Malignancy with benign course – basaloid squamous cell carcinoma: A rare diagnosis

Avik Dey Sarkar; Radhakrishna Mandal; Sanjay Kumar Daulat Thakur

doi:10.4103/jcor.jcor_10_22

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Year : 2022 | Volume : 10 | Issue : 3 | Page : 129-131

Malignancy with benign course – basaloid squamous cell carcinoma: A rare diagnosis

Avik Dey Sarkar¹, Radhakrishna Mandal², Sanjay Kumar Daulat Thakur²
¹ Department of Vitreo-Retinal Services, Aravind Eye Hospital, Madurai, Tamil Nadu; Department of Ophthalmology, Midnapore Medical College and Hospital, Midnapore, West Bengal, India
² Department of Ophthalmology, Midnapore Medical College and Hospital, Midnapore, West Bengal, India

Date of Submission	14-Jan-2022
Date of Decision	29-Mar-2022
Date of Acceptance	03-May-2022
Date of Web Publication	1-Dec-2022

Correspondence Address:
Avik Dey Sarkar
48, J. K. Mitra Road, Kolkata - 700 037, West Bengal
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/jcor.jcor_10_22

Abstract

A 45-year-old male presented with a 3 mm × 3 mm painless, slow-growing, gelatinous, freely mobile, benign-looking mass on the temporal side of the limbus in the conjunctiva of the right eye for 1 year. Other ophthalmic findings were unremarkable. Orbital computed tomography showed no orbital involvement or localized lymphadenopathy. The mass was excised with a healthy collar of 3 mm, and the sample was sent for histopathological examination (HPE). The patient was followed up with usual signs of healing. The case took a surprising turn when HPE showed areas of keratin pearls of squamous origin along with lobules of malignant cells displaying nuclear palisading and basal cell nests, thereby diagnosing basaloid squamous cell carcinoma of the conjunctiva. Positive staining for 34βE12 and negative for BCL-2 on immunohistochemistry confirmed it. Identification is extremely important due to its aggressive course with frequent recurrences and distant metastasis. It has been reported only thrice before in the literature.

Keywords: Basaloid squamous cell carcinoma, keratin pearl, no-touch technique

How to cite this article:
Sarkar AD, Mandal R, Daulat Thakur SK. Malignancy with benign course – basaloid squamous cell carcinoma: A rare diagnosis. J Clin Ophthalmol Res 2022;10:129-31

How to cite this URL:
Sarkar AD, Mandal R, Daulat Thakur SK. Malignancy with benign course – basaloid squamous cell carcinoma: A rare diagnosis. J Clin Ophthalmol Res [serial online] 2022 [cited 2023 Mar 24];10:129-31. Available from: https://www.jcor.in/text.asp?2022/10/3/129/362496

Conjunctival squamous cell carcinoma (SCC) is a rare, slow-growing, low-grade malignant tumor, which may arise from preexisting conjunctival carcinoma in situ.^[1] Basaloid SCC (BSCC) is an even rarer variant of SCC. This is predominant in men with an aggressive course and associated with poor prognosis. Usually, congenital long-standing mass over the limbal conjunctiva is diagnosed as limbal dermoid, whereas readily enlarging mass is taken as ocular surface squamous neoplasia. In our case, the lesion came to us as a diagnostic challenge as the appearance and history told an entirely different story from the actual rare diagnosis after histopathology.^[2]

BSCC of the conjunctiva is an extremely rare entity, only been reported thrice previously in the medical literature. Vasudev et al. stated a case series with two cases of BSCC in 2009 for the first time.^[3] Liu et al. in their case report named “Basaloid squamous cell carcinoma of the conjunctiva” have reported a case of BSCC of the conjunctiva in 2015.^[4] Mathur et al. recently in 2016 published a case of BSCC occurring at the limbus similar to our case.^[5] However, in contrast to all these previous documentations, in our case, the course of the disease has shown a rather confusing slow-growing pattern rather than an aggressive course.

Case Report

A 45-year-old male, farmer by occupation, presented to our outpatient department on December 06, 2018, with a painless, progressive lesion over the temporal side of the limbal conjunctiva of the right eye. The white-colored dark base lesion was slowly growing for the past 1 year [Figure 1]. The lesion with a dimension of approximately 3 mm × 3 mm was protruding out from the conjunctiva at the palpebral fissure. The mass was freely mobile and not encroaching toward the cornea. There was no orbital involvement or localized lymphadenopathy.

