Sebaceous gland carcinoma: analysis based on the 8th edition of American Joint Cancer Committee classification.

PURPOSE: To assess the prognostic value of T category of the 8th edition of American Joint Committee on Cancer (AJCC) classification in periocular sebaceous gland carcinoma (SGC). METHODS: Retrospective interventional case series of 119 cases. RESULTS: Based on the T category of 8th edition of AJCC classification, 119 periocular SGCs were classified into T1 (n = 33, 28%), T2 (n = 37, 31%) T3 (n = 17, 14%) and T4 (n = 32, 27%). There were no statistically significant differences in the rate of tumour recurrence based on T category. The outcome measures that showed significant increase with increase in T category included regional lymph node metastasis (3% for T1, 3% for T2, 12% for T3, and 44% for T4; p < 0.0001), systemic metastasis (0% for T1, 0% for T2, 12% for T3, and 25% for T4; p = 0.002) and death due to metastasis (0% for T1, 0% for T2, 12% for T3, and 22% for T4; p = 0.005). The 5-year Kaplan-Meier estimate rate for regional lymph node metastasis, systemic metastasis and metastasis-related death were all higher for the T4 category tumours (42%, p = 0.005; 34%, p = 0.0002; and 43%, p = 0.0001 respectively) compared to T1 (9%, 0%, and 0%), T2 (5%, 0%, and 0%) and T3 (10%, 17 and 8%) tumours. CONCLUSION: Primary tumour (T) category of the 8th edition AJCC classification predicts the prognosis of patients with periocular SGC. The rates of lymph node metastasis, systemic metastasis, and death is much higher in T4 tumours compared to T1, T2, and T3 tumours. There was no association between T category and tumour recurrence.

Pagetoid tumour spread in periocular sebaceous gland carcinoma: a comparative analysis in 130 patients.

PURPOSE: To compare the clinical features, histopathology features, treatment, and prognosis of patients with and without pagetoid tumour spread secondary to periocular sebaceous gland carcinoma (SGC). METHODS: Retrospective study of 130 patients with SGC who underwent conjunctival map biopsy. RESULTS: Of the 130 patients with SGC, 30 (23%) patients had histopathology proven pagetoid tumour spread. On multivariate analysis, increasing tumour basal diameter (p < 0.001) was predictive of pagetoid tumour spread. The odds ratio for tumour basal diameter in prediction of map biopsy positivity for pagetoid tumour spread was 1.13 (95% CI, 1.07-1.19). The sensitivity and specificity of clinicopathological correlation of pagetoid tumour spread was 57 and 90%. Overall, globe salvage was better in those without pagetoid tumour spread compared to those with pagetoid tumour spread (95% vs 33%; p < 0.0001). Comparing those with pagetoid tumour spread vs those without, the 5-year Kaplan-Meier estimate of systemic metastasis was 21% vs 4% (p = 0.15) and death was 28% vs 4% (p = 0.21), respectively. CONCLUSION: Increasing tumour basal diameter can predict the risk of pagetoid tumour spread. Every mm increase in tumour basal diameter increases the risk of pagetoid tumour spread by 13%. Map biopsy is recommended for all patients with periocular SGC's. Though the globe salvage rates are poor in those with pagetoid tumour spread, the occurrence of systemic metastasis and death are not significantly higher compared to those without pagetoid tumour spread.

Sebaceous Gland Carcinoma: Influence of Age at Presentation on Outcomes.

