Eyelid malignancies in young individuals: clinical peculiarities.

OBJECTIVE: The incidence of malignant eyelid tumors is considerably increasing, even in young patients. The purpose of this study was to identify particularities in individuals under 40 years of age affected by eyelid malignancies. METHODS: Clinical charts of patients under 40 years of age who underwent eyelid tumor excision from 2014 to 2020 in two reference centers, one in Brazil and one in the USA, were reviewed. Demographic and outcome measures included: age, gender, skin phototype, comorbidities, diagnosis, time until diagnosis, lesion location, recurrence and metastasis. In addition, associated characteristics, including chronic sun exposure, intentional tanning (outdoor or artificial), history of smoking, use of sunscreen, family history of skin cancer and exposure to pesticides or herbicides, were reviewed. RESULTS: A total of 24 malignant eyelid tumors from 17 patients were identified. Twelve (70.6%) patients were female, and the most prevalent tumor was basal cell carcinoma (62.5%). Three (17.6%) patients had xeroderma pigmentosum and presented with multiple lesions. Family history of skin cancer was reported by 47% of patients. Prolonged sun exposure was reported by 41.2%; history of smoking and intentional tanning were reported by 23.5 and 17.6%, respectively, and might have played a role in carcinogenesis. CONCLUSIONS: Although uncommon in young subjects, eyelid malignancies present some peculiarities in individuals under 40 years of age. Our results suggest that women with family history of skin cancer and history of chronic exposure to ultraviolet radiation are at risk. Association with genetic syndromes is also relevant among younger age groups.

The effect of direct cell injury inflicted by cryotherapy on eyelid sebaceous gland carcinoma cells: An ex-vivo experimental study.

PURPOSE: To evaluate the effect of direct cell injury of cryotherapy on eyelid sebaceous gland carcinoma cells by an ex vivo cryotherapy experiment. METHODS: It was a prospective interventional case series. Six patients with biopsy-proven nodular sebaceous gland carcinoma were included. After excision of the mass, a thin slice of the mass resembling the thickness of the conjunctiva was shaved off and was oriented over the broad end of a tissue forceps. Cryotherapy was applied to both its anterior and posterior aspects by the triple freeze-thaw technique. The mass was then labeled and sent separately for histopathological evaluation by fixation and staining. RESULTS: A total of six patients with a mean age of 58.2 ± 15.5 years were included. There were four females and two males. The mean duration of the lesion was 21.6 ± 17.51 months. All patients had involvement of the upper eyelid. The patients were clinically staged as T2b (n=2), T1a (n=2), T2c (n=1), and T3a (n=1) respectively. There was no regional lymphadenopathy or metastasis in any of the cases. The experimental cryo-tissue containing the cryo-treated lesion revealed the presence of viable tumor cells (>50%) in all six specimens. CONCLUSION: The direct cell injury caused by cryotherapy may not be sufficient to kill all the residual sebaceous gland carcinoma cells on the tumor bed.

The Köllner Tarsoconjunctival Flap for Lower Eyelid Reconstruction: Historical Perspective and Surgical Outcomes of 140 Cases.

PURPOSE: While Wendell Hughes popularized the tarsoconjunctival flap for lower eyelid reconstruction, most modern procedures are derived from the technique described by Köllner in 1911. This study reviews the history, techniques, and outcomes of a large series of patients treated with a modified Köllner flap. METHODS: In this observational cohort study, medical records and photographs were reviewed in patients undergoing surgery between 2005 and 2020. Patient demographics, complications, secondary interventions, and outcomes were evaluated. RESULTS: Marginal defect size ranged from 12 to 41 mm among the 140 study patients. Ancillary procedures included lower eyelid conjunctival flaps (n = 64) and septal orbicularis flaps (n = 68). The anterior lamella was reconstructed with skin grafts (n = 86), flaps (n = 10), or combined flaps/grafts (n = 44). Pedicle division was performed 23 to 84 days after primary repair. Subsequent interventions included steroid injection (n = 10), cryotherapy (n = 10), marginal erythema treatment (n = 9), and upper eyelid retraction repair (n = 6). Tearing (6.5%) and dryness (10%) were the most common postoperative symptoms, with most patients (78.6%) being asymptomatic. A good-excellent functional outcome was achieved in 94.3% and cosmetic outcome in 85.0% of cases. Defects <30 mm in width (P = 0.0001), defects not involving a canthus (P = 0.0158), and upper eyelid skin graft donor sites (P = 0.0001) were associated with better outcomes. CONCLUSIONS: The Köllner tarsoconjunctival flap is an effective technique to repair moderate-large lower eyelid defects, with the majority of patients achieving good-excellent cosmetic and functional outcomes. Factors associated with a poorer result include marginal eyelid defects ≥30 mm in width, defects involving a canthus, and non-upper eyelid skin graft donor sites. Most patients are asymptomatic after surgery.

