Recurrent ameloblastic fibroma in a 3-year-old boy

RECURRENT

AMELOBLASTIC F. HIXBSCH,

&YMO~D THO~IAS

FIBROMA

n.

STEPHESSOS,

Cwr.41~

IN A S-YEAR-OLD (1X ) I‘SK,”

TAETJTESAST

(DC)

BOY

.\sI)

VSN**

A

;\ AMEJIOBLASTOMA is an epithclial neoplasm which has becv~ chara+ terized by a marked tendency to recur, probably due for the most, part According to Small and Waldron,l approsit 0 inadequate surgical excision. mately 1 per cent of all cysts and t,umors of the jaws may be expected to lx Their statistics show that only 4 per cent of the patients in ameloblastomas. thr 1,036 ca.ses reported wcrc 9 years old or less at the time of discovery of the tumor. Published reports of cases occurring earlier than the sixth year of age are rare indeed. The following case report is presented w wpresenting one of the youngest patients with ameloblastic tumor tha.t havr bevn reportvd t.o dat,e, recurrent due to inadequate initia,l excision. Case Report In October, 1954, a 3-year-old Hospital, Philadelphia, Pennsylvania, mental area of the mandible.

white with

boy was brought to the Cnited Stat.ea Naval the complaint of a recurrent mass in t~he Ir-f~

The parents stated that when the patient was 1W years of age they had become COW c*erned over the noneruption of the left mandibular decitluous cuspid and lateral iuciqor teeth. Seeking medical and dental atlvice at that time, they were advised to “watch al111 wait” for eruption. Shortly before the boy’s second birthday, they noted a swelling in the area of the missing teet,h and once again they sought advice. Roentgenographic examination at that time revealed a small, multiloeular, cystic-appearing radiolucent area in association with the unerupted deciduous cuspid and lateral incisor teeth (Fig. 1). ‘l‘h~~ tentative diagnosis was dentigerous cyst. The tumor was enucleated at, a civilian hospital in February, 1954. The unerupted tleeitluous cuspid an11 lateral incisor, ax well as their Microscopic espermanent successors, were removed with the mass at that operation. (course was amination of the specimen revealed an “ atlalll:tllti110111a. ” The postoperative uneventful ant1 the operative site healed satisfactorily. Approximately one month prior to the patient’s appearance at the Uuited States Naval Hospital, the parents noticetl a recurrence of the swelling. Microscopic sections of the tissue removed in the February, 1954, operation were obtained and reviewed 1,~ The?- all concurred with the tliagnosis of ameloseveral general and oral pathologists. blastie fibroma. In view of the history of the previous simple enueleation, it aas assumed that the tumor mass represetl tecl :I rrc*urrtLrlt :~~rrc~l~~l~lnstor~~n,au11 llie I rratmr~rtl p1nnrrin.g was I~awtl 01, that prcmisr.

'708

RAYMOND

F. HUEBYCH

liND

THOMAS

D. STEPHENSON

Examination Findings.-The mandibular left deciduous cuspid and lateral incisor teeth were missing. In the area of the missing teeth there was noted a firm, yellow-pink mass, approximately 1 by 1.5 cm. in diameter (Fig. 2). This smooth, raised tumor extended from the lingual aspect of the alveolar crest laterally to the depth of the mucobuceal fold, causing a bulging of the labial cortical plate of the mandible. On palpation, moderate crepitation of the labial cortical plate was apparent.

Fig.

I.-Intra-oral

roentgenograms

showing the tumor months oltl.

Fig. Z.---Preoperative.

initially

photograph

when

the patient

was

YZ

of tumor.

A complete roentgenographic survey of the skull was negative for bony pathology except for the tumor mass in the body of the left mandible. A well-demarcated, multilocular radiolucent area, approximately 2 by 3.5 cm., was noted. This lesion extended from the alveolar crest inferiorly to the superior border of the inferior alveolar canal and mental fora.men (Fig. 3). Distally, it encroached on the mesial root of the first deciduous A mandibular molar and extended mesially to include the left deciduous central incisor. oeelusal roentgenogram revealed the lingual cortical plate to be intact, but the labial cortical plate was extremely thin and expanded laterally approximately 0.5 cm. The child

was admitted

to the hospital

on his third

birthday,

NOV. 23, 1954.

General Physical Findings.-The general physical findings were essentially negative (2) an with the following exceptions : (I) Grade 1. soft apical systolic cardiac murmur; shotty cervical lymph nodes with no apparent oral temperature of 99.8” F.; (3) bilateral tenderness; (4) several soft, nontender lymph nodes palpable in the right axilla; and (5) the previously described oral findings.

RECURRENT

A~~ELOBLASTIC

FIBROMA

IN

S-WEAR-OI~D

BOY

iO!)

Laboratory Findings.-Laboratory investigations were within the limits of normal, except for the white blood count which revealetl 12,000 leukocytes, of which 34 per cent were polymorphonuclear neutrophils, 62 per cent lymphocytes, and 4 per cent, eosinophila. Consultation with the Medical and Pediatric Services wau sought, because of thr The child was clearer1 for surgery, the lympho results of the differential leukocyte count. cytosis being explained on the basis of a recent upper respiratory infection episode.

