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Indian Journal of Ophthalmology Jul 2024This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current...
PURPOSE
This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current times and to compare these results with previous published data.
METHODS
It was a retrospective study. All exenterations performed at a tertiary eye care center over a period of 20 years (from January 2001 to June 2020) were retrospectively evaluated. Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathologic diagnosis.
RESULTS
A total of 352 cases (males:females = 222:130) who underwent exenteration were identified. Patients age ranged from 11 months to 87 years (mean: 43.86 years, median: 50 years). The most common indication for exenteration was found to be eyelid malignancy in 54.36%, followed by retinoblastoma in 18.75% and primary orbital tumors in 14.49%. Out of 156 cases of eyelid malignancies requiring exenteration, squamous cell carcinoma (SCC) was the most common histologic subtype ( n = 94, 60.26%), followed by sebaceous gland carcinoma ( n = 40, 25.64%) and basal cell carcinoma ( n = 20, 12.82%). The most common primary orbital tumors requiring exenteration were adenocystic carcinoma of the lacrimal gland in adults and rhabdomyosarcoma in the pediatric age group. Benign conditions requiring exenteration included fulminant fungal orbital infections and lymphangioma among others.
CONCLUSION
The number of exenterations performed have significantly increased in terms of absolute numbers. However, the ratio of exenteration to other tumor-related surgeries, mainly excision biopsy, has reduced compared to that reported from a previous study. The most common indication in our study remains eyelid malignancy followed by intraocular malignancy. However, SCC has emerged as the most common tumor histologic subtype requiring exenterations.
Topics: Humans; Retrospective Studies; Male; Female; Child; Middle Aged; Orbit Evisceration; Infant; Child, Preschool; Adult; Adolescent; Tertiary Care Centers; Aged; Aged, 80 and over; Young Adult; Orbital Neoplasms; Follow-Up Studies; Incidence
PubMed: 38454866
DOI: 10.4103/IJO.IJO_2716_23 -
Experimental Eye Research Apr 2024The accumulation of oleic acid (OA) in the meibum from patients with meibomian gland dysfunction (MGD) suggests that it may contribute to meibomian gland (MG) functional...
The accumulation of oleic acid (OA) in the meibum from patients with meibomian gland dysfunction (MGD) suggests that it may contribute to meibomian gland (MG) functional disorder, as it is a potent stimulator of acne-related lipogenesis and inflammation in sebaceous gland. Therefore, we investigate whether OA induces lipogenesis and inflammasome activation in organotypic cultured mouse MG and human meibomian gland epithelial cells (HMGECs). Organotypic cultured mouse MG and HMGECs were exposed to OA or combinations with specific AMPK agonists 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR). Lipogenic status, ductal keratinization, squamous metaplasia, NLRP3/ASC/Caspase-1 inflammasome activation, proinflammatory cytokine IL-1β production, and AMPK pathway phosphorylation in MG were subsequently examined by lipid staining, immunofluorescence staining, immunohistochemical staining, ELISA assay, and Western blot analyses. We found that OA significantly induced lipid accumulation, ductal keratinization, and squamous metaplasia in organotypic cultured MG, as evidenced by increased lipids deposition within acini and duct, upregulated expression of lipogenic proteins (SREBP-1 and HMGCR), and elevation of K10/Sprr1b. Additionally, OA induced NLRP3/ASC/Caspase-1 inflammasome activation, cleavage of Caspase-1, and production of downstream proinflammatory cytokine IL-1β. The findings of lipogenesis and NLRP3-related proinflammatory response in OA-stimulated HMGECs were consistent with those in organotypic cultured MG. OA exposure downregulated phospho-AMPK in two models, while AICAR treatment alleviated lipogenesis by improving AMPK/ACC phosphorylation and SREBP-1/HMGCR expression. Furthermore, AMPK amelioration inhibited activation of the NLRP3/ASC/Caspase-1 axis and secretion of IL-1β, thereby relieving the OA-induced proinflammatory response. These results demonstrated that OA induced lipogenic disorder and NLRP3 inflammasome activation in organotypic cultured mouse MG and HMGECs by suppressing the AMPK signaling pathway, indicating OA may play an etiological role in MGD.
Topics: Humans; Mice; Animals; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Sterol Regulatory Element Binding Protein 1; Oleic Acid; Meibomian Glands; AMP-Activated Protein Kinases; Lipogenesis; Epithelial Cells; Caspase 1; Cytokines; Metaplasia; Carcinoma, Squamous Cell; Interleukin-1beta
PubMed: 38453039
DOI: 10.1016/j.exer.2024.109851 -
Annals of Diagnostic Pathology Jun 2024Primary pulmonary salivary gland-type tumours (PPSGT) are rare lung neoplasms arising from submucosal seromucinous glands in the central airway.
