Fibroxanthoma of Ethmoid Sinus


Lesica, MD; Thomas R. Harwood, MD; Hidejiro Yokoo, MD

occurring in the ethmoid sinus 38-year-old man had the histological characteristic features of an atypical A tumor



fibroxanthoma. It recurred nine years after the initial excision, causing the clinical symptoms of progressive nasal obstruction.

Atypical fibroxanthoma is a tumor .¿ . usually found in solar-damaged skin of the head and neck.15 Histologically, the tumor is highly cellular and pleomorphic, but clinically, it behaves in





fibroxanthomas occurring in sites oth¬ er than the skin are rare.6·7 This report describes a patient with an atypical fibroxanthoma in the eth¬ moid sinus, which recurred nine years after the initial removal. REPORT OF A CASE A 38-year-old man was first admitted to the VA Research Hospital in January 1965, because of a slowly growing mass located over the medial superior wall of the right orbit. The patient first noticed the mass

for publication Feb 25, 1975. From the Laboratory Service, Veterans Administration Research Hospital, and the Department of Pathology, Northwestern University Medical School, Chicago. Reprint requests to the Laboratory Service, Veterans Administration Research Hospital, 333 E Huron St, Chicago, IL 60611 (Dr. Lesica).


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approximately two years before admission. About one year prior to admission results of a biopsy of this mass, done at another hospital, were reported as chronic granula¬ tion tissue. The mass continued to grow. The physical examination was essentially unremarkable except for the right orbit where a 2.5 cm in diameter round firm tumor could be palpated in the medial superior aspect of the right orbit. It was not fixed to the bone. Laboratory data on admission were unremarkable. X-ray films of the right orbit showed a questionable area of osteolysis in its roof. The paranasal sinuses were unremarkable except that the frontal sinus could not be visualized, in spite of repeated examinations. A right carotid angiogram was normal. The tumor was excised through an orbital incision on the right. The main portion of the tumor was located in the right ethmoid sinus. The surgical specimen consisted of two irregu¬ larly shaped portions of reddish-brown firm tissue, the larger measuring 2 x 1.5 x 1.5 cm. The histological diag¬ nosis of xanthogranuloma was made. The postoperative course was uneventful and the patient was discharged in a satisfac¬ tory condition. The patient was readmitted to the hospital in February 1974 with the signs and symptoms of progressive nasal obstruction of six months duration. Exami¬ nation of the nasal sinuses revealed a large firm tumor mass protruding from the middle meatus area on the right. A tomo¬ gram showed a tumor in the anterior ethmoid area. On Feb 15, 1974, the tumor was removed by means of polypectomy with ethmoidectomy. Postoperatively, the patient had an

uneventful recovery. The excised mass was polypoid, pinkish-gray, and firm, mea¬ suring 2.5 x 2.5 x 2.3 cm. The cut surface revealed pink-gray tissue with focal areas of yellow soft tissue. Microscopically, the histology of the tumor was identical to that of the previous specimen (Fig 1 and 2). It was composed of large elongated histio¬ cytic cells that resembled fibroblasts. These cells exhibited marked variations in size and shape of the nuclei, with occasional mitotic figures. Mixed with these cells were many lipid-laden histiocytes, with foamy cytoplasm and bundles of collagen fibers. A small number of inflammatory

Fig 1.—Top, Atypical fibroxanthoma of ethmoid sinus excised nine years ago. Tumor atypical histiocytes, fibroblasts, and occasional giant cells (hematoxylineosin, original magnification 150). Bottom, Higher magnification of top. Note pres¬ ence of a giant cell, atypical histiocytes, and a few lymphocytes (hematoxylin-eosin, original magnification 640). consists of

cells, predominantly lymphocytes, were present scattered between the tumor cells. At the

periphery of the lesion



gates of cholesterol-clefts surrounded by giant cells of the foreign body type. Exten¬ sive foci of necrosis were present within the tumor tissue. A diagnosis of fibroxanthoma was made.



