Sitara H. Hirji, Michelle M. Maeng, Ann Q. Tran, Wen-Hsuan W. Lin & Lora R. Dagi Glass
Cutaneous T-cell lymphoma (CTCL) may pose a diagnostic challenge for physicians, as clinical presentation and histologic analysis may mimic benign dermatologic conditions. The authors present a case of recurrent CTCL in which the recurrence was limited to the eyelid and misdiagnosed as a contact dermatitis. To the best of the authors’ knowledge, this is the first reported case of recurrent CTCL that has presented solely as dermatitis of the eyelid.
Andrea A. Tooley, Nailyn Rasool, Ashley Campbell & Michael Kazim
Purpose: The majority of pediatric patients with optic nerve gliomas (ONG) remain asymptomatic; however, a subset of patients suffer rapid, irreversible visual loss. The purpose of this study was to determine anatomic differences of ONG noted either by imaging or by intra-operative observation between patients with gradual visual dysfunction and those with rapid visual loss.
Methods: A retrospective review was performed in patients with visual loss secondary to ONG. The clinical records, pathology, and radiographic images were reviewed for all patients. The degree of folding or plication of the optic nerve (ON) was determined by analyzing the most acute angle present in the course of the ON.
Outcome measures: The primary outcome measure was the angle of plication or folding of the ON and the relationship of this to the course of visual dysfunction.
Results: Six patients with ONG were included in the study. A structural difference in the ON was identified in four patients with rapid vision loss compared with two patients with more gradual visual dysfunction. In patients with rapid progressive visual loss, the ONG had a 90-degree or more acute plication of the ON. Those with more gradual visual loss had more obtuse bends in the ONG.
Kalle Nummi, Tero T Kivelä
Aims To determine the incidence of retinoblastoma (Rb) and subsequent survival in the Finnish population during five decades.
Methods This retrospective observational cohort study comprised all patients with Rb born in Finland during 1964–2014 and diagnosed in 2018 (birth cohort analysis) or diagnosed in 1964–2014 (standard annual analysis), identified from the Finnish Cancer Registry and the national referral centre. We report age-adjusted incidences and survival according to cause of death.
Results Of children born in 1964–2014, 205 developed Rb, whereas 204 Rbs were diagnosed during these years; 196 belonged to both cohorts. Altogether 80 (38%) of the 213 children had heritable Rb and 19 (9%) had familial disease. The sex ratio was 1.34, suggesting male preponderance. Birth cohort analysis showed a median incidence of 6.2 per 100 000 live births (1:16 130) and less variability as compared with standard annual analysis (12.1, 6.5 and 4.4 per million children 0–4, 0–9 and 0–14 years of age, respectively). The incidence of heritable Rb increased with time, reflecting the increase in familial tumours. Five-year mortality rates from Rb were 6.2% and 7.6% for non-heritable and heritable diseases, respectively, and 35-year mortality rates from second malignancies were 0% and 14.3%, respectively. Family history predicted improved survival, whereas the period of diagnosis did not.
Conclusion The incidence of familial Rb has increased, along with improvement in survival in Finland in 1964–2014, whereas the overall incidence of Rb was stable. Long-term risk of dying of second malignancies after heritable Rb was in line with other countries.
Lata Singh, Mithalesh Kumar Singh, Moshahid Alam Rizvi, Neelam Pushker, Sameer Bakhshi, Seema Sen, Seema Kashyap
Background Immune checkpoint blockade strategies have gained attention in the treatment/prognosis of cancers by targeting the programmed death-1 (PD-1)/programmed death-ligand 1 (PD-L1) pathway alone or in combination with cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) blockade and are currently in clinical trials. The present study investigated the expression of the PD-1, PD-L1, CTLA-4, CD4 and CD8 proteins and their prognostic value in the tumour microenvironment of sebaceous gland carcinoma (SGC).
Methods The expression levels of PD-1, PD-L1, CTLA-4, CD4 and CD8 proteins were assessed in 52 cases of SGC by immunohistochemistry and validated by western blotting. mRNA expression was measured by quantitative real-time PCR. Kaplan-Meier curves and Cox proportional hazard models were used to analyse the correlation of protein expression with clinicopathological parameters and disease-free survival.
Results The expression of PD-L1 was found to be higher in tumour cells than in stromal cells. In univariate analysis, the expression of PD-1 in tumour-infiltrating lymphocytes (tPD-1) and PD-L1 in tumour cells was associated with reduced disease-free survival, whereas PD-L1 expression in stromal lymphocyte infiltration (sPD-L1) was associated with the increased survival of patients (p<0.05). However, by multivariate analysis, the expression of tPD-1 was found to be an independent prognostic factor for poor survival.
