Malignant tumors of the eye and ocular adnexa in Thailand: a six-year review at King Chulalongkorn Memorial Hospital

Transcription

1 Asian Biomedicine Vol. 3 No. 5 October 2009; Brief communication (Original) Malignant tumors of the eye and ocular adnexa in Thailand: a six-year review at King Chulalongkorn Memorial Hospital Fonthip Na Pombejara, Wasee Tulvatana, Keerati Pungpapong Department of Ophthalmology, Faculty of Medicine, Chulalongkorn University, Bangkok 10330, Thailand. Background: There are a limited number of reports on the epidemiology of malignant tumors of the eye and ocular adnexa in Thailand. Objective: To study the relative frequency and patient characteristics of various malignant tumors of the eye and ocular adnexa. Methods: Data from the medical records of the patients diagnosed with malignant tumors of the eye and ocular adnexa during 2000 to 2005, including the patients gender, age of presentation, and clinical presentation were collected. Histopathology results were classified by the anatomical location: eyelid, conjunctiva, orbit, lacrimal gland, and intraocular tissue. Pathology slides were reviewed. Results: Ninety patient data were studied. The most common presentation was mass (44.6%), followed by proptosis (10.8%), and ulcer (9.6%). Most of the tumors were in male. The most common site was the conjunctiva (35.6%), followed by the eyelid (27.8%), the lacrimal gland (14.4%), the eyeball (10%), and the orbit (8.9%). Conjunctival squamous cell neoplasia (84.4%) was the most common conjunctival malignancy. The average age was 56.4 years. Basal cell carcinoma (64%) was the most common eyelid malignancy. The mean age of presentation was 67.4 years. Lymphoma was a major malignancy of the lacrimal gland (84.6%) and the orbit (62.5%). Retinoblastoma was the most common intraocular malignancy with a mean age of 24 months. There was only one case of corneal malignancy, which was corneal intraepithelial neoplasia. Conclusion: The conjunctiva was the most common location of malignancy of the eye and ocular adnexa. The demographic data help ophthalmologists and epidemiologists to monitor trends of malignancy of the eye and ocular adnexa in this geographic region and provide comparison with other populations. Keywords: Eye, histopathology, malignant, ocular adnexa, tumor. Data on the epidemiology of the tumors of the eye and ocular adnexa among Thai population are limited. Most reports were case series of specific tumors or tumor in certain anatomic locations. A case series from Siriraj hospital reported of a 38-case collection during [1]. Shuangshoti et al. [2] The paper was presented in part at the Asia ARVO meeting on research in vision and ophthalmology in Singapore, March 2-5, Correspondence to: Wasee Tulvatana, MD, MSc, Department of Ophthalmology, Faculty of Medicine, Chulalongkorn University, 1873 Rama 4 Road, Patumwan, Bangkok 10330, Thailand. waseetulvatana@gmail.com reported 206 cases of retinoblastoma and intraocular melanoma in Kasantikul et al. [3] reported a case series of intraorbital extraocular tumors in Another series of 39 retinoblastoma was reported in 1989 [4]. A case series on malignant tumours of the eye and ocular adnexa were from the King Chulalongkorn Memorial Hospital, which collected the cases during [5]. The most recent article was published in 2002 reporting lacrimal region tumors in Northern Thailand [6]. Statistical records from the King Chulalongkorn Memorial Hospital showed the hospital prevalence of malignant neoplasm of the eye and adnexa was only 0.34% [7]. Although not commonly found, the