INTRODUCTION
Primary pulmonary salivary gland-type tumours (PPSGT) are rare lung neoplasms arising from submucosal seromucinous glands in the central airway.
METHODS AND RESULTS
We retrospectively analysed the clinicopathological features of 111 PPSGTs diagnosed at our institute between 2003 and 2021. The mean age at diagnosis was 43.8 years(range 6-78 years) and a male-to-female ratio of 2:1. On imaging, 92 % of cases had centrally located tumours and 37.3 % were early stage. The histopathological types included 70 cases (63 %) of mucoepidermoid carcinoma (MEC), 31 cases (27.7 %) of adenoid cystic carcinoma (ADCC), two cases of myoepithelial carcinoma, one case each of acinic cell carcinoma (ACC), clear cell carcinoma (CCC), epithelial myoepithelial carcinoma (EMC) and 5 others [including adenocarcinoma of minor salivary gland origin(n = 3), carcinoma with sebaceous differentiation(n = 1) and poorly differentiated carcinoma of salivary gland type(n = 1)]. The size of the tumours found in the resection specimens ranged from 1 cm to 13 cm, with an average size of 4.9 cm. High-risk attributes such as lymphovascular invasion (LVI), perineural invasion (PNI), pleural involvement, positive resection margins, and nodal metastasis were identified in 15.3 %, 15.3 %, 13.6 %,15.2 % and 6.7 % of cases, respectively. These attributes were found to be more frequent in ADCC than in MEC. Surgery was the main treatment modality [68/84 (80 %) cases]. ADCC cases had more recurrence and distant metastasis than MEC cases. The 3- year overall-survival (OS) and recurrence-free survival(RFS) were better in patients with age lesser than 60 years(p-value <0.0001), low pT stage (p-value 0.00038) and lower grade of MEC(p-value-0.0067).
CONCLUSION
It is crucial to have an acquaintance with the morphologic spectrum and immunophenotypic characteristics of PPSGT to recognize them in this unusual location. In tandem, it is crucial to differentiate them from conventional primary non-small cell lung carcinoma, as the management protocols and prognostic implications differ significantly.
Topics: Humans; Male; Middle Aged; Female; Retrospective Studies; Adult; Aged; Adolescent; Lung Neoplasms; Salivary Gland Neoplasms; Young Adult; Child; Carcinoma, Mucoepidermoid; Bronchial Neoplasms; Carcinoma, Adenoid Cystic
PubMed: 38447254
DOI: 10.1016/j.anndiagpath.2024.152283 -
Acta Dermatovenerologica Croatica : ADC Dec 2023Muir-Torre syndrome (MST) is a rare autosomal dominant subtype of hereditary non-polyposis colorectal carcinoma. The diagnosis is established based on the coexistence of...
Muir-Torre syndrome (MST) is a rare autosomal dominant subtype of hereditary non-polyposis colorectal carcinoma. The diagnosis is established based on the coexistence of sebaceous gland tumors and visceral organ malignancies. Mutations in the mismatch repair genes are responsible for Muir-Torre syndrome. Internal malignancies seen in MTS are most commonly colorectal, gastrointestinal system, endometrial, genitourinary system, breast, lung, brain, and hepatobiliary system malignancies. Detection of sebaceous neoplasia is essential in investigating Muir-Torre syndrome, allowing early detection of internal malignancies. Herein, we present the case of a patient with sebaceous adenomas, internal malignancies, and a new mutation detected during the genetic examination.
Topics: Humans; Muir-Torre Syndrome; MutS Homolog 2 Protein; Mutation
PubMed: 38439724
DOI: No ID Found -
Klinische Monatsblatter Fur... Feb 2024Although surgical therapy is often the first-line treatment for malignant eyelid tumors, pharmacological treatment approaches can also be included and pursued in the...
INTRODUCTION
Although surgical therapy is often the first-line treatment for malignant eyelid tumors, pharmacological treatment approaches can also be included and pursued in the treatment plan.
METHODS
Narrative review with a selective literature search on PubMed and Google Scholar.
RESULTS
Various pharmacological therapeutic principles are currently available. One option is the local application of agents within the tumor area. This can be achieved through cytostatically active drugs such as 5-fluorouracil for superficial basal cell carcinomas and precursors of squamous cell carcinomas, or through mitomycin C in specific cases of sebaceous gland carcinoma. Another form of pharmacological local therapy is local immunomodulation using Imiquimod for superficial basal cell carcinomas, actinic keratosis, and Bowen's disease. Furthermore, there are systemic pharmacological therapies like chemotherapies, for example in sebaceous cell carcinoma, or systemic immunomodulation using checkpoint inhibitors for example in basal cell carcinoma, squamous cell carcinoma, Merkel cell carcinoma, and melanoma. Additionally, targeted therapies offer yet another treatment modality that exploits the molecular biological characteristics of various tumor entities. Examples of this include Hedgehog inhibitors for basal cell carcinomas, EGFR inhibitors for squamous cell carcinomas, or BRAF inhibitors for melanomas. This review addresses these treatment options for malignant tumors of the eyelid and systematically organizes them for the reader.
