The Journal of P E D I A T R I C S
Letters to the Editor
Oculodentodigital syndrome To the Editor: Several inquiries followed the publication of our paper in this JOURNAL in July, 1963, volume 63, page 69, and we assume that other case reports will be forthcoming. We have chanced upon an article by Littlewood (J. M.) and Lewis (G. M.) entitled "The Holmes-Adie syndrome in a boy with acute juvenile rheumatism and bilateral syndactyly," in the Archives of Diseases o[ Childhood (38: 86, 1963). The boy described appears to have the facies and other signs of the oculodentodigital syndrome. An aspect of the syndrome we did not dilate upon was the n u m b e r of distal segments of the toes. Several correspondents have asked how commonly the number is reduced, from three to two in otherwise normal individuals. The n u m b e r of digital segments of the toes, other than the first, is extremely variable. The first toe nearly always has two segments, al-
though a three-phalanged form has been described. The two-phalanged form occurs quite commonly in the fifth toe (approximately 40 per cent of the time) in individuals of European ancestry. Its frequency in other racial groups has not been ascertained. The two-phalanged form has been found to occur in the fourth and fifth toes in about 2 per cent; in the third, fourth, and fifth toes in about 0.5 per cent; and in all toes in less than 0.1 per cent in this same racial group. For an excellent discussion of this problem see Venning, P.: "Variation of the digital skeleton of the foot," Clinical Orthopedics 16: 26, 1960. ROBERT
GORLtN~ D,D.S,~ M,S,
P R O F E S S O R A N D GIKAIRMAN~ DIVISION OF ORAL P A T H O L O G Y U N I V E R S I T Y OF M I N N E S O T A S C H O O L OF D E N T I S T R Y MINNEAPOLIS
14, M I N N .
Oral tumors of infancy To the Editor: We were most pleased to read the fine survey of oral tumors of infancy by Dr. S. Bhaskar ("Oral tumors of infancy and childhood," J. PEOIAT. 63: 195, 1963). The great number of cases analyzed by Dr. Bhaskar lends great validity to his findings. There are, however, a few points with which we would take exception: There is certainly no good evidence to indicate that the melanotic anlage tumor (we use this term to avoid controversy) is of odontogenic origin. While the vast majority have arisen in the maxilla and mandible, several other well-documented cases have been reported in the shoulder, skull, uterine broad ligament, epididymfs, etc. There seems to be little reason to infer that this tumor arises from embryonal dental structures simply because it is seen in juxtaposition to tooth germs .or even because it may contain
epithelial rests of Malassez, i.e., remnants of Hertwig's epithelial root sheath, (On this shaky ground, Willis indicates the so-called congenital epulis of the newborn [granular cell Schwannoma or "myoblastoma"] is of odontogenic origin.) Furthermore, we are certainly unable to detect a morphologic similarity between the melanotic anlage tumor and any stage of tooth development. Dr. Bhaskar suggested that the congenital epulis was an entirely different entity from the granular cell "myoblastoma." The differences rest largely on the very marked predilection for females, the failure of pseudoepitheliomatous hyperplasia, and the gingival location of the former. However, it should, be printed out that the "tumor" itself is identical in both lesions. There is, by the bye, good recent electrophotomicrographic and histochemical evidence to suggest strongly that the lesion is of neurogenic
Letters to the Editor
origin and should probably be called "granula cell Schwannoma." The presence of numerous epithelial rests therein again should not be taken as having any significance, since these rests are ordinarily present in considerable numbers in the jaw of newborn infants and are always present in the periodontal ligaments of man and animals. We would also take minor issue with the continued use of the term "adenoameloblastoma." Our preference would be ameloblastic adenomatoid tumor since the term employed by Dr. Bhaskar would imply that this tumor or hamar-
toma is a special type of ameloblastoma. It is quite different from the latter tumor, as Dr. Bhaskar has pointed out, in microscopic appearance and clinical behavior. It is cured by conservative curettage. We think that the continued use of such a term as "adenoameloblastoma', places unjust stigma on such a benign lesion. ROBERT j . GORLIN~ D.D.S., M.S. PROFESSOR AND C H A I R M A N DIVISION OF ORAL PATHOLOGY U N I V E R S I T Y OF M I N N E S O T A SCHOOL OF DENTISTRY M I N N E A P O L I S 14, M I N N .
Reply To the Editor: The questions raised by Dr. Gorlin are not new. They have been, and still are, a matter ot discussion. The origin of the so-called melanoameloblastoma is indeed debatable. However, on the basis of the clinical and some histologic features of this lesion, we prefer to include this among the tumors of the odontogenic apparatus. By far the largest number of these lesions occur in the jaws, and the fact that on a very rare occasion a lesion of similar morphology occurs elsewhere is no more a point against its odontogenic origin than the occurrence of an ameloblastoma-like lesion in the tibia (or even the skin) is a point against the origin of the common ameloblastoma from the enamel organ. Recently we have had the opportunity of studying epithelial odontogenic lesions which contained melanin, and this finding removes another small obstacle in the acceptance of the melanoameloblastoma among the family of odontogenic tumors. In regard to the so-called myoblastoma, I am aware of the fact that the granular cell myoblastoma is probably ~t granular cell Schwannoma (Cancer 15: 936, 1962). However, I do not think that the congenital epulis of newborn infant and the granular cell myoblastoma (lingual or elsewhere) are identical lesions. There are numerous clinical (age, sex, location, etc.) and histologic (type of granular cell, transitions from fibroblasts, absence of pseudoepitheliomatous hyperplasia, epithelial islands, calcifications, homogenous amorphous areas, etc.) differences between the two lesions. The similarity between the two lesions is based almost wholly upon a superficial resemblance between their "granular
cells." But similar cells are seen in some apical lesions of t h e tooth, or in cases of the traumatic granulomas of the tongue, or even in some types of an ameloblastoma (granular cell ameloblastoma). Therefore, it seems to me that it is shaky ground indeed to lump these lesions together merely because they contain granular cells w h i c h bear a resemblance to each other. W i t h regard to the adenoameloblastoma, I agree with Dr. Gorlin that it is a poor term because it contains the word "ameloblastoma." But the term "ameloblastic adenomatoid tumor" is not better, and for the same reasons. In fact, a study has just been completed in my departmeht on a large series of these cases, and we have attempted to show that these lesions in reality are follicular cysts with intracystic epithelial proliferations (J. Oral Surg., in press). These lesions are benign and local curettage is curative. The term "adenoameloblastoma," therefore, like many other terms in pathology, is a poor one, but well entrenched. Every surgeon who deals with these lesions, and every oral pathologist, is aware of its exact significance. To replace it with another, somewhat awkward and new term, seems unwarranted. Although I disagree with these comments, I am grateful for them, and for the author's interest. S. N. BHASKAR, D.D.S., M . S . , PH.D.~ C H I E F , D E P A R T M E N T OF ORAL PATHOLOGY UNITED STATES ARMY I N S T I T U T E OF DENTAL R E S E A R C H WALTER
CENTER W A S H I N G T O N 12, D. C.