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Angiomatoid Malignant Fibrous Histiocytoma of The Orbit
ANGIOMATOID MALIGNANT FIBROUS HISTIOCYTOMA OF THE ORBIT LUISA RODRIGUEZ CABALLERO, M.D.,
ANGEL CUNADO RODRIGUEZ,
AND A G U S T I N B U L L O N S O P E L A N A ,
M.D.,
M.D.
Salamanca, Spain
A 7-year-old boy had an angiomatoid malignant fibrous histiocytoma in his left orbit. After an adequate orbital exenteration, the tumor recurred and grew rapidly and destructively, filling the orbit. The patient died within six months. The ultrastructural study of the tumor showed some characteristics of histiocytes and fibroblasts, but also showed features, such as WeibelPalade bodies, that suggested an endothelial origin.
Malignant fibrous histiocytomas are a group of primitive connective cell tumors with cells characteristic of fibroblasts and histiocytes. This distinguishes them from other sarcomas of the soft tissues. Al though these tumors are rare, many cases have been reported. 1 " 3 The terminology varies from report to report because there are several variants or subtypes. Fibrous histiocytomas are found pri marily in the arms and legs, the abdo men, and the retroperitoneum. On rare occasions, they occur in the face and trunk. 4 Enzinger 5 recently reported 41 cases of a new variety, which he called angioma toid malignant fibrous histiocytoma. This variety, unlike the malignant fibrous his tiocytoma that usually occurs in old age, develops in young people (from 5 to 25 years old) and is often confused with a vascular tumor because of vascular spaces and areas of hemorrhage. Malignant fibrous histiocytoma of the From the Departments of Ophthalmology (Dr. Caballero) and Pathology (Drs. Cunado and Bullon), the University of Salamanca, Faculty of Medicine, Salamanca, Spain. Reprint requests to Luisa Rodriguez Caballero, M.D., Department of Ophthalmology, University of Salamanca, Faculty of Medicine, Salamanca, Spain.
orbit is rare. 6,7 We studied such a tumor in a 7-year-old boy, which we believe was of the angiomatoid type described by Enzinger. 0 CASE REPORT
A 7-year-old boy had had exophthalmos of the left eye for one month, along with visual loss in the left eye and slight headaches. His general health was good. Ophthalmologic findings—His visual acuity was R. E. : 6/6 (20/20) and L.E. : 6/60 (20/200). The papillae of the right eye were slightly pale, but the rest of the ophthalmologic findings were normal. In the left eye, the frontal segment, pupil, pupillary reflexes, and ocular motility were normal. The ophthalmoscopic examination showed hyperemia of the papillae and a blurring of the nasal margin, venous enlargement, and retinal folds in the paramacular region (Fig. 1). The axial exophthalmos measured 22 mm. The radiologie examination of the orbit showed an increase in the left sphenoidal fissure. Ten days later the exophthalmos had greatly in creased (Fig. 2). An examination showed conjunctival chemosis, comeal hypoesthesia, and ophthalmoplegia. The boy had severe headaches and his general condition had deteriorated. An orbital exenteration disclosed a violet tumor that filled the entire orbit. It resembled a vascular tumor. Twenty days later, there was a local recurrence of the tumor and the boy began to lose vision in his right eye. Within 15 days, amaurosis with paralytic mydriasis developed. The tumor grew rapidly and caused bone damage. Despite cytostatic treatment that initially decreased the tumor mass, the boy died. No autopsy was performed. Pathologic findings—The tumor had ill-defined borders and had invaded the fat and muscles of the
AMERICAN JOURNAL OF OPHTHALMOLOGY 92:13-15, 1981
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Fig. 2 (Caballero, Cufiado, and Bullon). Exophthalmos of the left eye ten days after the first examination.
