Central mucoepidermoid carcinoma in a child

512

CENTRAL

be avoided and appropriate should be obtained.

preoperative

consultation

CARCINOMA

IN A CHILD

2. Goeke .I. Hagan D. Goelzer S. et al: Lethal catatonia complicated by the development

References I. Castillo E. Rubin R. Holsboer-Trachsler

E: Clinical differentiation between lethal Catalonia and neuroleotic malignant svndrome. _ Am J Psychiatry 146:3. 1989

J Oral Mmllofac

MUCOEPIDERMOID

of neuroleptic malignant syndrome in a middle-aged female. Crit Care Mcd 19:I I. I99 I 3. Mann S. Caroff S. Bleier H. ct al: Lethal catatonia. Am J Psychiatry l43:II. 1986 3. Levenson .I: Ncuroleptic malignant syndrome. J Psychiatry 142: IO. 1985 5. Pearlman C: Neuroleptic malignant svndromc: ,A review 01‘thc literature. J Clin Psychiatrq 6:5. I986

Surg

52:512-515.1994

CentraI Mucoepidemoid in a Child

Carcinoma

iZSl&, MD, DDS, MS, FDSRCS (ENG), FRCS (ED),* ADRIAN W. SUGAR, BCHD, FDSRCSJ MARTIN A.P. MILLING, MA, FRCS (ENG & ED),* AND KEITH F. ASHLEY, BDS, FDSRCS (ENG & ED), FRCS (ED)§ ANDRAS

Mucoepidermoid carcinoma of the salivary glands is a well-known entity, representing 5% to 8% of all salivary tumors. ‘U However, central (primary intraosseous) mucoepidermoid carcinoma of the jaws is relatively rare, with only around 80 cases having been reported in the literature. the overwhelming majority occurring in the fourth and fifth decades of life. Central mucoepidermoid carcinoma in children is very rare. A case is reported that is only the sixth ever described in a child under the age of 16.

Report of Case An 1 l-year-old girl was referred for treatment to the Department of Oral and Maxillofacial Surgery. St Lawrence Hospital, Cheapstow, England. after a routine panoramic radiograph by an orthodontist (Fig I) showed a multilocular radiolucent lesion of the left angle of the mandible. There were no symptoms. but thorough clinical examination dem* Registrar in Oral and Maxillofacial Burns.

Plastic

and

Maxillofacial

Surgery. Welsh Centre for Surgery, St Lawrence Hospital.

onstrated buccal and lingual expansion of the mandible with no breach of the oral mucosa or lymph node involvement. The initial differential diagnosis included odontogenic cyst and ameloblastoma. A biopsy was performed before referral at County Hospital, Hereford. England. Two opinions about the diagnosis from general pathologists were clear-cell ameloblastoma and ameloblastic hbroma. However. two oral pathologists independently considered that the lesion was central mucoepidermoid carcinoma of the mandible. They thought that the general pathologists had been misled by the presence of the tooth germ of the third molar in one of the specimens. Computerized tomographic and magnetic resonance imaging scans (Fig 7) showed an expanding lesion of the left angle of the mandible that extended to the cortex but left it intact. It was difficult to determine whether the changes seen on the medial aspect of the mandible. extending to the tonsillar fossa. represented tumor invasion or postbiopsy surgical changes. Radical excision of the tumor. including the left mandible distal to the canine. left tonsillar region, and half of the soft palate, was performed. The condyle and the coronoid process were preserved. Suprahyoid node clearance was also performed on the affected side. Frozen sections examined by an oral pathologist confirmed that all of the margins were clear.

Chepstow. Gwent. United Kingdom. t Consultant Oral and Maxillofacial Surgeon. Welsh Centrc for Burns, Plastic and Maxillofarial Surgery, St Lawrence Hospital. Chepstow, Gwent, United Kingdom. $ Consultant Plastic Surgeon Welsh Centre for Burns, Plastic and Maxillofacial Surgery. St Lawrence Hospital. Chepstow. Gwent. United Kingdom. § Consultant Oral and Maxillofacial Surgeon. Department of Oral and Maxillofacial Surgery. County Hospital. Hereford. United Kingdom. Address correspondence and reprint requests to Dr Ezsias: Welsh Centre for Burns. Plastic and Maxillofacial Surgery. St Lawrence Hospital. Chepstow, Gwent NP6 5YX. UK.

0 1994 American Association of Oral and Maxillofacial Surgeons 027%2391/94/5205-0017$3.00/O

FIGURE I. Panoramic radiograph showing lucent lesion of left angle of mandihlc.

a multilocular

radio-

EZSIAS

ET AL

FIGURE 7. .I, Computerized tomographic scan showing expanding ill-defined soft tissue lesion without indication of invasion.

