JUVENILE MELANOMA OF T H E TONGUE
PAUL J E H N S T R O M , M . D . , AND GONZALO E . A P O N T E , M . D .
Department
of Pathology
and Clinical Laboratories, Jefferson
Philadelphia,
Pennsylvania
Medical
College
Hospital,
Children occasionally have pigmented, cutaneous or mucosal lesions that histologically resemble melanoblastomas occurring in adults. With rare exceptions,
these tumors in children fail to metastasize. Their clinical beliavior also differs
from that of melanoblastoma in other respects. For this reason, the term juvenile
melanoma has been used to designate the pigmented lesions in children. Its characteristics have been enumerated by Spitz.20 Perusal of the medical literature reveals sporadic instances of nevi arising in the labial, buccal, and gingival mucous
membranes of adults, but it seems that none has been described with its origin
in the mucosa of the tongue, either in adults or children. Insofar as the authors
are aware, this is the first published description of an unusual nevus in an oral
mucous membrane, that is, a juvenile melanoma of the tongue.
l i E P O H T O F CASE
Clinical data. A 7-ycar-old white girl had a "canker s o r e " on t,he left lateral margin of
her tongue, approximately midway in the anteroposterior dimension. T h e lesion seemed to
heal spontaneously, b u t a painless, rounded, saucer-like, nontender, sessile growth (coated
over the surface with a yellow, plastic exudate) was observed a few days later. The t u m o r
measured approximately 1 cm. in its greatest dimension (Fig. 1). No parabasal induration
or regional lymphadenopathy was observed. Clinically, the lesion was regarded as an ulcerated capillary hemangioma ("pyogenic g r a n u l o m a " ) . T h e entire mass was removed for
biopsy, and there was no evidence of residual or recurrent tumor when the patient was examined 10 months later.
Gross pathologic examination. A round, firm, elevated nodule, covered with a pink and
yellow exudate, was attached by a broad pedicle to the mucosa of an elliptical portion of
lingual tissue. The nodular mass measured 0.9 x 0.3 cm. Hemisectioning revealed t h a t the
mass was poorly circumscribed; the freshly cut surfaces were pink and gray, moist, and
moderately firm.
Microscopic examination. Study of the histologic sections revealed an ulcerated mucosa,
with associated granulation tissue t h a t superficially resembled an ulcerated capillary
hemangioma. A small focus of junctional activity was observed in the epithelium near the
border of the ulcer. (Fig. 2). T h e neval cells were predominantly segregated as large nests
and clusters, b u t , in some instances, they were single and tended to permeate the upper
portion of the lamina propria. Most of the cells were large, and varied from round to polygonal; a few of them were fusiform or spindle-shaped (Fig. 3). T h e cytoplasm of the cells
was relatively a b u n d a n t , faintly basophilic, and occasionally vacuolated. T h e nuclei were
prominent and relatively large, centrally placed, and vesicular in s t r u c t u r e , with small
clumps of chromatin distributed peripherally. Only an occasional mitosis was observed,
and multinucleated and giant neval cells wore not noted. Although a thorough examination
of numerous sections failed to reveal melanotic pigment, the cells were distinctly nevoid,
and the tumor was regarded as a juvenile melanoma of the tongue.
Received, May 14, 1956; revision received, J u n e 22; accepted for publication August 16.
Dr. Jcrnstrom is Assistant Professor of Pathology, and D r . Apontc is a Trainee of the
National Cancer I n s t i t u t e .
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JERNSTKOM AND APONTE
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Two special technics are useful in confirming the histologic identification of melanin,
namely, impregnation with silver and the reaction with dihydroxyphenylalanine (dopa).
Impregnation with silver results in a black pigment if melanin is present. On the other hand,
cells that contain the enzyme tyrosinase, thereby possessing the ability to form melanin,
take on a dark color in the reaction with dopa. Impregnation with silver does not demonstrate the site of formation of melanin, as does the reaction with dopa. Not all melanomas
are argontaffinophilic, but, owing to the fact that some are, histologic sections were treated
according to the technic proposed by Fontana. The cells of the tumor were not impregnated with silver by this method. The technic for the reaction with dopa requires the application of levorotatory 3,4-dihydroxyphenylalanine (0.1 per cent solution) to sections of
fresh tissue. Inasmuch as the nature of the tumor was not suspected clinically, or even at
the time of gross pathologic examination, fresh tissue was not available for testing with
dopa.
