Vismodegib as Eye-Sparing Neoadjuvant Treatment for Locally Advanced Periocular Basal Cell Carcinoma.

Locally-advanced periocular basal cell carcinoma (BCC) pose many therapeutic challenges due to the need to preserve functionality and cosmesis of the orbit and periocular area. Surgical excision and subsequent orbital exenteration are two recognized modalities of treatment. Vismodegib is currently an FDA-approved monotherapy for locally-advanced and metastatic BCC. We present a case of the use of vismodegib as neoadjuvant therapy prior to surgical excision of a locally-advanced periocular recurrent BCC in a 75-year-old male. The patient’s tumor successfully responded to vismodegib allowing surgical excision with clear margins. The orbit was saved in a patient who otherwise would have required complete orbital exenteration. J Drugs Dermatol. 20(5):552-554. doi:10.36849/JDD.5661.

Nordic treatment practices survey and consensus for treatment of eyelid sebaceous carcinoma.

BACKGROUND: The purpose was to describe the Nordic treatment practices and to reach a Nordic consensus for the treatment of sebaceous eyelid carcinoma. METHODS: The treatment practices data was collected by a questionnaire with 37 questions to the Nordic oculoplastic surgeons and analyzed. A PubMed MEDLINE database search was done to gather data on the published treatment practices and recommendations. A working group that consisted of in minimum one senior consultant from each leading Nordic University Eye Hospital was assigned. A structured interactive method was used to establish the consensus. RESULTS: Twenty-four doctors responded to the questionnaire. 23/24 (96%) of the respondents took a biopsy before surgery. Regional lymph node scanning was routinely done by 14/23 (61%) and a systemic screening of a metastatic disease by 13/23 (57%). 6/22 (27%) never took conjunctival mapping biopsies and 12/23 (52%) never screened for Muir- Torre. Respondents used Mohs surgery, frozen section or multi-stage excision with delayed closure, and 5-6 mm was the mostly preferred margin. Sentinel lymph node biopsy was a possible option for 9/22 (41%) and cryotherapy and Mitomycin C for 6/22 (27%) respondents. 50% of respondents considered radiation as a treatment option. 15/16 (94%) respondents always followed-up their patients, most for 5 years. Two thirds scanned regional lymph nodes during the follow-up. Consensus was reached for 18 statements representing three domains: preoperative work-up, treatment and follow-up. CONCLUSION: Treatment practices differ in between the five Nordic countries which have similar public health care systems. In the article the authors present a Nordic consensus for the treatment of eyelid sebaceous carcinoma.

Prognostic Factors, Treatment, and Survival in Primary Cutaneous Mucinous Carcinoma: A SEER Database Analysis.

BACKGROUND: Limited information exists on the demographics, tumor characteristics, and treatment in primary cutaneous mucinous carcinoma (PCMC). OBJECTIVE: The authors sought to describe prognostic factors, incidence rates, and the subsequent primary malignancy (SPM) risk in patients with PCMC. METHODS: Primary cutaneous mucinous carcinoma cases in the National Cancer Institute's Surveillance, Epidemiology, and End Results data (1972-2013) were analyzed to provide demographic, cancer-related, and treatment information and to calculate incidence and mortality. Patients were stratified by stage (local, regional, distant disease) for comparison. The risk of developing an SPM was calculated. RESULTS: Four hundred eleven PCMC cases were identified. The age-adjusted incidence was 0.04 cases per 100,000-person years. Blacks were disproportionately affected by PCMC (0.048; 95% confidence interval, 0.034-0.065; p < .001). Approximately 67.4% of patients had local disease, 10.5% had regional disease, and 5.8% had distant disease. Primary cutaneous mucinous carcinoma-specific mortality was independent of sex, age, race, primary site, histologic tumor grade, tumor size, tumor stage, or treatment. The overall frequency of developing a second primary malignancy was not increased in patients with PCMC. CONCLUSION: Although PCMC occurs with equally in both sexes, it may be more common in African Americans than previously recognized. Although eyelid PCMC may have a higher rate of distant metastasis, all patients need close follow-up.