Reconstruction of mandible was with a free nonvascularized. full-thickness iliac crest bone graft prepared with the aid of a template and held in position with an A0 reconstruction plate. This was covered orally with a fasciocutaneous radial forearm free flap (Figs 3-5). The histology of the resected specimen (Fig 6) satisfied the criteria for central mucoepidermoid carcinoma of the mandible. Both periodic acid-Schiff (PAS) and PAS-diastase stains demonstrated the presence of mucous within the cystic spaces and in mucous-secreting cells. Orthodontic treatment, including the prevention of overeruption of the maxillary teeth opposite the surgical site. commenced 10 months after surgery. At 4 years after surgery the patient remains free from clinical signs of recurrent disease.

lesion of the left angle region. B. Magnetic

resonance

image demonstrating

reviewed the literature and added a few cases.3.h-9 The last thorough review was undertaken by Grubka et al in 1983,“’ who found 38 reported cases in the mandible. Total cases were approaching 60 in 1984,’ ’ another 2 1 cases having been reported since then.“.” The ratio between mandible and maxilla is roughly 2: I, the most frequent site of occurrence being around

Discussion The intraosseous presentation of mucoepidermoid carcinoma is quite rare. The first detailed description and histologic analysis of mucoepidermoid carcinoma in the salivary glands was given by Stewart et al in 1945,’ who regarded it as a well-defined separate entity and used the term of mucoepidermoid to describe the two basic histologic features of these tumors. In 1963 Bhaskar’ reported two central mucoepidermoid carcinomas of the mandible, claiming that no such lesions were previously described. However, Hertz4 not only reported his four cases, but also found six previous reports. He. as well as Linnel’ credited Lepp as being the first to describe the mucous-secreting intraosseous tumors of the jaws in 1939. A number of investigators

FIGURE 3. Radial forearm free flap covering the bone graft. which is held by a large A0 reconstruction plate.

514

CENTRAL MUCOEPIDERMOID

CARCINOMA IN A CHILD

FIGURE 4. Radiograph of the mandible with A0 reconstruction plate and bone graft.

the third molar-angle region of the mandible. The female to male ratio is the same as the mandible maxilla ratio (2: 1). Eversole et al3 found that nearly 50% of the central mucoepidermoid carcinomas are associated with dental cysts or impacted teeth. The overwhelming majority of the cases were found in the fourth and fifth decades. Occurrence of central mucoepidermoid carcinomas in children is exceedingly rare and we have found only five reported cases in patients under the age of 16.‘.4.9,‘7.‘8Our patient, the sixth case, is the third youngest (Table 1). The pattern of the occurrence also seems to be somewhat different in childhood. Although the female to male ratio is the same as in adults (2: I), the mandible to maxilla ratio is 1: 1 in children. It is noticable that only one case has been reported in the first decade: thus it is unlikely that the etiology can be a developmental disturbance or that central mucoepidermoid carcinomas might be teratomas. It is also interesting that these tumors, although exceedingly rare, tend to crop up at puberty. which might suggest a hormonal influence. Hormones can have a marked effect on mucous-producing glands, including salivary glands. The criteria for diagnosis of intraosseus mucoepidermoid carcinoma are9.17:1) presence of intact cortical plates: 2) radiographic evidence of bone destruction;

FIGURE 5. Well-healed free radial forearm flap intraorally 2 months postoperatively.

FIGURE 6. Photomicrograph showing large. clear, mucus-secreting tumor cells intermixed with squamoid elements, a characteristic feature of mucoepidermoid carcinoma (hematoxylin-eosin stain, original magnification X40).

3) histologic confirmation; 4) positive mucin staining: 5) absence of a primary lesion in the salivary gland: and 6) exclusion of an odontogenic tumor. The pathogenesis of this tumor is still unknown. Bossart,” Linell,’ Barla-Szab6,20 and Alexander et al8 suggest a derivation from the oral epithelium which secondarily invades bone. Others’ believe in the inclusion of mucosal glandular tissue. Four possible origins have been considered7.’ ‘: 1) entrapment of retromolar mucous glands within the mandible, which later undergo neoplastic transformation: 2) embryonic remnants of the submandibular and sublingual glands trapped within mandible during development: 3) neoplastic transformation and invasion from the lining of the maxillary sinus: or 4) neoplastic transformation of mucous-secreting cells from the epithelial lining of dentigerous cysts associated with impacted (unerupted) third molars. Recurrence is common. Browand and Waldron” in 1975 quoted a recurrence rate of 26%. while Brookstone and HUVOS’~ in 1992 found 40% for conservative treatment by enucleation and 13% for radical excision. Metastases are rare, but not unknown, and usually are confined to regional lymph nodes.“,” However. Lebsack et alI6 recently ( 1990) reported a distant metastasis in the ipsilateral clavicle. Brookstone and HUVOS’~ suggested a new clinical staging for all central salivary gland tumors, including central mucoepidermoid carcinoma, that is based on the condition of the overlying bone rather than the tumor size. The World Health Organization classification,” after giving a detailed histologic description and discussing degree of differentiation, recognizes that these tumors may infiltrate locally and sometimes metastasize. They changed their earlier recommendation of the term “mucoepidermoid tumor” to “mucoepidermoid carcinoma.” Although this new recommendation was only made in 1991, many investigators had already been using the term mucoepidermoid carcinoma.‘“~“~‘3~‘6

515

EZSIAS ET AL

Table 1.