[Pleomorphic adenoma of the lacrimal gland in a young girl: a case report]. / Adenoma pleomorfo de glándula lagrimal: reporte de un caso pediátrico.

We present a case of a 13 year old girl with a diagnosis of Pleomorphic Adenoma (PA) located in the lacrimal gland of the left upper eyelid. The patient had a very satisfactory recovery after surgical excision of the tumor, with no recurrence after a two year follow-up. The clinical, radiological and histopathological characteristics of PAs are discussed.

Identification and regulation pattern analysis of long noncoding RNAs in meibomian gland carcinoma.

AIM: To identify long noncoding RNAs (lncRNAs) and to elucidate regulation patterns of lncRNAs in meibomian gland carcinoma (MGC). MATERIALS & METHODS: We used RNA-Seq, gene ontology, ClueGO, Ingenuity Pathway Analysis and co-expression network analyses to profile the expression and regulation patterns of lncRNAs and mRNAs in MGC. RESULTS: We identified 500 lncRNAs and 326 mRNAs as differentially expressed. Co-expression regulatory networks with lncRNAs and mRNAs were constructed. The differentially expressed mRNAs and lncRNAs were enriched by fundamental biological functions that are implicated in the inflammatory signaling pathway and tumor proliferation (IL6 and PTGS2). CONCLUSION: LncRNAs might play important roles via the competing endogenous RNA regulation pattern in MGC tumorigenesis and contribute to the molecular pathogenesis of MGC.

A case report of eyelid Merkel cell carcinoma occurring under treatment with nivolumab for a lung adenocarcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare neuroendocrine malignancy of the skin characterized by high aggressiveness. Four main factors are implicated in its development: immunosuppression, ultraviolet radiation, age and the Merkel cell polyomavirus (MCPyV). In recent years, immune checkpoint inhibitors have shown clinical activity in MCC treatment. CASE PRESENTATION: We report the case of an 82-year-old man with a lung adenocarcinoma diagnosis, who underwent immunotherapy with nivolumab as second-line treatment. Seven months after the diagnosis of lung cancer during the nivolumab treatment, the patient developed an eyelid MCC, initially misdiagnosed as a chalazion. A palliative radiotherapy was performed with clinical benefit. After a total of seven cycles of nivolumab, computed tomography showed a lung and cerebral disease progression. In addition, clinical conditions worsened leading to the patient's death 13 months after the initial lung cancer diagnosis. CONCLUSIONS: Cases of co-occurrence of MCC and non-small cell lung cancer (NSCLC) have rarely been reported. Interestingly, common risk factors may be postulated for both cancers. Considering the rarity of this adverse event, its short-term temporal relation with the administration of the drug, which makes a relation improbable, and the coexistence of other risk factors, which may provide plausible explanations, it is possible to conclude according to the WHO Adverse Reaction Terminology that a causal relation between the occurrence of this serious adverse event and the exposure to the drug is unlikely. However, the case deserves to be reported in the literature.

Thermal Burn Scar-Related Squamous Cell Carcinoma in the Eyelid.