Eyelid abscess masking underlying sebaceous cell carcinoma.

Sebaceous cell carcinoma is well known as the "great masquerader" mimicking other benign or malignant eyelid conditions and lesions. We present a case of a middle age male presenting with a subacute left upper lid abscess who was ultimately diagnosed with malignant sebaceous cell carcinoma after incision and drainage and treatment with broad spectrum antibiotics. This case highlights the deceptive clinical and radiographic appearance of this tumor and the importance of histologic examination in atypical or refractory periorbital abscesses.

Treatment of a case with giant eyelid syringomas.

He had a history of multiple asymptomatic flesh-colored papules, which had slowly increased in number and coalesced into plaques, on both upper and lower eyelids. Some of the plaques were giant and mostly coalescent in nature. We excised surgically the giant plaques which were approximately 1.5-2 cm in diameter symmetrically from upper and lower eylids and the remaining plaques were left to be treated with cauterization. Histopathological examination revealed syringoma. Both of the eyelids were observed to have a cosmetically adequate appearence after the surgical intervention.

Periocular infantile hemangiomas: Characteristics, ocular sequelae, and outcomes.

OBJECTIVES: To identify clinical factors associated with complications of periocular infantile hemangioma (IH) and monitor improvement in complication rates post-treatment. METHODS: Retrospective cohort study. Eighty-nine patients diagnosed with periocular IH at a pediatric dermatology clinic of a tertiary care center between 2001 and 2013 were included with parental approval. Parents were interviewed by telephone between July and September of 2015, then again in January 2018 to inquire about ophthalmologic follow-up. Electronic medical records were reviewed from January 2001 through January 2018. RESULTS: Sixty percent of patients demonstrated ocular sequelae, including astigmatism (33%), visual axis obstruction (29%), nasolacrimal duct obstruction (7%), ptosis (4%), amblyopia (3%), and strabismus (1%). Compared with superficial IH, deep and mixed IH had higher odds, 3.4 (P = 0.025) and 3.8 (P = 0.034), respectively, of developing ocular sequelae. All patients with astigmatism prior to involution of IH received systemic therapy, with a significant post-treatment decrease in the proportion of patients with astigmatism (40% to 18%, P = 0.027). Three-quarters of patients experienced complete IH involution by time of enrollment in kindergarten. Fifty-one (57.3%) patients received formal ophthalmologic evaluation confirmed through chart review or phone interview, with average follow-up duration of 51.2 months (range: 1.9, 99.3). CONCLUSION: Deep and mixed IH were more likely to demonstrate ocular complications than superficial IH. Rate of astigmatism decreased with systemic therapy. Our study suggests that patients with periocular IH have a lower rate of amblyopia now compared with the prepropranolol era and emphasizes the importance of early treatment of periocular IH to prevent permanent visual sequelae.

[Importance of Cryotherapy for Eyelid Papillomas]. / Kryotherapie in der Behandlung ausgedehnter Lidpapillome.

PURPOSE: Cryotherapy as a destructive procedure is an important alternative to the surgical excision of eyelid tumors. METHODS: We report our own experiences in the cryotherapy of large upper eyelid papillomas using spray- and contact-freezing. RESULTS/CONCLUSIONS: Cryotherapy gave excellent results in the treatment of unusually large papillomas. The preservation of collagen structures during cryotherapy provides a good aesthetic and functional outcome.

Impact of clinicopathologic factors on survival in patients with sebaceous carcinoma of the eyelid - a population-based analysis.

