< Current issue
Dermatopathology: Practical & Conceptual October - December 2003
New Heights: “Atypical” Spitz’s Nevus, “Malignant” Spitz’s Nevus, and “Metastasizing” Spitz’s Nevus: A Critique in Historical Perspective of Three Concepts Flawed Fatally
Joan M. Mones, D.O.
A. Bernard Ackerman, M.D.
Confusion from the outset
How the concept and the term “Spitz’s nevus” came to be
How the concepts and the terms “atypical,”“malignant,” and “metastasizing” Spitz’s nevus/tumor came to be
How the concept and the term "Spitz's nevus" came to be
The problem in diagnosis histopathologically of what today is known as "Spitz's nevus" surfaced for the first time in print in an article that appeared in the
French Bulletin of the Society of Dermatology and Syphilology
for March 3, 1910.
In that piece, Darier and A. Civatte wrestled with the enigma of "Naevus ou naevo-carcinoma chez on nourisson?" and sought earnestly to resolve it. What follows is a summary, in their own words (translated from French into English) of how they attempted to accomplish that desideratum:
"The lesion [located on the nose of an 8-month-old] which appeared when the child was 6 months old, grew rapidly, supposedly enlarging every day until the time that it was seen by dermatologists who noted that it was dome-shaped, papillomatous, 1.0 cm in diameter, and raised 2.03.0 mm above the surface of the skin. For the examiners, the lesion was too red, too hard, and too rapid in growth to be deemed a nevus. In favor of the fact that the tumor is malignant we have the rapid growth and unprecedented development of it."
The findings in sections of tissue from a biopsy specimen taken of the "red, dome-shaped, papillomatous" lesion on the nose of the child were as befuddling to Darier and Civatte as were the changes met with by them clinically. They noted by conventional microscopy some attributes of a nevus and others of lymphosarcoma. In the opinion of those authors, the cells that made up the tumor seemed to originate from the epidermis, were fusiform, possessed of a large clear nucleus, infiltrated the entire dermis, and were disposed at that latter site both as solitary units and in small compact masses. Because the large cells looked to them like those of connective tissue, their differential diagnosis was fibrosarcoma, lymphosarcoma, and alveolar-cell sarcoma. In many sections of tissue, groups of cells positioned in the papillary dermis resembled nests of a nevus. This disparity, namely, findings in some sections like those of a nevus and in others like those of a sarcoma, left the authors baffled. They simply could not decide, with surety, whether the tumor in the child was benign or malignant. At this juncture in their article, Darier and Civatte referred to a lesion Kyrle had reported on in what was claimed to be "a recent work" (for which, however, no citation was provided), the changes histopathologically resembling very much those in the red papule on the nose of their 8-month-old child. Kyrle was said to have told of cells like those of lymphosacroma in what otherwise was a typical nevus. Although Kyrle did not concern himself with the matter of whether the lesion was benign or malignant, he implied it was benign by calling it a "simple nevus." By contrast, Darier and Civatte, after grappling with the issue of the fundamental character of the neoplasm borne by the 8-month-old, admitted frankly their own uncertainty about the true nature of it. Because so many of the constituent cells were "malignant looking," they were inclined to consider the neoplasm malignant. This is how they phrased what for them was a conundrum they could not resolve:
"In favor of the hypothesis that the tumor is malignant we have the rapid growth and the impressive development of it. However, the convincing arguments in favor of malignancy, namely, spread to lymph nodes or metastases to the skin or even simple pigmentation, are lacking. We have all of the fears about malignancy, but no certainty of it."
In sum and in short, Darier and Civatte, soon after Kyrle, wrote of a proliferation of abnormal melanocytes, many of the cells of which were said to look "malignant," but that, nonetheless, behaved in benign fashion. Although they were unsure about whether the "tumor" was benign or malignant, as they acknowledged directly in the title of their piece ("Nevus or nevo-carcoma in a newborn?"), they favored malignancy on the basis mostly of cytopathologic attributes ("malignant-looking cells") of it.
Almost 20 years after the publication of Darier and Civatte, that is, in 1939, Pack and Anglem, both surgeons at Memorial Hospital in New York City, addressed the issue of "Tumors of the soft somatic tissues in infancy and childhood."
