- Email: [email protected]
High incidence of orbital malignant lymphoma in Japanese patients
clinical and laboratory investigations, the patient was diagnosed as having SSPE. He was hospitalized in Pediatrics Clinic and isoprinosine (100 mg/kg) and carbamazepine (10 mg/kg) were started. Neurologic symptoms and cognitive functions, however, deteriorated rapidly. After a 2-week hospital stay, he was discharged at the request of his family. He did not return for follow-up visits. Our case is of interest because it demonstrates a dramatic ocular involvement as the initial clinical presentation of SSPE which preceded the neurologic symptoms by 4 months. Such cases of SSPE with ocular onset can be easily misdiagnosed as hereditary dystrophy or toxoplasmosis in the absence of neurologic symptoms.2,4 The common fundus lesions of SSPE are papilledema, papillitis, optic atrophy, and macular degeneration of presumed infective etiology.1–5 Optic atrophy, which is a prominent finding in our case, has been reported to be directly related to the neurologic involvement.1 Macular degeneration, the most characteristic fundus lesion of SSPE, initiates as chorioretinitis with macular edema, hemorrhages, and white infiltrates, and leads to macular scarring, retinal pigment epithelial atrophy, and epiretinal membrane formation, as it is in our case.1,3,4 Clinicians should be aware that optic atrophy with macular changes including retinal pigment epithelial atrophy can be the initial presentation of SSPE even in the absence of neurologic signs and symptoms. REFERENCES
1. De Laey JJ, Hanssens M, Colette P, Geerts L, Priem H. Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations. Doc Ophthalmol 1983;56:11–21. 2. Salmon JF, Pan EL, Murray ADN. Visual loss with dancing extremities and mental disturbances. Surv Ophthalmol 1991; 35(4):299 –306. 3. Konizewski G, Ruprecht KW, Flugel KA. Necrotizing retinitis in subacute sclerosing panencephalitis. Klin Monatsbl Augenheilkd 1984:184(2):99 –103. 4. Zagami AS, Lethlean AK. Chorioretinitis as a possible very early manifestation of subacute sclerosing panencephalitis. Aust N Z J Med 1991;21:350 –352. 5. Tomoda A, Miike T, Miyagawa S, Negi A, Takeshima H. Subacute sclerosing panencephalitis and chorioretinitis. Brain Dev 1997;19:55–57.
High Incidence of Orbital Malignant Lymphoma in Japanese Patients Kenji Ohtsuka, MD, PhD, Masato Hashimoto, MD, PhD, and Yasuo Suzuki, MD, PhD PURPOSE: To review 244 cases of orbital tumors to determine pathologic findings in Japanese patients. DESIGN: Observational case series.
VOL. 138, NO. 5
We studied the pathology and origin of tumors in the orbit in 244 consecutive Japanese patients with orbital tumors at our institution from 1981 through 2002 (age 0 to 90 years, mean, 48.7 years; 114 men, 130 women). RESULTS: The most common tumors were lymphoproliferative diseases (n ⴝ 114, 42.5%), including malignant lymphoma (n ⴝ 59, 24.1%) and reactive lymphoid hyperplasia (n ⴝ 45, 18.4%), pleomorphic adenoma (n ⴝ 21, 8.6%), and cavernous hemangioma (n ⴝ 18, 7.4%). CONCLUSION: The incidence of lymphoproliferative diseases, especially malignant lymphoma, was very high in Japanese patients. (Am J Ophthalmol 2004;138: 881– 882. © 2004 by Elsevier Inc. All rights reserved.) METHODS:
M
ANY REPORTS HAVE REVIEWED THE PATHOLOGIC
profiles of orbital tumors mainly in white patients;1– 4 however, such pathologic profiles in large numbers are not available for Japanese patients. In this study, we reviewed pathology and origin of tumors in the orbit in 244 consecutive Japanese patients with orbital tumors at our institution from 1981 through 2002. Pathology, computed tomography (CT), and magnetic resonance imaging (MRI) files from Sapporo Medical University Hospital were reviewed for all patients with orbital tumors and space-occupying lesions, who were treated at our institution from January 1981 through December 2002. Two hundred forty-four consecutive Japanese patients with orbital tumors were included in this study (age 0 to 90 years; mean age 48.7 years, 114 men, 130 women). Computed tomography scans or MRIs of the orbit were performed on all patients. All patients who underwent biopsy or tumor resection and 19 patients with radiologically estimated orbital tumors were included. The 19 patients consisted of five