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Testicular Seminoma Metastatic to the Orbit
TESTICULAR SEMINOMA METASTATIC TO THE ORBIT JAMES A. RUSH, M.D.,
JAY J. OLDER, M.D., AND ALAN V. RICHMAN, Tampa, Florida
Bilateral exophthalmos, possibly caused by hormonal influence, has been docu mented in three patients with localized seminoma1"3 but, to our knowledge, no case of orbital seminoma metastatic from the testis has been previously docu mented. Three previously described cases of orbital tumor metastatic from the testes4,5 were choriocarcinomas rather than the less aggressive testicular neo plasm, the seminoma. We studied a case of testicular seminoma metastatic to the orbit in a patient whose ocular symptoms went into remission after orbital irradia tion.
M.D.
choroidal striae were present. The left eye was normal. Serum T3 and T4 values and chest roentgenograms were normal. A computed axial tomographic scan of the right orbit showed a diffused density. He began a three-week course of prednisone, with the dosage gradually decreased, on March 15, 1980. On April 14, 1980, visual acuity was R.E.: 6/60 (20/200) and L.E.: 6/6 (20/20). An afferent pupillary defect was present in the right eye and the optic disk was edematous. A mass was palpable through the upper eyelid. A computed axial tomographic scan showed a discrete mass in the right superior orbit. On April 15, 1980, a metastatic seminoma was found during an anterior orbitotomy and biopsy (Figure). Postoperatively, 3,000 rads of radioactive cobalt (^Co) was delivered to the right orbit. On May 14, 1980, visual acuity was R.E.: 6/12 (20/40). The optic neuropathy was improved but the choroidal striae persisted.
CASE REPORT A 28-year-old man was seen on March 12, 1980, for evaluation of painful exophthalmos of the right eye of four months' duration. He had been well until June 1979, when a seminoma of the right testis, which had been present for 18 months, was removed by radical scrotal orchiectomy. Preoperative and postoperative serum values for human chorionic gonadotropin, beta subunit, were 49 and 21 mU/ml, respectively (normal for males, 0 mU/ml). Radiation therapy was completed and he was well until November 1979, when transient right periorbital pain and eyelid swelling suddenly developed. Two weeks later diplopia and proptosis occurred; in February 1980 he noted blurred vision in his right eye. On March 12, 1980, his visual acuity was R. E. : 6/24 (20/80) and L.E.: 6/6 (20/20). The right eye was proptotic by 5 mm (Hertel exophthalmometer at base 106, R.E.: 21 mm and L.E.: 16 mm) and was displaced 10 mm inferiorly. No orbital mass was palpable superiorly. There was partial ophthalmoplegia of the right eye but no blepharoptosis or chemosis. The pupils were normal. The right optic disk was elevated, the posterior pole was indented, and
From the Departments of Ophthalmology (Drs. Rush and Older) and Pathology (Dr. Richman), University of South Florida, College of Medicine, Tampa, Florida. Reprint requests to James A. Rush, M.D., Univer sity of South Florida, College of Medicine, 12901 N. 30th St., Box 21, Tampa, FL 33612. 258
Figure (Rush, Older, and Richman). Photomicro graph of the metastasis of the right orbit, showing sheets of malignant cells with large nuclei, promi nent nucleoli, and a mild degree of nuclear anaplasia. There are a few mitoses. Most tumor cells have clear cytoplasm with well-demarcated cytoplasmic bor ders. Small lymphocytes are interspersed among the tumor cells. This pattern was typical for pure semino ma, and no histologie features of any other germ cell neoplasm were seen (hematoxylin and eosin, x 340).
