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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 4  |  Page : 534-536
Periocular metatypical cell carcinoma with scleral infiltration


1 Department of Ophthalmology, Government Medical College and Hospital, Chandigarh-160 032, India
2 Department of Pathology, Government Medical College and Hospital, Chandigarh-160 032, India

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Date of Web Publication1-Oct-2009
 

   Abstract 

A 60-year-old male, laborer by occupation, presented with an ulcerative lesion of medial canthus in the left eye along with infiltration and adhesion of lids to globe. He had decreased vision and restricted extraocular movements. Exenteration with skin grafting was done. Histopathologically it was diagnosed as periocular metatypical cell carcinoma with scleral infiltration. Any suspicious lesion should be diagnosed and treated early so as to prevent such extensive damage.

Keywords: Basosquamous carcinoma, metatypical cell carcinoma, scleral infiltration

How to cite this article:
Malik A, Bansal RK, Kumar S, Kaur A. Periocular metatypical cell carcinoma with scleral infiltration. Indian J Pathol Microbiol 2009;52:534-6

How to cite this URL:
Malik A, Bansal RK, Kumar S, Kaur A. Periocular metatypical cell carcinoma with scleral infiltration. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Aug 27];52:534-6. Available from: http://www.ijpmonline.org/text.asp?2009/52/4/534/56154



   Introduction Top


Basal cell carcinoma (BCC) is the most frequent periocular malignancy accounting for 80-90% of eyelid tumors. [1] Histologically, at least 20 subtypes of BCC have been described but the presence of squamous differentiation is associated with a higher incidence of recurrence and metastasis. [2] BCC with squamous differentiation is referred to as basosquamous or metatypical cell carcinoma (MTCC). Orbital invasion by a lid BCC occurs infrequently. Scleral infiltration is very rare. Medline search revealed only two case reports of scleral involvement by a lid BCC. [3],[4] We report a case of lid MTCC with orbital and scleral infiltration.


   Case Report Top


There was an eight-month history of a pigmented round nodule measuring approximately 2 x 2 mm near the medial canthus of the left eye in a 60-year-old male who was a laborer by occupation. To begin with it was painless and was slowly increasing in size. Thereafter it got ulcerated and gradually involved both the upper and lower eyelids. There was history of, off and on bleeding from the lesion. Since the past 15 days he had decreased vision, pain and inability to open the lids.

His right eye was pseudophakic with a vision of 20/30. The anterior and posterior segments were within normal limits. Examination of left eye showed visual acuity of light perception with inaccurate projection of rays. Digitally intraocular pressure was normal. Ocular movements were restricted in all gazes. Eyelashes were absent. An ulcerative lesion measuring 20 x 8 mm was seen extending from the medial canthus of the left eye towards the root of the nose. Its edges were indurated, pigmented with rolled out margins with some necrotic material and discharge seen at the base of the ulcer. The lesion was not mobile and was adherent to underlying structures. Temperature of nearby skin was normal. Both upper and lower eyelids were thickened, firm, indurated and adherent to the eyeball and to each other laterally (ankyloblepharon) [Figure 1]. Nasally, the bulbar conjunctiva appeared involved by the mass. There was ill-defined grey-white necrotic material on the cornea. Detailed intraocular examination was precluded by the adherent lids.

Ultrasound of left eye revealed multiple cystic areas with septations in the vitreous cavity. A soft tissue mass protruding from the retina with medium to high reflectivity was seen in the superior aspect. Computed tomography (CT) scan of left orbit (LE) revealed thickened lids, medial wall erosion, and few hyperdensities in the vitreous with a well-defined hyperdense lesion seen along the posterior wall on the temporal side [Figure 2]. Incisional biopsy from the ulcerated medial canthus revealed it to be basosquamous carcinoma.

Orbital exenteration with primary skin graft taken from thigh of the patient was done. Peroperatively, an area of medial wall erosion was seen. Gross examination of the exenterated globe revealed grey-white necrotic material intraocularly. No distinction could be made between anterior and posterior segments. Microscopic examination showed an invasive tumor arising from the overlying skin. There were nests and trabeculae of basaloid cells and at places of anaplastic squamoid cells showing intracellular and extracellular keratinization with a few keratin pearl formations [Figure 3]. The tumor was seen to involve the sclera [Figure 4]. Uveal tissue showed dense mixed inflammatory cell infiltrate. All resection limits and the optic nerve were free from the tumor. No intraocular tumor was seen. It was diagnosed as basosquamous/ metatypical cell carcinoma with scleral invasion.