CONCLUSION
Even though the data on these eye tumors is still limited, the reported case studies using systemic therapies for malignant eyelid tumors demonstrate the potential of this treatment modality. However, the need for further research is high especially concerning the combination of different therapy principles for increasing the effectiveness of eyelid tumor therapy.
PubMed: 38413000
DOI: 10.1055/a-2255-5937 -
Indian Journal of Pathology &... Apr 2024Skin adnexal or sweat gland neoplasms are rare adnexal tumors that pose a diagnostic challenge for both ophthalmologists and pathologists. Endocrine mucin-producing...
Skin adnexal or sweat gland neoplasms are rare adnexal tumors that pose a diagnostic challenge for both ophthalmologists and pathologists. Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is an uncommon low grade carcinoma of eccrine ducts with a predilection to occur in the periocular region in the elderly female. We present a rare case of 65-year-old healthy male who presented with a lobulated mass in the left eye lower lid, clinically suspected as sebaceous gland carcinoma, diagnosed as endocrine mucin-producing sweat gland carcinoma histopathologically.
Topics: Humans; Male; Aged; Sweat Gland Neoplasms; Eyelid Neoplasms; Mucins; Eyelids; Diagnosis, Differential; Carcinoma; Immunohistochemistry
PubMed: 38391357
DOI: 10.4103/ijpm.ijpm_278_21 -
European Journal of Ophthalmology Feb 2024To present the long-term outcome of the Cutler-Beard two-stage technique in patients with large full-thickness upper eyelid defects after tumor excision.
PURPOSE
To present the long-term outcome of the Cutler-Beard two-stage technique in patients with large full-thickness upper eyelid defects after tumor excision.
METHODS
The medical records of 24 patients with large full-thickness upper eyelid defects reconstructed with the Cutler-Beard technique from January 2000 to January 2021 were retrospectively reviewed. All the defects involved ≥ 60% of the horizontal length of the upper eyelid and extended vertically for at least 15 mm from the eyelid margin. Patients with follow-up < 24 months were excluded. Long-term postoperative complications, functional outcome, and patient's satisfaction at the end of the follow-up were evaluated.
RESULTS
Patients' age ranged from 36 to 88 (mean 66.0 ± 10.7 years) and 58.3% were females. Seven patients (29.2%) had had previous eyelid surgeries. The most common diagnosis was basal cell carcinoma (62.5%), followed by sebaceous gland carcinoma (12.5%), squamous cell carcinoma (8.3%), and Merkel cell carcinoma (8.3%). The mean duration of follow-up was 53.0 ± 16.9 months. Eleven patients (45.8%) developed upper eyelid entropion: 9 were treated conservatively with a therapeutic contact lens, 2 patients required a third operating stage. Most patients achieved a satisfactory functional and aesthetical outcome at the end of the follow-up.
CONCLUSIONS
Although non-bridging techniques are usually favored to reconstruct large upper eyelid defects, the Cutler-Beard bridge flap is a valuable technique in case of large horizontal defects where the vertical gap is greater than 15 mm. Further surgical steps are rarely necessary; however, alternative techniques should be considered following excision of highly malignant tumors.
PubMed: 38384118
DOI: 10.1177/11206721241234417 -
Modern Pathology : An Official Journal... Apr 2024High-risk human papillomavirus (hrHPV) and tumor-infiltrating lymphocytes (TILs) are known to have prognostic significance in oropharyngeal squamous cell carcinoma....
High-risk human papillomavirus (hrHPV) and tumor-infiltrating lymphocytes (TILs) are known to have prognostic significance in oropharyngeal squamous cell carcinoma. However, their significance in ocular sebaceous carcinoma (OSC) remains unverified because of the rarity of the condition. This study aimed to investigate the association between clinicopathologic features, biomarkers, and hrHPV infection and their potential to predict prognosis in OSC patients. We analyzed the clinicopathologic features of 81 OSC patients from Asan Medical Center between 2000 and 2022. Seventeen biomarkers and hrHPV were examined using immunohistochemistry and DNA in situ hybridization on tissue microarray cores. hrHPV was identified in 31 cases (38.3%). Univariate analysis revealed that hrHPV infection was associated with comedonecrosis (P = .032), high Ki-67 labeling index (≥30%, P = .042), lower expression of E-cadherin (P = .033), and loss of expression of zinc finger protein 750 (P = .023). Multivariate analysis revealed that loss of expression of zinc finger protein 750 (P = .026) remained an independently associated factor for hrHPV. Progression-free survival analysis was performed on 28 patients who were continuously observed for more than 5 years. During a median follow-up duration of 86 months, recurrence or metastasis developed in 14 patients (50%) within the survival cohort, occurring at a median time of 48 months after excision. Univariate analysis indicated that recurrence or metastasis was associated with tumor size (P = .010), high TILs (≥10%; P = .025), lymphovascular invasion (P = 0.043), site of origin (P = .025), and high expression of bcl-2-associated athanogene 3 (P = .039). Multivariate analysis demonstrated that high TILs (P = .017) and site of origin (P = .025) were independent prognostic factors. The prognosis of OSC was hrHPV-independent, and a better prognosis was associated with the site of origin in the order of the gland of Zeis, meibomian gland, and multicentric site, as well as with high TILs.
