1239

GRAY, MILLER, AND LOF-TUS

testing, must be consistent with the radiographic findings of a blowout fracture before repair is undertaken for the relief of diplopia.3 If the diplopia is due to an isolated extraocular muscle or cranial nerve palsy, the patient should be evaluated without orbital surgery (assuming the fracture is not markedly displaced). Under such circumstances, the diplopia is very likely to resolve spontaneously. However, if it persists, strabismus surgery can be performed at a later date. ’ Treatments for fourth nerve palsy have been classified according to increasing degree of intervention. The first modality involves supervised medical observation, and the second consists of ocular occlusion to eliminate one of the images. Sometimes a patch is used, but most often translucent tape is placed over the entire lens of one eye, or over the lower segment if the patient complains of diplopia only in downward and near gaze. The use of vertical prisms improves some patients. If diplopia persists, surgical correction involves weakening the antagonistic muscle in the eye that has the superior oblique involvement, ie, the ipsilateral inferior oblique muscle. Weakening can be accomplished by recessing the muscle 10 mm on the globe, along its direction of action.5 Tucking of the paretic superior oblique muscle is found to be of less value than inferior oblique and vertical rectus muscle surgery.6 The surgery should be deferred for 1 year to allow for possible further recovery or even adequate adaptation that may

occur during that interval.6 Although some studies of untreated unilateral posttraumatic fourth nerve palsy indicate that the condition is usually permanent,2,5 others report that spontaneous improvement generally occurs. ’ The case presented here suggests some caution in interpreting the “typical signs” of a blowout fracture. Even when a blowout fracture is confirmed, the possibility that vertical diplopia may be the result of an associated fourth nerve palsy should be considered. If the motility pattern is more compatible with paretic than restrictive strabismus, orbital exploration is not recommended.4

References 1. Wojno TH: The incidence of extraocular muscle and cranial nerve palsy in orbital floor blowout fractures. Ophthalmology 94682, 1987 2. Burger LJ, Kalvin NH, Smith JC: Acquired lesions of the fourth cranial nerve. Brain 93:567, 1970 3. Ruttum MS, Harris GT: Orbital blowout fracture with ipsilateral fourth nerve palsy. Am J Ophthalmol 100:343, 1985 4. Keane JR: Fourth nerve palsy opposite a black eye. J Clin Neuro Ophthalmol 1:209, 198 I 5. Younge BR, Sutula F: Analysis of trochlear nerve palsies. Diagnosis, etiology and treatment. Mayo Clin Proc 52: 11, 1977 6. Kawam E, Scott AB, Jampolsky A: Acquired superior oblique palsy, diagnosis and management. Arch Ophthalmol 77:76, 1967

Oral MaxlllofaC Surg 50:1239-1242,1992

J

Benign Fibrous Histiocytoma of the Oral/Perioral Regions: Report of a Case and Review of I7 Additional Cases PETER B. GRAY, DMD,* ARTHUR S. MILLER, DMD,t AND MICHAEL J. LOFTUS, DDS*

* Chief Resident, Department of Oral and Maxillofacial Surgery, Hahnemann University Hospital, Philadelphia, PA. 7 Department Chairman, Oral Pathology Section, Temple University School of Medicine, Philadelphia, PA. # Acting Chairman, Department of Oral and MaxilJofacial Surgery, Hahnemann University Hospital, Philadelphia, PA. Address correspondence and reprint requests to Dr Gray: Department of Oral and Maxillofacial Surgery, Hahnemann University Hospital, Broad & Vine Sts, Philadelphia, PA I9 102. 0 1992 American Association of Oral and Maxillofacial Surgeons 0278-2391/92/501

l-0021$3.00/0

Fibrous histiocytomas are uncommon tumors of the superficial and deep soft tissues that are reported to have a common origin from the tissue histiocyte.’ The neoplastic growth can assume many forms, and this has led to a diversity of applied names.’ This group of lesions has been shown to have a wide spectrum of clinical behaviors, from those with totally benign characteristics to those with malignant features. The latter may demonstrate multiple recurrences and widespread metastases.3“’ Benign fibrous histiocytomas occur most

