Pleomorphic adenoma of palpebral lobe of lacrimal gland masquerading as skin adnexal tumor of eyelid

Giriyan S S 1, Agrawal A2, Chandan R H3

1Dr. Sujata S Giriyan, Professor and Head, 2Dr. Akanksha Agrawal, Resident, 3Dr. Rajesh H Chandan, Associate Professor, All authors are affiliated with Department of Pathology, Karnataka Institute of Medical Sciences, Hubballi, Karnataka, India

Address for Correspondence: Dr. Akanksha Agrawal; Email: aki2003@gmail.com



Abstract

Occurrence of Lacrimal gland tumorsis rare. Benign epithelial tumors are more common of which pleomorphic adenoma is commonest. They usually occur in the orbital lobe of lacrimal gland however, they mayrarely occur in palpebral lobe. FNAC helps to rule out other pathologies and narrows the differential diagnosis. We describe a case of young female who presented with a nodular right upper eyelid swelling, clinically diagnosed as a skin-adnexal tumor. On FNAC, diagnosis of Chondroidsyringoma of skin adnexawas given. Histopathologically however it was confirmed to be Pleomorphic adenoma of lacrimal gland. Although rare, possibility of both chondroidsyringoma and pleomorphic adenoma of palpebral lobe of lacrimal gland should be considered in case of a mobile, nodular swelling in the upper eyelid. FNAC aids in diagnosis, however, histopathological examination remains the diagnostic gold standard.

Key words: Lacrimal gland, Epithelialtumors, Chondroidsyringoma, Pleomorphic adenoma



Manuscript received: 16th August 2017, Reviewed: 24th August 2017
Author Corrected: 1st September 2017, Accepted for Publication: 8th September 2017

Introduction

Lacrimal gland is a bi-lobedeccrine secretory gland located insupero-lateral and extra-conal regionof the orbit[1]. The orbital lobe is larger than the palpebral lobe. The lacrimal gland is a non-encapsulated gland composed of ducts and acini[2].

Tumors of lacrimal gland are rare in fine needle aspiration practice[3]. Amongst all, the most common epithelial tumor is pleomorphic adenoma [4,5]. Lacrimal gland pleomorphic adenoma occurs commonly in second to fifth decades of lifeand has slight female preponderance. The most frequent symptom is a painless palpable mass in the upper external quadrant of the orbit or less frequently a nodular mass in the eyelid[6].

FNAC helps to rule out lymphoma, pseudotumor or malignancy, and thus aids inearly diagnosis and to plan appropriate treatmenttohelp preserve vision and prevent malignant transformation in benign tumors [7].However, the definitive diagnosis is made on histopathological examination.

Case report

A 26years female presented with a nodular swelling in the right upper eyelid near the outer canthus of the eye of one month duration. It gradually increased in size and was not associated with pain or disturbance in vision. The sub-cutaneous swelling measured 0.5cm in diameter, firm in consistency, non-tender and freely mobile.
On fine needle aspiration cytology, the smears were cellular which showed presence of epithelial cells in clusters, sheets, ducts and singles with mild anisonucleosis and scant cytoplasm. Few bare nuclei with strands of myxoidstroma and occasional hyaline globules could also be appreciated. A diagnosis ofchondroidsyringomaof skinadenexa was given(figure1a & b).

The swelling was excised completely with appropriate margin and sent for histopathological examination.
Grossly, the resected tissue was sent in two bits. On cut section, one of the bits showed grey white areas and was firm in consistency.
Microscopically, there were clusters of epithelial cells arranged in groups and ducts surrounded by myoepithelial cells. Also myoepithelial cells were seen to be surrounded by and embedded inmyxoidstroma. A diagnosis of pleomorphicadenoma of lacrimal gland was made(figure2a b).

figure01
Figure 1a
 
figure02
Figure1b:

Figure 1:
FNAC 40x, (a:H&E, b: Wright’s); Epithelial cells in clusters, Myoepithelial cells in singles, Presence of Hyaline globules

figure03
Figure 2a
 
figure04
Figure 2b

Figure 2: 
HistopathologyH&E (a: Scanner, b: 40x) ;Tumor cells scattered in myxoidstroma, forming vague ducts and acini. Epithelial cells are in clusters and myoepithelial cells appear to ‘melt’ into the stroma.