1%. :i.-Intra-

Treatment

and extraoral Irnited

Naval

and Course.-The

one day preoperatively oral

roentgenograms

States

and continued

The anesthesia employed endotracheal int,ubation.

Hospital

OIYturnor

r?t the

when the patient itge

of

:X1 months.

was blnwht

patient was placed on parenteral procaine through the fifth post.operative day.

was nitrous oxide-ether-oxygen The right mandibular deciduous

to Ihe

penicillin

administered through an central incisor and left

710

R.4YMOND

B.

HUERSCH

AND

‘L’HOMi\S

D. STEPHENSON

mandibular deciduous first molar were extracted. An incision was made around the gingivae of the teeth ant1 along the alveolar crest from the seeontl tleciduous molar to the htterxl incisor. Ot)tiquc distal sitlc of the! rigtlt incisiotis tllcw wwc Jllatk 011 the lingual, ~uccal, and labial snrfacc+ of the mandil)le ~IOU’JI to the? reflection of the mncobnccal fold. The mucoperiostexl flaps were rctracte~l on the buwal mcl lingual sitles to expose the inferior bortler ant1 entire circrrmfrrenctt of the body of the mandible. With chisels and a dental handpiece with bone burs and diamond stones, a rectangular block of the body of the mandible was removed in one piece. This included the labial and lingual cortical plates down to the level of the inferior aspect of the inferior alveolar canal, leaving approximately 0.5 cm. of the inferior cortical border of the mandible intact. The specimen included the deciduous right lateral incisor and the uneluptetl permanent tooth butls of was obtainc~l the left first premolar a,nrl the right central incisor (Fig. 4). Hemostasis 1)~ placing Gelfoam saturatetl in a throml)in soluCion in the cavity over the bleeding points.

Ii.

A.

Fig.

4.-Photograph

of

#ross

sgecirrwn.

J,inxrml

an11

huccolahial

views.

(Ruler

in inches.)

Then, freeze-dry canc*c~llous INJIW (*hips, r~~constitutc~~l IvitlL normal saline antI 600,000 units of Tlrr soft tissLW aqueous crystalline p&cillin were placed into tlie 0pVrativc defect. flaps were trimmed, approximatetl closely, and sutured with 0000 chromic catgut sutures. The postoperative course was relatively uneventful. On the evening of the day of the operation the patient ran a rectal temperature of 100.8’ F., becoming afebrile on the morning of the first postoperative day. At this time the patient began taking liquids bJ On the fifth postoperative day he was discharged from mouth and appeared comfortable. The postoperative roen tgenogram is the hospital, cheerful and eating soft foo~ls well. shown in Fig. 5. The boy was followed at weekly- intervals for one month anfl then at monthly inThe bone graft appeared to have taken and roentgenographic examination in tervals. March, 1955, revealed consolidation of the chips and evidence of osteogenesis (Fig. 6). A space-maintaining partial denture was constructed in March, 1955, producing excellent fnnctional and esthetic results (Fig. 7). There was no evidence of recurrence through the sixth postoperative month.

Fig. 5.

Fig. chip Fig. and

5.7 -Hot s. 6.- -ROC wit dent

mtg mtg e 01

cha :ion

712

RAYMOND

3’. HIXBSCH

;IND

THOMAS

D. STEPHENSON

Pathology Report.-“There is very cellular, fibrous, connective tissue in which are islands and strands of odontogenic epithelium. The nuclei of the connective cells are irregular in shape and hyperchromatic. The prolit’erating fibrous tissue main component of the neoplasm. There is an adequate margin of normal tissue directions in the specimen submitted. ” (Captain R. A. Colby, DC, USN, TJnited Naval Dental School, National Naval Medical Center, Bethesda, Varyland.)

Diagnosis: “ Ameloblastic

fibroma,

mandible.

there tissue is the in all States

”

B. Fig.

7.-A,

Photograph

of

operative

site partial

four months denture.

postoperatively.

R,

Photograph

of

Discussion An ameloblastoma is a tumor primarily derived from ectodermal odontogenie epithelial tissue, possessing, to a greater or lesser degree, a mesodermal

RECURRENT

AMEI,OBLASTIC

VIBR(lMA

IS

:I-YEAR-OLD

BOY

7 1:;