Conclusion Our study highlights the prognostic outcome of PD-1 and PD-L1 protein expression in cells of tumour–stromal compartments. These results indicate that the PD-1/PD-L1 pathway mediates important interactions within the tumour microenvironment in SGC.
Osama M. Ahmed , Anthony K. Ma , Taha M. Ahmed & Renelle Pointdujour-Lim
We performed an epidemiological study of orbital lymphoma in the United States to determine how histological subtypes confer differing prognosis, and understand other factors associated with survival.
All patients in the Surveillance, Epidemiology and End Results database diagnosed with a histologically confirmed orbital lymphoma between 1973 and 2014 were included. Exclusion criteria included diagnosis at autopsy and the presence of other malignancies. Measures included patient demographic information, histological subtype and treatment modalities. Outcomes included overall and disease specific survival.
Of the 1504 cases identified, 702 were male (46.7%, mean age: 64.4 years, standard deviation [SD]: 15.3) and 802 were female (53.3%, mean age: 67.5 years, SD: 14.3). Mucosal associated lymphoid tissue (MALT) (49.5%) and diffuse large B cell lymphoma (DLBCL) (19.5%) were the two most common histologic subtypes. MALT lymphoma conferred the best prognosis (10-year cancer specific survival [CSS] 90.2%, 95% Confidence Interval [CI] 87.4% – 93.1%) and DLBCL conferred the worst prognosis (10-year CSS 68.6%, 95% CI 62.5% – 75.3%) (p < .001, log-rank test). Older age (Hazard Ratio [HR]: 3.71, 95% Confidence Interval [CI]: 2.94–4.66, p < .001), male sex (HR: 1.22, 95% CI: 1.039–1.441, p = .015), no radiation (HR: 1.72, 95% CI: 1.46–2.02, p < .001) and DLBCL histology were significant predictors of worse overall survival.
DLBCL histology confers the worst outcomes whereas MALT lymphoma confers the best outcome in orbital lymphoma. Age, gender, and radiation treatment also influence survival. These epidemiological results can be used clinically to communicate outcomes on the basis of patient characteristics and disease histology.
Douglas, Vivian Paraskevi; Douglas, Konstantinos A.A.; Cestari, Dean M.
Purpose of review
Optic nerve sheath meningiomas (ONSMs) are rare benign tumors of the anterior visual pathway which present with slowly progressive and painless vision loss and account for approximately 2% of all orbital tumors. This article provides an overview as well as an update on the ONSMs with regards to cause, epidemiology, clinical presentation, diagnosis, and management in adults and pediatric population.
The clinical presentation and prognosis of ONSMs can vary and largely depend on the location of tumor as well as the histologic type. Overall, the diagnosis is based on clinical presentation, examination, and neuroimaging findings. Nevertheless, delays in diagnosis or misdiagnosis are not uncommon and can result in higher morbidity rates. Recent advances in diagnostic as well as more effective and less-invasive treatment options are discussed in this review.
ONSMs are a rare cause of slowly progressive and inexorable visual loss. Although ONSM diagnosis depends on the characteristic clinical and radiologic findings, prompt diagnosis, and appropriate management is critical for favorable visual outcomes. Thus, current focus is optimizing diagnostic as well-treatment methods for patients with ONSMs.
David Ancona-Lezama, Lauren A Dalvin, Carol L Shields
Retinoblastoma management remains complex, requiring individualized treatment based on International Classification of Retinoblastoma (ICRB) staging, germline mutation status, family psychosocial factors and cultural beliefs, and available institutional resources. For this 2020 retinoblastoma review, PubMed was searched for articles dated as early as 1931, with an emphasis on articles from 1990 to the present day, using keywords of retinoblastoma, chemotherapy, intravenous chemotherapy, chemoreduction, intra-arterial chemotherapy, ophthalmic artery chemosurgery, intravitreal chemotherapy, intracameral chemotherapy, cryotherapy, transpupillary thermotherapy, laser, radiation, external beam radiotherapy, plaque radiotherapy, brachytherapy, and enucleation. We discuss current treatment modalities as used in the year 2020, including intravenous chemotherapy (IVC), intra-arterial chemotherapy (IAC), intravitreal chemotherapy (IvitC), intracameral chemotherapy (IcamC), consolidation therapies (cryotherapy and transpupillary thermotherapy [TTT]), radiation-based therapies (external beam radiotherapy [EBRT] and plaque radiotherapy), and enucleation. Additionally, we present a consensus treatment algorithm based on the agreement of three North American retinoblastoma treatment centers, and encourage further collaboration amongst the world’s most expert retinoblastoma treatment centers in order to develop consensus management plans and continue advancement in the identification and treatment of this childhood cancer.