AMERICAN JOURNAL OF OPHTHALMOLOGY 91:258-260, 1981
VOL. 91, NO. 2
TESTICULAR SEMINOMA
DISCUSSION
Neoplasms of the testes constitute only 1% of all malignant tumors in men but are the most common malignancies af fecting men 29 to 35 years of age.6 Malignant testicular tumors include semi noma, embryonal carcinoma, teratocarcinoma, and choriocarcinoma. Each usu ally occurs as a single cell type. 7 Almost one third of all cases are pure seminomas.8 The nonseminomatous neoplasms of the testes are invasive tumors. Orbital métastases from testicular choriocarcinomas have been reported.4'5 Testicular seminomas, however, are sensitive to radiation therapy, and patients treated with orchiectomy and lymph node irradi ation have a survival rate of more than 95%. They are unlikely to have orbital métastases. Bilateral exophthalmos without orbital métastases has been reported in three middle-aged patients with localized semi noma.1"3 In two of these patients, sym metrical, painless exophthalmos and re stricted ocular motility were the initial manifestations of a testicular seminoma. Conjunctival chemosis was striking, and thyroid exophthalmos was suspected, but serum T3 and T4 values were normal. The ocular symptoms went into remission in one patient who was treated with prednisone,2 and permanent resolution of the exophthalmos and chemosis occurred in both patients after orchiectomy. The authors thought a remote hormonal effect of the testicular neoplasm caused the proptosis in these patients.1'2 A third patient with seminoma who had bilateral exophthalmos, presumably of hormonal origin, had a mediastinal seminoma at autopsy examination, but no orbital tu mor.3 The bilateral orbital involvement in these cases differed from the unilateral orbital symptoms in our patient, in whom pain, diplopia, and proptosis without chemosis occurred. More than 90% of
259
cases of metastatic orbital carcinoma are unilateral; the primary symptoms are pain and diplopia.9 Our patient's symp toms resembled those caused by meta static orbital carcinoma and were dif ferent from those caused by bilateral, congestive proptosis of presumed endo crine influence reported in other patients who had localized seminomas.1"3 Less than 15% of all seminomas pro duce distant métastases10; these usually metastasize to lymph nodes, bones, and lung.11 Because our patient had not had the mass in his right testicle treated immediately, the diagnosis of the pri mary tumor was delayed, allowing tumor cells to seed his orbit before the delivery of abdominal irradiation. Henderson9 noted the significance of orbital symp toms developing in patients who have carcinoma; we emphasize that the orbit can also be the first site of disseminated seminoma. The prognosis of seminomas is ulti mately related to the cellular constituents of the tumor. One subgroup, the trophoblastic seminoma, which contains syncytiotrophoblastic cells that secrete hu man chorionic gonadotropin, is less sensitive to radiation therapy than the typical seminoma and may develop into the more aggressive choriocarcinoma.12 Although the histologie findings of the seminoma in our patient were those of a typical seminoma, we found increased serum levels of human chorionic gonado tropin, beta subunit. This may indicate a poor prognosis. Thirty percent of all seminomas me tastasize as a different histologie type, presumably because of unknown factors at metastatic sites that act upon persis tent stem cells and influence cellular dif ferentiation.12 The orbital biopsy in our patient did not show this evolution, however, and reports of good results with radiation therapy11 and chemotherapy13 in cases of pure metastatic seminoma
260
AMERICAN JOURNAL OF OPHTHALMOLOGY
suggest a favorable patient.
outcome
for
our
SUMMARY
Painful exophthalmos, ophthalmople gia, and visual loss in the right eye developed in a 28-year-old man four months after he underwent a radical orchiectomy and irradiation for a seminoma of the right testicle. Prednisone thera py failed to relieve the symptoms, and an orbital biopsy found a metastatic testicu lar seminoma. Orbital irradiation with radioactive cobalt reduced the exophthal mos, reversed the ophthalmoplegia, and improved his visual acuity. Previous cases of nonmetastatic exophthalmos in patients who had testicular seminoma have been reported, but, to the best of our knowledge, this is the first report of a patient who had orbital seminoma meta static from the testis. Successful irradia tion of the orbital focus can minimize ocular morbidity and may improve the prognosis. REFERENCES 1. Mann, A. S.: Bilateral exophthalmos in semino ma. J. Clin. Endocrinol. 27:1500, 1967.
