POSNICK ET AL 1009

6. Bjelle A: Cartilage matrix in hereditary pyrophosphate arthrop- athy. J Rheumatol 8:959, 1981

7. Terkeltaub RA, Ginsberg MH, McCarty DJ: Pathogenesis and treatment of crystal-induced inflammation, in McCarty DJ (ed): Arthritis and Allied Conditions. A Textbook of Rheu- matology (ed I I). Philadelphia, Lea & Febiger, 1989, pp 1691- 1710

8. de Bont LGM. Boering G, Liem RSB, et al: Osteoarthritis and internal derangement of the temporomandibular joint: A light microscopic study. J Oral Maxillofac Surg 44:634, 1986

9. Stegenga B, de Bont LGM, Boering G: Osteoarthrosis as the cause of craniomandibular pain and dysfunction: A unifying concept. J Oral Maxillofac Surg 47:249, 1989

10. de Bont LGM, Blankestijn J, Panders AK, et al: Unilateral con- dylar hyperplasia combined with synovial chondromatosis of the temporomandibular joint. Report of a case. J Maxillofac Surg 13:32, 1985

11. de Bont LGM, Liem RSB, Boering G: Synovial chondromatosis of the temporomandibular joint: A light and electron micro- scopic study. Oral Surg Oral Med Oral Path01 66:593, 1988

12. Pritzker KPH, Phillips H, Luk SC, et al: Pseudotumor of the temporomandibular joint: Destructive calcium pyrophosphate dihydrate arthropathy. J Rheumatol 3:70, 1976

13. Kamatani Y, Tagawa T, Hirano Y. et al: Destructive calcium pyrophosphate dihydrate temporo-mandibular arthropathy (pseudogout). Int J Oral Maxillofac Surg 16:749, 1987

J Oral Maxillofac Surg

50:1009-1015.1992

Recurrent Giant Cell Lesion of the Nasomaxillary Region in a Child:

Excision and Immediate Reconstruction With a Free Rectus Abdominis Muscle Transfer

JEFFREY C. POSNICK, DMD, MD,* HEATHER J. CLELAND, MBBS,t RONALD M. ZUKER, MD,* AND DAVID MOCK, DDS, PHD$

Giant cell lesions of the jaws are the most common benign jaw tumors in childhood,‘,2 yet they are poorly understood and their management is ill-defined. It is increasingly recognized that the innocuous reputation established for this tumor in the early literature3-5 does not always apply, but at present no histological markers of tumor behavior are available to assist clinicians in deciding on treatment. ‘3’ The difficulties are com- pounded when the lesion occurs in childhood and the likelihood of increased tumor aggressiveness’,6l7 must be weighed against the desire to avoid mutilating sur-

* Director, Craniofacial Program, Division of Plastic Surgery, De- partment of Surgery, University of Toronto and The Hospital for Sick Children: Faculty of Dentistry. University of Toronto. Toronto, Ontario.

t Division of Plastic Surgery, Department of Surgery, The Hospital for Sick Children, Toronto, Ontario.

$ Head, Division of Plastic Surgery, Department of Surgery, Uni- versity of Toronto and The Hospital for Sick Children, Toronto, Ontario.

5 Faculty of Dentistry. University of Toronto, Toronto, Ontario. Address correspondence and reprint requests to Dr Posnick: Cra-

niofacial Program, Division of Plastic Surgery, The Hospital for Sick Children, 555 University Ave. Toronto, Ontario, Canada M5G 1X8.

0 1992 American Association of Oral and Maxillofacial Surgeons

0276-2391/92/5009-0016$3.00/O

gery and unnecessary interference with growth in treating what remains an essentially benign condition.

A case is presented of an extensive, recurrent giant cell lesion of the maxilla in a 14-year-old girl that was managed with definitive excisional surgery and im- mediate free-flap reconstruction.

