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initial step in diagnosing this important, enigmatic disease. A complete evaluation with biopsies, and medical management and monitoring, are necessary to minimize morbidity and prevent mortality. Acknowledgment The authors wish to thank Drs Peter Larsen and Pierre Lavertu for providing the information regarding cases 1 and 2.

1. Wegener F: Uber generalisierte, septische Gefaesserkrankungen. Verh Dtsch Ges Pathol 29:202, 1936 2. Wegener F: Uber eine eigenartige Rhinogene Granulomatose mit besondere Beteiligung des Arteriensystems und der Nieren. Beitr Path01 Anat Allg 102:36, 1939 3. Leavitt RY, Fauci AS. Pulmonary vasculitis. Am Rev Respir Dis 134:149, 1986 4. Fauci AS, Haynes BF, Katz P, Wolfe SM: Wegener’s granulomatosis: Prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98:76, 1983 5. Cassan SM, Coles DT, Harrison EG Jr: The concept of limited forms of Wegener’s granulomatosis. Am J Med 49:366, 1970 6. Fienberg R: The protracted superficial phenomenon in pathergic (Wegener’s) granulomatosis. Hum Pathol 12:458, 1981 7. Handlers JP, Waterman J, Abrams AM, Melrose RJ: Oral features of Wegener’s granulomatosis. Arch Otolaryngol Head Neck Surg 111:267, 1985 8. Morgan AD, O’Neil R: The oral complications of polyartertis and giant-cell granulomatosis (Wegener’s granulomatosis). Oral Surg 9:845, 1956

49:29&3oct

9. Cawson RA: Gingival changes in Wegener’s granulomatosis. Br Dent J 118:30, 1965 10. Kakehashi S, Hamner JE, Baer PN, et al: Wegener’s granulomatosis: Report of a case involving the gingiva. Oral Surg 19:120, 1965 11. Brooke RI: Wegener’s granulomatosis involving the gingiva. Br Dent J 127:34, 1969 12. Scott J, Finch LD: Wegener’s granulomatosis presenting as gingivitis. Oral Surg 34:920, 1972 13. Edwards MB, Buckerfield JP: Wegener’s granulomatosis: A case with primary mucocutaneous lesions. Oral Surg 46:53, 1978 14. Israelson H, Binnie WH, Hurt WC: The hypcrplastic

References

J Oral Maxillofac

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15. 16.

17.

18.

19. 20. 21.

22.

gingivitis of Wegener’s granulomatosis. J Periodontol 52:81, 1981 Cohen PS, Meltzer JA: Strawberry gums: A sign of Wegener’s granulomatosis. JAMA 246~2610, 1981 Reed WB, Jensen AK, Konwaler BE, et al: The cutaneous manifestations in Wegener’s granulomatosis. Acta Derm Venereol43:250, 1963 Hansen LS, Silverman S Jr, Pons VG, et al. Limited Wegener’s granulomatosis: Report of a case with oral, renal and skin involvement. Oral Surg 60:524, 1985 Cawson RA, Eveson JW: Oral pathology and diagnosis. Color atlas with integrated text (ed 1). Philadelphia, PA, Saunders, 1987 (section 17.4) Sack KE: Wegener’s granulomatosis. West J Med 150:329, 1989 McDonald TJ, DeRemee RA: Wegener’s granulomatosis. Laryngoscope 93:220, 1983 Specks U, Wheatley CL, McDonald TJ, et al: Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener’s granulomatosis. Mayo Clin Proc 64:28, 1989 Nell B, Specks U, Ludemann J, et al: Anticytoplasmic autoantibodies: Their immunodiagnostic value in Wegener’s granulomatosis. Ann Intern Med 111:28, 1989

Surg

1991

Liposarcoma

Arising in the Cheek:

Report of a Case and Review of the Literature LCDR DAVID R. CHARNOCK, MC, USNR,* LCDR THORNTON JETT, DC, USN,t LCDR GREGORY HEISE, DC, USN,t AND CDR ROBERT TAYLOR, MC, USN* Received from the US Naval Hospital, Oakland, CA. * Department of Otolaryngology-Head and Neck Surgery; currently, Head, Department of Otolaryngology, Naval Hospital, Guam. t Oral Surgeon, Dental Department. 8 Chairman, ENT Department. The Chief, Navy Bureau of Medicine and Surgery, Washington, DC, Clinical Investigation Program, sponsored this case report 89-48-1970-103. The views expressed in this article are those of the author(s) and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the US Govemment. Address correspondence and reprint requests to LCDR Charneck: Department of Otolaryngology, Naval Hospital, Guam, FPO San Francisco, CA %630.