orbit. When cut, it was gray-white with many vascu lar spaces and small hemorrhagic cysts or foci. When examined under the microscope, the sec tions stained with hematoxylin and eosin showed a fasciculated proliferation of spindle-shaped cells with a cartwheel pattern (Fig. 3). Some cells were atypi cal, with voluminous hyperchromatic nuclei and some atypical mitoses. The atypical cells were usually
xanthomatous and had abundant clear cytoplasm (Fig. 4). Some areas of the tumor disclosed extensive sclerosis with strips of hyaline substance in the intercellular space. Ultrastructural findings—The characteristics of the cells that showed their fibroblastoid differentia tion were (in addition to their spindle-shaped ap pearance under the microscope) the dense chromatin in their nuclei, dark cytoplasm, the abundance of rugous endoplasmic reticulum, and collagen fibrils in the interstice. The characteristics that showed their histiocytic differentiation were lobulated or notched nuclei and dense bodies of a lysosomal type in their cytoplasm, numerous mitochondria, and pseudopodic or philopodic projections of cellular membranes. Some of the dense bodies were in the shape of
Fig. 3 (Caballero, Cufiado, and Bullon). Histologie section of the tumor shows numerous vascular spaces and extravascular erythrocytes. The tumor cells mixed with hyaline collagen are grouped together in a cartwheel pattern (hematoxylin and eosin, X31).
Fig. 4 (Caballero, Cufiado, and Bullon). Marked nuclear pleomorphism, cells with clear xanthomatous cytoplasm (asterisk), and atypical mitosis (arrows) (hematoxylin and eosin, x98).
Fig. 1 (Caballero, Cufiado, and Bullon). Hyperemia of the papillae, enlarged veins, and retinal folds in the macular region of the left eye.
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Fig. 5 (Caballero, Cufiado, and Bullon). Detail of the ultrastructure of the tumor. There are numerous dense intracytoplasmic Weibel-Palade bodies, some elongated in the form of rods (arrows). (x47,000).
Fig. 6 (Caballero, Cufiado, and Bullon). Detail of several Weibel-Palade bodies. One contains fine microtubular structures arranged longitudinally (ar rowhead), (x 141,000).
elongated rods and contained fine microtubular structures lying in a longitudinal direction, form ing the characteristic Weibel-Palade bodies (Figs. 5 and 6).
tumor's rapid and destructive growth was directed interiorly, toward the orbital vertex, as well as exteriorly, because the left sphenoidal fissure was enlarged. The amaurosis and paralytic midriasis that de veloped could also be explained as the result of damage to the optic nerve and chiasm by compression or invasion.
DISCUSSION
We believed that this was a case of angiomatoid malignant fibrous histiocyto ma. The hemorrhagic foci and vascular spaces of this tumor suggested a vascular tumor, 5 but the histologie characteristics did not coincide with those of a vascular tumor. The presence of fasciculated bun dles of fibroblastoid cells with a cartwheel pattern and the xanthomatous cells with markedly atypical characteristics suggest ed a malignant fibrous histiocytoma. Rhabdomyosarcoma can have similar characteristics, but we found no rhabdomyoblasts with striae or microfilaments under the electron microscope. Also, the cells did not have lipoblastoid character istics. The ultrastructural study provided data about the tumor's type 3,8 and perhaps its histogenesis. The presence of dense elon gated bodies and microtubular structures (Figs. 5 and 6) suggested Weibel-Palade bodies, 9 which are now considered specif ic identifiers of the endothelial cell. The clinical data indicated that the
REFERENCES 1. Stout, A., and Lattes, R. : Tumors of soft tissues. In Atlas of Tumor Pathology. Bethesda, Armed Forces Institute of Pathology, 1967, p. 107. 2. Kempson, R., and Kiriakos, M.: Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histocytoma. Cancer 29:961, 1972. 3. Taxi, J., and Battifora, H.: Malignant fibrous histiocytoma. A clinicopathologic and ultrastructural study. Cancer 40:254, 1977. 4. Weis, S., and Enzinger, F.: Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41:2250, 1978. 5. Enzinger, F.: Angiomatoid malignant fibrous histiocytoma. Cancer 44:2147, 1979. 6. Rodrigues, M., Furgiuele, F., andWeinreb, S.: Malignant fibrous histiocytoma of the orbit. Arch. Ophthalmol. 95:2025, 1977. 7. Verity, M., Ebert, J., and Hepler, R.: Atypical fibrous histiocytoma of the orbit. Electron micro scopic study. Ophthalmologica 175:73, 1977. 8. Fu, Y., Gabbiani, C , Kaye, C , and Lattes, R.: Malignant soft tissue tumors of probable histiocyte origin (malignant fibrous histiocytomas). General considerations and electron microscopic and tissue culture studies. Cancer 35:176, 1975. 9. Weibel, F., and Palade, G.: New cytoplasmic components in arterial endothelia. J. Cell Biol. 23:101, 1964.
