- Email: [email protected]
Cavitation in Ciliary Body Melanoma
Figure 1. The intraocular mass, which is rooted in the ciliary body (c), is arranged in a papillary pattern (hematoxylin and eosin; XlOO).
tal associations.2 History of trauma or inflammation was mentioned in some cases.3 In our patient, genetic counseling showed no remarkable abnormality. Epibulbar extension of adenocarcinomas of the ciliary body has been observed previously.1,3 In one series, five of 16 patients died from metastatic disease or tumor extension.4 We have been following up our patient for 3 years after enucleation, and prognosis seems good because neither extrascleral or optic nerve invasion were present. Rare intraocular tumors, such as medulloepitheliomas of the ciliary body, have been shown to be capable of masquerading as retinoblastoma.5 Adeno carcinoma of the ciliary body must also be included in the differential diagnosis of tumors originating from the ciliary body in young children. REFERENCES 1. Grossniklaus HE, Zimmerman LE, Kachner ML. Pleomorphic adenocarcinoma of the ciliary body. Ophthalmology 1990;97:763-768. 2. Papale J], Akiwama K, Hirose T. Adenocarcinoma of the ciliary body pigment epithelium in a child. Arch Ophthalmol 1984;102:100-103. 3. Rodrigues M, Hidayat A, Karesh ]. Pleomorphic adenocarci noma of ciliary epithelium simulating an epibulbar tumor. Am J Ophthalmol 1988;106:595-600. 4. Croxatto JO, Zimmerman LE. Malignant nonpigmented intra ocular tumors of neuroectodermal origin in adults: a review of 21 cases. In: Spencer WH, editor. Ophthalmic pathology, 3rd ed. Volume 2. Philadelphia: WB Saunders, 1985:1260-1262. 5. Hausmann N, Stefani FH. Medulloepithelioma of the ciliary body. Acta Ophthalmol 1991;69:398-401.
Figure 2. Reactive gliosis (g) is visible adjacent to the tumor area (t) in the sample obtained from the superotemporal perilimbal region (hematoxylin and eosin; X40).
2). A l t h o u g h some scleral vessels were invaded by
tumor cells, extrascleral and optic nerve invasion was not present. Immunohistochemical stain for cytokeratin and Fontana-Masson stain were negative. According to Zimmerman's classification,2 our pa tient's tumor could be considered papillary adenocarcinoma of nonpigmented ciliary epithelium. Though the cause of adenocarcinoma of the ciliary body is unknown, this type of tumor seems to occur sponta neously without any familial, genetic, or environmen VOL.123, No. 2
Cavitation in Ciliary Body Melanoma Cabrini T. Scott, MD, Gary N. Holland, MD, and Ben J. Glasgow, MD PURPOSE: To report a case of a cystic ciliary body melanoma that appeared clinically as a solid mass. METHODS: A 59-year-old woman underwent an iridocyclectomy for removal of a ciliary body melanoma in the right eye. The tumor was ana lyzed histopathologically for acid mucopolysaccharide production. RESULTS: Histopathology identified a ciliary body melanoma with cystic cavities. Contents of the cystic spaces did not stain with alcian blue.
