Clinicopathological study of meibomian carcinoma of eyelids – An experience of two years in a tertiary care center
Senjuti Dasgupta, Parul Jain, Nirmal K Bhattacharyya, Rojina Khatoon
Department of Pathology, Medical College, Kolkata, West Bengal, India
Correspondence Address:
Dr. Senjuti Dasgupta
Department of Pathology, Medical College, Kolkata, West Bengal
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/meajo.meajo_298_21
PURPOSE: Meibomian carcinoma is a rare and aggressive malignant neoplasm of the eyelids. The clinical presentation often mimics benign conditions thereby making the diagnosis challenging. The aim of the study was to analyze cases of meibomian carcinoma, the specimens of which were received, in the past 2 years in the pathology department.
METHODS: This retrospective observational study was undertaken for 2 years and included 9 patients of meibomian carcinoma. For each case, detailed history and clinical findings were retrieved from the hospital records. Histopathological examination was undertaken in all cases after preparing hematoxylin and eosin-stained slides from tissue blocks preserved in the department.
RESULTS: The mean age of the patients was 55 ± 15 years. Six (66.7%) patients were females, and the other three (33.3%) were male. Following surgery, gross examination of the specimens revealed that the mean size of the excised tumors was 2.45 ± 1.45 cm. The tumors were classified based on histopathological features according to growth pattern, cell type, and cytoarchitecture. Most cases had lobular growth pattern (5, 55.6% cases), consisted of epidermoid cells (5, 55.6% cases), and exhibited infiltrative cytoarchitecture (8, 88.9% cases).
CONCLUSION: Early diagnosis of meibomian carcinoma is important to reduce mortality from the aggressive tumor. The knowledge of clinicopathological aspects of the tumors that were biopsied in the department of pathology in the past 2 years will help in diagnosis and management of such tumors in future.
Keywords: Clinicopathological features, histopathological classification, meibomian carcinoma
Meibomian carcinoma is an aggressive tumor of the eyelids. It may arise from meibomian glands of the tarsal plate, caruncle, periocular skin, or glands of Zeiss. The incidence of meibomian carcinoma is only 1%–1.5%.[1] Although rare worldwide, meibomian carcinoma is now the third most common malignant tumor of eyelids after basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). SCC is on the rise and might be considered to be the second most common eyelid malignancies in some countries and the most common in others, like India.[2] Early diagnosis of meibomian carcinoma is often challenging since the clinical appearance often mimics benign conditions of eyelids such as chalazion and blepharoconjunctivitis.[3]
It is largely unknown why this carcinoma occurs commonly in the ocular adnexal structures.[4],[5] Even though the etiology of meibomian carcinoma has not been elucidated completely, an association with sunlight and radiation exposure has been reported.[3] Although it was previously believed that this disease is predominantly prevalent among Asians, not very long ago, greater incidence among Whites than Pacific Islanders or Asians has also been reported.[6] Muir–Torre syndrome has been known to predispose the development of sebaceous tumors due to the inactivation of genes MLH1 or MSH2.[7] p53 and RB are the other genes implicated in its pathogenesis.[8],[9] The association of meibomian carcinoma with Human Immunodeficiency Virus has been noted.[10]
It is absolutely essential to recognize the early symptoms and signs of meibomian carcinoma so that timely recognition of the tumor may save the patient's life. The present study was undertaken to highlight the clinicopathological characteristics of meibomian carcinoma cases encountered in the past 2 years in a tertiary care center of eastern India. This would help in diagnosis and management of similar cases in future.
MethodsA retrospective observational study was carried out for 2 years. Patients with meibomian carcinoma of eyelids who had submitted their specimens in the department of pathology, following surgery, for histopathological examination (HPE), during that time were included in the study. A total of 369 specimens of eyelid tumors were submitted to the department of pathology in the span of 2 years, among which only 9 cases were diagnosed as meibomian carcinoma. Out of these nine patients, 7 were considered to be under good prognostic criteria while poor prognostic criteria were found in two patients The clinical features which constitute poor prognostic criteria include duration of symptoms more than 6 months, tumors exceeding 10 mm in diameter, simultaneous involvement of both upper and lower eyelids and presence of orbital invasion.[11] Patients with any type of lesion of eyelids, other than meibomian carcinoma, were excluded from the study.
For each case, detailed history and clinical findings were retrieved from the hospital records. The contact information available in the records was used to obtain consent and also to follow-up the patients. The tissue blocks of meibomian carcinoma of eyelids which have been preserved over the last 2 years, were then retrieved. Hematoxylin and eosin (H and E) stained slides were prepared from the tissue blocks, and HPE was undertaken in each case. All data were meticulously recorded.
ResultsThe present study included 9 patients over a period of 2 years. The age of the patients ranged between 24 and 75 years with a mean of 55 ± 15 years. Six (66.7%) of the patients were female and the other three (33.3%) were male. The male-to-female ratio was 0.5:1.
All the patients presented with upper eyelid lesions. Six (66.7%) of the lesions were right sided and the rest (3, 33.3%) left sided. In five of the cases, the clinical suspicion was that of meibomian carcinoma from the onset. However, clinically, two cases were considered to be vascular lesions, one lacrimal gland neoplasm, and another melanoma.