Report of a Case

In May 1988, a 16year-old girl began to experience nasal obstruction and complained of mouth-breathing and snoring. She was initially treated for sinusitis. In October 1988, a swelling of the hard palate was noted during routine dental examination. This lesion was biopsied under local anesthetic, and the histological report described a lesion consistent with giant cell granuloma. Computed tomography (CT) scan showed a large soft tissue mass in the nasal cavity on the right, with bowing of the nasal septum and medial wall of the right maxillary sinus. Tumor had invaded and destroyed the right side of the hard palate (Fig 1). The lesion was initially treated via a right lateral rhinotomy by removal of the soft tissue mass and curettage of the bony lesion.

Postoperative follow-up showed that the right central and lateral incisors and cuspid were devitalized and mobile. An axial and coronal CT scan in April 1989 showed some evi- dence of bony healing of the hard palate, but extensive ab- normalities persisted, with lytic lesions of the maxilla ex- tending from the right posterior molar region across the midline to the contralateral cuspid region (Fig 2). These ab-

1010 MANAGEMENT OF RECURRENT GIANT CELL LESIONS

FIGURE 1. Coronal and axial CT scans of nasomaxillary tumor as it initially appeared.

normalities were thought to represent residual/recurrent tu- mor. The right maxillary and ethmoid sinuses were opaque.

The patient was referred to us at The Hospital for Sick Children in Toronto, Canada. Examination showed a well- healed lateral rhinotomy scar and the dental changes previ- ously noted (Fig 3). The rest of the physical examination findings were within normal limits. Laboratory tests were completed, showing a serum parathyroid hormone level of 28 mg/L (normal range, 10 to 65), a serum calcium of 2.46 mmol/L (normal range, 2.75 to 2.70), and a phosphate level of 1.3 1 mmol/L (normal range, 0.87 to 1.52), all indicating normal parathyroid gland function. Review ofthe histological slides from the original biopsy and surgical specimens con- firmed a benign giant cell lesion. In view of the extensive destructive nature of the lesion and the ineffectiveness of pre- vious surgery, it was decided that definitive excisional surgery was indicated. The patient was therefore also evaluated by a reconstructive microsurgeon, an orthodontist, a prosthodon- tist, and a psychiatrist.

In April 1989 the patient underwent tumor excision and reconstruction. A two-team approach was used. Via a right Weber-Ferguson incision incorporating the previous lateral rhinotomy incision (Fig 4) a subtotal maxillectomy was per- formed (Fig 5). This extended through the left cuspid tooth socket anteriorly and to the posterior border of the right maxilla (Fig 6). The left premolar and molar teeth and their alveolar bone were preserved, along with the whole of the soft palate. The right nasal turbinates and the nasal septum were removed. A dorsal nasal septal cartilaginous strut was preserved. The nasal process of the right maxilla seemed ab- normal and was therefore removed, along with the contained nasolacrimal apparatus, The right maxillary and ethmoid si- nuses were cleaned out. Frozen section examination of the margins was used extensively to ensure adequate tumor ex- cision (Figs 7, 8).

Following the resection surgery, a free right rectus abdom- inis muscle flap was raised, set into the midface defect in the roof of the mouth, and revascularized using the facial vessels.

FIGURE 2. Coronal and axial CT scans of recurrent tumor he- fore definitive excision.

POSNICK ET AL 1011

FKXJRE 3. Palatal view showing large tumor mass bulging through palate on both sides of midline.

Intraoral cover over the flap muscle was not attempted. The raw muscle surface was left to re-epithelialize from the ad- jacent oral lining. Stents were placed bilaterally along the nasal floor on top of the flap muscle in an effort to maintain a nasal airway while re-epithelialization of the nasal floor occurred.

Total operating time was 14 hours. The patient received a transfusion of seven units of blood. There were no intra- operative or postoperative surgical complications. However, despite extensive counselling before surgery, the patient be- came depressed after the operation because of the loss of her teeth, and required further psychiatric counselling. She was discharged 10 days after surgery.