In it, they confronted the same dilemma as had Darier and Civatte, that is, a diagnosis of melanoma by competent histopathologists yet behavior biologically of those neoplasms in children and in young adults in a wholly benign manner. This is how the surgeons/coworkers assessed the problem:
"Although malignant melanomas are found in infancy and childhood, they are of low-grade malignancy and seldom metastasize. Under the microscope these tumors may be indistinguishable from other melanomas occurring in adult life . . . yet their behavior is not in keeping with their morphological structure."
Pack and Anglem neither provided a description of findings histopathologically nor presented photomicrographs, and, furthermore, they gave no indication of understanding the reality that all but one of the children with reputed melanoma almost certainly had a nevus of the type that now is named for Spitz. In that one exception, the diagnosis was not at all in doubt: the child died from the effects of metastases of melanoma. Despite this limitation, Pack and Anglem were sufficiently prescient to advise that surgical treatment for melanomas in children should be different from that in adults. This is what they proposed in that regard:
"The malignant melanoma is removed by very wide surgical dissection, carrying the dissection below the deep fascia. In adults this procedure is routinely followed in six weeks' time by meticulous dissection of the regional lymph nodes draining the skin which is the site of the melanoma. . . . In infants and children, however, the routine dissection of regional lymph nodes is not done, because the tendency of melanomas in infancy and childhood to metastasize is latent."
Pack, in an editorial in 1948 given to the subject of "Pre-pubertal melanoma of skin,"
shared his thoughts about a puzzling circumstance that continued to trouble his colleague in pathology at Memorial Hospital, namely, Sophie Spitz (and also her husband, Arthur Allen, a pathologist stationed there, too), who, in the very same year engaged, in print, the very subject as had Pack. The surgeon, Pack, declared that what he called pre-pubertal melanoma (and Spitz soon was to designate "melanoma of childhood" and "juvenile melanoma") was not a malignant melanoma at all, but a nevus that often had attributes in common with melanoma histopathologically. Although Pack and Spitz must have known of the existence of one another at Memorial Hospital, both of them being interested inordinately in the subject of melanomas in young persons, neither of them gave any clue to that in their writings or any indication that even once they looked together at sections of tissue or that ever did they discuss with one another the particular matter of those peculiar proliferations of melanocytes in young persons. It is even questionable whether sections of tissue from any of the neoplasms removed surgically by Pack ever were seen by Spitz; the person at Memorial who served as Pack's personal pathologist was the head of the department of pathology there, Fred Stewart (personal communication, Leo Koss, M.D.). Although nowhere did he state, without equivocation, that "pre-pubertal melanomas" were benign, Pack, in an editorial in 1948, certainly implied that they were nevi.
This is some of what he said then about "pre-pubertal melanoma":
"None of these melanotic tumors of infancy and childhood has metastasized to regional lymph nodes, although many of them have been labeled as malignant melanoma by extremely competent pathologists . . . We might well adopt such term as prepubertal melanoma to indicate the group of tumors which resemble malignant melanoma histologically but do not behave as such."
Complicating the issue even further was an article by Spitz published in an issue of the
American Journal of Pathology
for 1948 under the title, "Melanomas of childhood."
) From a large group of children who had been diagnosed previously with malignant melanoma and catalogued as such in the files of the Memorial Hospital, Spitz identified 13 children, ranging in age from 18 months to 12 years, who had neoplasms that she considered to be authentic malignant melanomas. (
) This is how she expressed that conviction:
"However, there remained a group of cases in which a diagnosis of malignant melanoma seemed histologically sound. Over a period of years, the qualification has been added to reports of such lesions that they probably would not behave as malignant tumors. In order to distinguish these lesions both from the malignant melanoma of adults and the unequivocally benign nevus of childhood, the term 'juvenile melanoma' has been adopted. The term 'melanoma' in this paper, as in common usage, has been applied only as an abbreviation for malignant melanoma."
Fig. 2 The journal in which Spitz's seminal article appeared in 1948.
Fig. 3 Opening page of Spitz's seminal article in 1948.
