Diffuse large cell lymphoma of the lacrimal sac may mimic as acute dacryocystitis.

A 36-year-old male patient presented with a firm swelling in the left lacrimal sac region with a history of recurrent episodes of acute dacryocystitis, which partly resolved with systemic antibiotics. Computed tomography showed diffuse soft tissue mass without bony erosion in the same area. Incisional biopsy confirmed diffuse large cell lymphoma of non-Hodgkin's type by histopathology and immunohistochemistry. Oncologists did not detect systemic involvement, and the patient received six cycles of cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone (CHOP). Epiphora was resolved, and no recurrence of the lesion was seen with subsequent dacryocystorhinostomy with intubation and was in good health for up to 3 years of follow-up. Although primary lacrimal sac lymphoma is a rare entity, high suspicion, and prompt action in atypical cases can save lives from aggressive diffuse large cell lymphoma.

Pegylated interferon-alpha-2a for the treatment of ocular surface squamous neoplasia.

Ocular surface squamous neoplasia (OSSN) is the most common nonpigmented ocular surface neoplasm. Interferon-alpha (IFNα)-2b has been proved to be an effective immunotherapeutic agent to treat OSSN. We have used intralesional and topical (36 µg/ml) pegylated (peg) IFNα-2a to treat a recurrent surgically failed case of OSSN instead of IFNα-2b. In this case report, we have shared our initial experience with the novel use of peg-IFNα-2a to treat OSSN. According to our brief clinical experience, peg-IFNα-2a is nontoxic to ocular surface and has a promising role to treat OSSN.

Lacrimal Sac Tumors: A Case Series.

Objective: The aim of the study was to describe the clinical presentation, management strategies, and outcomes in a case series of primary lacrimal sac tumors. Methods: This retrospective study was conducted in Sheikh Fajilatunnessa Mujib Eye Hospital and Training Institute, Bangladesh, from July 1 to December 31, 2020, and included all patients who were evaluated, treated, and followed up for at least 6 months from January 2013 to October 2020. One patient developed a recurrence of the adenocarcinoma of the lacrimal sac after 1 year of primary treatment. Patients' demographic data were analyzed and reviewed from published articles on lacrimal sac tumors. We assessed patients clinically, followed by radiological evaluation. We also analyzed the biopsy technique, treatment modality, and recurrence. An oncologist reviewed all patients to prepare a plan for adjuvant treatment. Results: Ten patients with lacrimal sac tumors were included in this study. Swelling in the medial canthal region was the most common presenting feature (100%), followed by epiphora (60%) and pain (30%). Open biopsy was preferred over fine-needle aspiration biopsy. Incisional biopsy or complete excisional biopsy was performed for all suspected malignancies. Malignant tumors were found in 7 (70%) cases, and benign tumors in 3 (30%) cases. Non-Hodgkin's lymphoma (NHL) (40%) was the most common malignant lacrimal sac tumor. Mucosa-associated lymphoid tissue lymphoma was 75%, and diffuse large B-cell lymphoma was 25% among the cases of NHL. Patients with epithelial malignancy were treated with external beam radiation therapy, while NHL patients were treated with chemotherapy (CHOP regimen). Recurrence was noted in 1 case (10%) of epithelial malignancy after 1 year of treatment. Conclusion: Successful management of lacrimal sac tumors requires a high index of suspicion, as these are fatal tumors, often misdiagnosed as dacryocystitis. Nonepithelial malignancies are more predominant than epithelial malignancies, and hematolymphoid tumors are most frequent.

Clinicopathological Analysis and Surgical Outcome of Eyelid Malignancies: A Study of 332 Cases.