Purpose: To evaluate prognostic factors and survival of patients with sebaceous carcinoma of the eyelid through a population-based analysis. Methods: A total of 940 patients with primary sebaceous carcinoma of the eyelid were derived from the Surveillance, Epidemiology, and End Results (SEER) database in the United States from 1973 to 2013. Kaplan-Meier univariate analysis and Cox Regression multivariate analysis were performed to examine prognostic factors in overall survival (OS). 5- and 10-year survival rates, median survival, and prognostic variables with statistical significance were measured. Results: Kaplan-Meier analysis showed that OS is 66% and 44% at 5 years and 10 years respectively. Median OS is 9.4 years. Multivariate Cox regression analysis demonstrated that independent prognostic factors for OS are age at diagnosis (HR = 4.61 [95% CI 1.93-11.0], P = 0.001), surgical treatment (HR = 0.196 [95% CI 0.07-0.55], P = 0.002), combined surgical and radiation treatment (HR = 0.227 [95% CI 0.06-0.81], P = 0.023), and greater tumor size at diagnosis (HR = 3.381, [95% CI 1.77-6.45], P < 0.001). Conclusion: We report the largest population study to date to evaluate prognostic factors of patients with sebaceous carcinoma of the eyelid. Multivariate analysis shows that older age, and greater tumor size correlate with decreased overall survival, whereas surgical treatment or combined surgical and radiation treatment correlate with increased overall survival. Interestingly, tumor grade, lymph node involvement, and distant extent of tumor have not demonstrated to be independent prognostic factors for overall survival.

Eyelid Merkel cell carcinoma in a patient treated with golimumab.

PURPOSE: To describe a clinical case of biopsy-proven Merkel cell carcinoma of the eyelid following golimumab therapy for rheumatoid arthritis (RA). METHODS: Interventional case report. RESULTS: A 73-year-old woman with a history of chronic RA presented with a right upper eyelid mass. She had been treated with golimumab (tumor necrosis factor (TNF) inhibitors) injection therapy for the past 6 months. A biopsy showed findings suggestive of a Merkel cell carcinoma of the eyelid. CONCLUSIONS: Merkel cell carcinoma may be associated with anti-TNF treatment and should be included in the differential diagnosis of an eyelid tumor in patients treated with TNF inhibitors.

[Establishment of standardized diagnosis and treatment of eyelid sebaceous carcinoma in China].

Eyelid sebaceous carcinoma is a malignant tumor originating from Meibomian glands, Zeis glands, and other glands surrounding hair follicles. It is the second most common eyelid malignancy, following basal cell carcinoma, in China. Considering its diversified clinical manifestations and strong tendency for recurrence and metastasis, standardized cancer staging, surgical excision with frozen section control, and postsurgical follow-up has been internationally adopted with the aim to considerably reduce the risk of recurrence and metastasis. However, the lack of standardized and normalized staging, surgical treatment and follow-up in China inevitably caused high recurrence rate and low predictability of prognosis of eyelid sebaceous carcinoma. Therefore, it is of great significance to standardize the clinical diagnosis and treatment of eyelid sebaceous carcinoma in China. (Chin J Ophthalmol, 2018, 54: 481-483).

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1: Multidisciplinary Recommendations for Care.

TOPIC: Children and adults with neurofibromatosis type 1 (NF1), a common autosomal dominant condition, manifest a variety of ophthalmologic conditions. Plexiform neurofibromas (PNs) involving the eyelid, orbit, periorbital, and facial structures (orbital-periorbital plexiform neurofibroma [OPPN]) can result in significant visual loss in children. Equally important, OPPNs can cause significant alteration in physical appearance secondary to proptosis, ptosis, and facial disfigurement, leading to social embarrassment and decreased self-esteem. CLINICAL RELEVANCE: Although NF1 is a relatively common disease in which routine ophthalmologic examinations are required, no formal recommendations for clinical care of children with OPPNs exist. Although medical and surgical interventions have been reported, there are no agreed-on criteria for when OPPNs require therapy and which treatment produces the best outcome. METHODS: Because a multidisciplinary team of specialists (oculofacial plastics, pediatric ophthalmology, neuro-ophthalmology, medical genetics, and neuro-oncology) direct management decisions, the absence of a uniform outcome measure that represents visual or aesthetic sequelae complicates the design of evidence-based studies and feasible clinical trials. RESULTS: In September 2013, a multidisciplinary task force, composed of pediatric practitioners from tertiary care centers experienced in caring for children with OPPN, was convened to address the lack of clinical care guidelines for children with OPPN. CONCLUSIONS: This consensus statement provides recommendations for ophthalmologic monitoring, outlines treatment indications and forthcoming biologic therapy, and discusses challenges to performing clinical trials in this complicated condition.