From the title of her article, "Melanomas of childhood," and, as the preceding quotation reveals compellingly, it is evident that Spitz used the term "juvenile melanoma" as a synonym for malignant melanoma in children. This is what Spitz said she found in her analysis of the 13 childhood melanomas she culled from the archives of the Memorial Hospital:
"In the group of childhood melanomas (juvenile melanomas) there were 5 males and 8 females. . . All but one of the children are alive and have shown no evidence of recurrence either locally or in drainage sites. . . One of the 13 cases had been clinically malignant and the child is dead. . . This lesion occurred on the sole of the foot but was not described as involving the skin. After rapid growth over a period of 6 weeks, a soft white tumor, 2 cm in diameter, was resected from the plantar fascia. One month after the initial excision there was a bulky local recurrence, thrombosis of the femoral vein, and metastasis to inguinal lymph nodes. Within 4 months the child was dead of generalized metastases."
The "juvenile melanomas" Spitz depicted clinically in photographs were benign by criteria employed routinely in dermatology, being as they were relatively symmetrical, very well circumscribed, and seemingly uniform in color (
). Her photomicrographs, all "shot" at high magnification, showed nests in the epidermis positioned entirely at the dermoepidermal junction, maturation of melanocytes with progressive descent, and nuclei of melanocytes that were rather monomorphous (
), findings indicative of benignancy. Some nuclei of melanocytes, like those pictured in the photomicrograph on the upper right of our
, were large and housed a prominent nucleus, and other nuclei, in addition to being large, were pleomorphic. In brief, it was the cytopathologic attributes of "atypia" that compelled Spitz to the conclusion that "juvenile melanoma" truly was malignant melanoma.
Fig. 4 These four photographs (Spitz's figs.14) that appear in the seminal article of Spitz in 1948 show findings stereotypical of a nevus, namely, symmetry, sharp circumscription, smooth contour, and uniform pigmentation. Had Spitz been a dermatologist, rather than a general pathologist, she would have been able to integrate the features grossly with the findings histopathologically and avoided, thereby, the error of considering the proliferation of abnormal melanocytes to be that of a malignant melanoma.
Fig. 5 These two photomicrographs (Spitz's figs. 7 and 8) permit inference about why Spitz, in 1948, concluded, erroneously, that "melanomas of childhood" were malignant melanomas. They were "shot" by her at high magnification, thereby forcing a histopathologist to focus on the striking nuclear atypia of neoplastic melanocytes, a finding that Spitz equated with malignancy. This particular type of nevus, like all other kinds of nevi, is differentiated from melanoma best by assessing cytopathologic features in conjunction with the silhouette of the neoplasm in its entirety as that outline is assessed at scanning magnification.
In an attempt to reconcile the discrepancy in behavior biologically, to wit, 12 children alive and well and one child dead from metastases, with appearance histopathologically, i.e., melanoma, Spitz undertook to compare her 13 cases with (1) 50 "benign nevi"; (2) 17 malignant melanomas in adolescents or "young adults," their ages ranging from 14 to 19 years; and (3) one neoplasm removed from a 4-year-old girl and provided by Dr. Bjarne Pearson of the department of pathology of the University of Vermont, that neoplasm being malignant melanoma both clinically and histopathologically. Comparison of "juvenile melanomas" with nevi prompted Spitz to this conclusion:
"There are, then, definite cellular features of distinction between the juvenile melanoma and the ordinary benign nevus of children: (1) The pleomorphic structure of the juvenile melanoma is in contrast to the monotonous structure of the benign nevus of children; (2) In juvenile melanoma there are bizarre mononuclear or multinuclear giant cells totally unlike those formed by fused nuclei in the benign nevus; (3) The junctional change so prominent in the benign nevus is comprised of cells which are uniform, small, and closely packed whereas in the juvenile melanoma these cells are pleomorphic, larger, and form looser projections in the dermis; (4) Mitotic figures, occasionally seen in the juvenile melanoma, are rare in the ordinary nevus."