cases of optic nerve glioma, four cases of cavernous hemangioma, four cases of orbital varix, three cases of optic nerve sheath meningioma, two cases of hemorrhagic lymphangioma, and one case of fibrous dysplasia, which were estimated on the basis of characteristic findings of CT or MRI.5,6 Another 225 patients underwent biopsy or tumor resection at our institution and were verified histopathologically. Conventional light microscopic examination and immunohistochemical techniques were used for diagnosis. Provisional diagnosis of lymphoproliferative diseases were made on the basis of routine histology and immunohistochemistry for B and T cells. From 1990, molecular assessment using the polymerase chain reaction technique for immunoglobulin heavy chain gene and T-cell receptor gamma chain gene rearrangement was incorporated into a Accepted for publication May 25, 2004. From the Department of Ophthalmology, Sapporo Medical University, School of Medicine, Sapporo, Hokkaido, Japan. Inquiries to Kenji Ohtsuka, MD, PhD, Department of Ophthalmology, Sapporo Medical University, School of Medicine, S-1, W-16, Chuo-ku, Sapporo, 060-8543, Japan; fax: 81-11-613-6575; e-mail: kohtsuka@ sapmed.ac.jp
BRIEF REPORTS
881
liferative diseases, including malignant lymphoma and reactive lymphoid hyperplasia, were 42.5% of total orbital tumors. Other most common orbital tumors were pleomorphic adenoma (n ⫽ 21, 8.6%), cavernous hemangioma (n ⫽ 18, 7.4%), and dermoid cyst (n ⫽ 13, 5.3%). There are no major differences between Japanese and white patients in the occurrence of the most common orbital tumors. However, the incidence of lymphoproliferative diseases was higher in Japanese patients than in white patients. Among white patients, the incidence of malignant lymphoma and benign lymphoid hyperplasia is 5% to 15% and 4% to 10%, respectively.1– 4 It was 24.1% and 18.4%, respectively, in the present study. Polymerase chain reaction was performed in 73 of the 104 lymphoproliferative cases, and 58 cases (78%) were diagnosed as malignant lymphoma. Polymerase chain reaction was not carried out in 31 cases diagnosed before 1990 and only one case (3%) was diagnosed as definite malignant lymphoma. If polymerase chain reaction was performed on all cases, the incidence of malignant lymphoma may have been higher than 24.1%, and benign lymphoid hyperplasia may have been lower than 18.4%. These findings suggest that the high incidence of malignant lymphoma among Japanese patients is significant in contrast with white patients. The reason for this difference is uncertain. This difference may be the result of racial or environmental differences between Japanese and white populations.
TABLE 1. Pathologic Findings in 244 Patients With Orbital Tumors
Diagnosis
Malignant lymphoma Reactive lymphoid hyperplasia Pleomorphic adenoma Cavernous hemangioma Dermoid cyst Metastatic tumors Mucocele Schwannoma Adenocarcinoma Sebaceous cell carcinoma Squamous cell carcinoma Optic nerve sheath meningioma Optic nerve glioma Varix Hematoma Capillary hemangioma Hemorrhagic lymphangioma Epidermoid cyst Neurofibroma Osteoma Rhabdomyosarcoma Granular cell tumor Fibrous dysplasia Retinoblastoma Aneurysmal bone cyst Ectopic lacrimal dactal cyst Hemangiopericytoma Solitary fibrous tumor Fibrosarcoma Ewing’s sarcoma Amyloid tumor Eosinophilic granuloma Medulloepithelioma
Number of Patients
% of Total Tumors
59 45
24.1 18.4
21 18 13 8 8 7 6 6 6 5
8.6 7.4 5.3 3.2 3.2 2.8 2.5 2.5 2.5 2.0
5 4 4 3 3 2 2 2 2 2 2 2 1 1 1 1 1 1 1 1 1
2.0 1.6 1.6 1.2 1.2 0.8 0.8 0.8 0.8 0.8 0.8 0.8 0.4 0.4 0.4 0.4 0.4 0.4 0.4 0.4 0.4
REFERENCES
1. Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134 –155. 2. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit. A survey of 645 biopsies. Arch Ophthalmol 1984;102:1606 –1611. 3. Rootman J. Frequency and differential diagnosis of orbital disease. In: Rootman J, editor. Disease of the orbit. A multidisciplinary approach. Philadelphia, PA: JB Lippincott, 1989:119 –139. 4. Dermirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older population. Ophthalmology 2002;109:243–248. 5. Ohtsuka K, Hashimoto M, Akiba H. Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol 1997;123:396 –398. 6. DePotter P, Shields JA, Shields CL. MRI of the eye and orbit. Philadelphia, PA: JB Lippincott, 1995.
