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"Atypical" blue nevus, "malignant" blue nevus, and "metastasizing" blue nevus: a critique in historical perspective of three concepts flawed fatally.

The same errors that spawned, sustained, and continue to spur the notions of "atypical" Spitz's nevus, "malignant" Spitz's nevus, and "metastasizing" Spitz's nevus are animating of 3 other concepts flawed equally, namely, those of "atypical blue nevus," "malignant blue nevus," and "metastasizing blue nevus." Our intention here is to compel to the conclusion, by way of critique in historical perspective, that all neoplasms claimed to be "malignant blue nevus" and "metastasizing blue nevus;" in fact, are melanomas, that all "atypical blue nevi" are either a nevus or a melanoma, and that the trio of curious designations that serve as title of this work are mere evasions transparently from a diagnosis, straightforwardly, of 1 of only 3 possibilities, to wit, "blue nevus," melanoma, or melanoma in association with a "blue nevus." Rather than admit uncertainty forthrightly, those who employ circumlocutions that we deplore, such as those under scrutiny here, resort to linguistic maneuvers that, at first blush, seem to have the cachet of scholarship (the jargon used being in keeping with a slew of other well-accepted, but equally bogus diagnoses in [dermato]pathology, among those being "minimal deviation melanoma," "borderline melanoma," "nevoid melanoma," "potentially low-grade melanocytic neoplasm," and "melanocytic proliferation of uncertain biologic potential"). All those terms and phrases are constructed in a manner designed to make them appear to convey unbridled confidence on the part of a histopathologist, rather than what they are in actuality, that is, a cover abjectly for tentativeness. Scrutiny of the lingo, in very abbreviated form, just catalogued reveals it to be devoid of content utterly. For example, "malignant blue nevus" and "metastasizing blue nevus" not only are contradictions in terms, but they are outrageous violations of principles fundamental to classic Virchowian pathology. We seek here to debunk that claptrap in the same manner we did "atypical Spitz's nevus," "malignant Spitz's nevus," and "metastasizing Spitz's nevus." It is our hope that readers will consider our arguments worthy because they are logical, will find them convincing because they are irrefutable, and will incorporate the lessons communicated through them so that their own professional life, pathology as a discipline, and, ultimately, patients, are the beneficiaries.

Adult↗

"Atypical" Spitz's nevus, "malignant" Spitz's nevus, and "metastasizing" Spitz's nevus: a critique in historical perspective of three concepts flawed fatally.

Our purpose in undertaking this Arbeit was to review all articles published about "atypical" Spitz's nevus, "malignant" Spitz's nevus, and "metastasizing" Spitz's nevus, to criticize them in a fashion that illuminates, and to come to conclusions compellingly about those subjects. We found that an overwhelming majority of neoplasms that claimed to be "atypical Spitz's nevus," "metastasizing Spitz's nevus," and "malignant Spitz's nevus" were, in fact, melanomas ( Table 1). Moreover, in our estimation, those designations, and variants of them, like "atypical Spitz's lesion," "atypical dermal melanocytic lesion with features of Spitz's nevus," "atypical Spitzoid melanocytic neoplasm," and "problematic Spitzoid melanocytic lesion," are mere evasions from a diagnosis, straightforwardly, of either Spitz's nevus or melanoma. Diagnoses in pathology equally bogus are "minimal deviation melanoma," "borderline melanoma," "nevoid melanoma," "potentially low-grade melanocytic neoplasm," and "melanocytic lesion of uncertain biologic potential." Rather than admit uncertainty forthrightly, those who employ circumlocutions like those just mentioned resort to linguistic maneuvers that, at first blush, seem to be "academic" and constructed in such a way as to appear to convey confidence, rather than tentativeness, on the part of a histopathologist. On further scrutiny, however, each of those cliches is revealed to be devoid of content. For example, "malignant" Spitz's nevus and "metastasizing" Spitz's nevus not only are contradictions in terms, but they are outrageous violations of fundamental principles of classic Virchowian pathology, and "atypical" Spitz's nevus not only is a redundancy because the neoplasm was so atypical to Spitz, herself, she insisted (from the time she spawned the idea in 194 through 1951 it was a "malignant melanoma," but is abject intellectually, those who invoke it never setting forth, in clear-cut fashion, criteria for what constitutes a "typical" Spitz's nevus in contradistinction to an "atypical" one.

Diagnosis, Differential↗

A case of combined nevus: compound nevus and spindle cell Spitz nevus.