A 63-year-old woman presented with a large, ulcerokeratotic mass in her right lower eyelid that had grown over the last 2 months in a burn scar. She had experienced a thermal burn involving both inferior periorbital areas 35 years ago and received medical treatment. The lesion was excised with wide margins. The histologic diagnosis was squamous cell carcinoma. No recurrence was observed during a 22-month follow up. In the previous literature, a total of 10 cases of eyelid burn scar neoplasm, of which 9 were basal cell carcinoma, have been reported. To the authors' knowledge, this is the first reported case of thermal burn-related squamous cell carcinoma of the eyelid.

Eyelid lymphoma in a patient with a gold weight implant. / Linfoma palpebral en paciente con implante de pesa de oro.

CASE REPORT: A male patient with an exposure keratopathy caused by lagophthalmos. A gold weight was implanted in the right upper eyelid. Eight months later, he presented with erythema and swelling of right upper eyelid. An incisional biopsy was performed, reporting extranodal marginal zone B cell lymphoma. DISCUSSION: when a tumour at the site of a gold weight implant is refractory to treatment, it is essential to perform an incisional biopsy to establish the histopathological diagnosis. Ocular adnexal lymphomas are relatively common. The presence of foreign material can cause chronic inflammation that could be the stimulus for the development of a lymphoproliferative disorder.

Solitary Mastocytoma of the Eyelid in an Adult Patient With Prolidase Deficiency.

Prolidase deficiency and solitary mastocytoma of the eyelid are both exceedingly rare. Prolidase deficiency is an inherited connective tissue disorder that has systemic sequelae, such as intractable skin ulceration, poor wound healing, recurrent infections, and intellectual impairment. Cutaneous mastocytoma is an isolated, aberrant cutaneous aggregation of mast cells. A case of an adult with severe prolidase deficiency who developed cutaneous mastocytoma of the eyelid was presented. To the authors' knowledge, adult-onset solitary mastocytoma of the eyelid has never been reported previously.

Kaposi Sarcoma of the eyelid as an initial manifestation of AIDS.

Kaposi sarcoma (KS) is a multifocal systemic disease that originates in the vascular endothelium related to Human Herpes Virus 8 (HHV-8). In the early 1980s the first series of cases of disseminated Kaposi Sarcoma in HIV infected patients were reported. However, with the advent of highly active antiretroviral therapy (HAART) since 1997, these cases are less frequently observed by clinicians. We report the case of a 40-year-old woman, presenting with two asymptomatic purpuric nodules localized in the superior and inferior left eyelids, occluding the palpebral fissure, which were present for 4 months prior to presentation. The eyelid nodules were determined to represent KS, but there were no additional cutaneous lesions. Pulmonary and gastric KS involvement was documented. Antiretroviral therapy was initiated along with pegylated liposomal doxorubicin. The nodules gradually disappeared and her immune status eventually improved. Ocular and periorbital involvement of KS associated with HIV-1 infection as the initial clinical manifestations is a rare advent. This case is important as it illustrates that disseminated KS was not to be predicted by the number or the extension of cutaneous lesions.

Eyelid reconstruction in a child with xeroderma pigmentosum. / Reconstrucción palpebral en niño con xeroderma pigmentoso.

CLINICAL CASE: Seven-year-old male patient, affected by xeroderma pigmentosum (XP) who was referred to ophthalmology due to the presence of numerous tumours in his left eye that affected the eyelids and conjunctiva. He had a tumour in his lower eyelid that affected the free edge, creating a secondary ectropion, and several lesions with a melanocytic appearance in conjunctiva. A resection of the eyelid tumour and the conjunctival lesions were performed by placing a skin graft and amniotic membrane, respectively, as covering. DISCUSSION: XP patients have high probability of developing eye tumours. A thorough ophthalmic examination is necessary to establish an early diagnosis.

Periocular Skin Cancer in Solid Organ Transplant Recipients.