Reported Cases of Central Mucoepidermoid Tumors

Year Reported (Year Discovered)

Investigator

Age (yr)/ Sex

Site

Symptoms

in Children

Radiograph

Treatment

Follow-up

1952 (1928)

Hertz4

15/F

Hard palate

Swelling

Not given

Curettage

1961(195X)

Chaudry et aI”

16/F

Palate

Swelling

Negative

Partial maxillectomy

1963

Bhaska?

Swelling

Cystic lesion

Marsupialization, later excised

1975

Browand and Waldron’

15/M

Mandible, posterior body Maxilla

Swelling

En bloc excision

I2 years no

1992 1993 (1989)

Brookstone and Huvos” &siSs et al (this report)

IO/M II/F

Mandible body Mandible angle region

Not given Incidental finding

Multilocular radiolucency Osteolytic lesion Multilocular radiolucency

Radiotherapy En bloc resection

recurrence Lost to follow-up 4 years no recurrence

I/F

Waldron and Mustoe” recommended that “intraosseous mucoepidermoid carcinoma” be included in the primary intraosseous carcinomas of the jaw as type 4. We consider, along with others,8*“.‘2.‘4 that radical surgery with en bloc resection is the best course of treatment in view of the high incidence of recurrence after simple enucleation. References I. Stewart F’W. Foote FW. Becker WF: Muco-epidermoid 2.

3. 4. 5.

6. I. 8. 9. 10.

tumors of salivary glands. Ann Surg 122:820. 1945 Bhaskar SN: Central mucoepidermoid tumors of the mandible. Cancer 16721, 1963 Eversole LR, Sabes WR, Rovin S: Aggressive growth and neoplastic potential of odontogenic cysts. Cancer 35:270, 1975 Hertz J: Mucus-secreting tumours of the jaws. Acta Chir Stand 103:276, 1952 Linell F: Mucus-secreting and cystic epidermoid carcinomas of mucous and salivary glands. Acta Path01 Microbial Stand 25: 801, 1948 Smith RL. Dahlin DC. Waite DE: Muco-epidermoid carcinomas of the jawbones. J Oral Surg 26:387, 1968 Dhawan IK. Bhargava S, Nayak NC, et al: Central salivary gland tumors of jaws. Cancer 262 11, 1970 Alexander RW, Dupuis RH, Holton H: Central mucoepidermoid tumor (carcinoma) ofthe mandible. J Oral Surg 32:54 1, 1974 Browand BC, Waldron CA: Central mucoepidermoid tumors of the jaws. J Oral Surg 40:63 I, 1975 Grubka JM. Wesley RK, Monaco F: Primary intraosseous mucoepidermoid carcinoma of the anterior part ofthe mandible. J Oral Maxillofac Surg 4 I :389. 1983

Died 16 years later from repeated recurrences 3 years no recurrence Not given

I 1. Gingell JC, Beckerman T, Levy BA, et al: Central mucoepidermoid carcinoma. Oral Surg 57:436, 1984 12. Pincock JL, El-Mofty SK: Recurrence of cystic central mucoepidermoid tumor of the mandible. Int J Oral Surg 14:81, 1985 13. Grltz KW, Hadjianghelou 0: Prim& enossales Mukoepidermoidkarzinom im Kiefer-Gesichts-Bereich. Fortschr Kiefer Gesichtschir 31:81, 1986 14. Tornes K. Bang G, Moller P: Central mucoepidermoid tumour of the mandible. J Cranio-Max Fat Surg 15:38, 1987 15. Panzoni E, Nardi P. Ficarra G, et al: Tumore mucoepidermoide centrale della mandibola. Presentazione di un case. [Central mucoepidermoid tumour of the mandible. Presentation of a case.] Minerva Stomatol 38:4 17, 1989 16. Lebsack JP, Marrogi AJ, Martin SA: Central mucoepidermoid carcinoma of the jaw with distant metastasis. J Oral Maxillofac Surg 485 18, 1990 17. Brookstone MS, Huvos AC: Central salivary gland tumors of the maxilla and mandible: A clinicapathologic study of 11 cases with an analysis of the literature. J Oral Maxillofac Surg 50:229, I992 18. Chaudry AP, Dedolph TH, Vickers RA: Muco-epidermoid tumor arising from ectopic salivary glands in the maxilla: Report of case. J Oral Surg 19:521,1961 19. Bossart A: Zur Kenntniss einer ungewiihnlichen epithelialen Kiefergeschwulst (Hypemephroides Adamantinom). Schweiz Monatsschr Zahnheilkunde 55:9 1, 1945 20. Barla-Szabo L: Muco-epidermoid tumours of the oral cavity. Acta Morph01 Acad Sci Hung 5: 133, 1955 2 1. Seifett G, Sobin LH: Histological typing of salivary gland turnouts, in International Classification of tumours (ed 2). New York, NY, Springer-Verlag, 199 1 22. Waldron CA, Mustoe TA: Primary intraosseous carcinoma of the mandible with probable origin in an odontogenic cyst. Oral Surg Oral Med Oral Path01 67:716, 1989