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Sebaceous Carcinoma of the Eyelid: a Clinicopathological Study

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Sebaceous Carcinoma of the Eyelid: a Clinicopathological Study Br J Ophthalmol 1998;82:1049–1055 1049 Sebaceous carcinoma of the eyelid: a clinicopathological study Marlis Zürcher, Christoph R Hintschich, Alec Garner, Catey Bunce, J Richard O Collin Abstract from 0.2% to 4.7% of malignant epithelial eye- Background—Sebaceous carcinoma of lid tumours,11011and China, where the tumour the eyelid is rare. The diagnosis might be is reported to occur in 28% of the lid cancers.12 diYcult because of its ability to masquer- The purpose of our study was to analyse the ade as other periocular lesions. Prognosis natural history of the tumour and its response is still regarded as being poor compared to treatment in a series of patients in Britain. with most other malignant eyelid tumours with a mortality second only to malignant Patients and methods melanoma. The present study retrospec- From 1976 to 1992, 43 patients with histologi- tively analyses clinical and histopathologi- cally confirmed sebaceous carcinoma were cal findings and outcome in a series of treated at Moorfields Eye Hospital in London. patients with sebaceous carcinoma of the Data on patients’ history, clinical features, eyelid in Britain. therapy, and outcome were collected retro- Methods—43 patients with histologically spectively. In addition, all surviving patients confirmed sebaceous carcinoma treated except four, who were lost to follow up, were at Moorfields Eye Hospital between 1976 re-examined clinically. In 41 cases the histo- and 1992 were subjected to retrospective logical specimens were reviewed and assessed. analysis. Clinical data of all patients were Histological evaluation was based on the study reviewed from the charts; all surviving of conventional paraYn embedded sections patients except four cases lost for follow supplemented in most cases by oil red O up were re-examined. Histological speci- stained frozen sections. mens were reviewed in 41 cases. The binomial method was used to assess evi- Results—23 females and 20 males, mean dence that sebaceous carcinoma is more likely age 63 years (range 37–79), were treated. to occur in left versus right eye or upper versus Primary therapy was surgery in 37 and lower lid. We did not conduct any multivariate radiotherapy in six cases. After a median analyses when studying survival time and time follow up of 40 months (range 1–148) 30 to recurrence, bearing in mind that the power patients were alive without recurrences, of such tests relates to the number of events not four patients had died from the tumour, to the number of subjects.14 and one was alive with local recurrence and distant metastases. Four patients had Results died of non-tumour related causes. Histo- Characteristics of our study population are logically, unfavourable outcome was cor- presented in Table 1, patient details including Augenklinik, related with poor tumour diVerentiation sex, race, age at diagnosis, tumour position and Kantonsspital Luzern, and extensive invasion. size, time between first presentation and treat- CH-6000 Luzern 16, ment, modalities of therapy, complications, Switzerland Conclusion—Early diagnosis and conse- M Zürcher quent surgical therapy of sebaceous carci- and outcome are shown in Table 2. noma of the eyelid leads to a better Augenklinik der outcome and higher survival rates than PATIENTS’ HISTORY Ludwig- generally assumed. Even local recur- Twenty three women and 20 men were treated. Maximilians- rences can be treated successfully. How- Patients’ age at time of correct diagnosis Universität, ranged from 38 to 79 years (mean age 62.6 Munich, Germany ever, sebaceous carcinoma remains a C R Hintschich threatening disease, which leads to death years). Forty two patients were white with two in 9% and to mutilating exenteration in Indian, one Arab, one Maltese, and one Span- Moorfields Eye 23% of our patients. ish patient, and one patient was Asian from Hospital, London (Br J Ophthalmol 1998;82:1049–1055) China. The Asian, Maltese, and Spanish A Garner patients had lived in their countries of birth for J R O Collin at least 10 years. Sebaceous carcinoma of the eyelid may arise Details of the medical history were obtained Glaxo Department of Ophthalmic from the diverse sebaceous glands of the ocular in 33 patients. One patient had received radio- 1 Epidemiology, adnexa. Owing to its rarity and its ability to therapy for facial acne (dose unknown), one Moorfields Eye masquerade as other periocular lesions, diag- had a history of multiple basal cell carcinomas Hospital, London nosis of the disease might be diYcult.1–9 on his back, and one had been treated C Bunce Prognosis is still regarded as being poor surgically for an osteosarcoma of the femur 25 Correspondence to: compared with most other malignant eyelid years earlier. No patient had been exposed to Mr J R O Collin, Moorfields tumours with a mortality second only to malig- chemotherapy for any reason before manifesta- Eye Hospital, City Road, nant melanoma.1 7 10–13 Comprehensive studies, tion of the sebaceous carcinoma. London EC1V 2PD. however, are few and there is a substantial dif- Thirteen of 29 patients interviewed gave a Accepted for publication ference between surveys from America, where positive family history of cancer with four of 24 March 1998 the tumour is rare with an incidence ranging them having more than one aVected relative. 