Rhabdomyosarcoma of the oral and paraoral region

Rhabdomyosarcoma of the Oral and Paraoral Region

EiDMUND PETERS, DDS, MSc,* MARK COHEN, BDS, MDENT,t MARIO ALTINI, BDS, MDENT,' AND JILL MURRAY, MB BCH, FFPATH(SA)*

Oral rhabdomyosarcoma (RMS) was studied by analysis of eight such cases which presented over a 25- year period. Rhabdomyosarcoma was the fourth most common oral sarcoma (7.5%) from this period after osteosarcoma (32%), fibrosarcoma (19%), and chondrosarcoma (9%). In patients younger than 20 years, RMS was the second most common sarcoma (six cases) after osteosarcoma (ten cases). Combined analysis with 113 further cases documented in the literature showed the majority of cases (71.2%) were embryonal. The alveolar subtype was considered a distant second in frequency (12.3%) even though the pleomorphic subtype was apparently more common (16.4%). However, the pleomorphic cases were diagnosed before histologic criteria were established to discern this entity from other pleomorphic sarcomas and this data is probably unreliable. Site predilections were found for the soft palate, maxillary sinus and alveolus, posterior mandibular region, cheek and lip and possibly tongue. The gingiva and floor of mouth were uncommon sites. There was a predilection for occurrence in the first two decades with a decline in the third decade. Documentation of recent cases treated with a multidisciplinary approach indicated that lesions in the oral soft tissues have a good prognosis; 17 of 21 such cases showed no evidence of disease after a mean follow-up period of 7.2 years (SD = 4.4). In contrast, four of five cases in the posterior mandible resulted in death after a mean period of 1.1 years (SD = 0.3).

Cancer 63:963-966, 1989.

HABDOMYOSARCOMA (RMS) is the most common R soft tissue sarcoma in infants and children. It has a predilection for the head arid neck area, the genitourinary area and rlatroperitoneum and to a lesser extent, the ex- tremities.' In spite of the head and neck predilection, oral presentation has generally lbeen dealt with in case reports, in relatively small series aa as isolated examples within larger head and neck series and consequently, has not been well characterized. This study develops a profile of oral RMS from analysis of a series of eight such cases which presented at our institution and from the cumu- lative experience of 1 13 further cases documented throughout the literature.

Materials and Methods The relative frequency of RMS with respect to the total

cases of sarcoma in the oral cavity was assessed after a review of cases over a 25-year period from the files of the Department of Oral Pathology, University of the Wit- watersrand (Johannesburg,, South Africa) and the South

From the *Department of Oral Pathology and the $Department of Anatomic Pathology, School of 'Pathology, South African Institute for Medical Research and University of the Witwatersrand, Johannesburg; and the ?Division of Maxillofacial Surgery, Department of Surgery, Uni- versity of the Witwatersrand, Johannesburg, South Africa.

Address far reprints: Edmund ]Peters, DDS, MSc, Department of Oral Pathology, University of the Witwatersrand, Wits 2050, Johannesburg, South Africa.

Accepted for publication August 25, 1988.

African Institute for Medical Research. Hematoxylin and eosin-stained sections were available for all cases. Further sections of the suspected cases of RMS were recut, where necessary, and stained with periodic acid-Schiff (PAS)/ diastase to assess for glycogen, phosphotungstic acid hematoxylin (PTAH) to help identify cross-striations and the immunoperoxidase method (Vectastain ABC Kit, Vector Laboratories, Burlingham, CA) to assess for myoglobin. Only cases which were histologically classical of one of the four major histologic variants as originally defined by Horn and Enterline2 and described by Enzinger and Weiss' were accepted. This classification includes alveolar, embryonal, and pleomorphic subtypes; the fourth variant, the botryoid RMS, refers to the distinctive ap- pearance of an embryonal RMS within a hollow viscus. The demonstration of myoglobin or the presence of cross- striations in the appropriate histologic contexts were considered confirmatory but not essential for diagnosis except in the distinction of pleomorphic RMS. The presence of glycogen was considered strongly supportive evidence. The cases were independently diagnosed by at least two pa- thologists. Cases which could not be independently confirmed were classified as sarcomas of undetermined his- togenesis possibly RMS.

The clinical presentation was analyzed according to site, age, sex, race, stage, and survival.