Comparison of "juvenile melanomas" with malignant melanomas in patients 14 to 17 years of age led Spitz to these observations and inferences:
"The lesions in several of the fatal cases of young adults were extremely superficial and some had a qualitative histologic appearance far less malignant than many of the nonfatal juvenile melanomas . . . In general it was concluded that differentiation histologically between the juvenile and adult melanomas could not be made with certainty in most cases. The one feature, found in almost one-half the cases of juvenile melanoma, that seemed to permit histologic distinction from adult melanoma, was the presence of giant cells. In view of the survival of patients having this type of tumor, these have been regarded as an indication that the lesion is benign. This is so despite the fact that, except for the giant cells, such lesions have all the histologic criteria for the diagnosis of malignant melanoma."
The declaration of Spitz that on histopathologic grounds melanomas in young adults were "far less malignant than many of the nonfatal 'juvenile melanomas'" is evidence additionally that she considered "juvenile melanomas" to be malignant melanomas. The neoplasm on the base of the 4-year-old patient of Dr. Pearson, that Spitz thought was a "juvenile melanoma," later metastasized to inguinal lymph nodes bilaterally. This is how Spitz sought to integrate that particular happening and to synthesize the matter of metastasis with the usual benign behavior of "juvenile melanoma:"
"The cells of the primary lesion [of Dr. Pearson's 4-year-old patient] in this instance contained large, irregular, hyperchromatic nuclei with prominent vacuoles and acidophilic inclusions which would justify the diagnosis of malignant melanoma, regardless of the age. Bilaterally, the inguinal nodes showed a few clusters of metastatic cells in the peripheral sinuses. Although the follow-up in this child has been only for a period of 6 months and it is not possible to predict the outcome, it has been noted that generalized dissemination of the tumor has become evident in the fatal cases of young adults and in the one fatal case among the children within a very short time after the diagnosis has been made. It seems possible, however, that in some cases metastases to regional lymph nodes in children need not always indicate a fatal termination. This peculiarity of melanomas in children would seem to be indicated by the case included in the report of Webster, Stevenson, and Stout; this child, 8 years old, after local excision of a black lesion on the shoulder and subsequent metastases both to skin and cervical nodes which were resected, had survived at least 12 years without further recurrence."
As can be told from these lines of Spitz, she was perplexed thoroughly and groped valiantly for an explanation for why her own patient died so soon of metastatic disease, whereas the patient of Dr. Pearson was still alive, albeit with follow-up of only 6 months, and why the child reported on by Webster
had survived for 12 years when, in her judgment, all three neoplasms were "juvenile melanomas." In an effort to resolve what seemed to be unresolvable contradictions, Spitz ventured that "in some cases metastases to regional lymph nodes in children need not always indicate a fatal termination," a notion that would take on a life of its own in years to come, especially at the Armed Forces Institute of Pathology over the course of three decades, first by Helwig, then by K. Smith
and, most recently, by Lupton. Spitz surmised that it was the fact that sex hormones had yet begun to flow at puberty which was responsible for melanoma in pre-pubescents behaving better than did melanoma in post-pubescents. (
) This is how she expressed that idea:
"Inasmuch as there is a lack of constant morphologic evidence with which to explain the usually benign clinical behavior of histologically malignant melanoma occurring in childhood, an explanation based on sex-linked hormonal control would seem logically feasible . . . There is some evidence that even though metastases may occur in childhood, an inhibitory factor may exist before puberty to hinder either further dissemination or reception of metastatic cells by the viscera."
In retrospect, it is obvious that Spitz was comparing apples and oranges in a vain attempt to make sense of the curious, unpredictable behavior of what she designated juvenile melanoma. The 12 neoplasms that behaved in a benign fashion biologically in Spitz's original series all were nevi of the kind that today is known eponymically for Spitz. The neoplasm in the original series that metastasized and the neoplasms in the patients of Pearson and of Webster
were authentic malignant melanomas. Also evident from a careful reading of the section of the article given to "Summary and conclusions" by Spitz in her work about "Melanomas of childhood" is the belief that "juvenile melanomas" were malignant melanomas whose authentic behavior became manifest at puberty consequent to a surge of hormones. But that Spitz somehow was ambivalent about the true nature of "juvenile melanoma" can be intuited from her recommendation that it should be excised more conservatively than melanomas in adults. The uncertainty about the essential character of "juvenile melanoma," as Spitz telegraphed in her writing about it, was extraordinarily contagious and the effects of it are felt to this day. Listen to the words of Spitz more than half a century ago:
"Summary and Conclusions. Of 13 cases of juvenile melanoma in this series, only one (7.7 per cent) has had a clinically malignant and fatal course despite the similarity of the juvenile lesions to the malignant melanoma of adults. The juvenile melanoma may be distinguished histologically from adult melanoma in about one-half the cases by the presence of giant cells in the former which seldom occur in the latter. There is a precipitous rise in the capacity of melanomas to metastasize after puberty despite the histologic similarity to the usually non-metastasizing juvenile melanoma. The possible influence of sex-linked hormonal activation of the growth capacity of melanomas at the age of puberty seems a logical conclusion. Accordingly, since metastases from juvenile melanomas occur only rarely, conservative surgery, rather than the radical surgery usually indicated for adult melanomas, seems justified."