On review 22 months later, there was no evidence of tumor recurrence (Fig 9). Her nasal airway was patent, although she had occasional crusting and bleeding, and required night- time air humidification. She was dentally restored with a par- tial upper denture retained on her remaining four maxillary teeth (Fig 10). With this prosthesis, her occlusion was satis- factory and the patient reported that she was able to chew normally. Her speech articulation and velopharyngeal func- tion were normal and her facial appearance was excellent, esthetically equal to that before tumor excision (Fig 11). CT scan of the craniofacial skeleton showed no evidence of tumor recurrence (Fig 12). Abdominal examination showed a well- healed right paramedian scar with no hemiation and a normal contour.

Discussion

Giant cell lesion of the jaw is the term applied to a group of tumors of uncertain histogenesis and variable clinical behavior.6-8 References to it in the literature are inconsistent and conflicting; it is called a giant cell reparative granuloma,3-5 giant cell granuloma,1*7 giant cell tumor of the jaw,9,‘o and pseudo-giant cell tumor. ’ ’ These central bony tumors should not be confused with the soft tissue peripheral giant cell lesion or giant cell epulis, which is histologically similar, but considered to constitute a different clinical entity.

The giant cell lesion of the jaw typically occurs be- tween 20 and 25 years of age. However, it is not un- common in those less than 10 years old, and is the commonest benign jaw tumor of childhood.‘j2 It occurs more often in females and may exhibit hormonal de- pendence. ” The most common presenting symptom is a bony swelling, sometimes with associated pain and loosening or devitalization of teeth. Maxillary tumors may cause nasal obstruction or recurrent nose bleeds. Most tumors occur in the anterior jaw, and are more common in the mandible than the maxilla.3”,7,‘2

There are no pathognomonic radiologic features. The typical appearance is of a radiolucent lesion with well-defined margins and an overlying wispy opacifi- cation that may represent intralesional calcification. The lesion is unilocular, but may have scalloped mar- gins. There is bony expansion with cortical thinning, and cortical perforation may occur. Teeth in contact with the lesion usually do not show evidence of root resorption, but may lose the lamina dura.13 On CT scan, larger tumors may be identified, filling sinus cav- ities or the nasal vault, and causing expansion of bony walls; some destruction also occurs.‘4*‘5

Macroscopically, the lesion is composed of reddish, hemorrhagic, spongy tissue that may have a gritty con- sistency. ‘x3 Microscopic examination shows a loose, vascular stroma containing spindle-shaped fibroblastic cells, multinucleated giant celk., collagen, small areas of hemorrhage, and osteoid or new bone formation.12

FIGURE 4. Exposure is gained through the previous rhinotomy incision that extended through the upper lip.

1012 MANAGEMENT OF RECURRENT GIANT CELL LESIONS

The overall appearance is one of a benign process, with tumor expanding into but typically not invading the marrow spaces, although there is evidence of bony de- struction. The cells have varying degrees of maturity, with little evidence of mitotic activity or pleomorphism.

FIGURE 6. View of subtotal maxikctomy specimen.

FIGURE 5. View of right na- somaxillary region after tumor resection.

In general, the lesion can be distinguished histolog- ically from other bone tumors that contain giant cells, including fibrous dysplasia, aneurysmal bone cyst, and ossifying fibroma. However, it is histologically indis- tinguishable from the brown tumor of hyperparathy- roidism and from cherubism. Therefore, the differential diagnosis of these conditions rests on clinical, radio- logic, and biochemical features. In particular, all pa- tients must undergo appropriate investigation for hy- perparathyroidism, especially in the presence of multifocal or recurrent giant cell jaw lesions.16

FIGURE 7. The locally aggressive nature of the lesion is shown in this low-power photomicrograph. The lesion seems to infiltrate ad- jacent bone marrow spaces (hematoxylin-eosin stain, original mag- nification X50).

POSNICK ET AL 1013

FIGURE 8. A higher magnification shows multinucleated giant cells in a stroma of spindle-shaped fibroblastic cells (hematoxylin-eosin stain, original magnification X 125).