Combined nevi consisting of a Spitz nevus and an acquired nevus are unusual, and, to our knowledge, the combination of a spindle cell Spitz nevus and an overlying compound nevus has not been previously reported. We report a 17-year-old girl with a nodule on the left anterior lower extremity. The nodule was asymptomatic, firm, brown, symmetrical, dome-shaped, 8 mm in diameter, and not found with ulceration. Histological findings showed proliferation of spindle-shaped cells with an overlying compound nevus. The spindle-shaped cells were large, non-pigmented, uniform in size and shape, with rare mitoses and without nuclear atypia, and arranged in a storiform pattern in thick collagen bundles. They stained positively for S-100 and negatively for HMB-45. The lesion was considered to be a new type of combined nevus consisting of a spindle cell Spitz nevus and an overlying compound nevus.

Adolescent↗

Melanoma associated with blue nevus and melanoma mimicking cellular blue nevus: a clinicopathologic study of 10 cases on the spectrum of so-called 'malignant blue nevus'.

The term "malignant blue nevus" refers to a rare and heterogeneous group of melanomas that arise in several clinical settings. This includes melanomas arising in association with a common or cellular blue nevus and those arising de novo and resembling cellular blue nevi. We reviewed the clinicopathologic features of 10 cases of malignant blue nevi. Six cases proved to be de novo melanoma mimicking cellular blue nevus, but lacking a clear-cut benign component. Two melanomas arose in association with a common blue nevus, and two with a cellular blue nevus. The patients' (5 males, 5 females) ages ranged from 11 to 77 years (average age, 48.1 years). The head and neck was the most common location (6 of 10 patients), with five scalp tumors. Four tumors were located on the trunk; none was located on the extremities. Tumor size ranged from 0.5 to 2.2 cm (average size, 1.1cm). Most lesions had been present for many years before surgical removal. Pigmented dendritic cells were observed in 9 of 10 cases. The malignant and benign components were easily distinguished in the four cases that arose in association with a common or cellular blue nevus. Abrupt transition between a benign blue nevus and melanoma was readily recognized at scanning magnification as distinctive nodules of epithelioid to spindled cells with a sheet-like growth pattern. In all cases, malignancy was evidenced by increased mitotic rate, necrosis, nuclear atypia, pleomorphism, hyperchromasia, and prominent nucleoli. All 7 patients with follow-up information experienced recurrence (3 patients) or metastasis (4 patients). Three patients died of disease. Malignant blue nevus is a heterogeneous group of melanomas that are highly aggressive and often lethal, with a propensity for metastasis to the lymph nodes and lungs.

Adolescent↗

[Pigmented spindle cell nevus and pigmented Spitz nevus--clinical and histopathological study on pigmented Spitz nevus, and its differentiation from early melanoma by fluorescence method and measurement of 5-S-CD level in the lesion].

On the basis of clinical and histopathological studies on 17 patients who had been diagnosed as having pigmented Spitz nevus (PSN), pigmented spindle cell nevus (PSCN) was surmised to be a type of pigmented Spitz nevus. In order to distinguish pigmented spindle cell nevus and pigmented Spitz nevus from early melanoma, 5 PSCN cases and 12 PSN cases were analyzed by the fine-needle aspiration fluorescence method, touch fluorescence method and measurement of the 5-S-CD level in the lesion. With the touch fluorescence method, fluorescent tumor cells were detected in one case of PSN. With the fine-needle aspiration fluorescence method, fluorescent tumor cells were detected in one PSCN case and 2 PSN cases. In comparison with fluorescent melanoma cells, the detected fluorescent tumor cells were smaller in size and number and resembled melanocytes. The 5-S-CD level in the lesion was 50 ng/mg or less in all cases, whereas the level in melanoma is known to be a high 100 ng/mg or more. In the final analysis, measurement of the 5-S-CD level in the lesion was concluded to have the greatest utility for differential diagnosis of pigmented spindle cell nevus and pigmented Spitz nevus from early melanoma.

Adolescent↗

Nevus spilus (speckled lentiginous nevus) associated with a nodular neurotized nevus.

We report a case of nevus spilus with neurotized nevus studied by immunohistochemical methods using S-100, leu-6, glial fibrillary acid protein (GFAP), and myelin basic protein (MBP). Histologic findings of the speckled lesion showed irregular rete ridge elongation, increased epidermal melanocytes and melanin in the epidermis, and scattered nevus cell nests in the upper dermis, but showed neurotized nevus in the deep dermis, which has many features of neurofibroma. Diffuse expression of S-100 protein and MBP, focal staining with GFAP, and lack of staining with leu-7 were observed, Leu-7, positive only in neurofibromas and not in neurotized nevus, appears to be the more pertinent method for distinguishing neurotized nevus from neurofibroma.

