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MELANOTIC PROGONOMA

IN CHILDHOOD

Report of a Case of

‘¿Retinal

Anlage―Tumor of the

Maxilla in an Infant

Rute Medenis, M.D., Danely P. Slaughter, M.D., and Thomas K. Barber, D.D.S.

Departments of Pediatrics and Surgery, College of Medicine, and the Department of Pedodontics,

Collegeof Dentistry,Unieersityof illinois

M ELANOTIC PIIOGONOMA, a rare tumor frequently referred to as “¿retinal anlage― tumor, is peculiar to infants. This

tumor usually- orginates in the maxilla. It

is differentiated microscopically by a typi cal tissue pattern and the presence of pig

ment. Cases of the tumor have been re

ported ulider a variety of names, such as melanoma, mclanocarcinoma and melanotic odontogenic tumor. We have been able to

find reports of 25 cases of melanotic pro

gonoma (Table I).11s In 23 of these cases

the tumors originated in the maxilla or

mandible, in 1 in the area of the anterior fontanelle, atid in 2 at extracephalic sites. Despite the fact that melanotic progonoma

is a tumor pectiluir to childhood, only one case has been reported in a pediatric jour

nal. We wish to report another case of

melanotic progonoma occurring in the max

illa of an infant which was successfully

treated surgically. A brief summary of the reported cases is included in this paper.

CASE REPORT

A 5-month-old Caucasian female infant was admitted to the University of Illinois

Research and Educational Hospitals in

February, 1959, because of a firm swelling of the right upper alveolar ridge. The mass had first been noted by the parents 2 months prior to admission. It had enlarged progressively but had otherwise caused no symptoms. Physical examination on admis sion revealed a well-developed, well nourished, alert infant who did not appear

acutely or chronically ill. The abnormal

Fic. 1. Appearance of mouth before operation.

findings were confined to the right maxillary

area. A firm, nontender mass was present in the alveolar process, extending into the adjacent portion of the hard palate. Three partially erupted teeth were noted on the labial surface of the swelling (Fig. 1).

Intra-oral roentgenograms showed en largement of the upper alveolar process on the right, with some radiolucency centrally.

Roentgenograms of the skull revealed no additional abnormalities. Skeletal survey

films showed no evidence of metastases.

Leukocyte and erythrocyte counts and

results of urinalysis were normal.

A biopsy of the lesion was performed, and the diagnosis of “¿retinalanlage― tumor

was made. Three days later the involved maxilla and the alveolar ridge, including

the three teeth, were excised. The tumor

appeared to be encapsulated inferiorly

ADDRESS:(R.M.) Department of Pediatrics, 840 South Wood Street, Chicago 12, Illinois.

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A

Sourcege at On8et

(mo)Sex. SiteFollow-up

(yr)Krompecher'

(1918)..Maxilha4Mummery

& Pitts2 (l9@6)1FRight alveolar process anterior maxilla4Dudits

& Szabó' (1935)4FLeft anterior maxilla4Soderberg

& Padgett4 (1941)4

1F FRight

anterior maxilla Left maxilla6

5Halpert

& Patzer' (1949)6FAnterior maxillaWass6 (1948)5FMandible1Field cial,.7(1950)14FAnterior maxilla4Clarke & Parsons8(1951)3MRight maxilla74Martin

& Foote9 (1951)5FSkullI 4Battle

cial.'°

(1951—5@)14MMandible14Shafer

& FrisselP' (1953)4FAnterior maxilla54Notter

& SOderberg'2 (1953)MRight maxilla4Hllnerwadel'3 (1953)6FAnterior maxilla9McDonald & White'4 (1953—54)MMaxilla14Tiecke & Bernier'5 (1956)4MMandible..Tiecke & Bernier― (1956)6FMandible..Tiecke & Bernier'5 (1956)1@FMaxilla5Tiecke

& Bernier'6(1956)14FAnterior

maxillaTiecke

& Bernier―(1956)4MAnterior maxilla3Tiecke

& Bernier'6 (1956)3MAnterior

maxilla..Stowens― (1957)4MAnterior maxilla9Stowens― (1957)1FAnterior maxillaSStowens― (1957)@4FMaxilla4Eaton & Ferguson'1 (1956)5MEpididymis..Blanc ciaL'8(1958)5MRight shoulder..Present

report54FRight anterior maxilla14

TABLE I

REPORTED CASES OF “¿RETINALANLAGE―TUMOR OR MELANOTIC PROGONOMA

against the hard palate, superiorly against the floor of the orbit, and anteriorly against the maxilla.