Just one year later, in 1949, Spitz's husband, Allen, undertook to correct the notion that "juvenile melanomas," as both Spitz and he, himself, referred to them (
), were doubtlessly malignant, and he did that in these words that convey the first hint of retreat:
"Juvenile melanoma. There is a group of lesions occurring before puberty, which, despite their having the histologic characteristics of melanomas, do not behave as malignant lesions. These lesions are therefore qualified by the term juvenile melanomas."
Fig. 6 These two photomicrographs (Allen's figs. 21 and 22) that appeared in Allen's article of 1949 and diagnosed by him as "juvenile melanomas" show findings of a nevus that today is named eponymically for Allen's wife, Sophie Spitz.
In addition to that statement, Allen included "juvenile melanomas" among different types of "nevi," i.e., along with "junctional nevus," "compound nevus," "intradermal nevus," and "blue nevus" (that grouping also being illogical; the first three refer only to where nests of melanocytes are situated in sections of tissue, whereas the fourth identifies a particular type of proliferation of melanocytes that because it is unaffiliated with discrete nests or fascicles of melanocytes does not really qualify as a melanocytic nevus). To muddle matters further, Allen had this to say about the word "melanoma" as he chose to use it: "The unqualified term
has come to be used interchangeably with malignant melanoma and will be so employed in this report." Spitz, the year before, had utilized "melanoma," as in the diagnosis "juvenile melanoma," as "an abbreviation for malignant melanoma." Now Allen, the closest coworker of his wife, Spitz, was advocating just the reverse, that is, "juvenile melanoma" not being a malignant melanoma, but being benign and, by implication, a nevus. And to complicate matters still further, Allen, in 1949, continued to insist that "juvenile melanoma" could metastasize, if only episodically. This is how he put it:
"Notwithstanding the grave outlook which such a histological picture in an adult would portend, it is a remarkable fact that prior to puberty these lesions do not metastasize except in the rarest instances. Hence, they merit conservative surgical therapy. In a series of thirteen cases only one fatality was recorded and this in a case of a child at the borderline age of 12 years; metastasis did not occur in the remainder. Apparently even in the exceptional instance in which metastases occur prior to puberty, the ultimate course may differ from that expected in the adultwitness the case of the 8-year-old child with metastases to the skin and cervical lymph nodes, who survived at least twelve years without apparent additional metastases."
It is a bit shocking that in the late 1940s, general pathologists at the Memorial Hospital in New York City, one of the premier centers for the diagnosis and treatment of cancer in the world, would assert that a nevus could metastasize. Rather than "fessing up" to the reality that all of the melanocytic neoplasms that metastasized were authentic malignant melanomas, Allen promulgated stridently the erroneous notion that "juvenile melanomas" could, and did, metastasize. He also continued to advance the notion of Spitz that capability of "juvenile melanoma" to metastasize depended on hormonal factors linked to puberty. The reverberations of the failure of Spitz and Allen to adhere to time-honored principles of pathology, in particular, criteria for malignancy (capability of a neoplasm to kill by destruction of tissue locally or by the effects of metastases) are being felt today in the form of mystical concepts such as "atypical Spitz's nevus," "malignant Spitz's nevus," and "metastasizing Spitz's nevus."
