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Spitzoid Tumors: A Matter of Perspective?

2004; Lippincott Williams & Wilkins; Volume: 26; Issue: 1 Linguagem: Inglês

10.1097/00000372-200402000-00001

1533-0311

Lorenzo Cerroni,

Melanoma and MAPK Pathways

Imagine you are a dermatopathologist from another planet. You are careful, scrupulous, honest, and willing to learn from your mistakes. Say that on one special day, you get 100 specimens like the one depicted in Figure 1. You correctly recognize that they all represent tumors deriving from melanocytes and sign them out as benign melanocytomas (terminology, on your planet, is different, and there is only 1 term for all benign melanocytic nevi, but you still don't know how lucky this situation is). Say that after 5 years, you want to check whether your diagnoses were correct and call patients for follow-up information. You obtain this information for all of your patients (in this aspect, too, your planet is a remarkable one) and learn that 99 of them are alive and well, but the last one died of metastatic malignant melanocytoma a few months earlier. In an attempt to understand why this patient died of malignant disease (that is, to identify your mistake) and to find criteria that may allow you to distinguish the tumor of this unfortunate patient from those of the 99 showing a benign course, you review all 100 histopathologic specimens very carefully, but fail to find criteria for differentiating them: they all look alike. This failure is the cause of considerable dismay. After a while, being unable to clearly differentiate the 99 benign lesions from the 1 malignant tumor, you start to use a terminology different from the simple (and repeatable) one that you had used in the past. The articles of your planet's medical journals now begin to be infested by terms such as “minimal deviation malignant melanocytoma,” “melanocytic intraepidermal neoplasia,” “benign melanocytoma with architectural disorder,” and so forth.FIGURE 1.: (A) Malignant melanoma with asymmetric distribution of intraepidermal melanocytes and of inflammatory infiltrate in the papillary dermis. (B) Intraepidermal features of melanoma with solitary melanocytes with pleomorphic nuclei, some located in the granular and horny layers, predominating over nests. Note focal involvement of the papillary dermis.Say that after a long discussion in your medical community, you decide to turn to expert advice from another planet. You find out that in a distant solar system, there is a small, insignificant planet called Earth (although the inhabitants believe that they are very, very special; you laugh mildly, but this is another story. . .), in which benign and malignant melanocytic lesions are quite frequent. After inquiring about the best dermatopathologist to whom you should refer for advice, using your quantum teletransportation device, you safely bring that person to your planet and submit your problem to her (you happened to select a woman, unaware of the psychological implications on the ego of many male dermatopathologists on Earth, but this, again, is another story). All dermatopathologists on your planet are very excited while waiting for the interpretation of the terrestrial colleague, and when you finish translating her comment, there is a sudden silence filled with astonishment and surprise. In fact, it seems that you have missed all 100 diagnoses, not just 1, as you and your colleagues originally thought, as these lesions are all malignant melanocytomas. Say that your planet is inhabited by a species able of rational thinking, which means that not only a few, but all members of your community are capable of it (this is yet another remarkable difference between your and our planet). After a short period of dismay, frustration, and depression, you and your colleagues agree that the extraterrestrial dermatopathologist was right (unlike many of your extraterrestrial colleagues, you are able to agree that somebody else was right) and that you were simply looking at the problem from the wrong perspective. Trying to find criteria able to differentiate the single malignant from the 99 benign cases, you failed to recognize that they were all the same, that is, all malignant. In short, you confused diagnosis with prognosis, forgetting that a diagnosis of a malignant tumor does not necessarily mean that a given patient will die of that malignant neoplasm. Say, now, that on another day you get 100 specimens like that depicted in Figure 2. Once again, you correctly recognize them as melanocytic tumors, and sign out all of them as benign melanocytomas. Five years later, you look again at follow-up data and find out that 90 of your patients are fine, but that 10 had metastatic disease. Curiously enough, in spite of lymph node metastases, many of them didn't die and are still alive, but the fact remains that they had metastatic disease. The debate on how to distinguish the benign from the malignant lesions in your medical community starts again, and once more, terms like “borderline malignant melanocytoma,” “atypical benign melanocytoma,” and “malignant benign melanocytoma” (this last term, you must admit, is beyond your comprehension, but there seems to be no limit to the fantasy of your colleagues) infest your medical literature. One day, however, all of a sudden you remember the old story, and at the next meeting of your association (there is only 1 association of dermatopathologists on your planet, and only 1 meeting every year, but oddly enough, you and your colleagues seem to be happy with this state of affairs), you suggest that this may be again a problem seen from the wrong perspective, and that, perhaps, all these tumors are malignant... Did you get the story?FIGURE 2.: (A) “Spitzoid” tumor showing a polypoid nodule of melanocytes. (B) There is almost no junctional component and no involvement of the upper epidermal layers. (C) Sheets of atypical melanocytes could be observed in the dermis. This case has been published in the American Journal of Dermatopathology 23:237–241, 2001.The pathologist from another planet, of course, does not exist—neither does, unfortunately, the other planet, as far as I know. This story is clearly a provocation, and is not meant to change your concept of Spitz nevi, atypical Spitz tumors, malignant Spitz nevi, and spitzoid melanomas (if you happen to have a concept of it; if not, perusing through the literature will likely make you feel sick and in search of a job other than dermatopathology). My only purpose here was to look at the problem from a different perspective. Indeed, there are lesions that represent stereotypical examples of Spitz nevi and that are always benign. Criteria for diagnosis of so-called “spitzoid” tumors and for their differentiation from malignant melanoma, however, are still lacking, and we face the same problem every time one such specimen comes under our microscope. Clearly, this field of dermatopathology needs more than just a change of perspective, and perhaps only new techniques in the future will solve our differential diagnostic struggle. P.S.: In this article, I intentionally used the term “spitzoid.” Often, at dermatopathology meetings, those who use this terminology are told by other colleagues that this is an ugly deformation of Sophie Spitz's name and that the term “spitzoid” should never be used. The very same persons who make this objection, on the other hand, use without problems terms such as “pagetoid” (pagetoid reticulosis, pagetoid cells in pagetoid pattern, pagetoid spread, etc.), “bowenoid” (bowenoid papulosis), “kaposiform” (kaposiform hemangioendothelioma), “pacinian” (pacinian neuroma, pacinian corpuscle), “schwannoma,” and many others, all derived from the names of physicians related to the disease or the histopathologic features described by their name. And why shouldn't they use these terms? Similar ones are commonly used in many different fields of human life, and nobody ever objected: “Marxism”, “Newtonian,” and “Darwinian” are just a few examples. Is Sophie Spitz to be treated differently than John Templeton Bowen, Theodor Schwann, or Sir James Paget? Is the adjective “spitzoid” semantically worse than “pagetoid”? A “platonic” love is neither ugly nor offensive, nor does it disfigure the name of Plato. Therefore, let “spitzoid” be used just like all other adjectives derived from the names of famous persons: This innocent deformation of their names is the price they have to pay for their fame!