final diagnosis. The subclassifications of malignant lymphoma in this study were determined according to the Revised European American Lymphoma (REAL) system of nomenclature or the Working Formulation of Lymphoma Pathologic Classification Project. Among the 244 cases with orbital tumors, 213 (89%) were primary orbital tumors, 23 (9%) were secondary tumors, and eight (2%) were metastatic tumors. Table 1 shows the pathologic profile of all cases. Malignant lymphoma was the most common orbital tumor (n ⫽ 59, 24.1%). The subclassifications of malignant lymphoma in this study were as follows: mucosa-associated lymphoid tissue (MALT)-type extranodal marginal zone lymphoma (n ⫽ 44), follicular lymphoma (n ⫽ 8), diffuse large cell lymphoma (n ⫽ 5), plasmacytoma (n ⫽ 1), and natural killer/T-cell lymphoma (n ⫽ 1). The incidence of reactive lymphoid hyperplasia was 18.4%. Therefore, lymphopro882
AMERICAN JOURNAL
Sticky Eyelid Syndrome J. Scott Kortvelesy, MD, and George F. Buerger Jr., MD Accepted for publication May 25, 2004. From the John A. Burns School of Medicine, University of Hawaii, Straub Clinic and Hospital, Honolulu, Hawaii. Inquiries to J. Scott Kortvelesy, MD, John A. Burns School of Medicine, University of Hawaii, Straub Clinic and Hospital, 888 South King Street, Honolulu, HI 96813; fax: 808-522-4431; e-mail: [email protected] OF
OPHTHALMOLOGY
NOVEMBER 2004
1. De Laey JJ, Hanssens M, Colette P, Geerts L, Priem H. Subacute sclerosing panencephalitis: fundus changes and histopathologic correlations. Doc Ophthalmol 1983;56:11–21. 2. Salmon JF, Pan EL, Murray ADN. Visual loss with dancing extremities and mental disturbances. Surv Ophthalmol 1991; 35(4):299 –306. 3. Konizewski G, Ruprecht KW, Flugel KA. Necrotizing retinitis in subacute sclerosing panencephalitis. Klin Monatsbl Augenheilkd 1984:184(2):99 –103. 4. Zagami AS, Lethlean AK. Chorioretinitis as a possible very early manifestation of subacute sclerosing panencephalitis. Aust N Z J Med 1991;21:350 –352. 5. Tomoda A, Miike T, Miyagawa S, Negi A, Takeshima H. Subacute sclerosing panencephalitis and chorioretinitis. Brain Dev 1997;19:55–57.
High Incidence of Orbital Malignant Lymphoma in Japanese Patients Kenji Ohtsuka, MD, PhD, Masato Hashimoto, MD, PhD, and Yasuo Suzuki, MD, PhD PURPOSE: To review 244 cases of orbital tumors to determine pathologic findings in Japanese patients. DESIGN: Observational case series.