Curiously, Spitz herself, the author, in 1948, of "Melanomas of childhood," was conspicuously absent from authorship of the article in 1949 by her husband (with whom she collaborated so devotedly that she produced all of the photomicrographs that graced his tome devoted to
a work that Allen dedicated to her) (
), and she, despite murmurings to the contrary by Allen, continued to regard her 13 cases as malignant melanomas even though she avowed repeatedly that the behavior of them was benign. In fact, in 1951, in an article that appeared in the
Journal of the American Medical Women's Association
under the title, "Cutaneous tumors of childhood: Disparity between clinical behavior and histologic appearance,"
Spitz implied, once again, that melanomas in children were malignant. In that article, under the caption, "Nevi and melanomas" appear three headings, i.e., "Nevi in childhood," "Melanomas in childhood," and "Blue nevi," a clear distinction having been made there by her between nevi and melanomas in childhood (juvenile melanomas). This is some of what Spitz wrote in 1951 about "Melanomas in childhood":
"Occasionally, however, there occur in children pigmented lesions morphologically practically identical with the malignant melanomas of adults . . . In the records of Memorial Hospital, 13 of these 'juvenile melanomas' have been found and in only one from the sole of the foot in a female twelve years was the outcome fatal. Other cases too have been reported in children, in which there were recurrences and even metastases with survival, nonetheless, for many years . . . An explanation of this remarkable disparity between the histologic appearance and the clinical course of melanomas in children has been postulated on the basis of hormonal control of melanomas. There must exist during childhood a hormonal inhibition to the fatal spread of melanomas."
Fig. 7 Arthur Allen on the left and Sophie Spitz on the right at a "mint julep party" in 1950 at the Memorial Hospital in New York City. Only that duo is seen to be connected to one another spiritually in this photograph of a group of 12 members of the same department.
The photograph of the lesion in her Fig. 13 was the same one she presented as Fig. 4 in her original article about "Melanomas of childhood." The photomicrograph in her Fig. 14 (our
) had not been published before, but it, like the photomicrographs Spitz showed in her seminal article of 1948, was taken at a high magnification and exhibited findings of a nevus she diagnosed wrongly as malignant melanoma. The legend accompanying that photomicrograph reveals that by 1951 Spitz still seemed to think that "juvenile melanoma" was malignant melanoma. Because she was so ambivalent about the matter, she avoided, once again, confronting it head on. This is how her legend to Fig. 14 reads: "Juvenile melanoma. Note large neoplastic cells in all layers of the epidermis and invasion of dermis. Contrary to benign behavior in children, this structure in an adult would indicate a grave prognosis."
Fig. 8 Photomicrograph of a "juvenile melanoma" shown by Spitz in 1951. The legend indicates that as of that date she continued to believe that "juvenile melanomas" were malignant melanomas that behaved differently in children than in adults.
It was in 1953, just three years before her death on August 11, 1956, that Spitz, at last, stated clearly and unequivocally that "juvenile melanoma" was benign. She did that in an article, co-authored with Allen, about "Malignant melanoma: A clinicopathlogical analysis of the criteria for diagnosis and prognosis."
The coworkers conveyed the idea of benignancy of "juvenile melanoma" in five ways thus:
(1)"That there are special histological features of the benign juvenile melanomas from which it is possible to identify most of these lesions for what they are . . . We classify the lesions [of melanocytes] as follows:
1. Intradermal nevus (common mole)
2. Junctional nevus
3. Compound nevus
4. Juvenile melanoma
5. Blue (Jadassohn-Tièche) nevus
1. Melanocarcinoma (from junctional nevus or compound nevus)
2. Malignant blue nevus."
(3) In a section titled "Juvenile melanomas," under the heading, "Histological features of juvenile melanomas," Allen and Spitz wrote this:
"'Juvenile melanoma' is the term applied to those lesions occurring for the most part, BUT BY NO MEANS EXCLUSIVELY, prior to puberty. They are benign in behavior, but, as indicated, have the histological appearance, either indistinguishable only with the keenest evaluation, from those of adults. The percentage of juvenile melanomas that can be segregated by any individual pathologist from the adult melanocarcinomas on histologic grounds alone manifestly increases with increments of experience . . . On the basis of the histological criteria just outlined, it is possible, in about two thirds of the cases, to learn to differentiate the benign juvenile melanomas from the adult melanocarcinomas; in the remainder, this histological differentiation is not possible without knowledge of the age of the patient. However, if the prepubertal age of the child is made available to the pathologist, there then can be very little doubt that the lesion is benign if the exceptional patterns outlined in the following section are borne in mind."