ANGEL CUNADO RODRIGUEZ,
AND A G U S T I N B U L L O N S O P E L A N A ,
M.D.,
M.D.
Salamanca, Spain
A 7-year-old boy had an angiomatoid malignant fibrous histiocytoma in his left orbit. After an adequate orbital exenteration, the tumor recurred and grew rapidly and destructively, filling the orbit. The patient died within six months. The ultrastructural study of the tumor showed some characteristics of histiocytes and fibroblasts, but also showed features, such as WeibelPalade bodies, that suggested an endothelial origin.
Malignant fibrous histiocytomas are a group of primitive connective cell tumors with cells characteristic of fibroblasts and histiocytes. This distinguishes them from other sarcomas of the soft tissues. Al though these tumors are rare, many cases have been reported. 1 " 3 The terminology varies from report to report because there are several variants or subtypes. Fibrous histiocytomas are found pri marily in the arms and legs, the abdo men, and the retroperitoneum. On rare occasions, they occur in the face and trunk. 4 Enzinger 5 recently reported 41 cases of a new variety, which he called angioma toid malignant fibrous histiocytoma. This variety, unlike the malignant fibrous his tiocytoma that usually occurs in old age, develops in young people (from 5 to 25 years old) and is often confused with a vascular tumor because of vascular spaces and areas of hemorrhage. Malignant fibrous histiocytoma of the From the Departments of Ophthalmology (Dr. Caballero) and Pathology (Drs. Cunado and Bullon), the University of Salamanca, Faculty of Medicine, Salamanca, Spain. Reprint requests to Luisa Rodriguez Caballero, M.D., Department of Ophthalmology, University of Salamanca, Faculty of Medicine, Salamanca, Spain.
orbit is rare. 6,7 We studied such a tumor in a 7-year-old boy, which we believe was of the angiomatoid type described by Enzinger. 0 CASE REPORT
A 7-year-old boy had had exophthalmos of the left eye for one month, along with visual loss in the left eye and slight headaches. His general health was good. Ophthalmologic findings—His visual acuity was R. E. : 6/6 (20/20) and L.E. : 6/60 (20/200). The papillae of the right eye were slightly pale, but the rest of the ophthalmologic findings were normal. In the left eye, the frontal segment, pupil, pupillary reflexes, and ocular motility were normal. The ophthalmoscopic examination showed hyperemia of the papillae and a blurring of the nasal margin, venous enlargement, and retinal folds in the paramacular region (Fig. 1). The axial exophthalmos measured 22 mm. The radiologie examination of the orbit showed an increase in the left sphenoidal fissure. Ten days later the exophthalmos had greatly in creased (Fig. 2). An examination showed conjunctival chemosis, comeal hypoesthesia, and ophthalmoplegia. The boy had severe headaches and his general condition had deteriorated. An orbital exenteration disclosed a violet tumor that filled the entire orbit. It resembled a vascular tumor. Twenty days later, there was a local recurrence of the tumor and the boy began to lose vision in his right eye. Within 15 days, amaurosis with paralytic mydriasis developed. The tumor grew rapidly and caused bone damage. Despite cytostatic treatment that initially decreased the tumor mass, the boy died. No autopsy was performed. Pathologic findings—The tumor had ill-defined borders and had invaded the fat and muscles of the
AMERICAN JOURNAL OF OPHTHALMOLOGY 92:13-15, 1981
13
14
AMERICAN JOURNAL OF OPHTHALMOLOGY
JULY, 1981
Fig. 2 (Caballero, Cufiado, and Bullon). Exophthalmos of the left eye ten days after the first examination.