Discussion

Tumors of the lacrimal gland occur in<1 per 1,00,000population per year[8]. Lacrimal glandtumours comprise about 5 to 25% of all the orbital masses[9]. They are divided into epithelialand non-epithelialtumors. Epithelial tumors of the lacrimal gland and of which pleomorphic adenoma is the commonest, are more frequently encountered as compared to the non-epithelial tumors[1,2].The incidence of pleomorphic adenoma in the literature has been reported to be 9–27% of all the lacrimal gland tumours[5, 10].

Pleomorphic adenomas usually occur in the second and fifth decade of life, and incidence is slightly more in females[6].The present case, also a female of age 26years, presented with a nodular swelling in the right eyelid.Pleomorphic adenoma of the lacrimal gland usually manifests as a slowly progressive, painless superolateral orbital or a palpabral mass. The chronicity, the absence of pain, and the radiological evidence of no bony fossa erosion, suggest that a lacrimal gland mass is benign rather than malignant[6, 10].

These tumors are epithelial in origin. Ductal epitheliumdevelops into the epithelial component. The cellsin the myoepithelium form the stroma[11, 12]. Although pleomorphic adenomas commonly involve the orbital lobe of the lacrimal gland, they caninvolve the palpebral lobe in about 10% of cases[6]. The palpebral lobe tumors are freely movable, non-tender, and present for a shorter duration. They do not produce proptosis or bony changes[13, 14]. Pleomorphic adenomas arising in the palpebral lobe are excised with some normal lacrimal gland tissue[13]. Similar clinical picture was seen in present case where the patient presented with a small nodular mass in the eyelid of just one month duration.

FNAC reveals cellular smears mixed withstromal epithelial fragments dispersed singly and in sheetswith fibrillarchondromyxoidstroma. The cellular componentconsists of relatively uniform oval, plasmacytoid or spindle cells with round to oval, eccentric nuclei, bland finely granular chromatin with inconspicuous nucleoli. Cells with plasmacytoid appearance have eccentric nuclei withabundant cytoplasm. Spindle or rounded cells are present within the stromal fragments[7]. Fine needle aspiration of the present case revealed such cytological features(figure1a b).

Chondroidsyringoma is a tumor of sweat glands which presents with similar microscopic picture on fine needle aspiration showing biphasic population of cells in fibrillary and chondromyxoidstroma[15]. Since the site of nodular mass in present case is eyelid which also has sweat glands, Chondroidsyringoma forms one of the most important differential of pleomorphic adenoma of palpabral lobe of lacrimal gland[16]. Therefore, on FNAC, the possibility of present casebeing chondroidsyringoma was also considered.

Grossly, pleomorphic adenoma is grayish white, bossilated, solitary and well circumscribed from the normal gland. It is covered by a thin pseudocapsule formed by compressed connective tissue and reactive fibrosis. Small tumor nests may be seen outside the pseudocapsule (satellite lesions) and these cause a high incidence of recurrence when a margin of normal tissue is not removed with the tumor. The cut surface shows soft mucinous areas alternating with tough fibrous areas[11, 12].

Histopathological examination shows the mixture of epithelial and mesenchymal elements that led to the term benign mixed tumour[11]. The epithelial components are variably sized ducts containing an inner cuboidal to columnar epithelium and an outer flattened, spindle-shaped myoepitheliallayer. The myoepithelial cells undergo metaplasia to form myxoid tissue, cartilage, and bone(figure2a & b). Focal squamous metaplasia and keratin production may be observed[12, 17, 18].

The best management is complete excision of the tumor. To minimize any tumor seeding from microscopic extensions through the pseudocapsule, an adequate margin of the surrounding lacrimal gland should be taken and the tumour bed be inspected to prevent recurrences from satellite lesions[6, 17].

Conclusion

Pleomorphic adenoma of palpebral lobe of lacrimal gland, although uncommon should be considered as a differential diagnosis along with chondroid syringoma in cases of firm nodular eyelid swellings[19]. Pleomorphic Adenoma is the most common epithelial tumor of the lacrimal gland which occurs mainly in the orbital lobe and rarely in palpebral lobe. FNAC can help differentiating benign from malignant tumors. Definite diagnosis is given by histopathological examination. This allows appropriate and targeted approach towards the management of the tumor.

Funding: Nil, Conflict of interest: None initiated.
Permission from IRB: Yes

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How to ci te this article?

Giriyan S S, Agrawal A, Chandan R H. Pleomorphic adenoma of palpebral lobe of lacrimal gland masquerading as skin adnexal tumor of eyelid. Trop J Path Micro 2017; 3(3): 305-308. doi: 10.17511/ jopm.2017.i3.13.