Historical Perspective: Giersten, 1964; Kopf and Andrade, 1966

 
In 1964, Giersten22 recorded his experience with a 6-year-old boy who had a primary cutaneous melanoma that metastasized to regional lymph nodes (Fig. 5). This is what Giersten said and what he concluded:
 
"A malignant melanoma with metastases in a 6-year-old boy is reported. The tumor was situated on the anterior aspect of the right upper thigh, and metastases were found in the regional lymph nodes in the right groin. The tumor was composed of atypical nevus cells, epithelioid and spindle forms, probably derived from the epithelium. The histopathologic picture was semi-malignant only. The metastases were almost exclusively composed of extremely pleomorphic epithelioid cells. Because of the small number of mitoses the tumour was supposed to be of a low grade malignancy, with a fairly good prognosis. It is stressed that the pathologist will have to be careful in the evaluation of the biological potentialities of some of the pigmented tumours occurring in children."

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Fig. 5  Comment: On the basis of the findings in the photomicrographs in their figures 1 and 2, a histopathologist cannot make an unequivocal diagnosis of malignant melanoma, yet this was a melanoma in a child because it metastasized. The neoplasm pictured here is small, seemingly somewhat symmetrical, and made up of melanocytes whose nuclei appear to be small and monomorphous. Moreover, the changes in the epidermis are not definitive of melanoma in situ.
 
The statements of Giersten seem to imply the melanoma in this child somehow was related to a spindle and epithelioid cell nevus, among them reference to atypical "nevus cells" and to "epithelioid and spindle forms." Moreover, the neoplasm, which metastasized, was said to be "semi-malignant only" and to be "of a low grade malignancy." The finding of a "small number of mitoses" contributed to the impression that the child had "a fairly good prognosis." In fact, the reality of metastasis signified a grim prognosis for this child. The criteria employed by Giersten for diagnosis and prognosis of melanoma in a child were flawed seriously.
 
Kopf and Andrade, in an essay in 1966 titled, "Benign juvenile melanoma,"23 summarized their findings in 40 patients with that neoplasm and discussed, in detail, the differentiation of it histopathologically from malignant melanoma. They did that in these words:
 
"The features principally important in the differential diagnosis of malignant melanomas [from benign juvenile melanomas] are the marked cellular pleomorphism, bizarre nuclei, prominent nucleoli, giant cells, frequency and atypia of mitotic figures, epidermal disintegration by migration of the tumor cells leading to ulceration, inflammatory reaction and depth of invasion. . . . Malignant melanomas with a few features of juvenile melanoma represent a separate problem somewhat akin to malignant melanomas with 'junctional nevus' type of proliferation at the periphery or with nests of 'nevus cells' in the dermis. It has not been resolved whether these associations represent malignant tumors arising from benign tumor cells, whether the malignant and benign cells arise independently or whether such histologic interpretations are erroneous."
 
The criteria of Kopf and Andrade, claimed by them to be decisive for distinguishing benign juvenile melanoma from malignant melanoma histopathologically, simply do not work; each finding said to be characteristic of melanoma may be encountered in some benign juvenile melanomas. It is apparent that even by 1966, the nosologic status of benign juvenile melanoma had not been clarified definitively, including whether it was related in any way to development of melanoma.
 
Like Pack, Spitz, Allen, Allen and Spitz, and McGovern and Goulston before them, Kopf and Andrade made this declaration:
 
"True malignant melanomas in children, although exceptionally rare, show histologic features very similar to the adult type and have the same prognosis."