PURPOSE: To assess the relationship between age at presentation and outcomes of patients with periocular sebaceous gland carcinoma. METHODS: Retrospective case series of 241 patients. RESULTS: Of 241 cases, 29 (12%) were young adults (≤40 years), 122 (51%) were middle-aged adults (41-60 years), and 90 (37%) were older adults (>60 years). Based on the eighth edition of American Joint Committee on Cancer, most tumors belonged to T1 category (n = 78, 32%) on presentation. Wide excisional biopsy was the most common treatment modality (n = 183, 79%). There were no statistically significant differences in the clinical presentation, treatment approaches, and histopathology features amongst different age groups, except pagetoid spread which was higher in middle-aged adults (61%, p = 0.004). The incidence of tumor recurrence was higher in older age group compared to younger age groups, with 5-year, 10-year Kaplan-Meier estimate at 31%, 31% in young adults, 38%, 38% in middle-aged adults, and 45%, 100% in older adults (p = 0.03), respectively. The 10-year Kaplan-Meier estimate rate of locoregional lymph node metastasis, systemic metastasis, and death was higher in young adults (51%, 48%, and 48%, respectively) compared to middle-aged (30%, 17%, and 12%, respectively) and older adults (24%, 25%, and 27%, respectively), but the differences were not statistically significant. CONCLUSIONS: There is no difference in the clinical presentation of periocular sebaceous gland carcinoma based on age. However, the tumor recurrence rate is much higher in the older age group and death higher in younger patients despite the same treatment strategies in all age groups.

Solitary Fibrous Tumors of the Lacrimal Drainage System With Variable Orbital and Sinonasal Extensions: Combined External and Endoscopic Surgical Approach.

PURPOSE: To report techniques and outcomes of a combined external and endoscopic surgical approach in the management of solitary fibrous tumors of the lacrimal drainage system with variable orbital and sinonasal extensions. METHODS: Biopsy proven cases of solitary fibrous tumors of the lacrimal drainage system involving the lacrimal sac and the nasolacrimal ducts with an orbital, sinonasal, or intranasal extension were studied. Parameters assessed include patient demographics, clinical presentation and course, operative techniques, complications, postoperative adjuvant therapies and outcomes. RESULTS: The technique is described in 3 patients. Two patients had massive orbital extension and one had gross sinonasal extension. Nasolacrimal duct extension was involved in all the patients with intranasal extension in 2 patients. External approach was used to access the lacrimal sac and the orbital components while the endoscopic approach was used to assess the orbital clearance and manage the sinonasal extension and the nasolacrimal duct excision up to the inferior meatus. Following complete excision, additional biopsies were performed from the adjacent bones and sinonasal tissues. A detailed histopathology with immunohistochemistry is essential for not only the diagnosis but also to assess the margin clearance. There were no major postoperative complications. All the patients were advised for postoperative adjuvant external beam radiotherapy. At the last follow-up, none of the patients had any clinical or endoscopic evidence of a recurrence. CONCLUSIONS: A combined external and endoscopic approach en-block excision is most suitable management modality for solitary fibrous tumors involving the lacrimal sac and the nasolacrimal ducts with orbital and sinonasal extension.

Poorly differentiated primary adenocarcinoma of the lacrimal sac and the nasolacrimal duct.

Primary adenocarcinoma of the lacrimal drainage system is a rare malignancy. Poorly differentiated subtypes of this tumor demonstrate aggressive behaviors, and metastasis is not uncommon. We present a patient with poorly differentiated adenocarcinoma of the lacrimal sac and nasolacrimal duct with an orbital extension. The case was managed by a combined external and endoscopic approach excision followed by adjuvant radiotherapy.

Inverse Bell's phenomenon: a rare complication of levator resection surgery in a case of congenital ptosis.

A 27-year-old woman with moderate congenital ptosis and a positive Marcus-Gunn jaw winking reflex underwent levator resection surgery to correct the ptosis. Preoperatively, a normal Bell's reflex was documented. Postoperatively, she developed an inverse Bell's reflex and increased symptoms of ocular surface exposure. The Bell's reflex normalised in a week, with resolution of the corneal exposure. Reversal of the Bell's reflex can be an unforeseen complication following maximal levator resection. The early postoperative care in such cases is crucial, and the cornea must be protected from exposure changes. Accurate documentation of the Bell's phenomenon preoperatively is vital to recognise this rare event and plan management.