Journal of Neuro-Ophthalmology, June 1985, Volume 5, Issue 2

Malignant fibrous histiocytoma of the orbit.

A recent review of orbital tumors at the Armed Forces Institute of Pathology has shown that fibrous histiocytoma is the most common primary mesenchymal tumor of the orbit in adults. This surprising statistic is explained by the fact that the histologic classification of mesenchymal neoplasms has been revised extensively during the last two decades to include previously distinct tumors of fibrous tissue under the common histopathologic diagnosis of fibrous histiocytoma. We present the computed tomographic findings of a primary malignant fibrous histiocytoma of the orbit, along with a review of the literature.

J. Clill. N<'III'fH'phtlllllll/t'l. 5: Ilh-lIY, lYK5 © 1985 Raven Press, New York Malignant Fibrous Histiocytoma of the Orbit PABLO R. ROS, M.D. SEVIL KURSUNOGLU, M.D. JUAN F. BATLLE, M.D. JEROME j. SHELDON, M.D. JOEL GLASER, M.D. Abstract A recent review of orbital tumors at the Armed Forces Institute of Pathology has shown that fi­brous histiocytoma is the most common primary mesenchymal tumor of the orbit in adults. This surprising statistic is explained by the fact that the histologic classification of mesenchymal neoplasms has been revised extensively during the last two decades to include previously distinct tumors of fi­brous tissue under the common histopathologic di­agnosis of fibrous histiocytoma. We present the computed tomographic findings of a primary ma­lignant fibrous histiocytoma of the orbit, along with a review of the literature. Since the early 1960s, the term fibrous histio­cytoma has encompassed various mesenchymal tumors that were previously considered sepa­rate pathologic entities. The International His­tologic Classification of Tumors2 currently in­cludes under fibrous histiocytoma the following entities: dermatofibroma, sclerosing heman­gioma, nodular tenosynovitis or giant cell tumor of the tendon sheath, pigmented villonodular synovitis, atypical fibrous histiocytoma, der­matofibrosarcoma protuberans, fibroxanthosar­coma, xanthogranuloma, and malignant giant cell tumors of the soft tissues (Table 1). All of these benign and malignant lesions possess, as a common denominator, variable proportions of fibroblastic and histiocytic cells that are arranged in fascicles with a character-hom the Dl'partment ui Radiulugy (P.RR., S.K., ).).S .. J.G.), Mount SinJi Medic'll Center, Miami, Fluridd .•md Bascom Palmer EYl' Institute (J.F.B.), Midmi, f!.'rid,l 111'. P. R. Rus is Dauer fellow in Di,lgnostic R,ldiolog\'; his Cllr. rent address is Department oi Rddiulogic Pdtholog\', Arn1l'd !'<,rcl's Institute oi Pathology, Washington, D.C. . Wrill' lor rl','rillb /,,:). J. Sheldon M.D., Muunt SIIl,li t\kd­it'd I Cenll'r, 4]()() Alton [{o,ld, Mi.lI11i Ih·.I,·h, Fl ..1.1140, USA 116 istic "storiform" pattern. 3 The orbit appears to be a site of predilection for fibrohistiocytic Ie: sions, and several cases have been reported4.J as well as a series of 150 cases reviewed at the Armed Forces Institute of Pathology (AFIP).6 In spite of the increasing interest in malignant fibrous histiocytoma, the radiologic findings, particularly by computed tomography, of this neoplasm in the orbit have not been reported. The clinical, radiologic, and pathologic findings of a case of malignant fibrous histiocytoma are presented here, along \vith a review of the lit­erature. Case Report The patient was a 44-year-old man with a his­tory of slowly progressive loss of vision in the central field of the right eye associated with pain. The patient was othenvise healthy. Neuro-ophthalmological evaluation revealed a visual acuity of 20/25 + 2 in the right eye and 20/20 in the left eye. An afferent pupillary defect was present on the right. The patient missed 5/15 American Optical color plates with the right eve and onlv 1/15 with the left. There was lim­itation of upward gaze on the right with a re­striction of forced ductions. Goldmann visual fields demonstrated a superior, paracentral sco­toma in the right eye. Ophthalmoscopy re­vealed mild blurring of the right disc margins, Four millimeters of proptosis could be detected with Krahn exophthalmometrv on the right side. - Computed tomography was performed. A mass of soft tissue density was detected in the right orbital apex within the muscle cone. The mass