Topics: Humans; Prognosis; Lymphocytes, Tumor-Infiltrating; Carcinoma, Squamous Cell; Adenocarcinoma, Sebaceous; Biomarkers; Eye Neoplasms; Head and Neck Neoplasms; Sebaceous Gland Neoplasms; Human Papillomavirus Viruses
PubMed: 38369185
DOI: 10.1016/j.modpat.2024.100449 -
Indian Journal of Pathology &... 2024Sebaceous gland carcinoma is a rare and aggressive skin cancer derived from the sebaceous glands. Sebaceous carcinomas are divided into those occurring in ocular (75%)...
Sebaceous gland carcinoma is a rare and aggressive skin cancer derived from the sebaceous glands. Sebaceous carcinomas are divided into those occurring in ocular (75%) and extra-ocular locations. A 45-year-old female patient presented with rapidly growing swelling over the upper back region. It was provisionally diagnosed as an infected sebaceous cyst, and an excision biopsy was received in the pathology department. Histopathology was reported as sebaceous carcinoma, Grade II, Stage P T3 Nx. Immunohistochemistry was positive for epithelial membrane antigen. Sebaceous carcinoma accounts for 0.2-4.6% of all malignant cutaneous neoplasms, and the estimated rate of occurrence is only 1-2 per 1 million individuals per year. These tumors frequently present with a painless sub-cutaneous nodule, but they can also present as pedunculated lesions, irregular mass, or diffuse thickening of the skin. Hence, they are misinterpreted as other benign tumors or inflammatory conditions, thereby leading to delay in diagnosis, inappropriate treatment, increased morbidity, and mortality.
Topics: Female; Humans; Middle Aged; Adenocarcinoma, Sebaceous; Sebaceous Gland Neoplasms; Skin Neoplasms; Skin; Sebaceous Glands
PubMed: 38358213
DOI: 10.4103/ijpm.ijpm_860_21 -
International Journal of Dermatology Jun 2024Periocular sebaceous carcinoma (PSC) is a rare, aggressive, and potentially metastatic adnexal malignancy. Due to the ability of PSC to resemble several benign and... (Review)
Review
Periocular sebaceous carcinoma (PSC) is a rare, aggressive, and potentially metastatic adnexal malignancy. Due to the ability of PSC to resemble several benign and malignant conditions, diagnosis is often delayed or mistaken. In addition, even with a known diagnosis, choosing the right treatment is still an open debate. For this reason, we decided to review the most up-to-date literature on PSC and propose a dedicated procedural protocol to help clinicians when dealing with PSC. A PubMed search was carried out using the terms "Sebaceous Carcinoma", "Adnexal Periocular Cancer", "Sebaceous Carcinoma AND eyelid", "Periocular Sebaceous Carcinoma", and "Ocular Adnexa". Pertinent studies published in English from 1997 up to December 2022 were compared to the selection criteria and if suitable, included in this review. Through the initial search, 84 articles were selected. Of these, 36 were included in the final study. Several papers explored different diagnostic and therapeutic strategies regarding PSC diagnosis and management. In light of the current literature review and the multidisciplinary experience of three clinical centers, a dedicated procedural protocol is proposed. PSC diagnosis may be achieved through accurate clinical evaluation, but it requires histopathologic confirmation, which can be challenging. Dermoscopy, in vivo reflectance confocal microscopy, and optical coherence tomography may facilitate PSC clinical examination, while immunohistochemistry stains may support histological diagnosis. Appropriate disease staging is necessary before choosing the treatment, as local disease requires radically different treatment compared to advanced disease. In addition, recent innovations in nonsurgical treatments, including radio-chemotherapy, immunotherapy, and targeted therapy, may be a viable option in the most challenging cases.
Topics: Humans; Sebaceous Gland Neoplasms; Eyelid Neoplasms; Adenocarcinoma, Sebaceous; Neoplasm Staging; Dermoscopy; Diagnosis, Differential
PubMed: 38351466
DOI: 10.1111/ijd.17045