BRIEF REPORTS
269
Histologic findings suggested exudation, cavitary necrosis, or both rather than acid mucopolysaccharide production as pathogenic factors for cyst for mation. CONCLUSION: Cystic cavities can develop in a solid, malignant tumor and therefore should not be considered signs of a benign lesion. E EXAMINED A 59-YEAR-OLD WOMAN WHO HAD pain of 4 months' duration in her right eye. Before the onset of symptoms, a comprehensive examination by her ophthalmologist had been unre markable. Examination disclosed best-corrected visu al acuity of BE, 20/30 — 1. Intraocular pressure measured 13 mm Hg in both eyes. The pupils were symmetric in size and reactions. In the right eye, a pigmented tumor infiltrated the peripheral iris, the ciliary body, and the anterior chamber angle superotemporally (Figure 1). O n transpupillary transillumination, the tumor blocked light transmission. There was mild nuclear sclerosis of both lenses. The macula, peripheral retina, and optic disk appeared normal in both eyes. A- and B-scan ultrasonography of the right eye showed an iris mass in the superotemporal area, with low internal reflectivity and moderate vascularity. Posterior extension to the ciliary body was not recog nized (Figure 1, inset). A n iridocyclectomy was performed to remove the tumor. Gross examination of the iridocyclectomy specimen disclosed a tumor that involved the peripheral iris and the ciliary body, including seven contiguous ciliary processes. The bisected tumor contained numerous cavities (Figure 2, inset). Viewed microscopically, a malignant melanoma had invaded the stroma and muscles of the iris and ciliary body. Cystic spaces within the ciliary body contained serous fluid that failed to stain for acid mucopolysaccharides with alcian blue. The overlying Accepted for publication Aug 6, 1996. Jules Stein Eye Institute, Departments of Ophthalmology (C.T.S., G.N.H., B.J.G.) and Pathology (B.J.G.), UCLA School of Medicine; and Division of Ophthalmology, Charles R. Drew University of Medicine and Science (C.T.S.). Inquiries to Ben J. Glasgow, MD, Jules Stein Eye Institute, 100 Stein Plaza, UCLA, Room B-269, Los Angeles, CA 90095-7000; fax: (310) 794-2144; e-mail: [email protected]
270
Figure 1. Right eye, preoperatively, shows a raised lesion in the periphery of the iris and anterior chamber angle superotemporally between the 10- and 12-o'clock posi tions. (Inset) A-scan ultrasonography of the right eye using stand-off technique shows a lesion with low internal reflectivity (arrow).
Figure 2. Pigmented tumor of the ciliary body with cystic spaces (C) containing serous fluid and bordered by necrotic tumor cells (arrows) (hematoxylin and eosin, X 100). (Inset) Bisected gross specimen showing numer ous cystic cavities (C).
nonpigmented ciliary epithelium contained alcian blue material (positive control). Necrotic tumor cells lined the cavities (Figure 2). Spindle-shaped and epithelioid cells showed pleomorphism, mitotic fig ures, and tumor giant cells. Four previous cases of cystic melanomas of the
AMERICAN JOURNAL OF OPHTHALMOLOGY
FEBRUARY 1997
ciliary body have been reported.1,2 Of the four cases, two were clinically diagnosed as solid masses.1 A third tumor was clinically diagnosed as a solid mass initial ly, but 2 months later appeared cystic by clinical examination and ultrasonography. Before enucleation, the mass again appeared solid.2 Several hypotheses have been formulated regarding the basis for cystic changes in malignant melanomas. Tumor necrosis and hemorrhage,1'3 serous exudation,4 and elaboration of an acid mucopolysaccharide sub stance2 have been proposed. Necrosis and hemor rhage have been disputed as mechanisms by authors who, in contrast, have shown alcian blue staining indicative of an acid mucopolysaccharide substance within cysts of intraocular melanoma.2 Our case shows evidence of cavitary necrosis but no alcian blue-staining material. Upon reviewing the previously reported case, in which alcian blue staining was shown,2 one of the authors suggested that celloidin uptake of the alcian blue stain may have accounted for the previously published findings (Foos RY, Hogan Ophthalmic Pathology Society Meeting, September 17, 1995, Seattle, Washington). Cystic spaces in a ciliary melanoma may be unde tected on clinical examination1 or may alter the overall architecture of the tumor, leading to delayed diagnosis and treatment.2 With the advent of ultra sound biomicroscopy, it is important for clinicians to recognize that cystic change is not necessarily associ ated solely with benign lesions.5 One should be suspicious of a mass that shows cystic cavities or undergoes change from a solid to a cystic lesion. REFERENCES 1. Kennedy RE. Cystic malignant melanomas of the uveal tract. Ophthalmology 1948;31:159-167. 2. Zakka KA, Foos RY, Spencer WH, et al. Cavitation in intraocular malignant melanoma. Arch Ophthalmol 1982;100:112-114. 3. Reese AB. Pigmented tumors. In: Reese AB. Tumors of the eye, 3rd ed. New York: Harper & Row Publishers, 1976: 173-262. 4. Zimmerman LE. The uveal tract: malignant melanoma. In: Spencer WH, editor. Ophthalmic pathology: an adas and textbook, 3rd ed. Philadelphia: W. B. Saunders, 1986: 2072-2139. 5. Pavlin C], McWhae JA, McGowan HD, Foster FS. Ultra sound biomicroscopy of anterior segment tumors. Ophthal mology 1992;99:1220-1228.