(4) In the section captioned, "Postpubertal juvenile melanomas," Allen and Spitz advanced the notion of juvenile melanoma having capability to "convert" or "transform" into authentic malignant melanoma (
), while at the same time they persisted in maintaining that juvenile melanoma, in some instances, could not be distinguished histopathologically from true melanoma. This is how they phrased it:
" . . . in a small percentage of melanocarcinomas (5.9 per cent, or twenty-one of 362), there is noted residual evidence of a preexisting juvenile melanoma. Therefore, the second assumption that appears warranted is that some of the juvenile melanomas—apparently only a small proportion—become converted in later life into melanocarcinomas."
Fig. 9 Photomicrograph from an article of Allen and Spitz in 1953 showing what they claim is a melanocarcinoma but that almost certainly is a Spitz's nevus.
(5) In the section of their article dedicated to a "Basis for clinical-histological disparity of juvenile melanomas," Allen and Spitz said this:
"Despite the fact that most juvenile melanomas may be diagnosed on histologic features alone, it remains indisputable, as stated, that others of them, although benign, are utterly indistinguishable by histology alone from the unusual type of small adult malignant melanoma . . . In the rare examples of fatal, metastasizing melanocarcinomas of children, the question arises as to whether or not their endocrine status had in some ways been altered toward the adult pattern so as to provide tissue receptivity for the growth and metastasis of the neoplasm."
From perusal of quotations taken
from publications of Spitz in 1948, 1951, and 1953, and from Allen in 1949, it can be concluded that they, coworker spouses, were uncertain by virtue of ambivalence about the authentic character of "juvenile melanoma." After having first stated, without equivocation, that juvenile melanoma was malignant melanoma on the basis of findings histopathologically and of metastasis of one neoplasm biologically, Spitz, and, later, with Allen, was to aver that the "clinical course" of "juvenile melanoma" was different from that of malignant melanoma, the former behaving only rarely in malignant manner, and that "juvenile melanoma" had potential to "convert" into malignant melanoma, at the same time Allen, and Allen with Spitz, maintaining that although "juvenile melanoma" usually was "benign," it was "utterly indistinguishable" by conventional microscopy from malignant melanoma. Allen and Spitz showed an example in their Fig. 33 (our
) of a neoplasm that they considered to be a "melanocarcinoma" with residual "juvenile melanoma," but it was taken at too high magnification to convey accurately the silhouette and, thereby, a sense for what was the correct diagnosis. In short, Spitz and Allen were no more certain of the actual nature of the neoplasm under consideration here than were Darier and A. Civatte, the consequences of their confusion and their waffling repeatedly about the essential character of "juvenile melanoma" being very much an influence on the thinking about the subject by pathologists and dermatologists today. In retrospect, it is apparent that Spitz and Allen, being the general pathologists they were, were not conversant at all with rudiments of clinical dermatology; had they been, one glance at the "juvenile melanomas" as they presented themselves clinically would have convinced them that those lesions were nothing other than nevi.
In 1960, Kernan and Ackerman recommended that juvenile melanoma be renamed "spindle cell and epithelioid cell nevus," those two general pathologists reasoning that that neoplasm also "may occur in adults, and the term 'melanoma' may have a malignant connotation to the physician."
In 1961, Duperrat and Mascaro, dermatologists both, suggested the appellation "tumor de Spitz."
Five years later, in 1966, Kopf and Andrade, a dermatologist and a dermatologist/dermatopathologist, proposed the term "benign juvenile melanoma" because, to them, that appellation, "immediately indicates its clinical behavior and yet retains the original title of Spitz."
By employing the diagnosis benign juvenile melanoma, Kopf and Andrade hoped to "circumvent some of the confusion in which so-called juvenile melanomas have been reported to give rise to metastases." They went on to declare, correctly, that "such lesions undoubtedly are malignant melanomas and never should have been reported under this title."
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