Figure 1: (a) The OSSN mass on presentation at the limbus protruding out of the conjunctival surface. (b) 1-week postresection of the mass showing adequate healing

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After thorough examination and preanesthetic fitness, the patient was posted for surgical resection of the mass on December 14, 2018. Excision of the mass was done by “no-touch technique” with a collar of healthy conjunctiva around it for further 3 mm except the limbal side. The resected mass was 3 mm × 3 mm × 4 mm in size with two distinct zones. Its basal attachment with conjunctiva was brownish black and appendages growing outward were white in appearance. The sample was sent for histopathological examination (HPE) and immunohistochemistry (IHC) examination. On postoperative day 1, the wound seemed healthy and the underlying sclera did not show any gross involvement. The patient was discharged with regular medications. On postoperative week 1 and week 2, usual signs of healing were noted. The HPE suggested it to be a BSCC, an extremely rare entity. Slides expressed lobules of malignant basaloid cells displaying nuclear palisading, small cystic spaces filled with mucinous material associated with cellular nesting, lobular and trabecular arrangement of small crowded cells with scanty cytoplasm, and hyperchromatic nuclei noted on HPE [Figure 2]. On the other hand, the squamous counterpart exhibits hyperplasia, loss of goblet cells, and nuclear hyperchromasia with signs of dyskeratosis (keratin pearl).

Figure 2: (a) HPE section showing at ×5 of adequate staining of the slice and (b) ×10 of the section with encircled area in the left revealing basal cell nest and on the right the area within the square showing keratin pearl as squamous counterpart

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With limited resources of IHC in our setup, the sample showed diffuse positivity for 34βE12, a squamous epithelial marker, and was negative for BCL-2 that differentiates it from BCC. On receiving the HPE and IHC reports, the patient was posted for radiotherapy on the 27^th postoperative day. Till his last visit on March 11, 2020, the patient did not develop any recurrence, local lymphadenopathy, or distant metastasis [Figure 3].

Figure 3: Normal conjunctiva with mild hyperkeratosis in the last visit

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Discussion

BSCC was first described in the upper aerodigestive tract by Wain et al. in 1986.^[6] BSCC is characterized by nesting, lobular and trabecular arrangement of small crowded cells with scant cytoplasm, and hyperchromatic nuclei. The lobules of malignant basaloid cells often display peripheral nuclear palisading, high mitotic activity, comedonecrosis, and small cystic spaces filled with mucinous material, making these tumors difficult to differentiate from adenoid cystic carcinoma or from small-cell undifferentiated carcinoma. The most common sites to be affected are the larynx, hypopharynx, tonsils, and base of the tongue. Other less frequently affected sites are the nose, paranasal sinus, external ear, submandibular region, esophagus, lung, anus, vulva, vagina, and uterine cervix.^[7]

BSCC is an aggressive tumor with high rates of nodal (64%) and distant metastasis (44%). Soriano et al. found a six times higher risk of distant metastasis compared to usual SCC.^[8] It is always prudent to distinguish BSCC from adenoid cystic carcinoma, small-cell neuroendocrine carcinoma, and BCC as they have different management regimens. On IHC, BCCs are negative for epithelial membrane antigen (EMA) and are positive for BCL-2 and 2Ber-EP4. The cells in BSCC are positive for 34βE12 and EMA and focally positive for carcinoembryonic antigen. The cells show negativity for BCL-2. The entity shows higher recurrence in larger lesions with involvement of margins at excision biopsy.

The treatment of choice is complete surgical excision by involving “no-touch technique” with additional radiotherapy/adjuvant chemotherapy/cryotherapy. Chemotherapeutic agents include mitomycin-C, 5-fluorouracil, or interferon-α2B.^[9] Caujolle JP et al. have shown good results using dynamic phototherapy. A new therapeutic method – surgical removal and proton therapy for recurrent or invasive carcinomas – has been introduced recently.^[10]

Even though the mass presented to us with a benign-looking course for a longer duration, timely excision with adjuvant therapy after HPE and IHC confirmation helped the patient to skip any inadvertent complication due to its highly malignant nature. Hence, it is advisable to take any limbal pathology with heterogeneous appearance urgently and should be dealt promptly with utmost care. “No-touch technique” during resection helps avoid further dissemination of the disease providing good long-term safety.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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