VOL.123, No. 2
Metastatic Liposarcoma to the Orbit J. Fezza, MD, and J. Sinard, MD, PhD PURPOSE: To report a woman with blurred vision and proptosis who harbored an orbital liposarcoma that had metastasized from her abdomen. METHODS: The patient underwent an orbital biopsy, followed by postoperative intravenous corticosteroids and radiation. RESULTS: Pathology showed a dedifferentiated liposarcoma. The patient's visual acuity improved from 20/200 to 20/50 after treatment. CONCLUSIONS: Physicians should suspect the presence of this rare orbital tumor in a person with a history of liposarcoma who has proptosis. Liposarcomas usually exhibit a higher grade of malig nancy with recurrence, as shown in this patient.
A
75-YEAR-OLD WOMAN HAD A HISTORY OF A WELLdifferentiated abdominal liposarcoma, with ab dominal recurrences 9 and 11 years after her initial resection. Four years after her second recurrence, she was referred to us for decreased vision in her left eye for a duration of 6 weeks. Upon examination, visual acuity was LE, 20/200, and she had left eye proptosis with limited extraocular movements in all directions. Results of anterior segment examination were unre markable. Results of fundus examination were re markable for a choked and elevated left optic disk with blurred margins and surrounding intraretinal hemorrhages (Figure 1). A computed tomographic scan with contrast was performed to rule out an orbital mass. The scan showed a homogeneously enhancing left intraconal mass adherent to the medial rectus muscle. It measured 1.2 X 2.2 cm and displaced the optic nerve medially (Figure 2). The patient underwent a left lateral orbitotomy and biopsy of the tumor, which was infiltrating the medial rectus muscle and was adherent to the optic nerve.
Accepted for publication Sept 23, 1996. Departments of Ophthalmology (J.F.) and Pathology (J.S.), Yale University. Inquiries to J. Fezza, MD, 420 Elmington Ave, Apt 704, Nashville, TN 37205.
BRIEF REPORTS
271
tal associations.2 History of trauma or inflammation was mentioned in some cases.3 In our patient, genetic counseling showed no remarkable abnormality. Epibulbar extension of adenocarcinomas of the ciliary body has been observed previously.1,3 In one series, five of 16 patients died from metastatic disease or tumor extension.4 We have been following up our patient for 3 years after enucleation, and prognosis seems good because neither extrascleral or optic nerve invasion were present. Rare intraocular tumors, such as medulloepitheliomas of the ciliary body, have been shown to be capable of masquerading as retinoblastoma.5 Adeno carcinoma of the ciliary body must also be included in the differential diagnosis of tumors originating from the ciliary body in young children. REFERENCES 1. Grossniklaus HE, Zimmerman LE, Kachner ML. Pleomorphic adenocarcinoma of the ciliary body. Ophthalmology 1990;97:763-768. 2. Papale J], Akiwama K, Hirose T. Adenocarcinoma of the ciliary body pigment epithelium in a child. Arch Ophthalmol 1984;102:100-103. 3. Rodrigues M, Hidayat A, Karesh ]. Pleomorphic adenocarci noma of ciliary epithelium simulating an epibulbar tumor. Am J Ophthalmol 1988;106:595-600. 4. Croxatto JO, Zimmerman LE. Malignant nonpigmented intra ocular tumors of neuroectodermal origin in adults: a review of 21 cases. In: Spencer WH, editor. Ophthalmic pathology, 3rd ed. Volume 2. Philadelphia: WB Saunders, 1985:1260-1262. 5. Hausmann N, Stefani FH. Medulloepithelioma of the ciliary body. Acta Ophthalmol 1991;69:398-401.
Figure 2. Reactive gliosis (g) is visible adjacent to the tumor area (t) in the sample obtained from the superotemporal perilimbal region (hematoxylin and eosin; X40).