CD57 Antigens↗

[Combined nevus: malignant blue nevus and giant pigmented nevus (author's transl)].

A combined nevus occurring on the right temporal region of a 30-year-old man with multiple giant pigmented nevi on the head, trunk and extremities was surgically removed. Histologically, the cellular blue nevus found in the deep corium fulfilled the criteria of malignancy in so far as mitoses and foci of tumor necroses were present.

Adult↗

[Nevus of Ota. Presentation of a case associated with a cellular blue nevus with suspected malignant degeneration and review of the literature].

BACKGROUND: Nevus of Ota is a melanotic pigmentary disorder ("dermal melanocytosis"), mostly congenital or acquired, involving the skin innervated by the first and the second branch of the trigeminal nerve, hence its descriptive label of "nevus fuscus coerulaeus ophthalmic and maxillaris". In more than half of patients this condition is associated with "ocular melanocytosis" ("melanosis oculi") involving the conjunctiva, the sclera, the uveal tract and possibly the optic nerve. In some cases a condition of "orbital melanocytosis" with involvement of orbital fat and periosteum by dendritic melanocytes is on record as well as in some other patients an analogous condition of "leptomeningeal melanocytosis" is present. At histology tissues from the above sites are seen infiltrated by dendritic melanocytes which can vary in number from so scarce up to so numerous that a diagnosis of a blue nevus of the common type is warranted. Sometimes the finding of a variously pigmented typical cellular blue nevus in the skin and alternatively that of heavily pigmented melanocytoma in the eye ("nevus magnocellularis") or in the meninges (so-called "melanotic meningioma") are respectively observed. MATERIALS AND METHODS: A case of cellular blue nevus with histologically uncertain malignant potential in a nevus of Ota of 30 years duration in a white female patient aged 59 is described. The lesion which was surgically totally removed grossly appeared nodular shaped and 2 cm sized. Histologically it consisted of a fairly well-circumscribed proliferation of melanocytic spindle-shaped cells growing in a vaguely fascicular pattern. On the basis of random nuclear atypicalities and pleomorphism and additionally by virtue of the presence of a few scattered mitoses (one of which was atypical) but in absence of frank necrosis a diagnosis of unpredictable biologic behaviour seemed to be warranted. The patient was closely followed-up but no adjunctive therapy given. Four years after the excision and diagnosis no local recurrence or distant metastasis has been discovered. A computerized search of previously recorded cases of melanomas in nevus of Ota was made. CONCLUSIONS: Forty-eight cases of malignant melanomas complicating this clinico-pathological setting are on record, mostly in the uveal tract, followed by locations in central nervous system, skin, and retro-orbital fatty tissue. Melanomas arising in nevus of Ota tend to be low grade lesions that do infiltrate locally but rarely metastasize. The importance of a closely dermatological and ophthalmic surveillance of patients with nevus of Ota is emphasized.

Cell Nucleus↗

Congenital giant melanocytic nevus with pigmented epithelioid cells: a variant of epithelioid blue nevus.

Epithelioid-cell blue nevus is an unusual cytologic variant of blue nevus that has been recently described mostly in patients with Carney complex, although the lesion may also appear in patients with no evidence of Carney complex. This variant of blue nevus is composed of melanin laden large polygonal epithelioid melanocytes situated within the dermis. The neoplastic cells show no maturation with progressive descent and, in contrast with the usual stromal changes in blue nevi, epithelioid-cell blue nevus exhibits no dermal fibrosis. This report describes a congenital giant melanocytic nevus with pigmented epithelioid cells located on the back of a 2-year-old male. The lesion was present at birth and the patient had no evidence of Carney complex. Histopathologically, the lesion consisted of a large and entirely intradermal melanocytic nevus composed of heavily pigmented epithelioid melanocytes involving the full-thickness of dermis, but extending also to the subcutaneous fat and underlying soft tissues. Immunohistochemically, epithelioid neoplastic melanocytes expressed immunoreactivity for S-100 protein, HMB-45, Melan-A, NK1C3, and microphthalmia transcription factor (MiTF) antibodies. MIB-1 cellular proliferation marker was expressed in the nuclei of only a few scattered epithelioid melanocytes. This report demonstrates that epithelioid-cell blue nevus is a distinctive histopathologic variant of blue nevus that may also appear as a giant congenital melanocytic nevus.

Biomarkers, Tumor↗