Rhabdomyosarcoma cases from the literature which presented in the oral and paraoral region were reviewed

963

964 CANCER March 1 1989 Vol. 63

TABLE 1 . Clinical and Histologic Data of the Eight Confirmed Cases

Age/ Case Date sex Race Site SubtvDe Stage Treatment NED DOD

1 1972 20/M B Right angle, mandible E * * * * 2 1973 12/M A Right mandible, AL *

retromolar area

3 1977 I/M B Left cheek E 1 1 1 Chm, Dxt 10 yr

4 1979 16/M W Right posterior AL 1 1 1 Surg, Chm, Dxt mandible

5 1980 22/F B Right mandible, E 1 1 1 * alveolus

* *

0.8 yr

* *

6 1982 19/M B Left cheek and maxilla E 1 1 1 Chm, Dxt 2 yr recurrence: 1984

l 1984 15/F B Left maxillary sinus AL 1 1 1 Chm, Dxt and alveolus

and infraorbital area 8 1986 46/F B Right buccal fold, cheek AL 1 1 1 Chm, Dxt 1 Yr

0.6 yr

NED no evidence of disease; DOD died of disease; M male; F female; B: black; W white; A: Asian; E: embryonal; A: alveolar; Surg: surgery;

and categorized in a similar way. These cases were often culled from larger head and neck series or from articles attempting to illustrate tangential points, thus the data was not complete in all cases. In cases taken from head and neck series, a tabulation of the total cases was main- tained to assess the relative frequency of the oral cases. In some publications, the same or overlapping cases were utilized for different analyses3-’; only cases which could be clearly defined as unique were accepted in this study. The sites which were assessed included the cheek and lip, floor of mouth, gingiva, mandible, maxillary sinus and alveolus, palate and tongue.

Results

Analysis of Case Series

A total of 107 cases of sarcoma presenting in the oral cavity were found in our archival materiak the most common was osteosarcoma (32%) followed by fibrosarcoma (19%) and chondrosarcoma (9%). There were eight confirmed cases of RMS (Table 1) and a further four cases in which a primitive embryonal RMS was considered the most likely diagnosis as well as a single highly suggestive case of pleomorphic RMS which presented within the mandible. This case was not accepted because of the in- ability to demonstrate cross-striations. Further tissue sections were not available to assess for myoglobin which may have been confirmatory. The eight confirmed cases represented the fourth most common oral sarcoma (7.5%). A wide spectrum of sarcomas including three ameloblastic sarcomas comprised the remaining cases.

Histologically, the cases of RMS could be sub-typed as alveolar (four) and embryonal (four). The assessment of

Dxt: radiotherapy; Chm: chemotherapy. * Insufficient information.

special features supported the experience of previous Glycogen could be demonstrated in all cases;

cross-striations could be found in two cases (Cases 3 and 6), both embryonal; myoglobin was demonstrated in five cases (Cases 3 through 8).

There was a male predominance of five to three and a relatively high mean age of 19.6 years (SD = 10.9) with a range from 7 to 46 years. Six cases occurred in patients 20 years or younger and were exceeded only by cases of osteosarcoma (ten) in this group. The RMS cases generally presented in Stage 3 (Table 2). A further unexpected finding was that two of these cases (Cases 4 and 5) were central within the posterior mandible and two more (Cases 1 and 2) presented in the paramandibular soft tissues and posterior mandible.

Survival data is analyzed in conjunction with other documented cases in the next section.

Combined Analysis of Cases Reviewed in Literature and Case Series

A total of 1 13 RMS cases involving the oral and paraoral region were found in the l i terat~re .~~” Seventy-seven of these were derived from head and neck series which

therefore represented 19.5% of this total. The combined data with our series for a total of 12 1 cases is summarized as follows:

The majority of cases were embryonal (52), pleomorphic cases (1 2) were apparently the next most common and alveolar cases (nine), the least common.

Information concerning age was available in 70 cases; a mean age of 14.0 years (SD = 9.9) and median of 12

cumulatively totalled 394448.1 1,13,17,21,22,25. , the oral cases

No. 5 OIRAL AND PARAORAL RHABDOMYOSARCOMA - Peters et al. 965

years was found. The data graphically represented in Fig- ure 1 shows most cases distributed in the first two decades with a progressive decline into the third decade.

There was a male predilection of 36 to 24. The soft palate was invollved in 29 cases followed by

the maxillary sinus (23 cases), the posterior mandibular region (21 cases), the cheek and lip (21 cases), and the tongue ( 1 6 cases). The gingiva (three cases) and the floor of mouth (three cases) were uncommon sites. In three cases the lccation was stateid simply as mouth.25 In two cases, the data did not pennit assignment to a specific site.27

Survival in cases documented after 1968 which were treated with a multidisciplinary approach showed 19 of 2 1 patients with soft tissue lesions were alive after a mean follow-up time of 6.8 years (SD = 4.3) and 17 of these 19 showed no evidence of disease after treatment after a mean follow-up time of 7.2 years (SD = 4.4)."-13,'7720,26+27 I n contrast, fclur of five patients with lesions involving the posterior mandible died after a mean survival of 1 . 1 years (SD = 0.3),l1,14,19,24

Discussion

Site predilections within the head and neck have been defined for individual anatomic structures or for various anatomical area^'.^^'^'; the different organizations of data reflect recognition of the influence which anatomic site has on the biologic behavior of RMS and represent con- tinuing attempts to determine clinically useful organising principles. 'There is general (agreement that the orbit is the most commonly affected site. 1 9 2 8 3 2 9 The nasopharynx and middle ear :regions follow in frequency"28 although at least one major study suggests the soft tissue of the neck may be the second most commion site.6 The data from this study indicates that oral manifestations may occur in about a fifth of cases.