2 552 F. Na Pombejara, et al. tumors of the eye and ocular adnexa causes facial deformity and occasionally loss of visual function. In this study, we conducted a study to describe the frequency and patient characteristics of various malignant tumours of the eye and ocular adnexa from as an extended report from the previous study done in [5]. Material and methods The study protocol was approved by the Ethics Committee of the Faculty, Chulalongkorn University. The medical records of the patients with the diagnosis of malignant tumors of the eye and ocular adnexa were reviewed. The data were collected from the surgical records, out-patient cards, and in-patient files from the department of Ophthalmology, King Chulalongkorn Memorial Hospital between January 2000 and December We excluded the patients who were referred to other hospitals, in which the pathology tissues were not obtained, and non-thai citizens. Patients gender, age of presentation and clinical presentation were collected. Histopathology results were classified by the anatomical locations as eyelid, conjunctiva, orbit, lacrimal gland, and intraocular location. In the patients with indefinite diagnosis, the pathology slides were reviewed, and the diagnosis was confirmed by an ophthalmic pathologist. Results Ninety patients (55 men and 35 women) were studied. The mean age at presentation was 52.4 years (SD 21.8). The most common presenting symptom was mass formation (44.6%). The other manifestations were proptosis (10.8%), ulcer (9.6%), visual loss (8.3%), eye irritation (6%), eye pain (4.8%), red eye (3.6%), inflammation (3.6%), leukocoria (2.4%), abnormal pigmentation (1.2%), eye discharge (1.2%), recurrent hordeolum (1.2%), and abnormalities detected during routine eye examination (1.2%). The most common anatomical location of the malignant tumor of the eye and ocular adnexa was at the conjunctiva. The details of the tumor location, type, frequency and patient characteristics are shown in Table 1. Table 1. Characteristics of 90 cases of malignant tumours of the eye and ocular adnexa. Tumour location / type Number of cases Mean age of Male to (%) presentation female ratio (years, range) Conjunctiva 32 (35.6%) Squamous cell carcinoma and 27 (30.0%) 56.4 (28-89) 1.7:1 carcinoma in situ Lymphoma 4 (4.4%) 32.3 (22-42) 3:1 Malignant melanoma 1 (1.1%) 50 Eyelid 25 (27.8%) Basal cell carcinoma 16 (17.8%) 67.4 (39-86) 7:9 Squamous cell carcinoma 5 (5.6%) 59.5 (34-71) 3:2 Sebaceous carcinoma 4 (4.4%) 56.3 (35-70) 1:1 Lacrimal gland 13 (14.4%) Lymphoma 11 (12.2%) 49.5 (34-68) 7:4 Adenoid cystic carcinoma 1 (1.1%) 56 Malignant mixed tumour 1 (1.1%) 69 Eyeball 9 (10.0%) Retinoblastoma 6 (6.7%) 2.01 (0.08-3) 5:1 Malignant melanoma 3 (3.3%) 23 (13-36) 1:2 Orbit 8 (8.9%) Lymphoma 5 (5.6%) 66.2 (41-86) 4:1 Sebaceous carcinoma 1 (1.1%) 49 Nasopharyngeal carcinoma 1 (1.1%) 40 invading the orbit Squamous cell carcinoma 1 (1.1%) 43 Lacrimal sac 2 (2.2%) Squamous cell carcinoma 1 (1.1%) 71 Papillary transitional cell carcinoma 1 (1.1%) 79 Cornea: intraepithelial neoplasia 1 (1.1%) 68

3 Vol. 3 No. 5 October 2009 Malignant tumours of the eye and ocular adnexa in Thailand 553 Squamous cell carcinoma (SCC) and carcinoma in situ (CIS) were the most common conjunctival malignant tumors (84.4%). Most of the patients were male. Two cases (7.4%) of SCC and CIS had underlying human immune deficiency virus infection. Other two cases (7.4%) of SCC and CIS had underlying hepatitis-b virus infection. The majority of eyelid malignancies were basal cell carcinoma (64%), seen almost equally frequent in both sexes at the age range of 39 to 86 years. The lacrimal gland malignancies affected mainly in middle-aged patients with the mean age of 51.5 years. Lymphoma was the most common tumor found in this location (84.6%). For the intraocular region, retinoblastoma was found in two-third of the cases. The rest was malignant melanoma, which was mainly seen in adolescents. Two cases of retinoblastoma were brothers and both of them had bilateral tumors. The most common presentation of retinoblastoma was loss of vision (33%) and leukocoria (33%) followed by abnormalities detected during the family screening of retinoblastoma case (12%) and inflammation (12%). Lymphoma was the most common malignancy of the orbit (62.5%). Sebaceous carcinoma, nasopharyngeal carcinoma invading the orbit, and SCC were each found in one case and affected middleaged patients. Most of the tumors were found in male patients. There were only two