Microscopically, the tissue consisted of wide anastomosing strands of spindle shaped cells enclosing islets composed of two types of cells. One type was epithelial in character and heavily loaded with melanin. These cells lined irregularly shaped spaces, which contained cells of the second type, which were less uniformly shaped and contained very dark nuclei and little cytoplasm. These cellular nests were irregularly shaped and irregularly distrib

uted (Figs.2 and 3). Some areas of the

adjacent bone were partially replaced by the tumor tissue.

The child made an uneventful post-opera tive recovery and was discharged on March 14, 1959. The appearance of the mouth

at the time of discharge is seen in Figure 4. No recurrence of the tumor was found 1% years after the operative removal.

COMMENT

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Fic. 2. Photomicrograph of melanotic progonoma showing strands of spindle-shaped cells enclosing spaces lined with pigmented cells and containing cell nests.

_________

—¿

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than boys. The site of predilection of the tumor is the maxilla. In 18 cases it occurred in the maxilla and in 5 cases in the mandi ble; in 1 case it originated in the skull. In two instances the site of origin was ex

tracephalic—the shoulder area and the epi

18

Several theories have been proposed to

explain the development of this tumor. Some investigators believe that the neo plasm has an odontogenic precursor be cause of its almost exclusive location in the

jaws. Histologically and cytochemically,

however, melanotic progonoma does not resemble primitive dental tissue or tumors of established dental origin.19 Halpert and Patzer5 proposed “¿retinalanlage― as the

precursory site of the tumor, which results

from tissue being accidentally “¿pinched off―from the primitive optic cup and retina. They felt that the cup-shaped arrangement of the tumor pattern suggested the optic

cup and retina, and that the cells within the cup-shaped structures resembled neuro cytes. Martin and Foote9 and Clarke and Parsons8 accepted this theory. There are several facts, however, that are against this hypothesis. The retina is well developed at the time when the anlagen of the maxilla begin to form.20 It is rather improbable that more highly developed tissue could be

“¿pinchedoff―by relatively less developed

tissue. Moreover, no tissue resembling sclera or choroid has ever been found in these growths, and in no case have associated eye defects been present. Formation of a supernumerary optic cup would be re quired in order to explain the origin of a neoplasm containing retinal tissue. Such evi dence has not been found.16 Finally, the extracephalic sites of melanotic progonoma cannot be explained by this theory.

The vomeronasal organs of Jacobson have

been implicated as possible precursors of

these tumors.2' The majority of melanotic progonomata are located adjacent to the area occupied by the vomeronasal organs during their brief embryonic existence. The columnar cells of the olfactory epithelium

Fic. 4. Appearance of mouth after operation.

some extent the pigmented cells of the tu

mor. The less differentiated reserve cells of the vomeronasal organs are similar in ap pearance to the undifferentiated cells of the tumor. The neurons of the olfactory epithelium are regarded as the origin of the spindle-shaped cells. However, it seems very unlikely that the vomeronasal organs are the parent tissue of melanotic pro gonoma, because this theory does not ex plain the extracephalic sites of the tumors, and because histochemically the spindle shaped cells are not of neural nature.16

Stowens'6 proposed that atavism of de velopmental function could explain the origin of the tumor. On the basis of the histologic similarities between the vomero

nasal organs, lateral line organs in fish, and

pigmented tactile spots (all of which are of neuroectodermal origin) and the mela notic progonoma, he suggested that this tumor also is of neuroectodermal nature. If such atavism of the developmental func tion or the function of the organizer could occur, sensory neuroectodermal tissue could appear at a site where it is not normally found in the given species but where it is encountered during some period of life in the phylogenetic forms of the species.21 Thus tissue foreign to the area could de velop into a tumor. Stowens also proposed

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is consistent with the presence of extrace phalic locations of some of the tumors, but it remains unproved.

SUMMARY

A case of melanotic prognoma of the maxilla in a 5-month-old female infant is reported. The tumor was successfully re moved surgically, and there has been no recurrence. It is evident that the origin of melanotic progonoma, like that of many other tumors, is unknown. This tumor is benign and is amenable to surgery. It is therefore important that cases be recog nized early in childhood to insure optimal therapy.