BENIGN FIBROUS HISTIOCYTOMA OF THE MOUTH

1240 frequently on skin surfaces of the extremities, but may develop in deeper tissues or bone.2 There are few reported cases of benign fibrous histiocytoma in the oral cavity or perioral regions of the face.12-16 The purpose of this article is to report the details of a case of benign fibrous histiocytoma that arose in the upper lip, and to review the clinical features of 17 additional cases retrieved from the files of the Oral Pathology Laboratory at Temple University School of Medicine. Report of Case A 45-year-old Hispanic man was seen in the Oral and Maxillofacial Surgery Clinic at Hahnemann University Hospital for evaluation of a 2- to 3-cm nodular growth inside the right upper lip at the nasolabial angle. The patient had noticed a small mass in the area approximately 5 weeks earlier, but due to its painless state he sought no treatment. The mass steadily enlarged and he consulted his physician, who subsequently referred him for evaluation. Examination revealed a firm, palpable, nontender nodule of the deep soft tissue of the right upper lip. The mass was freely movable, and the overlying skin was without erythema, ulceration, or depression. Intraoral examination showed normal mucosa of the upper labial and vestibuiar regions. No regional lymphadenopathy was noted on palpation. The patient’s medical and surgical histories were unremarkable, and the patient denied any traumatic injury, radiation exposure, or excessive sun exposure of the perioral and oral regions. He was taking no medications and had no known allergies. His social history disclosed a significant smoking history and the regular consumption of alcohol. Radiographic evaluation revealed no maxillofacial intrabony pathosis. The nodular mass was removed using an intraoral approach following maxillary nerve block with a local anesthetic. A superficial, horizontal incision was made in the musosa of the upper lip and vestibule, and mucosal flaps were developed

FIGURE 2. Survey view showing well-delineated tumor mass and adjacent muscle (hematoxylin-eosin stain, original magnification X6).

and retracted. The mass was then easily accessed with blunt and sharp dissection (Fig 1). It was located deep within the orbicularis oris muscle and was not associated with bone or alar cartilage. The nodule appeared to be encapsulated and was easily removed from the surrounding soft tissue along with several adherent skeletal muscle fibers and minor salivary glands. A layered closure was performed reapproximating the orbicularis oris and associated fascia with resorbable, interrupted sutures. The mucosal margins were realigned and approximated with silk horizontal mattress sutures. The patient was subsequently administered oral antibiotics and analgesics. Healing of the surgical site was rapid and without complications. The excised tissue was submitted for gross and histologic examination. The excised mass was cut into multiple sections following formalin fixation, A survey view showed a well-delineated tumor mass, adjacent muscle, and minor salivary gland tissue (Fig 2). Further microscopic examination revealed uniform spindle-shaped cells with vesicular, ovoid nuclei interspersed throughout an eosinophilic collagenous stroma. There were regions where the tissue pattern was storifonn and whorled, and scattered foamy histiocytes (xanthoma cells) were noted, as were small capillaries and vascular slits. Lymphocytic infiltration was present in focal areas. A diagnosis of benign fibrous histiocytoma was made (Figs 3. 4).

Discussion

FIGURE I. Clinical photograph showing lesion being excised. The tumor was easily dissected from the surrounding connective tissue and muscle.

Fibrous histiocytoma is a generic term used to classify a broad group of proliferative lesions that are usually of benign character. It can assume many forms, and this has led to a diversity of names, such as dermatofibroma, sclerosing hemangioma, subepidermal nodular fibrosis, xanthoma, xanthofibroma, xanthogranuloma, and dermatofibrosarcoma protuberans. Kauffman and Stout” related these lesions to the differentiation potential of the tissue histiocyte, thereby establishing a common precursor. They reported that the histiocytic cells undergo bimodal differentiation, proliferating as both fibroblasts and phagocytic histiocytes.

GRAY, MILLER. AND LOFTUS

Stout and Lattes? and later Kempson and Kyriakos,4 in an attempt to simplify the complex nomenclature, recommended the term “fibrous histiocytoma” to refer to this entire class of lesions, both benign and malignant. Since that time, several subgroups under the fibrous histiocytoma category have been suggested. Soule and Enriquez’ delineated four subgroups of histiocytic tumors with aggressive behaviors based on the histologic appearance and level of malignant behavior, ie, tissue invasion, recurrence, and metastasis: atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytoma, and epitheloid sarcoma. In a more contemporary analysis, Hoffman and MartinezI separated these lesions into two broad categories, benign and malignant, and subdivided these into 1) benign fibrous histiocytoma, 2) atypical fibrous histiocytoma, 3) inflammatory fibrous histiocytoma, and 4) malignant fibrous histiocytoma. Benign lesions appear benign histologically and are cured by excision. The atypical fibrous histiocytoma subgroup tends to behave in a benign manner, with surgical excision usually also affording curative results. There were no instances of metastasis in their study, although some cases of local recurrence were reported. Their subgroups, inflammatory and malignant fibrous histiocytomas, fall into the realm of extremely aggressive neoplasms. These lesions are reported to be highly invasive, with numerous recurrences, metastases, and a fatal prognosis. The inflammatory fibrous histiocytoma growth pattern is consistent with low- to intermediate-grade malignancy. Although its name implies that it is a lesion of reactive inflammatory tissue, it is not. This term was derived from the histologic pattern, in which an intense inflammatory cell infiltrate was seen within the tumor stroma; it was noted that this attribute was unique only to this type of lesion. Malignant fibrous histiocytomas, they reported, are high-grade tumors with rapid, metastatic behavior, and have fatal results within 24 months