FEBRUARY, 1981
2. Taylor, J. B., Solomon, D. H., Levine, R. E., and Ehrlich, R. M.: Exophthalmos in seminoma. Regression with steroids and orchiectomy. J.A. M.A. 240:860, 1978. 3. Leyson, J. F.: Mediastinal seminoma associat ed with exophthalmos and gynecomastia. Urology 3:366, 1974. 4. Font, R. L., and Ferry, A. P. : Carcinoma meta static to the eye and orbit. III. A clinico-pathologic study of 28 cases metastatic to the orbit. Cancer 38:1326, 1976. 5. Jensen, O. A. : Metastatic tumors of the eye and orbit. A histopathological analysis of a Danish series. Acta Pathol. Microbiol. Scand. 212(Suppl.):201, 1970. 6. Slawson, R. G.: Radiation therapy for germinal tumors of the testes. Cancer 42:2216, 1978. 7. Kaplan, G., Cohen, B. B., and Roswit, B.: Malignant testicular tumors. Am. J. Roentgenol. Radium Ther. Nucl. Med. 66:405, 1951. 8. Maier, J. C , and Mittenmeyer, B.: Carcinoma of the testes. Cancer 39:981, 1977. 9. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1978, pp. 474-494. 10. Martin, L. S. J., Woodruff, M. W., Webster, J. H., and Pickren, J. W. : Testicular seminoma. A review of 179 patients treated over 50 years. Arch. Surg. 90:306, 1965. 11. Friedman, M., and Purkayastha, M. D.: Re current seminoma. The management of late metasta sis, recurrence, or a second primary tumor. Am. J. Roentgenol. Radium Ther. Nucl. Med. 83:25, 1961. 12. Merrin, C : Seminoma. Urol. Clin. North Am. 4:383, 1977. 13. Einhorn, L. H., and Donahue, J. P.: Chemo therapy for disseminated testicular cancer. Urol. Clin. North Am. 4:704, 1977.
JAY J. OLDER, M.D., AND ALAN V. RICHMAN, Tampa, Florida
Bilateral exophthalmos, possibly caused by hormonal influence, has been docu mented in three patients with localized seminoma1"3 but, to our knowledge, no case of orbital seminoma metastatic from the testis has been previously docu mented. Three previously described cases of orbital tumor metastatic from the testes4,5 were choriocarcinomas rather than the less aggressive testicular neo plasm, the seminoma. We studied a case of testicular seminoma metastatic to the orbit in a patient whose ocular symptoms went into remission after orbital irradia tion.
M.D.
choroidal striae were present. The left eye was normal. Serum T3 and T4 values and chest roentgenograms were normal. A computed axial tomographic scan of the right orbit showed a diffused density. He began a three-week course of prednisone, with the dosage gradually decreased, on March 15, 1980. On April 14, 1980, visual acuity was R.E.: 6/60 (20/200) and L.E.: 6/6 (20/20). An afferent pupillary defect was present in the right eye and the optic disk was edematous. A mass was palpable through the upper eyelid. A computed axial tomographic scan showed a discrete mass in the right superior orbit. On April 15, 1980, a metastatic seminoma was found during an anterior orbitotomy and biopsy (Figure). Postoperatively, 3,000 rads of radioactive cobalt (^Co) was delivered to the right orbit. On May 14, 1980, visual acuity was R.E.: 6/12 (20/40). The optic neuropathy was improved but the choroidal striae persisted.