Although the neoplasm did not resemble a granular-cell myoblastoma, this diagnosis was
discarded when the cells failed to manifest an affinity for the periodic acid-Schiff (PAS)
reagent. Sections treated with Masson's trichrome stain were no more specific than those
that were stained by the routine method with hemotoxylin and eosin.
Owing to the exceptionally rare occurrence of nevi in the tongue, sections of the tumor
were sent to Dr. Arthur Purdy Stout23 and to Dr. Fred W. Stewart. 22 Both consultants
agreed that the tumor was composed of neval cells, and they concurred in the diagnosis of
juvenile melanoma. Furthermore, Dr. Stewart expressed doubt that the tumor was benign.
DISCUSSION
Pigmentation of the oral mucous membranes is a well-recognized entity. It
is more frequent and more conspicuous in the darker races, but it may also be
observed in Caucasians, in normal and abnormal conditions.6 Masson13 stressed
the fact that melanoblasts are found in the oral mucous membranes of Caucasians more often than is generally believed, and an area that is rich in melanoblasts may not seem to be pigmented grossly. For example, Laidlaw and Cahn12
removed portions of grossly nonpigmented tissue from the gingival mucosa of
Negroes and Caucasians. Histologic sections revealed dendritic melanoblasts
in all of the specimens, chiefly in the basal layer of the mucosa. Spherical, unbranched forms similar to those observed in skin were not found. Furthermore,
these authors stated that Adachi1 observed the same type of melanoblasts in
labial mucous membranes. Finally, the clinical syndrome described by Jeghers
and his associates11 is characterized by pigmentation of the oral mucous membranes in association with polyposis of the gastrointestinal tract.
When one recognizes the rather frequent occurrence of melanoblasts in oral
mucosa, it is surprising that so few pigmented nevi have been observed in this
tissue. During an extensive histologic study of 14,609 nevi removed at Memorial
Hospital in New York City, Pack, Lenson, and Gerber17 encountered none from
the oral cavity. Greene and his associates10 described 3 nevi from the oral mucous
membranes in material studied at the Armed Forces Institute of Pathology.
These were located in the labial and muco-buccal folds above the upper left
incisor. The youngest patient in this small group was 37 years old. One of the
nevi was of the intradermal variety, and 2 were regarded as compound nevi.
Single instances of the intradermal type of nevus in gingival mucous membranes
were described by Allen and Bruce4 and by Bernier and Tiecke.7 The 2 patients
were white men who were, respectively, 37 and 53 years of age. In contrast to
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JUVENILE MELANOMA OF THE TONGUE
1343
FIG. 1. Artist's sketch of the juvenile melanoma of the
tongue. The lesion grossly resembled an ulcerated capillary
hemangioma ("pyogenic granuloma").
the relative rarity of pigmented novi in the oral mucosa, it is particularly interesting to note that the pigmented nevus is said to be the most common tumor
of the conjunctival mucous membranes.9
Malignant melanoma (or melanoblastoma) that is primary in mucous membranes is an uncommon, but not rare tumor. The most frequent site of involvement seems to be the conjunctiva, and such tumors are observed much less
often in the oral cavity. Moore and Martin 14 recently reviewed 12 malignant
melanomas of the upper respiratory tract and oral cavity, as observed at the
Memorial Hospital in New York City during a period of 19 years. Only 1 of
these was from the tongue; it was located at the base, and distant metastases
were present when the patient was first examined. The age, sex, and race of the
patient were not recorded. From a total of 95 malignant melanomas studied at
the University of Minnesota Hospital, Stewart, Hay, and Varco21 found only 2
that were primary in the oral cavity. There were other instances in which melanoblastomas involved the oral cavity secondarily, but these will not be discussed
in this paper.
The first published description of a melanoma of the oral mucous membrane
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JEKNSTKOM AND APONTE
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seems to be that by Weber in 1859.24 The lesion was regarded as a melanoma,
and it involved the palate. In 1941, Baxter 6 published a study of 55 examples of
melanomas of the oral mucous membranes; the youngest patient was 14 years
old. Only 4 were less than 25 years of age. The most frequent site of involvement
was the palate, and 1 of the tumors seemed to be primary in the tongue.
After thoroughly examining the medical literature from over the world, we
found 25 additional instances of oral melanomas. A few of these were not included
in the review by Baxter, 5 but most of them were published later. The most
frequent site of involvement was the hard palate. The only melanoma thought
to arise in the tongue was that described by Blackburn in a 90-year-old white
woman.8 Owing to inadequate or inconclusive data in the papers, we omitted
some of the reports from our survey. A total of 94 cases of oral melanomas
was reviewed. Most of the patients were more than 40 years of age. There
was no predilection for races. This type of lesion has been found in Caucasians,
Negroes, Indians, and Orientals. The most frequent site of involvement in the
94 instances was the palate, the tongue being involved in only 3 patients, all of
whom were adults.