Liposarcoma was first described by Virchow in 1857.’ Each year there are an estimated 5,100 new malignant soft-tissue tumors occurring in patients in the United States, 21% of which are liposarcomas.* These tumors are rare in the head and neck; most are located in the lower extremities and retroperitoneum. A study by Pack and Person3 from 1928 to 1954 found no reported cases of liposarcoma above the clavicles. To date, only 23 cases of liposarcoma in the oral cavity have been reported, 14 of which were located in the cheek.4 We describe the clinical and microscopic features, and treatment provided, of one additional case.

This a US government use.

Report of a Case Chinese male presented to the Oral Surgery Clinic in August 1988 with a 6-week history of an

work. There are no restrictions on its

0278-2391 I91 /4903-0015$0.00/0

A 63-year-old

CHARNOCK

ET AL

enlarging, painless mass of the right cheek. He denied other symptoms and the remainder of his history was noncontributory. Physical examination showed a 2 x 3cm, firm. oval, mobile, submucosal mass anterior and inferior to Stensen’s duct in the right cheek. An excisional biopsy was performed through a transoral approach, which showed an irregularly defined mass I .2 cm in diameter, similar in consistency to a lipoma (Fig 1). The microscopic diagnosis was well-differentiated liposarcoma, subsequently confirmed by the Armed Forces Institute of Pathology (AFIP) (Fig 2). The patient was referred to the Department of Otolaryngology for evaluation. Physical examination revealed a well-healed scar and a 2-cm, firm nodule in the area of previous biopsy. Computerized tomography did not show any discrete mass. Results of a metastatic evaluation and additional physical examination were normal. Under general anesthesia, a wide excision of the mass from skin to mucosa was performed, with preservation the right oral commissure. The incision for resection was placed in the nasolabial fold. The microscopic examination showed positive tumor margins adjacent to the right oral commissure. The diagnosis was again confirmed by AFIP to be welldifferentiated liposarcoma. The patient elected close observation in our Tumor Clinic, with further surgery and radiation therapy held in reserve. To date there is no evidence of residual or recurrent disease.

Discussion The behavior of liposarcomas of the head and neck is similar to liposarcomas occurring in other regions of the body, and is directly related to its histology.’ It is thought that liposarcomas arise de novo and not from degenerating lipomas. Lipomas arise from subcutaneous fat, whereas liposarcomas appear to originate from deeper tissue.’ Fewer than 4% of liposarcomas occur in the head and neck. They are twice as common in men, with a peak incidence in the fourth to sixth decade.5 When compared to adults, lipoblastic tumors in children are unusual in any location. Liposarcomas are slow-growing and symptoms are dependent

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FIGURE 2. Representative photomicrograph Note large lipoblast in center (hematoxylin-eosin magnification x 250).

of specimen. stain, original

on size and location.

In 1988, Gertner reported a case of liposarcoma of the larynx requiring a total laryngectomy and postoperative radiation therapy.‘j In 1980, Jones reported a case of liposarcoma involving the parotid gland in which the patient died after 24 months despite combined surgery and chemotherapy.’ The youngest reported case of a liposarcoma of the cheek is in a 6-month-old infant.* The microscopic appearance of liposarcoma COJrelates directly with prognosis. Liposarcoma was first described by Virchow in 1857; Ewing, in 1935, was the first to classify it according to cell type.’ Ewing’s classification was modified and expanded by Stout in 1944. I0 The current World Health Organization classification system, proposed by Enzinger in 1962,” consists of four subtypes; welldifferentiated, myxoid, round-cell, and pleomorphic, each having a distinct microscopic appearance and j-year prognosis (Table l).’ The myxoid type is the most common and accounts for almost one half of all liposarcomas. The cells resemble fetal fat with a distinctive capillary vasculature. The welldifferentiated, adult type of liposarcoma is sometimes divided into two subgroups: 1) a “lipomaTable 1. Recurrence Rate and Survival for Different Histologic Types of Llposarcoma Type Well-differentiated Myxoid Pleomorphic Round-cell

FIGURE I.

Gross specimen removed by excisional biopsy.

Recurrence Rate (%) 53 53 73 85

Data adapted from Enzinger and Winslow.”