Luis García Onrubia, Gabriela Estefanía Pacheco-Callirgos, Alejandro Portero-Benito, Ciro García-Álvarez, Ester Carreño Salas, M Fe Muñoz-Moreno, Elena García-Lagarto, María Antonia Saornil-Álvarez
To report the spectrum and frequency of conjunctiva tumours in an ocular oncology unit analysing the clinical profile of benign, precancerous and malignant conjunctival lesions.
A retrospective case series of 462 consecutive patients diagnosed at the Ocular Oncology Unit of the University Hospital of Valladolid from 1992 to 2017.
Among 462 consecutive patients, the tumour was classified as melanocytic in 252 (54.5%) and non-melanocytic in 210 (45.5). Two hundred forty-eight males (mean age 51.63 (SD = 23.20)) and 214 females (mean age 48.27 (SD = 21.77)) were included. Mean patient age at diagnosis was 50.07 years (range = 1-92 years). The majority of tumours were benign (n = 307 (66.5%)) followed by precancerous (n = 103 (22.3%)) and finally by malignant ones (n = 52 (11.3%)). Benign lesions were predominantly found in younger individuals rather than premalignant (p < 0.05) and malignant ones (p < 0.05). Most of the melanocytic lesions were benign (88.5%), most epithelial ones were precancerous (61.4%) and most lymphoid lesions were malignant (56.3%). Tumours involving one or four quadrants of the ocular surface usually were benign, unlike tumours involving three quadrants that were malignant (16 (48.5%) p < 0.05). The majority of benign lesions were detected on females (n = 163 (53.1%)) by routine examination (n = 178 (86.4%)). However, main complaint in malignant tumours was the growth of the lesion (n = 39 (76.5%)).
Most of the conjunctival tumours were melanocytic, mostly benign, closely followed by those of epithelial origin, with a predominance of precancerous lesions. Melanocytic, epithelial and lymphoid tumours accounted for over 90% of cases. A trend was identified with benign lesions being found in younger female patients on routine examination.
Stine Dahl Vest, Lauge Hjorth Mikkelsen, Frederik Holm, Geeta K. Vemuganti, Bradley A. Thuro, Steffen Heegaard
To characterize the clinical features of subtype-specific lacrimal gland lymphoma and their effect on patient survival.
Multicenter retrospective interventional case series.
Patient data were collected from 6 international eye cancer centers from January 1, 1980, through December 31, 2017. All patients with histologically verified primary or secondary lymphoma of the lacrimal gland were included. Primary endpoints were overall survival (OS) and disease-specific survival (DSS).
A total of 260 patients with lacrimal gland lymphoma were identified. The median age was 58 years and 52% of patients were men. Non-Hodgkin B-cell lymphomas constituted 99% (n = 258) and T-cell lymphomas constituted 1% (n = 2). The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n = 177, 68%), follicular lymphoma (FL) (n = 26, 10%), diffuse large B-cell lymphoma (DLBCL) (n = 25, 10%), and mantle cell lymphoma (MCL) (n = 17, 7%). Low-grade lymphomas (EMZL and FL) were most commonly treated with external beam radiotherapy (EBRT), whereas high-grade lymphomas (DLBCL and MCL) were treated with chemotherapy in combination with rituximab and/or EBRT. The prognosis was relatively good with a 5-year OS and DSS of 73.8% and 87.5%, respectively. Lymphoma subtype was a statistically significant predictor for DSS, with EMZL (5-year DSS: 93.4%) having the best prognosis and DLBCL (5-year DSS: 52.6%) having the poorest.
This is the largest reported collection of data of subtype-specific lacrimal gland lymphoma. The subtype distribution of lacrimal gland lymphoma resembles that of the ocular adnexa. Prognosis is good and the histologic subtype is a significant predictor for disease-specific survival.
Bilge Batu Oto, Ahmet Murat Sarıcı, Osman Kızılkılıç
To evaluate the treatment outcomes of an alternative retrograde superselective ophthalmic artery catheterization (intra-arterial chemotherapy [IAC]), while treating retinoblastoma patients.
A retrospective review of IAC for 21 treatment-naïve eyes (21 patients, primary group) and 10 eyes of previously treated 9 patients (secondary group). Statistical analysis was performed using Number Cruncher Statistical System 2007, Kaplan–Meier survival analysis, and Fisher’s exact test.
Total fluoroscopy time ranged from 3 to 12 minutes. Globe salvage was 71.4% (15/21 eyes) and 80% (8/10 eyes) in the primary and secondary groups, respectively. Globe salvage rates were recorded as 100%, 100%, 70%, and 0% for group B, C, D, and E patients, respectively.
Retrograde IAC is effective in tumour control with shorter fluoroscopy time and acceptable complication rates both for naïve and treated patients. Furthermore, controlling retinoblastoma in advanced group D eyes was efficacious.