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Dermatopathology: Practical & Conceptual January - March 2002
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New Concept: Melanomas in Prepubescent Children: Review Comprehensively, Critique Historically, Criteria Diagnostically, and Course Biologically
Joan M. Mones, D.O.
A. Bernard Ackerman, M.D.
Abstract
Definition of “Prepubescent” and of Proven “Melanomas” for Purposes of This Treatise
Historical Perspective: Darier and A. Civatte, 1910
Historical Perspective: Coe, 1925; Pack and Anglem, 1939
Historical Perspective: Pack et al., 1947; L. Ackerman and del Regato, 1947
Historical Perspective: Pack, 1948; MacDonald, 1948
Historical Perspective: Spitz, 1948
Historical Perspective: Allen, 1949
Historical Perspective: Spitz, 1951; Pack and Scharnagel, 1951
Historical Perspective: Truax and Allen, 1953; Allen and Spitz, 1953
Historical Perspective: Becker, 1954; McWhorter and Woolner, 1954
Historical Perspective: McWhorter et al., 1954; Hendrix, 1954; Dobson, 1955
Historical Perspective: Allen, 1960
Historical Perspective: Hoagland and Hughes, 1960
Historical Perspective: Pontius and Dziabis, 1961; McGovern and Goulston, 1963
Historical Perspective: Giersten, 1964; Kopf and Andrade, 1966
Historical Perspective: Responses of Allen to Kopf and Andrade, 1966
Historical Perspective: Skov-Jensen et al., 1966; Zwaveling et al., 1966; Saksela and Rintala, 1968
Historical Perspective: Lerman et al., 1970
Historical Perspective: Trozak et al., 1975; Shanon et al., 1976
Historical Perspective: Helwig, 1975
Historical Perspective: Speculations of Helwig, 1975
Historical Perspective: Boddie, et al., 1978
Historical Perspective: Stomberg, 1979; Pratt et al., 1981
Historical Perspective: Flemming and Ruggins, 1985; Bader et al., 1985
Historical Perspective: Peters and Goellner, 1986
Historical Perspective: Moss and Briggs, 1986; Melnick et al., 1986; Chapman et al., 1987
Historical Perspective: Donner et al., 1988
Historical Perspective: Fisher et al., 1988
Historical Perspective: K. Smith et al., 1989: “Malignant Spitz’s Nevus”
Historical Perspective: Partoff et al., 1989; Roth et al., 1990
Historical Perspective: Allen, 1991
Historical Perspective: Temple et al., 1991
Historical Perspective: Crotty et al., 1992
Historical Perspective: A. H. Mehregan and D. A. Mehregan, 1993
Historical Perspective: Tate et al., 1993
Historical Perspective: Chun et al., 1993; Bartoli et al., 1994; Nitta et al., 1995
Historical Perspective: Barnhill et al., 1995
Historical Perspective: Barnhill et al., 1995: “Metastasizing Spitz’s Tumor”
Historical Perspective: Barnhill et al., 1995: “Atypical Spitz Tumor”
Historical Perspective: Lartigau et al., 1995
Historical Perspective: Whiteman et al., 1995; Handfield-Jones and N. Smith, 1996
Historical Perspective: Spatz et al., 1996; Naasan et al., 1996
Historical Perspective: Scalzo et al., 1997; Eady, 1997; Crotty, 1997; Zhu et al., 1997
Historical Perspective: Wu and Lambert, 1997; Milton et al., 1997
Historical Perspective: Spatz and Avril, 1998
Historical Perspective: Barnhill, 1998; Spatz and Barnhill, 1999
Historical Perspective: Barnhill et al., 1999
Historical Perspective: Rapini, 1999
Historical Perspective: Strojan and Lamovec, 2000; Davis, 2000; Neville et al., 2000
Historical Perspective: Kogut et al., 2000; Patterson et al., 2000; Zuckerman et al., 2001
Historical Perspective: Conti et al., 2001; Fabrizi and Massi, 2001
Summary: Major Sources of Error in Interpretation
Summary: Major Errors of Pack, Spitz, and Allen
Summary: Major errors of Helwig, K. Smith, and Barnhill
Our Experience
Clinical Appearance
Histopathologic Findings (
Figure 18
)
Histopathologic Findings (
Figure 19
)
Histopathologic Findings (
Figure 20
)
Histopathologic Findings (
Figure 21
)
Histopathologic Findings (
Figure 22
)
Histopathologic Findings (
Figure 23
)
Histopathologic Findings (
Figure 24
)
Histopathologic Findings (
Figure 25
)
Differences Histopathologically Between Melanomas in Prepubescents and in Postpubescents: Scanning Magnification
Differences Histopathologically Between Melanomas in Prepubescents and Postpubescents: Higher Magnification
Histopathologic Differential Diagnosis
Biologic Behavior
Synthesis
Purpose of This Endeavor and the Essence of the Message
Conclusions
Addendum and Caveat
Postscript
Acknowledgements
References
SEE ALSO
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melanoma
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Historical Perspective: Pack, 1948; MacDonald, 1948
In an editorial given to the subject of "Pre-pubertal Melanoma of Skin,"
5
Pack, in 1948, shared his thoughts about a conundrum that continued to vex his colleague in pathology at Memorial Hospital, Sophie Spitz (and her husband, Arthur Allen), who wrote about the same subject that same year.