Periocular Neoplasia in the Horse.

Periocular neoplasia is common in horses. Treatment of the periocular skin and ocular adnexal structures can be technically challenging. Common neoplastic conditions, a treatment algorithm, surgical principles, and therapeutic modalities are reviewed. Regardless of the type of neoplasia found or the treatment that is applied, success is most likely when the neoplastic tumor is small.

Sebaceous Carcinoma of the Eyelid.

BACKGROUND: Periocular sebaceous carcinoma (PSC) is a rare but aggressive neoplasm that tends to clinically and histopathologically mimic other conditions. PSC can be challenging to diagnose using histomorphology alone given its overlap with 2 more common tumors that occur in this area (basal cell carcinoma [BCC] and squamous cell carcinoma [SCC]). Use of immunohistochemistry can help resolve this differential diagnosis. METHODS: A review of the literature was performed, focusing on the epidemiology, morphology, and immunohistochemical features of PSC. RESULTS: The most useful immunostains in the differential diagnosis of PSC are epithelial membrane antigen, Ber-Ep4, androgen receptor (AR), and adipophilin. To discern PSC from BCC, one should use EMA, Ber-Ep4, AR, and adipophilin, whereas discerning PSC from SCC can be achieved by evaluating AR and adipophilin. In addition, p53 and ERBB2 (formally known as HER2/neu) are other potentially useful immunohistochemical markers for the differential diagnosis of PSC. CONCLUSIONS: Use of new immunohistochemical techniques, as well as the elucidation of molecular alterations, such as the presence of ERBB2 amplification, will advance our understanding of PSC.

Primary cutaneous CD30+ anaplastic large cell lymphoma treated with radiotherapy and methotrexate with development of xanthomas at the sites of prior disease.

Primary cutaneous anaplastic large cell lymphoma is a rare type of cutaneous T-cell lymphoma, and the involvement of the ocular adnexa is extremely rare. Secondary xanthoma-like changes after radiation therapy or chemotherapy have been rarely reported in association with large-cell T-cell anaplastic lymphoma. We report one case of a primary C-anaplastic large cell lymphoma affecting the eyelid with fast progression with multiple nodules in various anatomic sites and development of xanthoma-like lesions after treatment.

Primary Signet-Ring Cell/Histiocytoid Carcinoma of the Eyelid: A "Binocle" Presentation of the "Monocle Tumor".

Primary cutaneous signet-ring cell carcinoma is a rare and aggressive neoplasm which diffusely involves dermis and subcutis of the eyelid or axillae. Neoplastic cells show a signet-ring cell or histiocytoid morphology in variable number, and can be found intermingled among collagen bundles, sparing the epidermis. This neoplasm typically appears in the eyelids of elderly men, in the form of a painless infiltration and swelling but with no other specific clinical feature, and frequently causes diagnostic retardation and worse prognosis. Frequent involvement of both eyelids of the same eye has given it the name of monocle tumor. Only 29 cases have been described in English literature to date, of which 7 developed metastases, mainly on regional lymph nodes. The authors present a case of involvement of contralateral eyelid, which has only been described previously in 2 cases. The immunohistochemical profile of the involvement in the contralateral eye, and the absence of other metastasis, suggest that it is locally spread from the initial lesion. However the possibility of being a second primary tumor or metastasis cannot be readily ruled out.

Carbon dioxide laser combined with botulinum toxin A for patients with periorbital syringomas.