BACKGROUND: Eyelid tumours are common in our ophthalmic practice. Malignancy cases account only for one-fourth of all eyelid tumours. The most aggressive eyelid malignancy is sebaceous gland carcinoma, but its occurrences are rare in western countries. We found sebaceous gland carcinoma is as common as basal cell carcinoma in our clinical practices. Hence, it is essential to build awareness about the more aggressive eyelid malignancies to reduce morbidity and mortality. AIM: To assess the relative frequency of eyelid malignancies in the Bangladesh population, state their clinical features and outcome of management strategies and build awareness about the more aggressive eyelid malignancies to reduce morbidity and mortality. METHODS: This was a retrospective case series study of 332 patients in Bangladesh. This study analyzed all the recorded data of the histologically proven primary eyelid malignancies and followed them up for at least six months from 2014 to 2019 (6 years). All patients were managed by surgical excision with tumor-free margins verified on histopathology, either the frozen section or excision biopsy with 2-3 mm microscopic view of normal tissue followed by eyelid reconstruction. Computer-based statistical software SPSS was used for the analysis, and an appropriate test of significance (chi-square) was used for the statistical analysis. RESULTS: Sebaceous gland carcinoma (SGC) was the highest in occurrence, at 42%, followed by 38% basal cell carcinoma (BCC), 18% squamous cell carcinoma (SqCC), and 02% malignant melanoma (MM). The mean age at presentation of SGC, BCC, SqCC, and MM were 57.41 years, 62.56 years, 64.73 years, and 59.28 years, respectively. Female (59%) was slightly more preponderance over the male (41%) for SGC than other malignancies. Pigmentation was associated with malignant melanoma (100%) and BCC (81%). Statistically, a significant difference was found between eyelid malignancies, including location, size, pigmentation, recurrence, and invasiveness. The recurrence rate was low lower in the patients who underwent frozen section biopsy (3%) for margin clearance than those who underwent excision biopsy (21.5%) in the follow-up time. Conjunctival map biopsy (8%) was performed as an essential tool for excluding the pagetoid spread of SGC. A new reconstruction method named triangular-shaped musculocutaneous tail flap was performed in 33 (11%) patients to reconstruct the moderate eyelid defect following local resection. CONCLUSION: Sebaceous gland carcinoma (SGC) was the highest occurrence found to be the highest occurrence among all eyelid malignancies in Bangladesh. SGC is more aggressive and the recurrence rate was higher than BCC and SqCC.

Primary Conjunctival Pigmented Basal Cell Carcinoma with Increased Numbers of Intra-Tumour Melanocytes, Mimicking Melanoma: A Case Report and Review of the Literature.

A 60-year-old, South-Asian male patient presented with a dark brown lesion on his left conjunctiva surface near the limbus for 2 years. Examination showed a globular, dark brown-coloured mass near the temporal limbus at 4 to 5 o'clock position, measuring 3 × 3 × 2 mm. There were a few scattered flat pigmented lesions near the mass and also a few prominent vessels. The mass underwent wide local excision, with a clear margin, followed by double freeze cryotherapy of the conjunctival margins along with the application of Mitomycin C. The bare sclera was covered by amniotic membrane. Histology surprisingly revealed a nodular basal cell carcinoma (BCC) containing melanin. The melanin was present in the tumour cell cytoplasm, within melanophages and within dendritic, Melan A-positive melanocytes. The BCC was immunohistochemically positive for BerEP4 and negative for EMA. The clinically observed smaller nodules flanking the main pigmented BCC corresponded histologically to pigmented superficial growth pattern BCC connected to the under surface of the conjunctival epithelium. This is the first report of a pigmented BCC of the conjunctiva, mimicking a conjunctival melanoma. Pigmented BCC should be considered in the differential diagnosis of pigmented tumours of the conjunctiva.

Results of Intralesional Bleomycin Sclerotherapy for Treatment of Orbital Lymphangiomas at a Tertiary Eye Care Centre in Bangladesh.

Purpose: The purpose of our study was to evaluate the role of intralesional bleomycin injection for the treatment of orbital lymphangiomas. Methods: This was a prospective interventional case study. Twelve patients diagnosed with orbital lymphangiomas received intralesional bleomycin injection at a dose of 0.5 mg/kg body weight (maximum 15 mg) along with lignocaine 2%. Those who required retreatment were injected at intervals of 4 weeks. The outcome was reviewed radiologically and using serial photographic comparison. Result: Proptosis and lid swelling were the most frequent presentations. The number of injections ranged from 1 to 3. Fifty percent of our patients showed complete resolution, and the other 50% showed more than 70% resolution as evidenced radiologically and on digital photography. Conclusion: No significant ophthalmic or systemic side effect was noticed in our study group. Therefore, intralesional bleomycin injection can be considered as first-line therapy for treatment of orbital lymphangiomas.