The ameloblastic fibroma is classified by Thoma’ a.s a. mix4 odontogenic tumor of both ectodermal and mesodermal origin, the predominant component being fibroma. The five most commonly accepted possibl(L source of origin for ameloblastic tumors are: (1) from epithelial cell rests of Hertwig’s sheath or the debris of Malasscz in the adult periodontal rnc>tnbrane and marrow spaces; (2) from genetic disturbance of the tissue forming the enamel organ; (3) from epithelial proliferations in odontogenic cysts; C-411 from the oral surface epithelium; and (5) from displaced epithelium with odontogenic propensities in other regions of the body, such as the tibia, h,vpophysis, and ovary. Tncidenee appears to be almost equally distributed between the WSW. slightly more common in the male. The majority of the cases have bf>etlr NJport,ed as occurring in patients between the ages of 10 and 35 years. Numot~ous cases, however, have been reported as late as the sixt.h and sevent,h decades of life. The mandible appears to bc a more common site than the maxilla. Due to the slow growth and delayed detection of t,he tumor, the report,ed apt incidence may tend to be misleading. Diagnosis is positive only on confirtnation by microscopic exa.mination. Roentgenographic differential diagnosis is of limited aid. A history of a slowgrowing, expansive tumor of the jaws, rsprcially in cases of noneruption oi deciduous or permanent tect,h, should make the clinician suspicious of the possibility of an ameloblastic neoplasm. Prcoperativc biopsy. therefore, is OF invaluable aid in treatment planning. Opinion concerning the classification of the arncloblastorua as t.o relaiiy(b benignity or malignancy is widely varied. Perhaps the most feasible vicbwpoint places the ameloblastoma on the border line between a benign and a malignant Itsion, benign in that it rarely ltlctastasizes and ma.lignant it-t that, it almost always recurs if not excised widely enough. Although roentgenographic and clinical examination ma.y lead to the bclicf that the lesion is wtlll demarcated, microscopic examination of bone immediately adja.rent to the t8umor will show amPloblast,ic rclls within the tltarrow spaces of apparrntly clinically sound boric. There are a frw well-authenticated cases on rcc~rtl that demonstrate the ability of the amcloblastnnl;l to mrtastasizc to regional lymph nodes and to more distant st,ructurcs. Treatments proposed have ranged from simple ~~nuclt~ation to htGsc4on or radical “en bloc” procedures. Enuclcation appears t.o be the least, S~WPSSful treatment, resulting in the greatest nultlbt~r of recurrences. Whilcb radical excision of the lesion, togethe t* with a grnc~~ms portion of su~8rounding strut.turcs, docas result in a high number of CWPS, thtl opc>ra.tor is faced with the problems of gross disfigurement. Tt is felt that where there remains sufficient sound bone surrounding the lesion, which has neither mctastasizrtl IIO~ invaded the soft tissues, the treatment of choice is peripheral osteotomy 01’ marginal resection (excising the lesion together with 0.5 to 1 cm. of clinica,lly normal surrounding bone), thus removing the invasive epithelial pegs with the main tumor mass and resulting in thr least possible deformity consistent with a&yuate removal. c0mp011e11t.

Summary Treatment of choice in an early, operable ameloblastic tumor of the jaws appears to be marginal resection. Removal of an adequate margin of clinically sound bone, maintenance of bony continuity if possible, and avoidance of radical disfiguring surgical procedures present humane treatment consistent with minimal chance of recurrence. Early discovery, histopathologic examination of biopsied tissue for positive diagnosis, and adequate excision are the keynotes of successful therapy for ameloblastic tumors of the jaws. References of the Jaws, ORAL SURG., ORAT, MEL)., 1. Small, I. A., and Waldron, C. A.: Ameloblastoma AND ORAL PATH. 8: 281-297, 1955. 2. Thoma, K. H.: Oral Pathology, ed. 3, St. Louix, 1950, The C. V. Mosby Company, 1’1). 1273-1292. 3. Thoma K H * Oral Pathology, ed. 2, St. Louis, 1952, The C. V. Mosby Company, pp. li93-i3I8’ and 1337-1351. Tumors of Bone, Am. .J. Cancer 26: 702-707, 4. Geschickter, C. F., and Copeland, M. M.: 1936. .?. Ewing, J.: Neoplastic Diseases, ed. 4, Philadelphia, 1940, W. B. Saunders Compan.v, pp. 770-776. Pathology of Tumors, St. Louis, 1948, The C. V. Mosby Company, pp. 6. Willis, R. A.: 308-315. in a 3-Year-Old Roy, ORAL SURG., 7. Hunter, H. A., and Nikiforuk, G.: Ameloblastoma ORAL MED., AND ORAL PATH. 7: 906, 1954. in Relation to Tooth Development, Australian J. Dent. S. Manley, E. B.: Adamantinoma 58: 137-150, 1954. of Ameloblastomas, ORAL SXJRG.,ORAL MED., ANI) 9. Aisenberg, Myron S.: Histopathology ORAL PATH. 6: 1111-1128, 1953. 10. Villa, V. G.: A Case of Ameloblastoma Derived From Adult Oral Epithelium, ORAL SIJRG., ORAL MED.! AND ORAL PATH. 6: 1216-1258, 1953. Dentlgerous Cyst With Ameloblastoma, ORAL SURG., ORAI, Mm)., A~‘I) Il. Bailey, J. W.: ORAL PATH. 4: 1122-1126, 1951. 12. Ward, G. E.: Tumors of the *Jaws, ORAL R:RG., ORAL MEU., AND Oaar, PATII. 5: 675-704, 1952.

13. Karsner H T * Human pp.‘370-37.1.

Pathology,

ccl. 7, Philatlelphia,

1949, J. B. J,ippiucott

Company,