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American Journal of Dermatopathology:
From Dermatopathology: Practical and Conceptual

Melanomas in Prepubescent Children: Review Comprehensively, Critique Historically, Criteria Diagnostically, and Course Biologically

Mones, Joan M. D.O.; Ackerman, A. Bernard M.D.

Section Editor(s): Ackerman, A. Bernard Editor

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Abstract

Our series was comprised of 11 children age 10 years or younger (6 were younger than age 5) with primary cutaneous melanoma. All of the melanomas occurred de novo and all metastasized; one child died. In no instance was melanoma a clinical consideration, and in none did the histopathologist who first “signed out” the case make a diagnosis of melanoma. Despite the inability of clinicians and pathologists to diagnose correctly, with repeatability, melanomas that develop in children yet to be pubescent, those neoplasms, nonetheless, are melanomas and, therefore, criteria employed currently for diagnosis of melanoma, especially clinically, must be refined in order that they be applicable equally to melanomas in pre- and postpubescents. The vaunted ABCDs (Asymmetry, Border irregular, Color variability, Diameter >6.0mm) surely do not work for melanomas that appear in children who are prepubescent. Additionally, melanomas that occur in these children have distinctly different architectural and cytopathological features from those that arise in postpubescents, often being confused as they are by conventional microscopy with a Spitz's nevus. As a rule, melanomas in prepubescent children grow much more rapidly then those in adults but, like them, have the capability to disseminate widely and cause death.

Authentic primary cutaneous melanomas, as proved by metastasis, do arise de novo in children despite assertions to the contrary (1–83). Befuddlement that has enveloped the matter of melanomas in children for nearly 100 years stems directly from three major factors: (1) confusion about the subject that began with Darier and Civatte (123) and was sustained by Pack, Spitz, and Allen and Spitz (2,3,5,7–10) (2) from the unrelenting efforts of Allen for more than 50 years later to rewrite history in such a way that he and Spitz would emerge heroic by virtue of having understood from the outset that what came to be designated Spitz's nevus truly was a nevus rather than the malignant melanoma that Spitz insisted repeatedly that it is, and, (3) in the 1940s, 50s, and 60s, lack of criteria that really work for diagnosis histopathologically of melanoma and of nevi of various kinds, among them that nevus called by Spitz, bewilderingly, “juvenile melanoma” (12,18,46). Regrettably, Spitz (7,9), Allen (8,18), and Allen and Spitz (12) never “fessed up” to the mistaken interpretation of Spitz in her original article (vida infra). In subterranean fashion, Allen, even as late as the 1990s, pretended that Spitz and he had gotten it right the whole time, having recognized from the very beginning the nevus for what it is (46).

For purposes of our study we elected to define “prepubescent” as 10 years of age or younger; our series includes only primary cutaneous melanomas that developed in children who appeared to be prepubescent and in whom metastasis ensued. A review of prepubescent melanoma that deals only with patients who fulfilled our criteria for prepubescence would not consist of even a single sentence, the reason being that, to the best of our knowledge, no such series has ever been published. Ours is the largest series reported on to date of de novo melanomas, proven by metastasis, which began in children who presumably were prepubescent. None of the melanomas in our series arose in association with a nevus of any kind, including a congenital one (84–114) in a fetus acquired in utero from a mother who herself had metastases of melanoma (115–119), in children with xeroderma pigmentosa (120), in conjunction with another malignant neoplasm in a child who was immunocompromised (121), or in a zone of previous irradiation (102). In sum and in short, despite reams written about the subject of “melanomas of childhood,” it is impossible for a serious, conscientious, discerning student of the subject to be anything but confounded. And that is precisely why we undertook this endeavor in regard to melanomas in prepubescent children, highly cognizant as we were of the failures of the past and determined that our work be elucidating and illuminating and, thereby, worthy.

© 2003 Lippincott Williams & Wilkins, Inc.

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