Upper lid mass excision was done under general anesthesia with Cutler–Beard procedure in all but one case. The right orbit exenteration with forehead flap reconstruction was undertaken in that case. This aggressive procedure was done since a previous incisional biopsy report was available in this case, which was dated 3 months back. A differential diagnoses of meibomian carcinoma and SCC was rendered in that report. Later, a (contrast-enhanced computed tomography [CECT]) was done and it showed homogeneously enhancing mass lesion at superolateral quadrant of right orbit and lacrimal gland region measuring 3.6 cm × 3.1 cm × 7.1 cm. The lesion was found to protrude out to the globes. On the basis of these reports (incisional biopsy and CECT), a decision of the right orbit exenteration with forehead flap reconstruction was undertaken. In another case, small-sized masses were excised from the same site on three prior occasions, and each time the mass was histopathologically diagnosed to be sebaceous adenoma.
Following surgery, the specimens were sent to the department of pathology in 10% neutral buffered formalin. Gross examination of the specimens revealed that the size of the excised tumors ranged between 0.8 and 4.5 cm with a mean of 2.45 ± 1.45 cm. The histopathological diagnoses of the cases were rendered as meibomian carcinoma. Microscopic examination revealed that the growth pattern of the cases was trabecular in 3 (33.3%) cases, lobular in 5 (55.6%) and BCC like in 1 (11.1%) case. The tumor cell type was basaloid in 1 (11.1%) case, basosquamous in 3 (33.3%), and epidermoid in 5 (55.6%) cases. The cytoarchitecture was infiltrative in 8 (88.9%) cases and nodular in 1 (11.1%) case [Figure 1].
Figure 1: Meibomian carcinoma with lobular arrangement of epidermoid cells and infiltrative growth pattern (H and E, ×400). Inset shows scanner view of the tumor (H and E, ×100)Immunohistochemical analysis was undertaken in the case where there was clinical suspicion of melanoma. The tumor cells were found to be EMA positive and S100 negative. Accordingly, the case was finally diagnosed as a case of meibomian carcinoma.
All the nine cases have been followed up for between 6 and 18 months. So far, all patients are doing well.
DiscussionMeibomian carcinoma has a poor prognosis. Shields et al. reported that only 32% of cases of meibomian carcinoma were appropriately diagnosed during the first clinical examination. Further, on initial histopathologic analysis, only 50% of cases were correctly diagnosed.[12] Cicinelli and Kaliki were of the opinion that timely diagnosis and therapeutic intervention may improve the prognosis of this dismal disease.[2]
It has been emphasized that the varied clinical presentations of mebomian carcinoma and its histopathologic mimics must be appreciated to achieve the objective of appropriate diagnosis and management of the lesion.[4] In the early stage of the disease, pagetoid spread of meibomian carcinoma without tumefaction may lead to its resemblance with blepharoconjunctivitis. Chalazion is also a common misdiagnosis of this tumor. The histopathologic differentials include BCC and Merkel cell carcinoma.[3] In the present study, vascular lesion, lacrimal gland neoplasm, and melanoma were the differentials on clinical examination. In cases where the posterior extent of the tumor could not be delineated on clinical examination, CECT of the orbit was undertaken. Fine-needle aspiration cytology of lymph nodes was performed in those cases where local lymph nodes were found to be palpable. Systemic metastases were ruled out in all cases by the following investigations – chest X-ray, ultrasound of the whole abdomen and liver function tests. Immunohistochemical analysis was required to rule out the possibility of melanoma in one case.
Cicinelli and Kaliki classified meibomian carcinoma based on histopathological features according to growth pattern, cell type, and cytoarchitecture.[2] The same system of classification has been used in the present study.
Meibomian carcinoma occurs commonly in the elderly with the mean age of incidence being 73 years.[6] Even though the maximum age found in the present study was 75 years, the mean age was 55 years. Etiology could not be delineated in any of the cases. According to Sung et al. this aggressive malignant lesion can occur even in the absence of any prior history of radiation therapy, occurrence of retinoblastoma or syndrome association.[3]
Meibomian carcinoma has a high recurrence rate ranging between 9% and 36%.[13] It also has a significant metastatic potential. The regional spread has been reported to structures such as nasolacrimal duct, lacrimal sac, and also the regional lymph nodes (both preauricular and submandibular). Rarely, the distant spread may occur to lungs, liver, bones, and brain.[1]
In institutes where facilities for intraoperative frozen section are available, the technique helps to ensure clean surgical margins during the excision of malignant eyelid lesions. Meibomian carcinoma is notorious for recurrence, especially if resection margins are not free of tumor. Hence, the frozen section technique helps to prevent recurrence. However, the use of frozen section prolongs the time of surgery, increases the expenses and also requires the presence of an experienced pathologist at the center.[14]
The most common treatment of meibomian carcinoma is Mohs micrographic surgery, which is also most effective. Some cases however require more radical approach including orbital exenteration. The success of other modalities of treatment is rather limited.[15] The factors which impart a worse prognosis in this tumor include involvement of both upper and lower eyelids, a large tumor size (≥10 mm), persistence of symptoms for more than 6 months, poorly differentiated morphology on histopathologic examination, lymphovascular invasion, multicentric origin, extension into the orbit and pagetoid spread of tumor cells.[16] Till date, the follow-up of patients included in the present study has not shown any evidence of recurrence. But further follow-up for a longer period is crucial so as to detect any recurrence at its earliest.
ConclusionEarly diagnosis of meibomian carcinoma is essential to reduce mortality from the aggressive tumor. Studies like the present one are necessary for meticulous analysis of clinicopathological aspects of meibomian carcinoma which in turn will help in diagnosis and management of such tumors in future.
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