PURPOSE: To determine the proportion of solid organ transplant recipients developing periocular nonmelanoma skin cancer and to describe the morbidity of these cancers in transplant recipients. DESIGN: Cohort study. PARTICIPANTS: Consecutive patients undergoing solid organ transplantation at the Cleveland Clinic between 1990 and 2008. METHODS: The charts of all patients receiving a solid organ transplant from 1990-2008 evaluated in the dermatology department for a subsequent biopsy-proven head and neck malignancy through April 2015 were reviewed. Patients with a periocular region nonmelanoma skin cancer (NMSC) or a nonperiocular NMSC causing a complication requiring eyelid surgery were included. Charts were reviewed for demographic data; transplant date, type, and source; immunosuppressive agents received at diagnosis; and type of NMSC, number of nonperiocular NMSCs, ophthalmologic findings, and periocular sequelae after the repair. MAIN OUTCOME MEASURES: Primary outcome measures included the type, location, final defect size, tumor-node-metastasis classification, presence of perineural invasion, and reconstruction technique(s) used for each periocular NMSC. Secondary outcome measures included the type and treatment of ocular sequelae due to nonperiocular facial NMSC. RESULTS: A total of 3489 patients underwent solid organ transplantation between 1990 and 2008. Of these, 420 patients were evaluated in the dermatology clinic for biopsy-proven NMSC of the head and neck during the study period, and 11 patients (15 malignancies) met inclusion criteria. Nine patients developed 12 periocular malignancies and 3 patients required eyelid surgery for facial malignancies outside the periocular zone. All 11 patients developed a squamous cell carcinoma (14 malignancies), and 1 patient (1 malignancy) also developed a periocular basal cell carcinoma. There was orbital invasion in 4 cases and paranasal and/or cavernous sinus invasion in 3 cases. Two patients underwent exenteration. Seven cases required reconstruction with a free flap or graft. Periocular sequelae included lower eyelid ectropion (6 malignancies), dry eye and/or exposure symptoms (8 malignancies), unilateral vision loss (3 malignancies), and facial nerve paresis (5 malignancies). CONCLUSIONS: Squamous cell carcinoma affecting the periocular region represents a risk of solid organ transplantation and may produce significant ocular morbidity, including the need for major eyelid reconstruction, globe loss, and disfiguring surgery.

Transpalpebral proton beam radiotherapy of choroidal melanoma.

BACKGROUND: Collateral damage to upper eyelid margin during proton beam radiotherapy (PBR) for choroidal melanoma may cause squamous metaplasia of the tarsal conjunctiva with keratinisation, corneal irritation, discomfort and, rarely, corneal perforation. We evaluated transpalpebral PBR as a means of avoiding collateral damage to the upper eyelid margin without increasing the risk of failure of local tumour control. METHODS: Retrospective study of consecutive patients who underwent PBR for choroidal melanoma between 1992 and 2007 at the Royal Liverpool University Hospital and the Douglas Cyclotron at Clatterbridge Cancer Centre, UK. RESULTS: Sixty-three patients were included in this study. Mean basal tumour diameter and tumour thickness were 11.8 mm and 3.6 mm, respectively. PBR mean beam range and modulation were 26.5 mm and 16.9 mm respectively. The eyelid margin was included in the radiation field in 15 (24%) eyes. The median follow-up was 2.5 years. Local tumour recurrence developed in 2 (3.2%) patients. In these two cases that developed tumour recurrence the transpalpebral treatment did not involve the eyelid margin. Six (9.5%) patients died of metastatic disease. No eyelid or ocular surface problems developed in any of the 48 patients who were treated without eyelid rim involvement, while 7 of the 15 patients with unavoidable irradiation of the eyelid rim developed some degree of madarosis. These seven patients all received more than 26.55 proton Gy to the eyelid margin. Symptoms, such as grittiness occurred in 12% of 48 patients without eyelid margin irradiation as compared with 53% of 15 patients whose lid margin was irradiated. CONCLUSIONS: Transpalpebral PBR of choroidal melanoma avoids eyelid and ocular surface complications without increasing failure of local tumour control.

Periocular sebaceous cell carcinoma following chemical burn: a unique case of a Marjolin ulcer.