Five-Year Survival 1%) 85 77 21 18

LIPOSARCOMA

300 like” tumor occasionally confused with the atypical lipoma, and 2) a sclerosing lipoma often found in the retroperitoneum. ’ The round-cell type is characterized by an excessive proliferation of round cells with scanty lipid formation; it is more undifferentiated than the adult or myxoid types. The least common is the pleomorphic type, which exhibits extreme pleomorphism and bizarre giant cells; like the round-cell type, its prognosis is poor. Most liposarcomas grow by infiltration; they appear to be circumscribed, but do not have a true capsule. The less well-differentiated types commonly metastasize to the lungs, but rarely to lymph nodes. Treatment The recommended treatment for liposarcoma is wide excision. Radiation is reserved for tumors that are nonresectable or require conservative surgery. DeVita12 recommends postoperative radiation therapy for all sarcomas. Friedman,13 Enterline,’ and Edland’ have suggested that the more welldifferentiated variants of liposarcoma are more radiosensitive. Enterline reviewed 53 cases of liposarcoma and suggested that the well-differentiated and myxoid types respond to radiation therapy. The number of cases reviewed by Enterline was small and his classification included the round-cell variant with the myxoid group. Three of seven patients in Enterline’s myxoid group had no response to radiation therapy. Saunders reported a series of four liposarcomas involving the head and neck, and suggested that more advanced lesions should receive postoperative radiation therapy.15 Chemotherapy has not been proven to be of benefit for the treatment of liposarcoma. Summary The following points can be made about liposarcoma of the oral cavity: 1) it is rare and slow growing; 2) it is often mistaken for a benign lesion; 3) there is a direct correlation of microscopic appearance with biological behavior and prognosis; 4) treatment is primarily surgical, with radiation used

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for selected cases; and 5) the better-differentiated tumors seem to respond more favorably to radiation. References 1. Bat&is G, Regezi JA, Rice DH: The pathology of head & neck tumors. Fibroadipose tissue and skeletal muscle tissue. Head Neck Surg 3: 145, 1980 2. Torasian MH, Friedrich C, Goldbold J, et al: Soft tissue sarcoma: Initial characteristics and prognostic factors in patients with and without disease. Semin Surg Oncol4: 13, 1988 3. Pack GT, Pierson JK: Liposarcoma: A study of 105 cases. Surgery 36:687, 1954 4. Watanabe I: Liposarcoma arising from the cheek of the aged: Report of a case and review of the literature. Gerodontics 1: 148, 1985 5. Otte T, Kleinsarrer 0: Liposarcoma of the head and neck. Arch Otorhinolaryngol 232:265, 1981 6. Gertner R, Podoshin L, Fradis M, et al: Liposarcoma of the larynx. J Laryngol Otol 102:838, 1988 I. Jones JK, Baker HW: Liposarcoma of the parotid gland. Arch Otolaryngol 106:497, 1980 8. Suzuki H, Nagayama M, Kaneda T, et al: Liposarcoma of the cheek of an infant. J Oral Maxillofac Sure 42: 180.1984 9. Enterline HT: Liposarcoma: A clinical and-pathological study of 53 cases. Cancer 13:932, 1%9 10. Stout AD: Liposarcoma: The malignant tumor of lipoblasts. Ann Surg 119:119, 1944 11. Enzinger FM, Winslow DJ: Liposarcoma: A study of 103 cases. Virchows Arch [Pathol Anat] 355367, 1962 12. DeVita VT Jr: Cancer Principles and Practice of Oncology (ed 2), ~012. Philadelphia, PA, Lippincott, 1985, pp 12611262 13. Friedman N, Egan JW: Irradiation of liposarcoma. Acta Radiol54:225, 1960 14. Edland RW: Liposarcoma: A retrospective study of 15 cases, a review of the literature and discussion of radiosensitivity. Am J Roentgen01 Radium Ther Nucl Med 103:778, 1968 IS. Saunders JR, Jaques DA, Casterline PF, et al: Liposarcoma of the head and neck. Cancer 43:162, 1979 16. Latter R: Tumors of Soft Tissue (series 2). Washington, DC, Armed Forces Institute of Pathology, 1982, p 146 17. Sadeghi EM, Sack JJ: Liposarcoma of the oral cavity. Clinical, tissue culture, and ultrastructure: Study of a case. J Oral Pathol 11:263, 1982 18. Yochanas R, Horowitz Y, Oberman M, et al: Liposarcoma of buccal mucosa. Int J Oral Surg 6:226, 1977 19. Herefer EP: Liposarcoma of the cheek. J Oral Surg 34: 1039, 1976 20. Dahl EC: Liposarcoma of the head and neck. J Oral Maxillofac Surg 40:674, 1982 21. Amarjit S, Singh A, Nagpal B, et al: Liposarcoma of the cheek. J Oral Surg 36:811, 1978 22. Thawley SE, Panje WR, Batsakis JG: Comprehensive Management of Head and Neck Tumors, vol 2. Philadelphia, PA, Saunders, 1987, p 1270

Liposarcoma arising in the cheek: report of a case and review of the literature.

The following points can be made about liposarcoma of the oral cavity: 1) it is rare and slow growing; 2) it is often mistaken for a benign lesion; 3)...
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