2). A l t h o u g h some scleral vessels were invaded by
tumor cells, extrascleral and optic nerve invasion was not present. Immunohistochemical stain for cytokeratin and Fontana-Masson stain were negative. According to Zimmerman's classification,2 our pa tient's tumor could be considered papillary adenocarcinoma of nonpigmented ciliary epithelium. Though the cause of adenocarcinoma of the ciliary body is unknown, this type of tumor seems to occur sponta neously without any familial, genetic, or environmen VOL.123, No. 2
Cavitation in Ciliary Body Melanoma Cabrini T. Scott, MD, Gary N. Holland, MD, and Ben J. Glasgow, MD PURPOSE: To report a case of a cystic ciliary body melanoma that appeared clinically as a solid mass. METHODS: A 59-year-old woman underwent an iridocyclectomy for removal of a ciliary body melanoma in the right eye. The tumor was ana lyzed histopathologically for acid mucopolysaccharide production. RESULTS: Histopathology identified a ciliary body melanoma with cystic cavities. Contents of the cystic spaces did not stain with alcian blue.
BRIEF REPORTS
269
Histologic findings suggested exudation, cavitary necrosis, or both rather than acid mucopolysaccharide production as pathogenic factors for cyst for mation. CONCLUSION: Cystic cavities can develop in a solid, malignant tumor and therefore should not be considered signs of a benign lesion. E EXAMINED A 59-YEAR-OLD WOMAN WHO HAD pain of 4 months' duration in her right eye. Before the onset of symptoms, a comprehensive examination by her ophthalmologist had been unre markable. Examination disclosed best-corrected visu al acuity of BE, 20/30 — 1. Intraocular pressure measured 13 mm Hg in both eyes. The pupils were symmetric in size and reactions. In the right eye, a pigmented tumor infiltrated the peripheral iris, the ciliary body, and the anterior chamber angle superotemporally (Figure 1). O n transpupillary transillumination, the tumor blocked light transmission. There was mild nuclear sclerosis of both lenses. The macula, peripheral retina, and optic disk appeared normal in both eyes. A- and B-scan ultrasonography of the right eye showed an iris mass in the superotemporal area, with low internal reflectivity and moderate vascularity. Posterior extension to the ciliary body was not recog nized (Figure 1, inset). A n iridocyclectomy was performed to remove the tumor. Gross examination of the iridocyclectomy specimen disclosed a tumor that involved the peripheral iris and the ciliary body, including seven contiguous ciliary processes. The bisected tumor contained numerous cavities (Figure 2, inset). Viewed microscopically, a malignant melanoma had invaded the stroma and muscles of the iris and ciliary body. Cystic spaces within the ciliary body contained serous fluid that failed to stain for acid mucopolysaccharides with alcian blue. The overlying Accepted for publication Aug 6, 1996. Jules Stein Eye Institute, Departments of Ophthalmology (C.T.S., G.N.H., B.J.G.) and Pathology (B.J.G.), UCLA School of Medicine; and Division of Ophthalmology, Charles R. Drew University of Medicine and Science (C.T.S.). Inquiries to Ben J. Glasgow, MD, Jules Stein Eye Institute, 100 Stein Plaza, UCLA, Room B-269, Los Angeles, CA 90095-7000; fax: (310) 794-2144; e-mail: [email protected]
270
Figure 1. Right eye, preoperatively, shows a raised lesion in the periphery of the iris and anterior chamber angle superotemporally between the 10- and 12-o'clock posi tions. (Inset) A-scan ultrasonography of the right eye using stand-off technique shows a lesion with low internal reflectivity (arrow).
Figure 2. Pigmented tumor of the ciliary body with cystic spaces (C) containing serous fluid and bordered by necrotic tumor cells (arrows) (hematoxylin and eosin, X 100). (Inset) Bisected gross specimen showing numer ous cystic cavities (C).