cases of lacrimal sac malignancy, which were SCC and papillary transitional cell carcinoma. One case of corneal malignancy was found, which presented as an isolated corneal intraepithelial neoplasia in a 68-year-old woman. Discussion There were an increased number of cases with malignant tumor of the eye and ocular adnexa diagnosed at King Chulalongkorn Memorial Hospital in this study, compared with the previous study, which found 128 cases in a 12-year collection [5]. This increment may be due to the real accretion of the incidence, a larger number of patients attending our service during the study period, or better data retrieval. The major limitation of this study was due to the retrospective nature of data collection. Incomplete information may result in underestimation of cases. Since our setting is a tertiary medical care, a referral bias may skew the frequencies of some tumor types. Therefore, the incidence of the diseases cannot be definitely estimated. In this study, the first three most common locations for malignant tumors of the eye and ocular adnexa were conjunctiva, eyelid, and lacrimal gland. The most affected sites were different from the data during from our hospital, which showed intraocular site, conjunctiva and eyelid as the first three most common locations [5] and were different from data in India [8], France [9], Singapore [10], and USA [11]. Our data showed that SCC and CIS were the most common conjunctival malignant tumor. The male to female ratio of SCC and CIS was 1.7 to 1, which was lower than that reported in the United States (5 fold higher in male patients) [12]. Although there was a report from Tanzania showing a relationship between HIV infection and conjunctival squamous cell neoplasia [13], we could not evaluate this association because HIV test was not always included in the preoperative laboratory tests in our setting. Basal cell carcinoma (BCC) was the most common eyelid malignancy followed by SCC and sebaceous carcinoma. This finding was comparable to the report from a study in Taiwan [14]. However, the frequency of BCC was 64% in our patients, which was lower than the 90.8% prevalence reported in USA [15, 16]. Mean age at the presentation of BCC was 67.4 years in our study, which did not correspond with the findings of an increased prevalence of BCC among the lower age groups in Italy [17]. Lymphoma was the most common lacrimal gland malignancy in our study. This was different from previous studies that showed adenoid cystic carcinoma and malignant mixed tumor as the most common lacrimal gland malignancy [15, 16]. Retinoblastoma has been the most common intraocular malignancy but the mean age at the presentation (24 months) was slightly older than previously reported (22 months). One study in India showed an older presenting age (41 months) [18]. Since better survival was observed in retinoblastoma patients diagnosed at the age younger than two years [19, 20], we might have to consider implementing a policy to detect these patients at an earlier phase. Interestingly, uveal melanoma was seen in a young age group. The mean age of presentation was only 23 years whereas the study from the United States showed that uveal melanoma was most often found in the sixth decade of life [21]. We should continue to monitor the trend of this disease.

4 554 F. Na Pombejara, et al. We found a large increase in the frequency of orbital lymphoma (62.5%) in this study as compared with 9.1% in the previous study [5]. Sebaceous carcinoma and SCC were seen in only one case in each tumor. We considered them as primary orbital tumors since the patients presented as masses in the orbit without any other lesions. There were only a few reports [22, 23] on lacrimal sac tumors as it was a very uncommon site for malignancy. However, it is recommended that the specimens from dacryocystorhinostomy should be sent for histopathologic study only in selected cases due to the low probability of malignancy [24]. Isolated corneal intraepithelial neoplasia was also a very rare malignancy. The case found in this study was the only case in the last 18 years at our hospital. Conclusion The frequencies of malignancy of the eye and ocular adnexa were classified by anatomy. Our data could serve as a reference for this geographic region for future research, and could guide physicians and policy makers in planning resources for screening, treatment, and prevention of malignancy of the eye and ocular adnexa. The authors declare no conflict of interest in this study. References 1. Taweepanich S. Cancer of the eye, ear, nose and throat in Siriraj Hospital during Siriraj Hospital Gazette. 