Various qualitative histologic differences are said to distinguish the giant cell tumor of long bones from the giant cell lesion of the jaws.3,7 Whereas this is true for most lesions, considerable overlap exists. In particular, 10% of giant cell jaw lesions are histologically indistin- guishable from the typical giant cell tumor of long bones. Instances of jaw lesions that have behaved in an aggressive fashion and were histologically similar to typical long-bone giant cell tumors have been taken as evidence that true giant cell tumors occur in the jaws. 9-“.‘7,‘8 However, in contrast to long-bone giant cell tumors,” there has been no documented evidence

FIGURE 10. View of patient with prosthesis in place.

of a giant cell jaw lesion of benign appearance metas- tasizing, and indeed only one well-documented report of a malignant giant cell tumor occurring in the jaw.2o Although the exact relationship of giant cell lesions in these different sites remains obscure, it seems appro- priate to distinguish between them on clinical grounds at least.

As described by Jaffe,3 and subsequently by others,4.5 the giant cell lesion of the jaws is innocuous, and can be treated simply and successfully by curettage. This view has predominated, despite subsequent reports of tumors displaying aggressive local behavior and high recurrence rates, especially in children.‘.2.7*8,10*‘6 There

FIGURE 9. Intraoral view showing healing of surgical site with no clinical evidence of re- currence.

1014 MANAGEMENT OF RECURRENT GIANT CELL LESIONS

FIGURE I 1. Full-face view 22 months after resection, with pros- thesis in place.

are few large series that adequately document treatment and recurrence rates. Andersen et al*l report a 13% recurrence rate in 32 patients treated with curettage. It is evident that curettage, the generally recommended treatment for these lesions and their recurrences,‘L will be inadequate treatment in a significant number of cases, as illustrated by the case presented here.

Recent reports 6,7 have failed to identify any histo- logical features of value in predicting the clinical be- havior of the lesion. Notably, it could not be demon- strated that jaw lesions histologically similar to most giant cell tumors of long bone were more likely to be- have in an aggressive fashion or to recur after treatment. Our case demonstrates that a tumor with the typical histological appearance of the tumor characterized by Jaffe3 as a reactive reparative lesion may exhibit locally destructive and aggressive behavior. Histoenzymic and ultrastructural studies may eventually help us under- stand the true origin, role, and nature of giant cells in these lesions, and provide a basis for appropriate treat- ment.6,8

In the absence of histological markers, clinical fea- tures alone must form the basis for treatment decisions. A lesion that is large at the time of initial diagnosis, and is associated with extensive bony destruction (es- pecially if it occurs in a young person), requires exci- sional surgery rather than simple curettage.* Decisions about appropriate treatment of recurrent lesions must take into account the adequacy of the initial treatment,

FUJIMURA AND ENOMOTO 1015

rate of growth, and speed of recurrence. When defin- itive excisional surgery is indicated, the ability to pro- vide functional and esthetic reconstruction is essential. Radiotherapy has not been shown to be effective in treating giant cell jaw lesions.8~‘7 It is inappropriate therapy for benign lesions, especially in children, be- cause of long-term interference with growth and the potential for development of malignancy.4

References

I. Dehner LP: Tumors of the mandible and maxilla in children. I. Clinicopathologic study of 46 histologically benign lesions. Cancer 31:364. 1973

2. Chuong R, Kaban LB: Diagnosis and treatment of jaw tumors in children. J Oral Maxillofac Surg 43:323, 1985

3. Jaffe HL: Giant-cell reparative granuloma, traumatic bone cyst, and fibrous (fibro-osseous) dysplasia of jawbones. Oral Surg 6:159, 1953

4. Austin LT, Dahlin DC, Royer RQ: Giant-cell reparative gran- uloma and related conditions affecting the jaw bones. Oral Surg Oral Med Oral Path01 12:1285, 1959

5. Waldron CA, Shafer WG: The central giant cell reparative gran- uloma of the jaws. An analysis of 38 cases. Am J Clin Path01 45:437, 1966

6. Chuong R, Kaban LB, Kozakewich H, et al: Central giant cell lesions of the jaws: A clinicopathologic study. J Oral Maxillofac Surg 44:708, 1986