extended through the superior orbital fis­sure into the cavernous sinus (Figs. la and b). Therapy with 80 mg prednisone daily was ad­ministered during a 4-day trial. The patient no­ticl'd subjective improvement of the orbital pain but worsening of the vision. The visual acuity dropped to 20/30 + 2 in the right eye. The limi- TABLE 1. Synonyms of Fibrous Histiocytoma Subepidermal nodular fibrosis Dermatofibrom,l Sderosin~ heman~iom,l Pro~ressiYl' rl'(Urrl'nt dl'rmatofibrnma Xanthofibr,'m,l Fibn'us 'I.,lnthosar(oma JuYenile 'l.antho~ranulom,l Atypi(al fibr,'us histi,,(Ytom,l Atypi(al fibro'l.,lnthoma Nodular tl'nosyn,,,,itis Pi~mented n"dlll.1r tl'n"s\'lll'yitjs Giant (ell tllm,'r "t s"t! ti-sslll'S ,lIld It'nd'Hls Ne\'l,jd histi'KYtoma Ne\'l)'l.anth"l'nd"thl'li,'m,l Reti(lll"histi,,(\·t,'ma Rl'tklll"(Yti( ~ranllioma Dermat"fibrosar(oma pr,'tllbl'r,lnS Epitht'1i"id sarcoma Data fwm rd. 2. tation of upward gaze persisted and ophthal­moscopy revealed frank papilledema with for­mation of concentric chorioretinal folds as de­scribed by Paton in the peripapillary region. The trial of steroids was deemed unsuccessful and surgical exploration and biopsy were rec­ommended. The orbit was approached through a modified Kronlein incision. A white, com­pressible tumor mass was seen and palpated in the posterior aspect of the orbit within the muscle cone. The tumor was firmly attached to the orbital apex so that complete extirpation was not possible. The tumor was debulked and sub­mitted for pathologic examination. Frozen sec­tions of the tumor were diagnosed as fibroxan­thoma; however, review of the permanent sec­tions demonstrated the spindle-shaped cells arranged in fascicles typical of fibrous histiocy­toma. Postoperatively, the patient did well. The vi­sion in the right eye improved to 20110 - 2. Discussion Font and Hidayattf> reviewed 150 cases of fi­brous histiocytoma of the orbit submitted to the AFIP and concluded that it is the most common mesenchymal tumor of the orbit in adults. Fi­brous histiocytoma is a mesenchymal tumor that frequently involves the skeletal muscle, fascia, and adipose tissue. 7 However, its pre­dilection for the orbit is not widely recognized. In fact, it is more common than cavernous hem­angiomas, Schwannomas, or meningiomas of the orbit. The concept of fibrohistiocytic tumors is de­rived from the questionable assumptions that June 1985 Ros et al. histiocytes may act as facultative fibroblasts and that primitive mesenchymal elements may give rise to both fibroblasts and histiocytes. 7,9 How­ever, Kojima III presented histochemical evi­dence that some histiocytes are activated forms of fibroblasts, thus lending support to the hy­pothesis of fibrohistiocytic neoplasms. Fibrous histiocytomas of the orbit usually ap­pear in the fifth or sixth decade of life and do not have any predilection for sex or race. The most common signs and symptoms of the tumor are proptosis, palpable mass, decreased vision, and pain. Visual field defects and signs of optic nerve compression are also frequent. The tumor can also present as an external oph­thalmoplegia. Histopathologically, the tumor is classified as benign, locally aggressive, or malignant on the basis of its biologic behavior. Representative il­lustrations from cases unrelated to the one de­scribed here depict the storiform pattern of fi­broblasts and histiocytes without atypia seen in the benign form (Fig. 2) and the pleomorphism and giant cells typical of the malignant fibrous histiocytoma (Fig. 3). Primary orbital malignant fibrous histiocytoma is the least frequent form, accounting for only 17 of the 150 cases reviewed at the AFIP. Malignant fibrous histiocytomas of the orbit are usually larger at the time of initial examination than the benign and locally ag­gressive ones. The symptoms are usually acute rather than chronic, complete surgical removal is often difficult, and the recurrence rate is very high. Overall, the prognosis for the malignant variant of the tumor is very poor, due to its high recurrence rate, its radioresistance, and wide­spread metastases. The radiologic method of choice for evalua­tion of malignant fibrous histiocytoma is com­puted tomography. Its appearance in the orbit is similar to that of fibrohistiocvtic tumors in other locations, usually presentilig as masses of muscle density with possible central areas llf de­creased