CASE REPORT A 28-year-old man was seen on March 12, 1980, for evaluation of painful exophthalmos of the right eye of four months' duration. He had been well until June 1979, when a seminoma of the right testis, which had been present for 18 months, was removed by radical scrotal orchiectomy. Preoperative and postoperative serum values for human chorionic gonadotropin, beta subunit, were 49 and 21 mU/ml, respectively (normal for males, 0 mU/ml). Radiation therapy was completed and he was well until November 1979, when transient right periorbital pain and eyelid swelling suddenly developed. Two weeks later diplopia and proptosis occurred; in February 1980 he noted blurred vision in his right eye. On March 12, 1980, his visual acuity was R. E. : 6/24 (20/80) and L.E.: 6/6 (20/20). The right eye was proptotic by 5 mm (Hertel exophthalmometer at base 106, R.E.: 21 mm and L.E.: 16 mm) and was displaced 10 mm inferiorly. No orbital mass was palpable superiorly. There was partial ophthalmoplegia of the right eye but no blepharoptosis or chemosis. The pupils were normal. The right optic disk was elevated, the posterior pole was indented, and
From the Departments of Ophthalmology (Drs. Rush and Older) and Pathology (Dr. Richman), University of South Florida, College of Medicine, Tampa, Florida. Reprint requests to James A. Rush, M.D., Univer sity of South Florida, College of Medicine, 12901 N. 30th St., Box 21, Tampa, FL 33612. 258
Figure (Rush, Older, and Richman). Photomicro graph of the metastasis of the right orbit, showing sheets of malignant cells with large nuclei, promi nent nucleoli, and a mild degree of nuclear anaplasia. There are a few mitoses. Most tumor cells have clear cytoplasm with well-demarcated cytoplasmic bor ders. Small lymphocytes are interspersed among the tumor cells. This pattern was typical for pure semino ma, and no histologie features of any other germ cell neoplasm were seen (hematoxylin and eosin, x 340).
AMERICAN JOURNAL OF OPHTHALMOLOGY 91:258-260, 1981
VOL. 91, NO. 2
TESTICULAR SEMINOMA
DISCUSSION
Neoplasms of the testes constitute only 1% of all malignant tumors in men but are the most common malignancies af fecting men 29 to 35 years of age.6 Malignant testicular tumors include semi noma, embryonal carcinoma, teratocarcinoma, and choriocarcinoma. Each usu ally occurs as a single cell type. 7 Almost one third of all cases are pure seminomas.8 The nonseminomatous neoplasms of the testes are invasive tumors. Orbital métastases from testicular choriocarcinomas have been reported.4'5 Testicular seminomas, however, are sensitive to radiation therapy, and patients treated with orchiectomy and lymph node irradi ation have a survival rate of more than 95%. They are unlikely to have orbital métastases. Bilateral exophthalmos without orbital métastases has been reported in three middle-aged patients with localized semi noma.1"3 In two of these patients, sym metrical, painless exophthalmos and re stricted ocular motility were the initial manifestations of a testicular seminoma. Conjunctival chemosis was striking, and thyroid exophthalmos was suspected, but serum T3 and T4 values were normal. The ocular symptoms went into remission in one patient who was treated with prednisone,2 and permanent resolution of the exophthalmos and chemosis occurred in both patients after orchiectomy. The authors thought a remote hormonal effect of the testicular neoplasm caused the proptosis in these patients.1'2 A third patient with seminoma who had bilateral exophthalmos, presumably of hormonal origin, had a mediastinal seminoma at autopsy examination, but no orbital tu mor.3 The bilateral orbital involvement in these cases differed from the unilateral orbital symptoms in our patient, in whom pain, diplopia, and proptosis without chemosis occurred. More than 90% of
259
cases of metastatic orbital carcinoma are unilateral; the primary symptoms are pain and diplopia.9 Our patient's symp toms resembled those caused by meta static orbital carcinoma and were dif ferent from those caused by bilateral, congestive proptosis of presumed endo crine influence reported in other patients who had localized seminomas.1"3 Less than 15% of all seminomas pro duce distant métastases10; these usually metastasize to lymph nodes, bones, and lung.11 Because our patient had not had the mass in his right testicle treated immediately, the diagnosis of the pri mary tumor was delayed, allowing tumor cells to seed his orbit before the delivery of abdominal irradiation. Henderson9 noted the significance of orbital symp toms developing in patients who have carcinoma; we emphasize that the orbit can also be the first site of disseminated seminoma. The prognosis of seminomas is ulti mately related to the cellular constituents of the tumor. One subgroup, the trophoblastic seminoma, which contains syncytiotrophoblastic cells that secrete hu man chorionic gonadotropin, is less sensitive to radiation therapy than the typical seminoma and may develop into the more aggressive choriocarcinoma.12 Although the histologie findings of the seminoma in our patient were those of a typical seminoma, we found increased serum levels of human chorionic gonado tropin, beta subunit. This may indicate a poor prognosis. Thirty percent of all seminomas me tastasize as a different histologie type, presumably because of unknown factors at metastatic sites that act upon persis tent stem cells and influence cellular dif ferentiation.12 The orbital biopsy in our patient did not show this evolution, however, and reports of good results with radiation therapy11 and chemotherapy13 in cases of pure metastatic seminoma
260
AMERICAN JOURNAL OF OPHTHALMOLOGY
suggest a favorable patient.