7. Auclair PL, Cuenin P, Kratochvil FJ, et al: A clinical and his- tomorphologic comparison of the central giant cell granuloma

and the giant cell tumor. Oral Surg Oral Med Oral Path01 66: 197, 1988

8. Bondi R, Urso C, Santucci B, et al: Giant cell lesion of the jaw. Case report. Tumori 74:479, 1988

9. Shklar G, Meyer I: Giant-cell tumors of the mandible and maxilla. Oral Sum Oral Med Oral Path01 14:809. 196 1

IO. Small GS, Rowe NH: A “true giant cell tumor” in the mandible? J Oral Surg 33:296, 1975 _

11. Leban SG. Leoow H. Stratiaos GT. et al: The aiant cell lesion of jaws:‘Neoplastic or reparative’ J Oral Su< 29:398, 197 1

12. Lucas RB: Giant cell lesions, in Lucas RB (ed): Pathology of Tumors of the Oral Tissues, (ed. 4) Edinburgh, Scotland, Churchill Livingstone, 1983, p 262

13. Homer K: Central giant cell granuloma of the jaws: A clinico- radiological study. Clin Radio1 40~622, 1989

14. Som PM, Lawson W. Cohen BA: Giant-cell lesions of the facial bones. Radiology 147: 129, I983

15. Rhea JT, Weber AL: Giant-cell granuloma of the sinuses. Ra- diology 147:135, 1983

16. Smith PG, Marrogi AJ, Delfino JJ: Multifocal central giant cell lesions of the maxillofacial skeleton: A case report. J Oral Maxillofac Surg 48:300, 1990

17. Smith GA, Ward PH: Giant-cell lesions of the facial skeleton. Arch Otolaryngol 104:186, 1978

18. Quick CA, Anderson R, Stool S: Giant cell tumors ofthe maxilla in children. Laryngoscope 90:784, 1980

19. Hutter RVP. Worcester JN, Francis KC, et al: Benign and ma- lignant giant cell tumors of bone. A clinicopathological analysis of the natural history of the disease. Cancer 15:653, 1962

20. Mintz GA, Abrams AM, Carlsen GD, et al: Primary malignant giant cell tumor of the mandible. Report of a case and review of the literature. Oral Surg 5 1: 164. 198 1

21. Andersen L, Fejerskov 0, Philipsen HP: Oral giant cell granu- lomas. A clinical and histological study of-129 new-cases. Acta Path01 Microbial Stand 8 1:606, 1973

J Oral Maxillofac Surg

50:1015-1017,1992

Lipoma of the Tongue With Cartilaginous Change:

A Case Report and Review of the Literature

N. FUJIMURA, DDS, PHD,* AND S. ENOMOTO, DDS, PHDt

Although lipoma can occur in any part of the body, it is infrequently found in the oral cavity.lm3 It is a be- nign lesion composed of mature fat tissue that is ar- ranged in lobules that are separated by septa of fibrous

Received from the Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tokyo Medical and Dental University, Tokyo.

* Formerly, Instructor; currently, in private practice. 7 Chief and Professor. Address correspondence and reprint requests to Dr Fujimura: De-

partment of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tokyo Medical and Dental University, 5-45, Yushima, 1-Chome, Bunkyo-ku, Tokyo 113, Japan.

0 1992 American Association of Oral and Maxillofacial Surgeons

0278-2391/92/5009-0017$3.00/O

tissue and surrounded by a thin fibrous capsule. Some variants of lipoma have been classified histologically according to the kind and the amount of the tissue other than fat that is present.294 The most common is fibrolipoma, which contains an increased amount of fibrous connective tissue between the fat cells. Others include angiolipoma with an excess of capillaries, and myxolipoma with wide areas of myxoid change. Li- poma with osseous or cartilaginous change is the rarest variant. Overall, there are 13 cases of this variant that were reviewed by Allen’ and 4 cases presented by Katzer.6 In the oral cavity, only five such lipomas have been reported.‘-” Thus, this case is the sixth intraoral lipoma with osseous or cartilaginous change to be de- scribed in the literature.