Melanotic tumors that were interpreted as melano-ameloblastomas, or as
tumors of the retinal anlage, in the oral cavities of infants were reviewed by
Shafer and Frissell.18 All of these infants were less than G months of age. Nine of
the 10 lesions involved the maxilla, and I the mandible. The biologic evidence
seemed to support the contention that the tumors originated from the retinal
anlage rather than odontogenic tissue. Such tumors seem to be benign, and no
recurrences were observed up to 7 years after conservative surgical excision
was performed. The patient described by Notter and Soderberg16 may have had
a tumor of this same category, although it was not regarded as such. The growth
occurred in the maxilla of a 2-month-old infant. Similarly, Soderberg and
Padgett 19 observed 2 tumors that resembled melano-ameloblastomas in the
maxillas of infant girls, respectively, 1 and 3 months of age.
In 1954 McWhorter and Woolner15 published a review of 102 case reports of
malignant melanomas (melanoblastomas) in children, prior to the introduction
of the term juvenile melanoma in 1948. These authors thought that only 13 of the
series were documented sufficiently to justify the diagnosis of malignant melanoma. They omitted a few instances in which the tumor was presumably transmitted from the mother to the fetus by way of the placenta (i.e., congenital
melanoma). In addition to the 13 instances published in the literature,
McWhorter and Woolner studied 5 from the material at the Mayo Clinic.
All 18 were believed to be primary in cutaneous surfaces. The entire series of
papers included descriptions of 11 additional pigmented tumors that were
regarded as juvenile melanomas, but none of these were thought to be primary
in a mucosal surface.
In the original series of juvenile melanomas described by Spitz20 (1 of which
proved later to be malignant), there was not a single instance in which the tumor
was primary in mucosa. Cytologic criteria were enumerated for some of the
melanomas of childhood that may be regarded with impunity as juvenile mel-
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JUVENILE MELANOMA OF THE TONGUE
1345
F I G . 2 (upper). Photomicrograph illustrating the characteristic arrangement of the classic neval cells in the juvenile melanoma of the tongue.
Hematoxylin and eosin. X 100.
F I G . 3 (lower). Photomicrograph to illustrate the cellular configuration
of the distinctly nevoid growth of the tongue. Hematoxylin and eosin.
X 430.
anomas, thereby implying that the clinical course would be uneventful. Spitz
thought that the histocytologic criteria were reliable for distinguishing approximately two-thirds of the benign juvenile melanomas from the melanoblastomas
ordinarily observed in adulthood. On the other hand, she thought that clinical
data were required for distinguishing the remaining third. Allen2 believes that
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JKKNSTKOM AND A P O N T t i
Vol. 26
there are exceedingly few, truly malignant melanomas in children (he refers to
6 instances), and that these may be distinguished microscopically on the basis
of excessive atypism in their histologic structure.
We did not find any previously published instance of a duly substantiated
example of juvenile melanoma arising in the oral mucous membrane, particularly that of the tongue. Melanotic tumors seem to occur only rarely in the oral
mucosa of children, but, according to the literature, they do not seem to occur
in the tongue. Usually, the true biologic character of such tumors has proved to
be malignant (by clinical observation), or the tumors were not conclusively
identified as bona fide melanotic tumors.
SUMMA RY
1. A tumor is described that is believed to be the first example of a juvenile
melanoma arising in the tongue. Clinically, it was atypical for a melanotic neoplasm of the oral mucous membrane {i.e., unusual course and location), but the
cytologic and histologic features were characteristic of a juvenile melanoma.
2. Pigmentation of the oral mucous membranes and benign and malignant
melanotic lesions of mucous membranes are discussed in relation to juvenile
melanoma.
SUMMAKIO I N
iNTERLINGUA
1. Le autores describe un tumor, reguardate como le prime exemplo de un
melanoma juvenil del lingua. Chnicamente le tumor esseva atypic pro neoplasma
melanotic del mucose membrana oral {i.e., su curso e su sito esseva inusual), sed
le aspectos cytologic e histologic esseva characteristic de melanoma juvenil.
2. Es discutite le pigmentation del mucose membrane oral e melanotic lesiones
benigne e maligne de membranas mucose in relation a melanoma juvenil.
Acknowledgment.
clinical data.
T h e authors wish to thank Or. John D. Reese for permission to use t h e
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