outcome
for
our
SUMMARY
Painful exophthalmos, ophthalmople gia, and visual loss in the right eye developed in a 28-year-old man four months after he underwent a radical orchiectomy and irradiation for a seminoma of the right testicle. Prednisone thera py failed to relieve the symptoms, and an orbital biopsy found a metastatic testicu lar seminoma. Orbital irradiation with radioactive cobalt reduced the exophthal mos, reversed the ophthalmoplegia, and improved his visual acuity. Previous cases of nonmetastatic exophthalmos in patients who had testicular seminoma have been reported, but, to the best of our knowledge, this is the first report of a patient who had orbital seminoma meta static from the testis. Successful irradia tion of the orbital focus can minimize ocular morbidity and may improve the prognosis. REFERENCES 1. Mann, A. S.: Bilateral exophthalmos in semino ma. J. Clin. Endocrinol. 27:1500, 1967.
FEBRUARY, 1981
2. Taylor, J. B., Solomon, D. H., Levine, R. E., and Ehrlich, R. M.: Exophthalmos in seminoma. Regression with steroids and orchiectomy. J.A. M.A. 240:860, 1978. 3. Leyson, J. F.: Mediastinal seminoma associat ed with exophthalmos and gynecomastia. Urology 3:366, 1974. 4. Font, R. L., and Ferry, A. P. : Carcinoma meta static to the eye and orbit. III. A clinico-pathologic study of 28 cases metastatic to the orbit. Cancer 38:1326, 1976. 5. Jensen, O. A. : Metastatic tumors of the eye and orbit. A histopathological analysis of a Danish series. Acta Pathol. Microbiol. Scand. 212(Suppl.):201, 1970. 6. Slawson, R. G.: Radiation therapy for germinal tumors of the testes. Cancer 42:2216, 1978. 7. Kaplan, G., Cohen, B. B., and Roswit, B.: Malignant testicular tumors. Am. J. Roentgenol. Radium Ther. Nucl. Med. 66:405, 1951. 8. Maier, J. C , and Mittenmeyer, B.: Carcinoma of the testes. Cancer 39:981, 1977. 9. Henderson, J. W.: Orbital Tumors. Philadel phia, W. B. Saunders Co., 1978, pp. 474-494. 10. Martin, L. S. J., Woodruff, M. W., Webster, J. H., and Pickren, J. W. : Testicular seminoma. A review of 179 patients treated over 50 years. Arch. Surg. 90:306, 1965. 11. Friedman, M., and Purkayastha, M. D.: Re current seminoma. The management of late metasta sis, recurrence, or a second primary tumor. Am. J. Roentgenol. Radium Ther. Nucl. Med. 83:25, 1961. 12. Merrin, C : Seminoma. Urol. Clin. North Am. 4:383, 1977. 13. Einhorn, L. H., and Donahue, J. P.: Chemo therapy for disseminated testicular cancer. Urol. Clin. North Am. 4:704, 1977.