A site predilection within oral regions has not been well established; some authors have stated that the predomi- nant site is the palate followled by tongue?8 whereas others indicate the: tongue is the mlost common site.24 Our results do not show a pronounced site predilection although pal- atal involvement was somewhat more common and gingiva and floor of mouth were unusual locations.

The frequent presentatiton in the posterior mandibular region, including apparently, several intraboney cases,8'10,14,'6,19,24,30 is noteworthy; this does not appear to have been ,recognized as a relatively common site within the mouth. Some of the intraboney cases could represent extension from soft tissue into bone or metastatic deposits from occult primary sites; however, there should not be theoretical objections to primary lesions. Rhabdomyo- sarcoma is considered to result from malignant change of primitive mesenchymal (cells rather than differentiated

TABLE 2. Staging of Rhabdomyosarcoma (According to the Intergroup Rhabdomyosarcoma Study)

1 . Localized disease, tumor completely resected, regional nodes not

2. Localized disease with microscopic residual disease or regional

3. Incomplete resection with gross residual disease 4. Metastatic disease

involved

disease with or without microscopic residual disease

muscle. 13,22 Thus, histologically typical RMS has been described in other areas where striated muscle is absent such as the common bile duct or the urinary bladder.' A further case of multifocal RMS occumng in bone marrow, which suggests the pluripotentiality of this tissue, has been rep~rted.~ ' Our cases and the documentations in the literature suggest that mandibular lesions may not be an uncommon site of occurrence.

The histopathologic categorization of RMS into alveolar, embryonal, and pleomorphic subtypes is generally accepted albeit with reservations due primarily to overlapping histologic patterns. The need to adhere to unequivicol criteria has been emphasized; in some studies including ours, this has resulted in the reassignment of sarcomas from the clearly defined RMS categories into groups which indicate the lack of histologic differentiati~n.~?'~ Most of our cases were embryonal in keeping with the recognized predilection of this subtype for the head and neck area. The cases of pleomorphic RMS are probably considerably overestimated; all cases were derived from a single review article in 1962.6 The concept of the malignant fibrous histiocytoma, which would be a major differential diagnosis, had not been established at that time. It is possible these cases should be reclassified using current criteria. Even so, recent textbooks still indicate pleomorphic RMS

Age FIG. 1 . Histogram showing age distribution.

966 CANCER March 1 1989 Vol. 63

is a major histologic type in the oral region, especially the tongue.28 In this connection, if the cases of pleomorphic RMS are disregarded,6 the tongue would represent a relatively unusual location for RMS.

The mean age of 14.0 years derived from this study is comparable to a mean of 16.1 years found in a somewhat similar study in 1963, although these authors also found a median age of 6 years.’ Our results show a median of 12 years with most cases distributed in the first two decades and declining in the third decade. This data suggests that the pronounced predilection for children generally associated with RMS in the head and neck6332 may not occur to the same degree in the oral region.

The prognosis is influenced chiefly by the clinical stage and anatomic site of the tumor. Also, the alveolar subtype has been associated with a worse prognosis.8933 The use of combination surgery, chemotherapy, and radiotherapy has dramatically improved survival rates over the last 20 years. Disease-free survival periods of 9 1 % after 3 years have been published for Stage I, 11, and I11 patients with orbital lesions; prognosis is excellent even without surgery. Comparable survival rates of 75% for other head and neck areas and 45% for parameningeal sites have been established. The parameningeal sites, which include nasopharynx, nasal cavity, paranasal sinuses, pterygopalatine, and infratemporal fossa and middle ear have been associated with extension directly to the central nervous system (CNS) in 35% of cases within 1 year of diagnosi~.~~?~’ In this regard, the behavior of RMS within the specific anatomical site of the oral region has not been well defined. However, soft tissue oral lesions have been associated with an excellent progno~is,~’ a finding supported by this study. In contrast, lesions involving the posterior mandible had a markedly worse prognosis. The more successful man- agement of RMS with chemotherapy and radiation therapy has resulted in various distinctive oral stigmata. These are generally the result of interference with the developing dentition or jaws in children and include microdontia, hypoplastic enamel, underdeveloped roots resulting in delayed or premature exfoliation of the primary and secondary teeth, radiation caries, and micrognathia. 8,36,37

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Rhabdomyosarcoma of the oral and paraoral region

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