BACKGROUND: Although various destructive methods have been described for treating syringomas, they are often associated with significant scarring and recurrence. In 2007, multiple-drilling method using carbon dioxide (CO2) laser was introduced as an alternative modality to gain good cosmetic results. OBJECTIVE: To retrospectively evaluate the effectiveness of CO2 laser combined with botulinum toxin A (BTXA) as treatment for syringomas. MATERIAL AND METHODS: Forty-eight patients with periorbital syringomas were treated with topical application of BTXA immediately after CO2 laser treatment. Forty-four patients were treated with CO2 laser only. RESULTS: Patients who were treated with CO2 laser combined with BTXA required significantly (p = 0.038) fewer treatment sessions compared with those treated with CO2 laser only. When the clinical improvements of the two treatment sessions were compared, the combined therapy (CO2 laser and BTXA) had significantly (p = 0.044) higher rate of results showing good (disappearance of 60-80% of lesion) or excellent (80-100%) than the CO2 laser only therapy (87.5% vs. 70.5%). CONCLUSIONS: CO2 laser combined with BTXA showed better effect than CO2 laser only.

Malignant hair follicle tumors of the periorbital region: A review of literature and suggestion of a management guideline.

Malignant hair follicle tumors are rare skin adnexal malignancies that have a predilection for the head and neck region. They can be categorized into a number of different subtypes. Histologically, they are distinct from their benign counterpart. To the best of our knowledge, there is no extensive review of these malignancies, especially in the periorbital region. We aim to provide a literature review and a guideline for management of these malignant tumors in the periorbital region. Database from Medline, PubMed, Embase, and Google Scholar were consulted. A total of 16 cases from the literature on hair follicle malignancies in the periorbital region were included in this review. The clinical presentations, diagnostic patterns, investigations used, and best management approach of these tumors are discussed. The American Joint Committee on Cancer (AJCC) 7(th) edition carcinoma of the eyelid staging system was used to describe their behaviors. We recommend wide excision surgery and a close follow-up for these tumors. Tumors presenting with a late stage require work-up for distant metastasis and consideration for exenteration procedures. The role of radiotherapy and chemotherapy in this context is still uncertain.

Squamous Cell Carcinoma of the Caruncle.

Squamous cell carcinoma (SCC) of the lacrimal caruncle is a rare entity. The authors report the management and outcomes of 3 cases of caruncle SCC. Case 1 underwent wide margin surgical excision with adjuvant topical chemotherapy for a poorly differentiated SCC. He later developed regional lymph node metastasis and required modified radical neck dissection. Case 2 underwent wide margin surgical excision with cryotherapy and adjuvant topical chemotherapy for an invasive moderately differentiated SCC. She later developed a recurrence and underwent orbital exenteration. Case 3 was a moderately differentiated SCC treated with wide margin excision alone and had no recurrence during 5-year follow up. Careful surveillance of caruncle SCC is required, given the observed propensity for local recurrence and/or regional metastasis.

Accuracy of Biopsy in Subtyping Periocular Basal Cell Carcinoma.

PURPOSE: To determine the accuracy of initial biopsy in the diagnosis of basal cell carcinoma (BCC) histologic subtype. METHODS: Retrospective histopathologic review of patients with a diagnosis of primary periocular BCC from 2006 to 2013 inclusive. RESULTS: A total of 174 primary BCCs were identified. BCCs were classified as nodular, superficial, or aggressive (including mixed cases with an aggressive component). Punch biopsies were used in 41% of cases, while the remaining patients underwent shave or incision biopsies. The final histologic subtypes at excision were nodular (59%), superficial (7%), nodular and superficial (7%), and aggressive (51%). The overall concordance between the BCC subtype identified in the biopsy specimen and the subsequent excision specimen was 54%. In total, there were 51 cases (29%) of BCC, which included aggressive subtypes, of which 52% of initial biopsies failed to detect an aggressive component. There were 45 cases (26%) of mixed BCC, and an aggressive histologic subtype was present in 73% of these cases. CONCLUSIONS: The accuracy of initial biopsy for BCC histologic subtype at excision is highest for nodular BCC. For aggressive BCC, biopsy was able to detect the aggressive component in only 48% of cases. This may have implications for choice of treatment modality.