Advanced Lower Eyelid Sebaceous Gland Carcinoma with Deep Orbital Extension and Skin Metastasis
Mansourehjamshidian Tehrani, Afsane malekpour, Abolfazl kasaee and Ameli K*
Department of Ophthalmology, Tehran University of Medical Sciences, Iran
Submission: August 08, 2017; Published: September 22, 2017
*Corresponding author: Ameli K, Department of Ophthalmology, Tehran University of Medical Sciences, Farabi Eye Hospital, Kargarjonoubi St, Qazvin Sq, Tehran, IR Iran, Zip code: 1336616351; Tel: +989133164233; Fax: +982155409095; Email: k_ameliz@razi.tums.ac.ir
How to cite this article: Mansourehjamshidian T, Afsane m, Abolfazl k, Ameli K. A Rare Presentation of Ocular Tuberculosis: A Case Report. JOJ Ophthal. 2017; 5(1): 555654. DOI: 10.19080/JOJO.2017.05.555654.
Abstract
Sebaceous Gland Carcinoma (SGC) is considered as a rare cutaneous carcinoma. It is estimated to have less than 1% prevalence rate among all eyelid tumors. They reveal high potential to simulate various inflammatory conditions. A 77 year-old woman was presented with chief complaint of vision loss in right eye. Her symptoms developed during the last year with gradual visual acuity loss and multiple nodules on the right side of her face. Spiral Computed Tomography (CT) Scan of the Orbits revealed a large occupying soft tissue mass in the right orbital cavity with deep skin invasions. Biopsy findings confirmed poorly differentiated adeno carcinoma of the right lower eyelid. In this article a rare case of lower eyelid sebaceous adeno carcinoma is described. Early suspension in all patients complaining periocular masses or lesions could significantly decline the rates of mortality in patients with these types of malignancies
Dear Editor
Sebaceous Gland Carcinoma (SGC) is considered as a rare cutaneous carcinoma. It is estimated to have less than 1% prevalence rate among all eyelid tumors [1]. These lesions mostly originate from sebaceous glands of the face, including the ones in the eyelids. They reveal high potential to simulate various inflammatory conditions (i.e Chalazion), thus it is essential to include these tumors in the differential diagnosis of all eyelid lesions, particularly in more susceptible population groups. In the present article, we present a very rare case with SGC on her lower right eyelid with deep metastasis to the skin [1,2].
Case Presentation
A 77 year-old caucasian woman was referred to the Ophthalmic Plastic unit of the Farabi University Hospital of Tehran in February 2016 with chief complaint of vision loss in right eye. Her symptoms developed during the last year with gradual visual acuity loss, multiple nodules on the right side of her face and lately ongoing swelling on the right periorbital region. Upon the first ocular examination, swollen right eyelids particularly in the lower eyelid along with multiple ulcerative nodules and different sized satellite lesions on the right cheek and the right side of her nose were detected (Figures 1A).These lesions measuring from 1cm*1cm to 3cm*3cm were painful and extended from front to lateral aspect of the face to the deep lateral view, in temporal region just in front of the right ear. Due to annoying symptoms and complaints of the patient, no further ophthalmic examinations were performed on the right eye. The left eye revealed No Light Perception (NLP) on the examination probably due to corneal phthisis and corneal scar. Her vital signs were stable. The past medical history was only positive for controlled systemic hypertension. Also A thorough review of systems and physical examination revealed multiple skin lesions on the right lower limb just like the ones on the face. No Organomegalies or lymphadenopathies were detected. A wide differential diagnosis list, particularly considering malignancies, including Basal Cell Carcinoma, Sabaceous Gland Carcinoma, Squamous Cell Carcionoma were made before further evaluations. Then, the patient underwent Spiral Computed Tomography (CT) Scan of the Orbits which showed a large occupying soft tissue mass in the right orbital cavity with bone and skin invasions. Based on the site of the lesions on the CT scan, an incisional biopsy was performed on the right eyelid and lower limb lesions for Histopathological evaluations. Also an Immuno histo chemistry study was done for the patient. Histopathological findings revealed poorly differentiated adeno carcinoma with marked necrosis, skin infiltrations and vascular invasions (Figure 1B & C). Immunohistochemistry showed neoplastic cells were positive for, Carcinoembryonic antigen (CEA), Creatine kinase (CK), Epithelial membrane antigen (EMA) (Figure 1D-1F). Unfortunately the patient had a cardiac arrest in the hospital and died before further ophthalmological evaluations and procedures.
Discussion
Sabaceous Gland Carcinomas (SGC) is defined as highly aggressive, lethal malignancies with high tendency to present in periocular region. They arise from sebaceous glands like Meibomian glands of the eyelids [3,4]. It is estimated that these carcinomas have about 30% of mortality rate but the prognosis varies individually based on different factors like the tumor size or its sites of involvement and the time of first diagnosis. Women reveal higher rates of the disease than men. Also reports show 6th and 7th decade as the most frequent age range for presenting sebaceous carcinomas in patients [4,5]. These type of malignancies require high suspicion for diagnosis, considering the different differential diseases [5,6]. These include: Basal Cell Carcinoma, Blepharoconjunctivitis, Chalazion, Conjunctival carcinoma in situ, Cutaneous Squamous Cell Carcinoma, Lacrimal Gland Tumors and Leukoplakia. Successful diagnosis is made following the proper biopsy [5,6]. The typical histological demonstration is the invasion of the dermis by lobules of poorly defined sebaceous cells or basaloid/squamoid cells. Apart from the aggression and malignancy potential, these tumors tend to metastasis to different organs like lungs, brain, skin and liver rapidly [4-7], Vascular, lymphatic and orbital invasion, poor histological differentiation pattern, pagetoid invasion of eyelid epithelium, high infiltration pattern and the estimated symptom presentation of more than 6 months are among the most definitive parameters of prognosis evaluation [6,7]. There are various management strategies for the treatment including local surgical removal, radiotherapy and chemotherapy which might be performed based on the stage of the cancer at the time of diagnosis and possible metastasis sites [7,8]. We described a patient with lower eyelid carcinoma and deep skin involvement. The progression of the disease in our patient could be due to late diagnosis or the low compliance of the patient to perform the follow-up and treatment processes. Upper eyelids are more frequently involved in sebaceous eyelid tumors than the lower ones. The incidence of orbital extension is 6 to 30 percent in different reports and it is associated with about 70% of mortality rate [7,8]. The classical treatment in such rare patients is to remove the affected tissue with proper margin and to inspect other organs for the metastasis based on the pathological report and the stage of the tumor. The follow up course is for four years from the time of primary treatment and it is applied because of the high rate offer assurance in such carcinomas [7,8].
Conclusion
We report a rare case of lower eyelid sebaceous gland carcinoma with rare dermatological extension in a 70 year old patient in the advanced stage at the time of diagnosis. We recommend early suspension in all patients complaining periocular masses or lesions regarding these types of tumors to prevent high mortality rates in delayed diagnosed cases.
References
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