inspection gross, thereby excluding any possibility of diagnosing it with surety clinically. Not a single photograph of a lesion clinical purported to be one of pigmented epithelioid melanocytoma was printed in the article.