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BRIEF REPORT
Year : 2004  |  Volume : 52  |  Issue : 2  |  Page : 156-7

Recurrent Mucinous Carcinoma of the Eyelid


Medical and Vision Research Foundation, Sankara Nethralaya, Chennai, India

Correspondence Address:
S Krishnakumar
Medical and Vision Research Foundation, Sankara Nethralaya, Chennai
India
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Source of Support: None, Conflict of Interest: None


PMID: 15283224

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  Abstract 

A case of recurrent mucinous carcinoma of the lid in a 40-year-old male is reported. Clinical differential diagnosis and histopathologic features are discussed.

Keywords: Eyelid, mucinous carcinoma, recurrence


How to cite this article:
Krishnakumar S, Rambhatla S, Subramanian N, Mahesh L, Biswas J. Recurrent Mucinous Carcinoma of the Eyelid. Indian J Ophthalmol 2004;52:156

How to cite this URL:
Krishnakumar S, Rambhatla S, Subramanian N, Mahesh L, Biswas J. Recurrent Mucinous Carcinoma of the Eyelid. Indian J Ophthalmol [serial online] 2004 [cited 2020 Dec 2];52:156. Available from: https://www.ijo.in/text.asp?2004/52/2/156/14603

Primary mucinous carcinoma is a rare malignant tumour that typically arises in the periorbital area. [1],[2],[3] It is a slow growing tumour that recurs after traditional surgical excision. [1],[2],[3] Recurrent eyelid mucinous carcinoma tends to be locally destructive with a regional metastatic rate of 3.5% and a distant metastatic rate of 3%. [1],[2] We report a case of recurrent primary mucinous carcinoma of the eyelid.


  Case report Top


A 40-year-old male presented with a recurrent swelling in the right upper lid of 6 months' duration. He gave a history of similar swelling in the same eyelid two and half years earlier. Eight months before he had undergone incision and curettage elsewhere for a suspected chalazion. The swelling recurred after 6 months. It was excised and then reported as mucinous carcinoma of the lid. When the tumour recurred another time after 16 months, he was referred to our institute for further management.

Ophthalmic examination revealed a best-corrected vision of 6/6, N6 in both eyes. There was a swelling on the lateral 1/3rd of the right upper lid margin with obvious loss of cilia [Figure - 1]. The swelling was non-tender, soft in consistency, the margins were ill defined and was anterior to the tarsal plate. The palpebral conjunctiva was free from mass and there was no regional lymphadenopathy. The patient underwent complete excision of the lid tumour with frozen section control and the lid reconstruction was done by Tenzel's semicircular flap under general anesthesia.

Histopathological examination revealed a lid tumour enclosing numerous islands of tumour cells in pale staining mucin in the dermis [Figure - 2]. The tumour cells were cuboidal with eosinophilic cytoplasm and centrally located nuclei. The cells were cohesive and mitosis was inconspicuous. Some cells showed ductal lumen differentiation [Figure - 3] Alcian blue stain at a pH of 2.5 was positive. The margins were clear of tumour cells. A diagnosis of mucinous adenocarcinoma of the eyelid was made.

The patient was reviewed by an oncologist and was investigated for evidence of primaries in the lung or prostate or the gastrointestinal system; these were found normal. At the 10-month follow-up visit there was no recurrence of the lesion.


  Discussion Top


Mendoza and Helwig first described mucinous eccrine carcinoma primary to the skin in 1971.[1] It is a rare tumour that typically affects patients in their sixties with a male: female ratio of 2:1.[2] More than two-thirds of those affected are Caucasians.[1],[2] The carcinoma is usually found in the head or neck region, although the trunk has been involved in some cases. [1],[2],[3],[4],[5]

According to a literature review, 40% of the lesions involve the eyelid and lesions are typically asymptomatic and slow growing, although rapidly enlarging lesions have been described.[1],[2] Before a mucinous carcinoma is considered to be primary in the lid, the possibility of a metastasis from the gastrointestinal tract, breast, pancreas, ovary, lung, kidney and the prostate must be excluded.[4]

Clinically the appearance of mucinous carcinoma varies. They are solitary, 0.7 to 2.5 cm nodules or papules that may transilluminate. The definitive diagnosis is based on the ultrastructure. Primary mucinous carcinoma has distinctive histochemical and ultrastructural features. [4],[5],[6],[7] The tumour is composed of small, irregular clusters of tumour cells around a ductal lumen in mucinous stroma. The tumour cells have a centrally placed, cuboidal nucleus and eosinophilic cytoplasm with little mitosis.