   Discussion Top


Periocular BCC is one of the most common lid malignancies and its most common location is lower eyelid followed by the medial canthus, upper eyelid and lateral canthus. [5] They have to be clinically differentiated from squamous cell carcinoma (SCC) and  Meibomian gland More Details carcinomas (MGC) of the lid. The most common form of BCC is noduloulcerative and it has typical raised and rolled out edges, while SCC has sharply defined indurated and elevated borders. Dilated blood vessels can be seen over the lateral margins in BCC whereas SCC has no surface vascularization. BCC is locally destructive while SCC is associated with distant metastasis. MGC presents as a nodular or a spreading form causing diffuse thickening of lids. It occurs most commonly on the upper lid and has pagetoid spread. It can present as recurrent chalazion or blepheroconjunctivitis.

Medial canthal BCC is known to be locally invasive due to anatomic proximity of the skin to the orbit. [2] Our patient also had medial canthal tumor which invaded the orbit and lead to fixation of the tumor mass to bone, limitation of ocular movements and erosion of the medial wall.

Scleral invasion by BCC carcinoma is extremely uncommon. Sclerochoroidal invasion by a recurrent lid BCC has been reported only in one case. [3] Chew reported a case of infiltration of the right globe and the retro-orbital region by a recurrent lid BCC. [4] Intraocular invasion by a lid BCC which extended through the cornea into the anterior chamber has been reported in a patient of leprosy. [6] The histopathological variant of BCC of these tumors was not known.

MTCC was first realized in 1910 as a histological variant in a series of rodent ulcers, in which basal cell and squamous cell tumors were present side by side without a transition zone. It is a continuum of BCC and SCC. Since the basal cell is a pluripotent cell and SCC is the more aggressive tumor, it is possible that BCC differentiates into SCC. [6] It is known to have a worse prognosis than BCC with a potential for metastasis. Accurate tumor typing has important implications for the patient since these tumors have different modes of behavior and metastatic potential.

MTCC of the periocular region is a rare malignancy with a reported incidence of 1-2% of all skin carcinomas. [7] It is an aggressive form of BCC and is associated with greater incidence of recurrence, orbital invasion and metastasis. Maloney reported that this variant of BCC has a greater potential for metastasis probably because of its squamous component. [8]

To the best of our knowledge this is the first case report of primary MTCC of lid with scleral invasion. Infiltration of the eyeball though very rare has been seen mostly in recurrent cases. Our patient had a primary tumor which on histopathology was found to be of metatypical variety with scleral invasion. These lesions should be recognized and treated early and need more close follow-up.

 
   References Top

1.Aurora AL, Blodi FC. Lesions of the eyelids: A clinicopathological study. Surv Ophthalmol 1970;15:94-104.   Back to cited text no. 1      
2.Leibovitch I, McNab A, Sullivan T, Davis G, Selva D. Orbital Invasion by periocular basal cell carcinoma. Ophthalmology 2005;112:717-23.  Back to cited text no. 2      
3.Mantovani E, Doro D, Milizia E, Steindler P. Recurrent eyelid basal cell carcinoma with sclerochoroidal infiltration: echographic findings. Ophthalmologica 1998;212:40-1.  Back to cited text no. 3      
4.Chew R. Destruction of the orbit and globe by recurrence of basal cell carcinoma. Optometry 2007;78:344-51.  Back to cited text no. 4      
5.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;106:746-50.  Back to cited text no. 5      
6.Aldred WV, Ramirez VG, Nicholson DH. Intraocular invasion by basal cell carcinoma of the lid. Arch Ophthalmol 1980;98:1821-2.  Back to cited text no. 6      
7.Karatas Silistreli O, Ayhan M, Aytug Z, Gorgu M, Demirdover C. Periocular metatypical cell carcinoma: clinicopathologic correlation, management, and follow-up of 35 patients. J Plast Reconstr Aesthet Surg 2006;59:1280-7.  Back to cited text no. 7      
8.Maloney ML. What is basosquamous carcinoma? Dermatol Surg 2000;26:505-6.  Back to cited text no. 8      

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Correspondence Address:
Archana Malik
Department of Ophthalmology, Government Medical College and Hospital, Chandigarh-160 032
India
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DOI: 10.4103/0377-4929.56154

PMID: 19805965

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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