1050 Zürcher, Hintschich, Garner, et al Table 1 Characteristics of study population during the course of the disease until the defi- nite histological diagnosis was made was found Study factor No % in five patients. Sex The time interval between first documented Male 20 47 symptoms and diagnosis ranged from 1 month Female 23 53 Race to 15 years (median 12 months). Only 13 White 42 98 patients (30%) were diagnosed within 6 Chinese 1 2 months of the first symptoms. After the defini- Size of tumour (mm) 5–10 11 27.5 tive diagnosis had been established, therapy 11–20 13 32.5 was instituted at a median time of 1 month. 21–30 6 15 The follow up beginning after the first treat- DiVuse 10 25 Eye aVected ment at Moorfield’s ranged from 1 month to Left 27 63 12.3 years (median 3.3 years). Four patients Right 16 37 Tumour on upper lid 29 67 were lost to follow up after 1, 2, 60, and 87 Tumour on lower lid 17 40 months, respectively. Tumour on conj/cornea 14 33 Tumour in orbit 2 5 DiVuse tumour 1 2 THERAPY Age (years) The primary treatment at Moorfields Eye Hos- Median 63 pital was mainly a surgical approach. Thirty Range 37–79 Follow up time (months) seven patients were operated on, and only six Median 40 were treated by radiotherapy. Surgery con- Range 1–148 sisted of extensive excision with a macroscopi- cally tumour free margin of at least 4 mm. The The tumours included four cases of gastric surgical margin was evaluated histologically by carcinoma, two of breast carcinoma, and one frozen section in 10 patients and by Mohs’ each of cancer of the skin, brain, lung, prostate, technique in one patient. Primary orbital throat, and liver. exenteration had to be performed in eight patients. CLINICAL FEATURES Twenty seven of the carcinomas were found on OUTCOME the left, 16 on the right side (p=0.12; At the end of the survey 30 patients were alive binominal test). The tumour was localised in without detectable tumour recurrences. One the upper eyelid in 26 patients, and less patient was alive but had a local recurrence and frequently in the lower eyelid in 14 patients distant metastases. Eight patients had died (p=0.08). Both lids were involved in three during the period of follow up. In four there cases. The conjunctiva was aVected in 14 was a tumour related cause. One patient died patients, three of them additionally had corneal from metastatic disease of her eyelid tumour epithelial changes. At the time of diagnosis two (no 29), and three from causes related to their patients showed intraorbital tumour, two had lid carcinoma, but which were not proved by local lymph node metastases, and one had a necropsy (patient nos 19, 31, and 41). In the distant metastasis. other four cases death was due to broncho- Tumours were graded as small (<10 mm) in genic carcinoma in two and to unknown causes 11 cases, of medium size (10–20 mm) in 14 in the other two. All four patients who died cases, and large (>20 mm) in six cases. In 10 from sebaceous carcinoma had received pallia- patients, the tumour was diVuse or multifocal, tive radiotherapy as primary treatment. The imitating a blepharoconjunctivitis, and could two patients dying from unknown causes had not be measured accurately. In two cases no undergone orbital exenteration. None of the measurements were available. patients treated surgically died from tumour The diagnoses were made on histological related causes. examinations based on specimens obtained by Four patients were lost to follow up—two incisional biopsy in 14 cases (one combined immediately after the treatment, one after 5 with conjunctival biopsy), full thickness eyelid years, and another after 7 years of recurrence- biopsies in 23 cases (mainly excisional biop- free follow up. sies), and three excisional biopsies which were Twelve patients (28%) suVered from either not further specified. A conjunctival biopsy local recurrences and/or distant metastases. Six only was performed in two eyes. In one patient (14%) of them developed only a local tumour the diagnosis was made from the exenteration recurrence after 2 to 48 months (median 9 specimen. months). Preauricular lymph node metastases At the time of referral a correct initial were seen in six patients 6–24 months (median diagnosis had been established in only eight of 14 months) after initial treatment.
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