orbit. When cut, it was gray-white with many vascu lar spaces and small hemorrhagic cysts or foci. When examined under the microscope, the sec tions stained with hematoxylin and eosin showed a fasciculated proliferation of spindle-shaped cells with a cartwheel pattern (Fig. 3). Some cells were atypi cal, with voluminous hyperchromatic nuclei and some atypical mitoses. The atypical cells were usually
xanthomatous and had abundant clear cytoplasm (Fig. 4). Some areas of the tumor disclosed extensive sclerosis with strips of hyaline substance in the intercellular space. Ultrastructural findings—The characteristics of the cells that showed their fibroblastoid differentia tion were (in addition to their spindle-shaped ap pearance under the microscope) the dense chromatin in their nuclei, dark cytoplasm, the abundance of rugous endoplasmic reticulum, and collagen fibrils in the interstice. The characteristics that showed their histiocytic differentiation were lobulated or notched nuclei and dense bodies of a lysosomal type in their cytoplasm, numerous mitochondria, and pseudopodic or philopodic projections of cellular membranes. Some of the dense bodies were in the shape of
Fig. 3 (Caballero, Cufiado, and Bullon). Histologie section of the tumor shows numerous vascular spaces and extravascular erythrocytes. The tumor cells mixed with hyaline collagen are grouped together in a cartwheel pattern (hematoxylin and eosin, X31).
Fig. 4 (Caballero, Cufiado, and Bullon). Marked nuclear pleomorphism, cells with clear xanthomatous cytoplasm (asterisk), and atypical mitosis (arrows) (hematoxylin and eosin, x98).
Fig. 1 (Caballero, Cufiado, and Bullon). Hyperemia of the papillae, enlarged veins, and retinal folds in the macular region of the left eye.
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Fig. 5 (Caballero, Cufiado, and Bullon). Detail of the ultrastructure of the tumor. There are numerous dense intracytoplasmic Weibel-Palade bodies, some elongated in the form of rods (arrows). (x47,000).
Fig. 6 (Caballero, Cufiado, and Bullon). Detail of several Weibel-Palade bodies. One contains fine microtubular structures arranged longitudinally (ar rowhead), (x 141,000).
elongated rods and contained fine microtubular structures lying in a longitudinal direction, form ing the characteristic Weibel-Palade bodies (Figs. 5 and 6).
tumor's rapid and destructive growth was directed interiorly, toward the orbital vertex, as well as exteriorly, because the left sphenoidal fissure was enlarged. The amaurosis and paralytic midriasis that de veloped could also be explained as the result of damage to the optic nerve and chiasm by compression or invasion.
DISCUSSION
We believed that this was a case of angiomatoid malignant fibrous histiocyto ma. The hemorrhagic foci and vascular spaces of this tumor suggested a vascular tumor, 5 but the histologie characteristics did not coincide with those of a vascular tumor. The presence of fasciculated bun dles of fibroblastoid cells with a cartwheel pattern and the xanthomatous cells with markedly atypical characteristics suggest ed a malignant fibrous histiocytoma. Rhabdomyosarcoma can have similar characteristics, but we found no rhabdomyoblasts with striae or microfilaments under the electron microscope. Also, the cells did not have lipoblastoid character istics. The ultrastructural study provided data about the tumor's type 3,8 and perhaps its histogenesis. The presence of dense elon gated bodies and microtubular structures (Figs. 5 and 6) suggested Weibel-Palade bodies, 9 which are now considered specif ic identifiers of the endothelial cell. The clinical data indicated that the
REFERENCES 1. Stout, A., and Lattes, R. : Tumors of soft tissues. In Atlas of Tumor Pathology. Bethesda, Armed Forces Institute of Pathology, 1967, p. 107. 2. Kempson, R., and Kiriakos, M.: Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histocytoma. Cancer 29:961, 1972. 3. Taxi, J., and Battifora, H.: Malignant fibrous histiocytoma. A clinicopathologic and ultrastructural study. Cancer 40:254, 1977. 4. Weis, S., and Enzinger, F.: Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41:2250, 1978. 5. Enzinger, F.: Angiomatoid malignant fibrous histiocytoma. Cancer 44:2147, 1979. 6. Rodrigues, M., Furgiuele, F., andWeinreb, S.: Malignant fibrous histiocytoma of the orbit. Arch. Ophthalmol. 95:2025, 1977. 7. Verity, M., Ebert, J., and Hepler, R.: Atypical fibrous histiocytoma of the orbit. Electron micro scopic study. Ophthalmologica 175:73, 1977. 8. Fu, Y., Gabbiani, C , Kaye, C , and Lattes, R.: Malignant soft tissue tumors of probable histiocyte origin (malignant fibrous histiocytomas). General considerations and electron microscopic and tissue culture studies. Cancer 35:176, 1975. 9. Weibel, F., and Palade, G.: New cytoplasmic components in arterial endothelia. J. Cell Biol. 23:101, 1964.