VOL. 138, NO. 5
We studied the pathology and origin of tumors in the orbit in 244 consecutive Japanese patients with orbital tumors at our institution from 1981 through 2002 (age 0 to 90 years, mean, 48.7 years; 114 men, 130 women). RESULTS: The most common tumors were lymphoproliferative diseases (n ⴝ 114, 42.5%), including malignant lymphoma (n ⴝ 59, 24.1%) and reactive lymphoid hyperplasia (n ⴝ 45, 18.4%), pleomorphic adenoma (n ⴝ 21, 8.6%), and cavernous hemangioma (n ⴝ 18, 7.4%). CONCLUSION: The incidence of lymphoproliferative diseases, especially malignant lymphoma, was very high in Japanese patients. (Am J Ophthalmol 2004;138: 881– 882. © 2004 by Elsevier Inc. All rights reserved.) METHODS:
M
ANY REPORTS HAVE REVIEWED THE PATHOLOGIC
profiles of orbital tumors mainly in white patients;1– 4 however, such pathologic profiles in large numbers are not available for Japanese patients. In this study, we reviewed pathology and origin of tumors in the orbit in 244 consecutive Japanese patients with orbital tumors at our institution from 1981 through 2002. Pathology, computed tomography (CT), and magnetic resonance imaging (MRI) files from Sapporo Medical University Hospital were reviewed for all patients with orbital tumors and space-occupying lesions, who were treated at our institution from January 1981 through December 2002. Two hundred forty-four consecutive Japanese patients with orbital tumors were included in this study (age 0 to 90 years; mean age 48.7 years, 114 men, 130 women). Computed tomography scans or MRIs of the orbit were performed on all patients. All patients who underwent biopsy or tumor resection and 19 patients with radiologically estimated orbital tumors were included. The 19 patients consisted of five cases of optic nerve glioma, four cases of cavernous hemangioma, four cases of orbital varix, three cases of optic nerve sheath meningioma, two cases of hemorrhagic lymphangioma, and one case of fibrous dysplasia, which were estimated on the basis of characteristic findings of CT or MRI.5,6 Another 225 patients underwent biopsy or tumor resection at our institution and were verified histopathologically. Conventional light microscopic examination and immunohistochemical techniques were used for diagnosis. Provisional diagnosis of lymphoproliferative diseases were made on the basis of routine histology and immunohistochemistry for B and T cells. From 1990, molecular assessment using the polymerase chain reaction technique for immunoglobulin heavy chain gene and T-cell receptor gamma chain gene rearrangement was incorporated into a Accepted for publication May 25, 2004. From the Department of Ophthalmology, Sapporo Medical University, School of Medicine, Sapporo, Hokkaido, Japan. Inquiries to Kenji Ohtsuka, MD, PhD, Department of Ophthalmology, Sapporo Medical University, School of Medicine, S-1, W-16, Chuo-ku, Sapporo, 060-8543, Japan; fax: 81-11-613-6575; e-mail: kohtsuka@ sapmed.ac.jp
BRIEF REPORTS
881
liferative diseases, including malignant lymphoma and reactive lymphoid hyperplasia, were 42.5% of total orbital tumors. Other most common orbital tumors were pleomorphic adenoma (n ⫽ 21, 8.6%), cavernous hemangioma (n ⫽ 18, 7.4%), and dermoid cyst (n ⫽ 13, 5.3%). There are no major differences between Japanese and white patients in the occurrence of the most common orbital tumors. However, the incidence of lymphoproliferative diseases was higher in Japanese patients than in white patients. Among white patients, the incidence of malignant lymphoma and benign lymphoid hyperplasia is 5% to 15% and 4% to 10%, respectively.1– 4 It was 24.1% and 18.4%, respectively, in the present study. Polymerase chain reaction was performed in 73 of the 104 lymphoproliferative cases, and 58 cases (78%) were diagnosed as malignant lymphoma. Polymerase chain reaction was not carried out in 31 cases diagnosed before 1990 and only one case (3%) was diagnosed as definite malignant lymphoma. If polymerase chain reaction was performed on all cases, the incidence of malignant lymphoma may have been higher than 24.1%, and benign lymphoid hyperplasia may have been lower than 18.4%. These findings suggest that the high incidence of malignant lymphoma among Japanese patients is significant in contrast with white patients. The reason for this difference is uncertain. This difference may be the result of racial or environmental differences between Japanese and white populations.