nonpigmented ciliary epithelium contained alcian blue material (positive control). Necrotic tumor cells lined the cavities (Figure 2). Spindle-shaped and epithelioid cells showed pleomorphism, mitotic fig ures, and tumor giant cells. Four previous cases of cystic melanomas of the
AMERICAN JOURNAL OF OPHTHALMOLOGY
FEBRUARY 1997
ciliary body have been reported.1,2 Of the four cases, two were clinically diagnosed as solid masses.1 A third tumor was clinically diagnosed as a solid mass initial ly, but 2 months later appeared cystic by clinical examination and ultrasonography. Before enucleation, the mass again appeared solid.2 Several hypotheses have been formulated regarding the basis for cystic changes in malignant melanomas. Tumor necrosis and hemorrhage,1'3 serous exudation,4 and elaboration of an acid mucopolysaccharide sub stance2 have been proposed. Necrosis and hemor rhage have been disputed as mechanisms by authors who, in contrast, have shown alcian blue staining indicative of an acid mucopolysaccharide substance within cysts of intraocular melanoma.2 Our case shows evidence of cavitary necrosis but no alcian blue-staining material. Upon reviewing the previously reported case, in which alcian blue staining was shown,2 one of the authors suggested that celloidin uptake of the alcian blue stain may have accounted for the previously published findings (Foos RY, Hogan Ophthalmic Pathology Society Meeting, September 17, 1995, Seattle, Washington). Cystic spaces in a ciliary melanoma may be unde tected on clinical examination1 or may alter the overall architecture of the tumor, leading to delayed diagnosis and treatment.2 With the advent of ultra sound biomicroscopy, it is important for clinicians to recognize that cystic change is not necessarily associ ated solely with benign lesions.5 One should be suspicious of a mass that shows cystic cavities or undergoes change from a solid to a cystic lesion. REFERENCES 1. Kennedy RE. Cystic malignant melanomas of the uveal tract. Ophthalmology 1948;31:159-167. 2. Zakka KA, Foos RY, Spencer WH, et al. Cavitation in intraocular malignant melanoma. Arch Ophthalmol 1982;100:112-114. 3. Reese AB. Pigmented tumors. In: Reese AB. Tumors of the eye, 3rd ed. New York: Harper & Row Publishers, 1976: 173-262. 4. Zimmerman LE. The uveal tract: malignant melanoma. In: Spencer WH, editor. Ophthalmic pathology: an adas and textbook, 3rd ed. Philadelphia: W. B. Saunders, 1986: 2072-2139. 5. Pavlin C], McWhae JA, McGowan HD, Foster FS. Ultra sound biomicroscopy of anterior segment tumors. Ophthal mology 1992;99:1220-1228.
VOL.123, No. 2
Metastatic Liposarcoma to the Orbit J. Fezza, MD, and J. Sinard, MD, PhD PURPOSE: To report a woman with blurred vision and proptosis who harbored an orbital liposarcoma that had metastasized from her abdomen. METHODS: The patient underwent an orbital biopsy, followed by postoperative intravenous corticosteroids and radiation. RESULTS: Pathology showed a dedifferentiated liposarcoma. The patient's visual acuity improved from 20/200 to 20/50 after treatment. CONCLUSIONS: Physicians should suspect the presence of this rare orbital tumor in a person with a history of liposarcoma who has proptosis. Liposarcomas usually exhibit a higher grade of malig nancy with recurrence, as shown in this patient.
A
75-YEAR-OLD WOMAN HAD A HISTORY OF A WELLdifferentiated abdominal liposarcoma, with ab dominal recurrences 9 and 11 years after her initial resection. Four years after her second recurrence, she was referred to us for decreased vision in her left eye for a duration of 6 weeks. Upon examination, visual acuity was LE, 20/200, and she had left eye proptosis with limited extraocular movements in all directions. Results of anterior segment examination were unre markable. Results of fundus examination were re markable for a choked and elevated left optic disk with blurred margins and surrounding intraretinal hemorrhages (Figure 1). A computed tomographic scan with contrast was performed to rule out an orbital mass. The scan showed a homogeneously enhancing left intraconal mass adherent to the medial rectus muscle. It measured 1.2 X 2.2 cm and displaced the optic nerve medially (Figure 2). The patient underwent a left lateral orbitotomy and biopsy of the tumor, which was infiltrating the medial rectus muscle and was adherent to the optic nerve.
Accepted for publication Sept 23, 1996. Departments of Ophthalmology (J.F.) and Pathology (J.S.), Yale University. Inquiries to J. Fezza, MD, 420 Elmington Ave, Apt 704, Nashville, TN 37205.
BRIEF REPORTS
271