FIGURE 3. Photomicrograph showing overall pattern of swirling clusters of fibroblasts. presence of lymphocytes, and large foamy histiocytes (right; hematoxylin-eosin stain, original magnification X 100).

1241

FIGURE 4. Photomicrograph showing vesicular, ovoid nuclei in the fibrocytic cells. Scattered lymphocytes are seen at the upper border (hematoxylin-eosin stain, original magnification X 450).

of diagnosis. It is our opinion that fibrous histiocytomas constitute neoplastic entities, with the benign form representing one end of a continuum. We do not feel that these tumors represent an inflammatory process. There is no universal agreement regarding the naming of these lesions. Based on a selected review of the literature and clinical data, we prefer a simplification of the nomenclature and favor classification of these neoplasms into three groups: 1) benign fibrous histiocytoma, 2) atypical fibrous histiocytoma, and 3) malignant fibrous histiocytoma. These designations are based on the clinical presentations and histologic features. It is our belief that further subdivision does not add clarity. Benign lesions consist of uniform, frequently encapsulated masses, which demonstrate cellular regularity, a relative absence of pleomorphism, and little mitotic activity on histologic examination. They tend to occur cutaneously or in superficial tissues,” and do not recur following excision. Atypical fibrous histiocytoma occurs more often in deeper tissues. It has benign characteristics histologically, with well-differentiated cells, a lack of pleomorphism, and few mitoses. However, because of their benign appearance, these lesions may be misleading. Clinically, they behave more aggressively, with reported local recurrences’6 and isolated metastases.21 Malignant fibrous histiocytoma has a highly aggressive behavior. Histologically, it possesses high degrees of cellular atypia, with hyperchromatic nuclei, pleomorphism, poorly differentiated cells, considerable mitotic activity, and areas of necrosis with invading inflammatory cells. It occurs predominantly in deeper tissues, recurs locally, and displays metastasis. The malignant characteristics of fibrous histiocytomas are well documented, with numerous case reports of neoplasms demonstrating highly aggressive behaviors and multiple metastases.18 Some of these tumors have been found in the head and neck region and in

1242

BENIGN FIBROUS HISTIOCYTOMA OF THE MOUTH

the oral cavity. *A’Fortunately, only approximately 1% of fibrous histiocytomas tend to behave in a malignant manner; however, when they do, they carry a very poor prognosis for long-term survival. Early diagnosis and aggressive surgical therapy remains the treatment of choice. Using our classification, a diagnosis of atypical fibrous histiocytoma must be carefully eliminated. Because these lesions appear benign histologically, it is only when they recur or demonstrate metastatic behavior that they may be classified as the atypical form. Due to the clinically aggressive nature of these lesions, surgical excision and close follow-up evaluation is recommended as the treatment of choice for all benign fibrous histiocytomas to avoid undertreating the atyp ical form. Periodic follow-up examinations should focus on ruling out a tumor recurrence. Benign fibrous histocytomas usually occur on the extremities; children and young adults are primarily affected.” Prolonged sun exposure and traumatic injury have been alleged to be possible causative agents. These tumors are uncommonly reported in the oral cavity or perioral region. Our review of the literature found cases reported by Kjaerheim and Stokke,12 Friedlander and Zeff,13 Hillis and Beasley,14 Hutchinson and Friedberg,” and Hoffman and Martinez.16 However, we found 18 cases, including the present one, that were accessioned by the Oral Pathology Laboratory, Temple University School of Medicine, between 1986 and 199 1 (Table 1). Twelve patients were female and 6 were male, a ratio of 2: 1. The patients ranged in age from 12 years to 7 1 years, with a median age of 55 years: in 2 patients, the age was unknown. Our findings

Table 1.