absorption values representing central necrosis. I I Computed tomograph\' can abl) demonstrate bony erosions, muscle or nerve in­filtration, and extension into surrounding stnlC­tures such as the cavernous sinus. Although some malignant fibrous histil1Cv­tomas may contain, on histl)logic examinatioi1, lipid-filled vacuolL's within the cvtoplasm of the cells, fat is not dl'lectable bv cl1mpult'd tl1n1llg­raphy. This is a very useful differential feature that allows distinctilll1 l1f fibrous histil)cvtllInas from other ml'sl'IKhym,ll tuml1rs of th-e l1l'bit such as lipl1mas ,1Ild iipl1s,lI'cl1m<ls, in which lu­cent are,lS repn'senting f,lt ,He commonIv presen t. . Other ,1 Ill'rna tiVl' imaging modalities can be 117 Figure 1. a: A plain, axial computt'd tnmngraphic SC,ln dl'l1lnl1stratt's ,1 sntt tissut' mass (arrow) in the right orbital apex contiguous with and locatl'd supt'rior and latl'ral til tlw "ptic nt'rvt' within tht' musclt' cone. b: A repeat, axial scan after the intravenous administrdtion "I lOll ml (-10 gl "I illdindtt'd Cllntr,lst agt'nt l1t'm"nstriltt's ring t'nhancement with extension of the mass through th,' superior orbital fissurt' (arrow) intll tht' middlt' crani,,1 tllSSd cllntigullus with tht' cavernous sinus. 118 Journal of Clinical Neuro-ophthalmology Figure 2. Ph,'tomicwgr,lph ,'I benign libn'us hisllocVI,'ma "i the ,'rbit. :",'te the t\'pic,]1 st,)ritnrm pattern, Thert' is no t'\"idence "i dt\'pia or mit,)tie aell\'it\'. ( ... 00,) used in malignant fibrous histiocytoma of the orbit, such as scintigraphy, angiography, and ultrasonography. The diagnostic information that can be obtained by computed tomography is far superior to any other modality, in our opinion. 12 The radiological differential diagnosis of or- Figure 3. Photomicrograph of malignant fibrous histiocy­toma of the orbit. Note the nuclear pleomorphism and bi· zarre giant cells typical of this tumor. (x 70,) June 1985 Ros et al. bital malignant fibrohistiocytoma should in­clude neurofibromatusis, neurilemoma, heman­giopericytoma, nodular fascitis, rhabdomyosar­coma, lymphoma, hemangiuma, melanoma, and Ilwtastasis. h ,7 Acknowledgment Wl' appreciatl' the collaboration of Dr. A. A, Hy­dayatt of the Ophthalmic Pathology Branch, AFIP, for providing the microscopic illustrations that ap­pear in this article, We extend our appreciation also to Dr. J, Lawton Smith for his encouragement and guidance, References 1. Stout, A. P" and Lattes, R,: Tulltors of the 50ft Tissues, (Atills of TUlllor Pathology. ser.' 2, Fasc.) Armed Forces Institute of Pathology, Wash­ington. D,C., 1967, 2. Enzinger, F, M" Lattes, R., and Torloni, M,: His­tological Typing of 50ft Tissue TUn/ors, (International Histological Classification of TUlllors. no, 3,) World Health Organization. Geneva, 1969, 3. Kaufman, S, L, and Stout, A. P,: Histioclftic Tu­lItors (Fi/1Ylllls xallthollta 1I1ld HistlOC1/1t1l1111) 'ill Chil-drt'/ 1. ClIllcer 14: 469-482. 1961. . 4, Rodrigues, M, M" Furgiveie, F, P., and Weinreb, S,: Malignant fibrous histiocytoma of the orbit. Arch, 0l'hthallllol, 95: 2025, 1977. 5, Jakobic, F. A., Howard, G, M" Jones, I. S" et al.: Fibrous histiocytomas of the orbit. Am, ,. Ophthaillto/. 77: 333, 1974. 6, Font, R, L, and Hidavatt, A, A.: Fibrous histio­cytoma of the orbit: a' clinical pathological study of 150 cases, Hullt, Patlwl, 13: 199-209, 1982, 7, Weiss, S, W" and Enzinger, F, M,: Malignant fi­brous histiocytoma, an analysis of 200 cases. Callcer 41: 2250-2260, 1978. . 8, Ozzello, L, Stout, A, P" and Murrav, M, R,: Cultural characteristics of malignant fibrous his­tiocytomas and fibrous :>.anthomas. 16: :UI-334. 1963. 9, Fu, Y, S" Gabbiill1ai, G" K,1\', G, I" .md Lattes. R,: Malignant soft tissut' tUIll0rs l,f pwbable his­tiocytic origin: genl'ral (l'nsideratillI1s ill1d t'lt'c­tron minoscopic and tissue culture studies. Callc('/' 35: 170-298, lY75, 10. Kojima, M.: Tumor growth of the reticuloendo­thelial system. Acta Patlw/. ",", 263, 273-294, 1976, 11. Bernardino, M, E" .ling, B, S" Thomas, .I. L., Lindell, M, M., ,md Gornoza, .I,: TIll' e:>.tremitv soft tissue lesitlll-n)mparali\'l' study l,f ultf,i­sound, computed tOlllograph\' and :>',;rographv, Radiolo:{I/139: 53-58, IYHI. 12. Ros, P. R., Viamonte, M., and R\,wlin, A, M,: Malignant fibrous histiocytoma: rnesenchymal tumor of ubiquitolls origin, A.'.R, 142: 753':'759. 1984. 119