The mucin is diastase resistant, periodic acid schiff positive, hyaluronidase resistant and alcian blue positive (pH 2.5). Ultrastructurally, the tumour is composed of an admixture of pale and dark staining cells containing mucin droplets. Histochemistry supports the eccrine derivation of the tumour.[5] Adnexal tumours are believed to originate from multipotent undifferentiated stem cells and is known to differentiate along particular pathways, which may be multiple.

Primary mucinous carcinomas of the lid have a relatively benign clinical course. Lymph node metastases or distant invasion is rare. [1],[2],[3] The reported rate of regional metastasis is 10%.[2] The benign course of this tumour has been related to the uncontrolled secretion of mucin, indicative of a high degree of cellular differentiation.

Although metastasis is rare, the tumour does have a tendency to recur, particularly in the periorbital area. The recurrence rate of tumours on the eyelids after excision is 40%.[5] Very rarely the tumours can invade muscle, periosteum, parietal bone, duramater, and transverse sinus.[2], [8]

Primary mucinous carcinoma of the eyelid is treated by surgical removal, with a wide local excision[1],[2] to Moh's surgery.[2] Regional lymph node dissection is required only if lymphadenopathy is present. Frequent follow-up is recommended to observe any signs of recurrence.[3] Radiotherapy and chemotherapy are ineffective in treating primary mucinous carcinoma and its metastasis. The primary tumour can be present from 2 months to 12 years before metastasizing. [6],[7],[8]

Primary mucinous carcinoma of the lid has not been reported earlier in the Indian literature though not rare in the Western literature. [1],[2],[3],[4] Our case is interesting for the following reasons: the young age of patient, the initial mistaken diagnosis of chalazion, and multiple recurrence.

Our case indicates that primary mucinous carcinoma of the lid should feature in the differential diagnosis of a lid mass mimicking chalazion and the material should be subjected to histopathological study. Excisional biopsy should be done with frozen section control to ensure complete removal of the tumour mass. Careful longterm follow-up is also mandatory.



 
  References Top

1.
Mendoza S, Helwig EB. Mucinous (adenocystic) carcinoma of the skin. Arch Dermatol . 1971; 103:68-78.  Back to cited text no. 1
    
2.
Wright JD, Font RL. Mucinous sweat gland adenocarcinoma of the eyelid. A clinicopathological study of 21 cases with histochemical and electronmicroscopical observations. Cancer 1979; 44:1757-68.  Back to cited text no. 2
[PUBMED]    
3.
Gardner TW, O' Grady RB. Mucinous adenocarcinoma of the eyelid. Arch Ophthalmol 1984;102:912.  Back to cited text no. 3
    
4.
Wat Kin W, Silva EG,Gershenson DM. Mucinous carcinoma of the ovary. Pathologic prognostic factors. Cancer 1992;69:208-12.  Back to cited text no. 4
    
5.
Headington JT. Primary mucinous carcinoma of the skin. Histochemistry and electron microscopy. Cancer 1977:39:1055-1063  Back to cited text no. 5
    
6.
Karimipour DJ, Johnson TM, Kang S, Wang TS, Lowe L. Mucinous carcinoma of the skin. J Am Acad Dermatol 1996;34:323-26.  Back to cited text no. 6
    
7.
Fukamizu H, Tomita K, Inoue K, Takigawa M. Primary mucinous carcinoma of the skin. J Dermatol Surg Oncol 1993;19:625-28.  Back to cited text no. 7
[PUBMED]    
8.
Yeung KY, Stinson JC. Mucinous carcinoma of sweat glands with widespread metastasis. Case report with ultrastructural study. Cancer 1977;39:2556-62.  Back to cited text no. 8
[PUBMED]    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]


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