Primary Orbital Cystic Pilocytic Astrocytoma.

PURPOSE: To describe the clinical, radiological and histopathological features of a highly unusual primary orbital glioma. PROCEDURES: A 6-year-old female presented with right-sided proptosis that revealed a well-defined intraconal mass on CT imaging with intact orbital bony walls. During the removal of the lesion, it dissected away freely from the muscles and was separated from the optic nerve and globe. There was no communication with the brain. RESULTS: Histology showed histological features highly favouring a pilocytic astrocytoma enveloped by meninges. No non-neoplastic brain tissue was identified. CONCLUSION: This is the first documentation of a primary orbital pilocytic astrocytoma not anatomically connected with the brain. We speculate that the tumour had arisen from developmental brain heterotopia.

Eyelid Metastatic Thyroid Papillary Carcinoma.

PURPOSE: The aim of this study was to report the clinical and histopathological features of an eyelid metastasis in a 70-year-old male with no prior medical history. METHODS: A 70-year-male presented with a painless, hard, red nodular growth in his left lower medial eyelid of 6 months duration, involving the canaliculus and punctum. He did not complain of any other ocular or systemic abnormality. The clinical differential diagnosis included lymphoma or a primary adnexal tumour. The lesion was excised under local anaesthetic cover and sent for routine histopathological analysis. RESULTS: The histology revealed a carcinoma involving the posterior and anterior lamella of the lower lid. It was composed of papillary and follicular areas. The tumour nuclei were optically clear, overlapping, had nuclear grooves and were associated with eosinophilic colloid material. The tumour cells were strongly positive for nuclear thyroid transcription factor-1 and thyroglobulin (which also stained some of the eosinophilic colloid material). The histological and immunohistochemical features were those of metastatic papillary carcinoma of the thyroid. Unfortunately, the patient's thyroid has yet to be examined because of loss to follow-up. CONCLUSION: To the best of our knowledge, this is the first report of thyroid papillary carcinoma metastasizing to the eyelid as the presenting feature of the disease. A review of the literature of metastatic thyroid carcinoma to the eyelids is presented, to place the findings of this case report in context.

Lacrimal sac rhinosporidiosis: clinical profile and surgical management by modified dacryocystorhinostomy.

PURPOSE: To describe the clinical profile and surgical management of patients with lacrimal sac rhinosporidiosis. METHODS: This is a retrospective interventional case series. Eighteen patients, who were clinically diagnosed as isolated lacrimal sac rhinosporidiosis between October 2009 to January 2012, were included in the study. Detailed history, including whether there was exposure to stagnant bathing water, was noted. All patients underwent modified dacryocystorhinostomy (DCR) under general anaesthesia. RESULT: Out of 18 patients, 14 were male and 4 were female. Rhinosporidiosis was more prevalent in the 25 to 34 years age group. The most common clinical presentation was a doughy swelling over the lacrimal sac area, present in all cases. Twelve patients (66.67%) had a history of bathing in stagnant water. After modified DCR operation, all patients were followed for one year and only two patients had recurrence of their disease. CONCLUSION: Patients with lacrimal sac rhinosporidiosis usually present with a soft, doughy swelling in the lacrimal sac area. Bathing in stagnant water is a common risk factor. A modified DCR yields excellent outcomes.

ALK-1 positive orbital inflammatory myofibroblastic tumour (IMT) associated with prominent numbers of IgG4 plasma cells - a case report.

A 14-year-old boy presented with a 2-year history of a supraorbital mass, associated with loss of vision and phthisis. A lack of response to systemic steroids led to an incisional biopsy. This showed a spindle cell lesion with a prominent inflammatory component, with numerous plasma cells. The spindle cells were positive for anaplastic lymphoma kinase (ALK-1). Over 90% of the plasma cells were surprisingly positive for IgG4. The presence of ALK-1 positivity within the spindle cells, coupled with the prominent inflammation indicated a diagnosis of ALK-1 positive inflammatory myofibroblastic tumour (IMT-the neoplastic member of the so called inflammatory pseudotumours). However, the level of IgG4 positivity within the plasma cell population would have otherwise lead to a diagnosis of IgG4 related disease, if the ALK-1 positive spindle cells population was not represented. Recent literature from systemic IMT has alluded to the presence of IgG4 plasma cell positivity in IMT and argues that in the absence of other supporting histological features of IgG4 disease (phlebitis and lymphoid aggregates), as in this case, the presence of IgG4 plasma cells, even in high numbers should not lead to a kneejerk diagnosis of co-existing IgG4 disease. This case report is the first to make this association in the orbit and argues that in the presence of IMT, the IgG4 plasma cells are not necessarily pathogenic.