7
Pack averred what he called pre-pubertal melanoma (and Spitz was to designate "melanoma of childhood") was not a malignant melanoma at all, but a nevus* that often had features in common histopathologically with melanoma. Pack and Spitz doubtlessly knew of the existence of one another at Memorial Hospital and of the interest of each other in melanomas in young persons, but neither of them gave any indication that they ever looked together at sections of tissue that housed those proliferations of melanocytes or that they ever even discussed the subject. It is questionable whether sections of tissue from any of the neoplasms removed surgically by Pack were seen by Spitz; the person at Memorial who acted as Pack's personal pathologist was the head of the Department of Pathology there, namely, Fred Stewart (personal communication, Leo Koss, M.D.). Although nowhere in Pack's writings did he state, without equivocation, that "pre-pubertal melanomas" were benign, he surely referred to them as nevi in the editorial in 1948. This is some of what Pack wrote in that editorial about "pre-pubertal melanoma":
"There is one important type of pigmented nevus which bears such a close resemblance to malignant melanoma that it is not possible clinically [sic] to distinguish between the two. This nevus may be found in children from age of one to the time of puberty . . . When this tumor is excised in infancy or childhood and submitted to pathologists for diagnosis, they are frequently unable to state whether the neoplasm is benign or malignant, inasmuch as the histological criteria, exclusive of invasion and permeation of vessels, are practically identical with the malignant variety of melanoma as encountered in adults."
Even though "practically identical" was an overstatement, Pack, in the editorial, modified further his recommendations for surgical treatment of what he continued to refer to as melanomas in prepubescent children from
"very wide surgical dissection, carrying the dissection below the deep fascia,"
a procedure he advocated in 1939 for persons 16 years of age or younger, to a much more limited procedure. This is what he advised:
"A microscopic diagnosis of melanoma in a skin tumor removed during infancy and childhood does not call for an extension of radical surgery such as wide excision and skin grafting and dissection of regional lymph nodes provided the child has not entered the postpubertal stage."
Pack seemed to believe that a "prepubescent melanoma" had the potential to become malignant melanoma by virtue of the affects of hormones generated at puberty. The evidence for that inference comes from these remarks by him:
". . . It is our belief, therefore, that the malignant melanoma in its derivation from the pigmented nevus, is a tumor closely related to the endocrine system and markedly influenced by the activity of the endocrines, notably the gonads, the suprarenal cortex, and the hypophysis. In keeping with this opinion, we have often observed malignant melanomas to occur in adolescent girls and boys just after the age of puberty, and furthermore, we have commented on the extremely rapid growth and wide dissemination of these tumors at this early age . . ."
Also in 1948, MacDonald told of her experience with 349 patients whose melanoma was recorded in the registry of the State of Connecticut during the period of 1936 through 1946.
6
Six patients were younger than 15 years of age and three of them were younger than 10. One male child, 6 years of age, had a melanoma on a knee that metastasized. A 2-year-old and an 8-year-old, both girls, had melanomas on the left leg and right shoulder, respectively, and neither of those neoplasms showed evidence of having metastasized after follow-up periods of 8 and 4 years, respectively. The other three children, all older than 10 years, namely, 13, 14 and 13, all had metastases to regional lymph nodes, one of those children having succumbed to the disease. No description of histopathologic findings was provided and no photomicrographs were shown; in all instances in which metastases occurred, however, the diagnosis histopathologically was melanoma. In the two youngsters in whom there were no metastases, the possibility exists that the neoplasms really were a "juvenile melanoma."
*The word "nevus," unmodified, is synonymous with melanocytic nevus throughout this work.
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