Atypical fibroxanthoma is a noduloulcerative tumor, which usually oc¬ curs in solar-damaged skin of the head and neck of elderly persons.15 Occa¬ sionally it is seen in irradiated skin.14 Fretzin and Helwig5 noted a clinical variant of this tumor among a much younger age group that characteristi¬ cally involved unexposed areas of skin of the body.6 As mentioned earlier, the atypical fibroxanthoma is highly cellular and pleomorphic, resembling a sarcoma. It

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highly atypical histology, the clinical behavior of the atypical fibroxan¬ thoma is benign. They do not metastasize and recur only rarely.2-5 When they recur, a second excision usually leads to a complete cure.4 In our case,

the tumor recurred nine years after the initial surgical removal. There is no sign of recurrence ten months after the second operation. The nature of the atypical fibroxanthoma is not certain. It may represent a reactive process or a benign neoplasm of histiocytic cells.3 Recognition should be given to the existence of this rare tumor involving the paranasal sinuses, and in partic¬ ular to its benign clinical behavior and

diagnostic difficulty.


Fig 2.—Recurrent atypical fibroxanthoma, showing similar histologie features (hema¬ toxylin-eosin, original magnification 649).

is primarily composed of three cellular elements: histiocytes, fibroblasts, and giant cells. Among these elements histiocytic cells may appear quite

bizarre, having a single or multiple large hyperchromatic nuclei with prominent nucleoli.15 Some of them have abundant vacuolated cytoplasm. Mitotic figures may be encountered frequently. Because of their highly cellular and pleomorphic appearance, atypical fibroxanthomas have been erroneously called malignant mesen¬ chymal tumors, spindle-cell squamous cell

carcinomas, reticulum cell sarco¬ malignant melanomas, which

mas or

in some cases resulted in unnecessary radical surgery.1 :l·4 The majority of atypical fibroxanthomas so far report¬ ed in the literature occurred in the

skin.15 They have been described in the trachea (one patient) and retroperitoneum (seven patients) by Soule and Enriquez." Barney7 reported a case of atypical fibroxanthoma of temporal bone in a 43-year-old man. A case of malignant fibrous histiocytoma of maxilla in a 46-year-old man was reported by Spector and Ogura.8 These authors implied that the tumor could be classified as an atypical fibrous histiocytoma (atypical fibro¬ xanthoma). A fibrous histiocytoma involving the paranasal sinus of a 3year-old boy was reported by Townsend et al," but this lesion did not show atypical features. Thus, although rare, the paranasal sinuses appear to be one of the sites that may be involved by fibrous histiocytic tumors. Despite the

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1. Vargas-Cortes F, Winkelmann RK, Soule EH: Atypical fibroxanthoma of the skin. Mayo Clinic Proc 48:211-218, 1973. 2. Hudson AW, Winkelmann RK: Atypical fibroxanthoma of the skin: A reappraisal of 19 cases in which the original diagnosis was spindlecell squamous carcinoma. Cancer 29:413-422, 1972. 3. Kroe DJ, Pitcock JA: Atypical fibroxanthoma of the skin: Report of ten cases. Am Clin Pathol 51:487-492, 1969. 4. Kempson RL, McGavran MH: Atypical fibroxanthoma of the skin. Cancer 17:1463-1471, 1964. 5. Fretzin DF, Helwig EB: Atypical fibroxanthoma of the skin: A clinicopathologic study of 140 cases. Cancer 31:1541-1552, 1973. 6. Soule EH, Enriquez P: Atypical fibrous histiocytoma, malignant fibrous histiocytoma, malignant histiocytoma and epithelioid sarcoma: A comparative study of 65 tumors. Cancer 30:128\x=req-\ 143, 1972. 7. Barney PL: Atypical fibrous histiocytoma (fibroxanthoma) of the temporal bone. Trans Am Acad Ophthalmol Otolaryngol 76:1392-1393, 1972. 8. Spector GJ, Ogura JH: Malignant fibrous histiocytoma of the maxilla: A report of an unusual lesion. Arch Otolaryngol 99:385-387, 1974. 9. Townsend GL, Bryan III N, Weiland LH, et al: Fibrous histiocytoma of paranasal sinuses. Arch Otolaryngol 98:51-52, 1973.

Atypical fibroxanthoma of ethmoid sinus.

A tumor occurring in the ethmoid sinus in a 38-year-old man had the histological characteristic features of an typical fibroxanthoma. It recurred nine...
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