REFERENCES

1. Krompecher, E.: Zur Ilistogenese und Mor phologie der Adamantinome und sonstiger

Kiefergesch@velste. Beitr. Path. Anat., 64:165, 1918.

2. Mummery, J. H., and Pitts, A. T.: A melanotic

epithelial odontome in a child. Brit. Dent.

J., 47:121, 1926.

3. Dudits, A., and Szabó, B.: Kongenitales Mel anokarzinom des Oberkiefers. Mschr. Kinder heilk., 63:294, 1935.

4. Soderberg, N. B., and Padgett, E. C.: Two un usual melanomas of the alveolus and maxilla.

Amer. J. Orthodontics (Oral Surg. Sect.), 27:270, 1941.

5. Halpert, B., and Patzer, R.: Maxillary tumor

of retinal anlage. Surgery, 22:837, 1947.

6. Wass, S. H.: Melanotic adamantinoma of the

mandible in a child aged 5 months. Proc.

Roy. Soc. Med., 41 :281,1948.

7. Field, H. J., Ackerman, A., and Kannerstein, M.: Melanotic tumor of the maxilla in an in fant: case report. J. Newark Beth Israel

Hosp., 1:286, 1950.

8. Clarke, B. E., and Parsons, H.: An embryologi

cal tumor of retinal anlage involving the skull. Cancer, 4:78, 1951.

9. Martin, H., and Foote, F. W., Jr. : A third

case of so-called retinal anlage tumor. Can cer, 4:86, 1951.

10. Battle, R. J. V., Hovell, J. H., and Spencer, H.:

Pigmented adamantinomata. Brit. J. Surg.

39:368, 1951-52.

11. Shafer, W. C., and Frissell, C. T.: The mela

noameloblastoma and retinal anlage tumors.

Cancer, 6:360, 1953.

12. Notter, C., and Soderberg, C. : Em Fall von

sog. “¿MelanocarcinomaCongenitum Proces sus Alveolaris―(Krompecher). Acta Radiol.,

40:54, 1953.

13. Hünerwadel, M. : Em Kongenitaler Melano

tischer Tumor am Oberkiefer eines Sechs

monatigen Sauglings. Schweiz. Mschr. Zahnh., 63:1029, 1953.

14. MacDonald, A. M., and White, M.: Pig

mented congenital epulides of neuro-epithe hal origin (the description of a further case

with successful excision and survival). Brit.

J. Surg., 41:610, 1954.

15. Tiecke, R. W., and Bernier, j. L.: Melanotic ameloblastoma. Oral Surg., 9:1197, 1956. 16. Stowens, D.: A pigmented tumour of infancy:

the melanotic progonoma. J. Path. Bact., 73:43, 1957.

17. Eaton, \V. L., and Ferguson, J. P.: A retino

blastic teratoma of epididymis: case report.

Cancer, 9:718, 1956.

18. Blanc, W. A., Rosenblatt, P., and Wolff, J. A.: Melanotic progonoma (“retinal anlage― tu mor) of the shoulder in an infant. Cancer,

11:959, 1958.

19. Sognnaes, R. F.: Microstructure and histo chemical characteristics of the mineralized

tissues. Ann. N. Y. Acad. Sci., 60:545, 1955.

20. Patten, B. M: Human Embryology, Ed 2.

New York, Blakiston, 1953, p. 428. 21. Borchers, E.: Geschwulste der Kieferknochen.

Neue Deutch. Chir., 53:466, 1932. 22. DuShane, G. P.: In The Biology of Melanomas,

edited by M. Gordon et al. New York, The

New York Academy of Sciences, 1948. 23. Mathias, E.: Zur Lehre von den Progono

blastomen, Virchow. Arch. Path. Anat., 236:

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1962;29;600

Pediatrics

Rute Medenis, Danely P. Slaughter and Thomas K. Barber

Anlage" Tumor of the Maxilla in an Infant

MELANOTIC PROGONOMA IN CHILDHOOD: Report of a Case of "Retinal

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1962;29;600

Pediatrics

Rute Medenis, Danely P. Slaughter and Thomas K. Barber

Anlage" Tumor of the Maxilla in an Infant

MELANOTIC PROGONOMA IN CHILDHOOD: Report of a Case of "Retinal

http://pediatrics.aappublications.org/content/29/4/600

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