Benign Fibrous Histiocytomas

Case

Age

Sex

Anatomic Location

I

42 65 59 31 50 71 45 49 70 12 60 68 59 66 37 35

M M M F F F F M F F M F F F F F F M

Right buccal mucosa Right buccal mucosa Right body of mandible I_& side of tongue Right dorsum of tongue Left buccal mucosa Right lower lip Right maxillary vestibule Left buccal mucosa Right anterior mandibular ridge Left mandibular vestibule Right buccal mucosa Left mandibular vestibule Left body of mandible Inferior cortex, left mandible Left mandibular vestibule Right anterior maxillary gingiva Right upper lip

2 3 4 5 6 7 8 9 10 II 12 13 14 15 16 17 18*

* Indicates case reported.

indicate that oral and perforal benign lesions occur in patients of an older age group of approximately 40 to 60 years. The buccal mucosa/vestibular region was the most common reported location (9 cases). Two cases were reported in the tongue, 2 on the gingiva/alveolar ridge, 2 central within the mandible, 1 on the inferior cortex of the mandible, and 1 each on the upper lip and lower lip. The finding in our series of 2 cases with lesions that were central within the mandible is somewhat unusual. Cale et a12’reported a case in the maxilla and cited three previous cases reported in the jaw bones. Surgical excision was the treatment of choice in the 18 cases and, to date, no recurrences have been reported to us. References 1. Bat&is JG: Tumors of the Head and Neck (ed 2). Baltimore, MD, Williams & Wilkins. 1979. pp 264-269 2. Stout AP. Lattes R: Tumors of the soft tissues, in Atlas of Tumor Pathology (fast 1). Washington, DC. Armed Forces Institute of Pathology, 1967 O’Brien JE, Stout AP: Malignant fibrous xanthoma. Cancer 17: 1445, 1964 Kempson RL, Kyriakos M: Fibroxanthosarcoma of the soft tissue. Cancer 29:961, 1972 Soule EH, Enriquez P: Atypical fibrous histiocytoma, malignant histiocytoma and epitheloid sarcoma. Cancer 30: 128, 1972 Solomon MP, Sutton AL: Malignant fibrous histiocytoma of the soft tissues of the mandible. Oral Surg Oral Med Oral Path01 35:653, 1973 I. Fu YS, Gabbaini GI, Lattes R: Malignant soft tissue tumors of probable histiocytic origin (malignant fibrous histiocytomas): General considerations and electron microscopic and tissue culture studies. Cancer 35: 176, 1975 8. Blitzer A, Lawson W, Biller H: Malignant fibrous histiocytoma of the head and neck. Laryngoscope 87:1479, 1977 9. Slootweg PL, Muller H: Malignant fibrous histiocytoma of the maxilla. Oral Surg Oral Med Oral Path01 44:560, 1977 IO. Bras J. Batsakis JG, Luna MA: Malignant fibrous histiocytoma of the oral soft tissues. Oral Surg Oral Med Oral Path01 64: 57. 1987 Il. DiLascio JP, Devlin GP, Doyle JL: Aggressive fibrous histiocytoma of perioral soft tissues: Report of case. J Oral Maxillofac Surg 39:i34, 1981 12. Kjaerheim A, Stokke T: Juvenile xanthogranuloma of the oral cavitv. Oral Sure Oral Med Oral Path01 38:4 14. 1974 13. Friedlander A, Zel? S: Sclerosing hemangioma of the tongue. J Oral Maxillofac Surg 33:2 12, 1975 14. Hillis RE, Beasley JD: Fibrous histiocytoma of the lip. J Oral Med 30~81, 1975 15. Hutchinson JC, Friedberg SA: Fibrous histiocytoma of the head and neck. Laryngoscope 88:1950. 1978 16. Hoffman S, Martinez MG: Fibrous histiocytomas of the oral mucosa. Oral Surg Oral Med Oral Path01 52:277, 198 1 17. Kauffman SL, Stout AP: Histiocytic tumors (fibrous xanthoma and histiocytoma) in children. Cancer 14469, 1961 18. Geist J, Azzopardi M, Domanowski A, et al: Thoracic malignant fibrous histiocytoma metastatic to the tongue and skin of face. Oral Surg Oral Med Oral Path01 69: 199, 1990 19. Cale AE, Freedman PD, Kerpel SM, et al: Benign fibrous histiocytoma of the maxilla. Oral Surg Oral Med Oral Path01 68: 444, 1989 20. Enzinger FM, Weiss SW: Soft Tissue Tumors (ed 2). St Louis, MO, Mosby, 1988, pp 223-233 21. Hakim M, Pai R, Fine G, et al: Fibrous histiocytoma of the trachea. Chest 68:367, 1975