1960; 12(7-8): Shuangshoti S, Panyathanya R. Retinoblastoma and uveal melanoma. A study of 206 cases. J Med Assoc Thai. 1973; 56: Kasantikul V, Ruangwattanaporn M, Shuangshoti S. Intraorbital extraocular tumors: a study of 56 cases. Chulalongkorn Med J. 1988; 32: Shuangshoti S, Chaiwun B, Kasantikul V. A study of 39 retinoblastomas with particular reference to morphology, cellular differentiation and tumour origin. Histopathology. 1989; 15: Tulvatana W, Paisuntornsug T, Keelawat S, Sansopha L, Karnsawai S. Malignant tumors of the eye and ocular adnexa at King Chulalongkorn Memorial Hospital: a twelve-year review ( ). Chulalongkorn Med J. 2001; 45: Leeungurasatien P. Lacrimal gland tumors in a medical center. J Med Assoc Thai. 2002; 85: Statistical Unit, Department of Radiology, Faculty of Medicine, Chulalongkorn University. Tumor registry statistical report 2003; p Sunderraj P. Malignant tumours of the eye and adnexa. Indian J Ophthalmol. 1991; 39: Scat Y, Liotet S, Carre F. Epidemiological study of 1075 malignant tumors of the eye and adnexa. J Fr Ophtalmol. 1996; 19: Lee SB, Au Eong KG, Saw SM, Chan TK, Lee HP. Eye cancer incidence in Singapore, Br J Ophthalmol. 2000; 84; Swanson MW, Cloud G. A retrospective analysis of primary eye cancer at the University of Alabama at Birminghan Part 1: Eye and orbital cancer. J Am Optom Assoc. 2001; 62: Sun EC, Fears TR, Goedert JJ. Epidemiology of squamous cell conjunctival cancer. Cancer Epidemiol Biomarkers Prev. 1997; 6: Poole TR. Conjunctival squamous cell carcinoma in Tanzania. Br J Ophthalmol. 1999; 83: Lin HY, Cheng CY, Hsu WM, Kao WH, Chou P. Incidence of eyelid cancers in Taiwan: 21-year review. Ophthalmology. 2006; 113: Cook BE Jr, Bartley GB. Epidemiologic characteristics and clinical course of patients with malignant eyelid tumors in an incidence cohort in Olmsted county, Minnesota. Ophthalmology. 1999; 106: Margo CE, Waltz K. Basal cell carcinoma of the eyelid and periocular skin. Surv Ophthalmol. 1993; 38: Delfino S, Innocenzi D, Di Lorenzon G, Scalvenzi M, Montesarchio V, Feroce F, et al. An increase in basal cell carcinoma among the young: an epidemiological study in a middle-south Italian population. Anticancer Res. 2006; 26: Schultz Kr, Ranade S, Neglia JP, Ravindranath Y. An increased relative frequency of retinoblastoma at a rural regional referral hospital in Miraj, Maharashtra, India. Cancer. 1993; 72: Saw SM, Tan N, Lee SB, Au Eong KG, Chia KS. Incidence and survival characteristics of retinoblastoma in Singapore from J Pediatr Ophthalmol Strabismus. 2000; 37: Tamboli A, Podgor MJ, Horm JW. The incidence of retinoblastoma in the United States: 1974 through Arch Ophthalmol. 1990; 108: Egan KM, Seddon JM, Glynn RJ, Gragoudas ES, Albert DM. Epidemiologic aspects of uveal melanoma. Surv Ophthalmol. 1988; 32: Tucker N, Chow D, Stockl F, Codre F, Burnier M. Clinically suspected primary acquired nasolacrimal

5 Vol. 3 No. 5 October 2009 Malignant tumours of the eye and ocular adnexa in Thailand 555 duct obstruction: clinicopathologic review of 150 patients. Ophthalmology. 1997; 104: Anderson NG, Wojno TH, Grossniklaus HE. Clinicopathologic findings from lacrimal sac biopsy specimens obtained during dacryocystorhinostomy. Ophthal Plast Reconstr Surg. 2003; 19: Merkonidis C, Brewis C, Yung M, Nussbaumer M. Is routine biopsy of the lacrimal sac wall indicated at dacryocystorhinostomy? A prospective study and literature review. Br J Ophthalmol Dec; 89(12):