This study reports a unique case of a Marjolin ulcer in a patient who presented with periocular sebaceous cell carcinoma 16 years after a lye chemical burn. A 68-year-old woman presented with a long history of irregular and tender right upper and lower eyelid margins, periocular erythema, madarosis, trichiasis, ptosis, and chemosis, occurring many years after chemical burn to the area. After eyelid biopsy established the diagnosis of sebaceous cell carcinoma, subsequent map biopsies of the conjunctival surface revealed extensive sebaceous cell carcinoma with pagetoid spread, necessitating anterior exenteration for definitive treatment. This report describes the first case in which a sebaceous cell carcinoma has arisen in the area of a chemical burn. Sebaceous cell carcinomas should be included in the spectrum of Marjolin ulcers, and ophthalmologists should be aware of this entity when evaluating patients with a history of a burn to the eyelids.

[Clinical study and risk factors for recurrence of basal cell carcinoma of the eyelid: results of a Tunisian series and review of the literature]. / Étude clinique et facteurs de risque de récidive des carcinomes basocellulaires des paupières : résultats d'une série tunisienne et revue de la littérature.

PURPOSE: To report our results of treatment of eyelid basal cell carcinomas and evaluate risk factors for recurrence. PATIENTS AND METHODS: We carried out a retrospective study of 172 basal cell carcinomas in 168 patients, treated in the Ophthalmology department of Sousse University Medical Center (Tunisia), from January 1987 to July 2012. Initial treatment was surgical excision with a standard margin of 4mm or a single radiation treatment. When excision was incomplete, we had the choice between further excision, radiotherapy or a "wait and see" approach. Mean follow-up in our study was 11.5 months with range from 1 month to 14 years. RESULTS: We treated 169 tumors in 165 patients, with three patients out of 168 having refused the proposed treatment. Primary radiotherapy was performed in 4 cases in patients presenting with large tumors and refusing disfiguring surgery. For the 165 other tumors (95.9%), surgical excision was performed. Surgical margins were clear in 106 cases (64.2%) and positive in 37 cases (22.4%). No residual tumor was noted in 2 cases for which biopsy was initially performed, and margins could not be visualized in 20 cases (12.1%) due to the small size or fragmentation of the specimen. For the incompletely excised tumors, we performed a second excision in three cases (8%), radiotherapy in 11 cases (29.7%) and a "wait and see" approach in 22 cases (59.4%). The recurrence rate was 6.9%, with a mean time of 32.8 months until recurrence. CONCLUSION: Basal cell carcinoma represents the most frequent malignant tumor of the eyelids. Surgery remains the standard treatment. The "wait and see" approach offers an interesting option for the management of incompletely excised basal cell carcinoma, especially with low risk lesions.

Ultraviolet light and ocular diseases.

The objective of this study is to review the association between ultraviolet (UV) light and ocular diseases. The data are sourced from the literature search of Medline up to Nov 2012, and the extracted data from original articles, review papers, and book chapters were reviewed. There is a strong evidence that ultraviolet radiation (UVR) exposure is associated with the formation of eyelid malignancies [basal cell carcinoma (BCC) and squamous cell carcinoma (SCC)], photokeratitis, climatic droplet keratopathy (CDK), pterygium, and cortical cataract. However, the evidence of the association between UV exposure and development of pinguecula, nuclear and posterior subcapsular cataract, ocular surface squamous neoplasia (OSSN), and ocular melanoma remained limited. There is insufficient evidence to determine whether age-related macular degeneration (AMD) is related to UV exposure. It is now suggested that AMD is probably related to visible radiation especially blue light, rather than UV exposure. From the results, it was concluded that eyelid malignancies (BCC and SCC), photokeratitis, CDK, pterygium, and cortical cataract are strongly associated with UVR exposure. Evidence of the association between UV exposure and development of pinguecula, nuclear and posterior subcapsular cataract, OSSN, and ocular melanoma remained limited. There is insufficient evidence to determine whether AMD is related to UV exposure. Simple behaviural changes, appropriate clothing, wearing hats, and UV blocking spectacles, sunglasses or contact lens are effective measures for UV protection.