Management of floppy eyelid associated with Down's syndrome: a case report.

PURPOSE: To report a case of floppy eyelid associated with Down's syndrome in a 2-year-old child. CASE REPORT: A 2-year-old child associated with Down's syndrome presented with a history of eversion of both upper lids during sleep and while crying since birth. There was no history of sleep apnea and no mechanical stimuli over eyelids. Examination under anesthesia revealed gross laxity with thick and rubbery tarsi in both the upper and lower lids. Vascularized corneal scar was present in both eyes. The laxity in the upper eyelids was corrected by pentagonal wedge resection and in the lower lids by lateral tarsal sling. No recurrence was observed up to 6-month follow-up. CONCLUSION: Although floppy eyelid is more common in middle-aged obese patients, it should be carefully looked for children particularly while dealing with Down's syndrome.

Two-Stage Correction of Blepharophimosis Syndrome: Analysis of Surgical Outcome.

PURPOSE: To evaluate the outcome of surgical correction of blepharophimosis syndrome. DESIGN: A retrospective, non-randomized, interventional case series. METHODS: Ten patients undergoing surgical correction of blepharophimosis syndrome in 2 stages were reviewed. In the first stage, correction of epicanthic fold and telecanthus was done by either Y-V plasty or Roveda procedure with or without medial canthal tendon shortening. In the second stage, ptosis correction was done by bilateral frontalis sling. The preoperative and postoperative ratio of intercanthal distance (ICD) to horizontal palpebral fissure length (HPFL) was compared to assess the outcome of surgical correction of blepharophimosis. The ratio was graded as good (ratio < 1.3), suboptimal (ratio between 1.3 and 1.5), and poor (ratio > 1.5). The outcome of ptosis correction was graded as good, moderate, and poor. RESULTS: Of 10 patients, 5 (50%) underwent Y-V plasty, and 5 (50%) underwent Roveda procedure. Medial canthal tendon shortening was done in 5 patients (50%) having a preoperative ICD of 35 mm or more. After surgical correction, mean reduction in ICD was 4.8 mm (P < 0.0001), and mean improvement in HPFL was 5.45 mm (P < 0.0001). The mean ICD to HPFL ratio changed from 2.02 to 1.31 after surgery, which was statistically significant (P < 0.0001). Two patients (20%) had a ratio greater than 1.5. Nine patients (90%) had either good or moderate ptosis correction after bilateral frontalis sling with silicone rod. CONCLUSIONS: Functionally and cosmetically acceptable outcome can be achieved after surgical correction of blepharophimosis syndrome in 2 stages.

Orbital lipofibromatosis in a child: a case report.

PURPOSE: Lipofibromatosis is a rare, benign, soft tissue tumor that typically involves extremities particularly in children. We report a young girl with lipofibromatosis involving the superotemporal quadrant of the left orbit. CASE REPORT: An 8-year-old girl presented with a history of a gradually expanding mass in the superotemporal quadrant of the left orbit over several years. Orbital imaging showed a soft tissue mass superolateral to the globe with possible infiltration into the lacrimal gland and lateral rectus muscle. The lesion was excised preserving adjacent structures. Histopathological examination revealed that the lesion consisted of adipose tissue with fibroblastic elements, being consistent with a diagnosis of lipofibromatosis. No recurrence of the lesion was seen in 18 months follow-up. COMMENT: Orbital involvement of lipofibromatosis as reported here is indeed a very rare entity. Diagnosis can be confirmed by histopathological analysis. As the lesion has a tendency to infiltrate into surrounding structures, surgical excision without hampering important structures is a treatment of choice.