TABLE 1. Pathologic Findings in 244 Patients With Orbital Tumors
Diagnosis
Malignant lymphoma Reactive lymphoid hyperplasia Pleomorphic adenoma Cavernous hemangioma Dermoid cyst Metastatic tumors Mucocele Schwannoma Adenocarcinoma Sebaceous cell carcinoma Squamous cell carcinoma Optic nerve sheath meningioma Optic nerve glioma Varix Hematoma Capillary hemangioma Hemorrhagic lymphangioma Epidermoid cyst Neurofibroma Osteoma Rhabdomyosarcoma Granular cell tumor Fibrous dysplasia Retinoblastoma Aneurysmal bone cyst Ectopic lacrimal dactal cyst Hemangiopericytoma Solitary fibrous tumor Fibrosarcoma Ewing’s sarcoma Amyloid tumor Eosinophilic granuloma Medulloepithelioma
Number of Patients
% of Total Tumors
59 45
24.1 18.4
21 18 13 8 8 7 6 6 6 5
8.6 7.4 5.3 3.2 3.2 2.8 2.5 2.5 2.5 2.0
5 4 4 3 3 2 2 2 2 2 2 2 1 1 1 1 1 1 1 1 1
2.0 1.6 1.6 1.2 1.2 0.8 0.8 0.8 0.8 0.8 0.8 0.8 0.4 0.4 0.4 0.4 0.4 0.4 0.4 0.4 0.4
REFERENCES
1. Kennedy RE. An evaluation of 820 orbital cases. Trans Am Ophthalmol Soc 1984;82:134 –155. 2. Shields JA, Bakewell B, Augsburger JJ, Flanagan JC. Classification and incidence of space-occupying lesions of the orbit. A survey of 645 biopsies. Arch Ophthalmol 1984;102:1606 –1611. 3. Rootman J. Frequency and differential diagnosis of orbital disease. In: Rootman J, editor. Disease of the orbit. A multidisciplinary approach. Philadelphia, PA: JB Lippincott, 1989:119 –139. 4. Dermirci H, Shields CL, Shields JA, Honavar SG, Mercado GJ, Tovilla JC. Orbital tumors in the older population. Ophthalmology 2002;109:243–248. 5. Ohtsuka K, Hashimoto M, Akiba H. Serial dynamic magnetic resonance imaging of orbital cavernous hemangioma. Am J Ophthalmol 1997;123:396 –398. 6. DePotter P, Shields JA, Shields CL. MRI of the eye and orbit. Philadelphia, PA: JB Lippincott, 1995.
final diagnosis. The subclassifications of malignant lymphoma in this study were determined according to the Revised European American Lymphoma (REAL) system of nomenclature or the Working Formulation of Lymphoma Pathologic Classification Project. Among the 244 cases with orbital tumors, 213 (89%) were primary orbital tumors, 23 (9%) were secondary tumors, and eight (2%) were metastatic tumors. Table 1 shows the pathologic profile of all cases. Malignant lymphoma was the most common orbital tumor (n ⫽ 59, 24.1%). The subclassifications of malignant lymphoma in this study were as follows: mucosa-associated lymphoid tissue (MALT)-type extranodal marginal zone lymphoma (n ⫽ 44), follicular lymphoma (n ⫽ 8), diffuse large cell lymphoma (n ⫽ 5), plasmacytoma (n ⫽ 1), and natural killer/T-cell lymphoma (n ⫽ 1). The incidence of reactive lymphoid hyperplasia was 18.4%. Therefore, lymphopro882
AMERICAN JOURNAL
Sticky Eyelid Syndrome J. Scott Kortvelesy, MD, and George F. Buerger Jr., MD Accepted for publication May 25, 2004. From the John A. Burns School of Medicine, University of Hawaii, Straub Clinic and Hospital, Honolulu, Hawaii. Inquiries to J. Scott Kortvelesy, MD, John A. Burns School of Medicine, University of Hawaii, Straub Clinic and Hospital, 888 South King Street, Honolulu, HI 96813; fax: 808-522-4431; e-mail: [email protected] OF
OPHTHALMOLOGY
NOVEMBER 2004