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Clinical and histopathologic case of an eyelid eccrine poroma, a benign adnexal neoplasm rarely found on the periorbital skin.

Syringomas are benign adnexal neoplasms that may induce psychological stress when they are large or disfiguring or present in delicate regions such as the periorbital area. Despite the availability of various lasers for syringomas, no consensus has been established on the optimal laser setting and side effects of these therapies. The current review aims at understanding the efficacy and safety of various laser therapies available for the treatment of syringomas. A literature search was carried out using PubMed and Ovid databases for articles published from Jan 2000 through Mar 2022. Screening the eligible articles yielded 27 studies, comprising clinical studies, case series, and case reports, which were included in this review. The CO 2 laser is the most widely used ablative laser therapy but is usually associated with adverse events. Pinhole and multiple drilling methods using CO 2 laser yielded excellent cosmetic results with minimal adverse effects. Fractional lasers reduced the downtime and complications compared to non-fractionated ones. Non-ablative fractional lasers could be advantageous in terms of easy operation, minimal side effects and moderate recovery period compared with ablative lasers. Large clinical trials are needed to generate strong evidence to guide clinicians in choosing the most appropriate laser therapy for syringoma treatment.

Mantle cell lymphoma is a rare malignancy to present in the orbit, comprising only 1-5% adnexal lymphomas. Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is an equally uncommon adnexal tumor of sweat gland origin that may present on the eyelid. Herein we present a case of a 77-year old man with no previous cancer history who presented with painless progressive left globe proptosis and an enlarging left upper lid margin lesion, ultimately determined upon biopsy to be simultaneous orbital mantle cell lymphoma with systemic involvement and isolated eyelid EMPSGC. The pathogenesis, clinical manifestation, and management for each rare disease entity are reviewed and concept of collision tumors is discussed.

Mixed tumour of the skin is a rare entity also known as chondroid syringoma and pleomorphic adenoma. These usually present as slow-growing skin nodules with a smooth surface, clear boundaries, and no ulceration. Case series exist describing pleomorphic adenomas in the periocular region including the lids and orbit, separate to the more familiar lacrimal gland pleomorphic adenoma. These may arise from accessory or ectopic lacrimal gland tissue but in the eyelids are more likely to arise from sweat glands in the skin. Histopathological analysis of these lesions is important to identify complete excision, minimising recurrences and in identifying rare but potential malignant transformation. We describe the clinical features and outcomes in three cases of pleomorphic adenoma with two at the medial canthus (including one recurrence) and one in the brow region.

Background: Endocrine mucin-producing sweat gland carcinoma is a rare, under-reported cutaneous adnexal tumor that is often misdiagnosed and has an unknown incidence of metastasis., Objective: To determine the incidence of metastasis and tumor recurrence, as well as diagnostic accuracy and current trends in treatment modality., Methods: A search was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Tumor pathology and clinical data concerning demographics, presentation, diagnosis, treatment and follow-up were assessed., Results: A total of 36 publications with 110 cases were identified. Initial pathological diagnosis was incorrect in 45.5% of cases. One case of metastatic disease was reported. The incidence of locoregional recurrence was 10.6% over a mean follow-up period of 21.3 months. Of cases with known methods of resection, 34.6% were resected by excisional biopsy, 42.8% were resected by wide surgical excision, and 31.3% were cleared by Mohs micrographic surgery., Limitations: The low reported incidence and level of evidence was suboptimal with only case reports and retrospective case studies being reported., Conclusion: Reported cases of this pathology demonstrate poor diagnostic accuracy. High rates of misdiagnosis and inadequate definitive treatment suggest the need for more comprehensive work-up and management of lesions suspicious for this pathology.

Introduction: Primary Apocrine adenocarcinomas (PAA) are very infrequent tumors that are often confused initially with benign lesions. Little is known about this disease and there is still much to be clarified. We present a case of PAA on the eyelid successfully treated with surgery alone and a literature review regarding what is currently described about this disease., Methods: Noncomparative, retrospective case report of a patient with PAA on the eyelid succesfully treated with surgery alone and a literautre review., Results: A 91-year-old man with a 2 months lesion on the upper left eyelid was treated with surgery alone with oncological margins of 5mm. The Hystopathology diagnosis was a PAA of the eyelid and free margins were obtained. After 12 months of follow-up, the patient does not show any signs of local recurrence or distant metastasis. A review of the literature suggests these tumors are located more frequently in the axilla (50%) and secondly in the head and neck (35%), with similar distribution in the upper (41%) and lower eyelid (45%). The most commonly used treatment is surgical excision, but radiotherapy and chemotherapy have also been used with variable results., Conclusions: PAA is a very rare and aggressive tumor. Because it is so infrequent, treatments are based on the sporadic cases encountered in the literature. As more cases are reported, more can be elucidated about the characteristics of this tumor, its behavior and best treatment choice and this may allow progress in the understanding and management of this disease.

A major review of the literature of syringocystadenoma papilliferum's (SCAP's) presentation and management is presented. In addition, a case report of this unique diagnosis presenting as a corneal abrasion in a 66-year-old-male is included. This benign adnexal tumor of the apocrine glands is most commonly found in the face and neck. When found on the eyelids, these lesions are commonly misdiagnosed as basal cell carcinoma or cysts. Diagnosis is made based on histopathology. Treatment is complete excision of the lesion and this has a low recurrence rate. While predominantly benign, there have been cases of basal cell carcinoma development or other malignant transformations. Of the 26 reported cases of SCAP of the eyelids, none have caused a corneal abrasion. The authors present the only known presentation of eyelid SCAP, causing corneal abrasions, and provide a review of literature with discussion of clinical presentation, natural history, diagnosis, and treatment of this rare, eyelid lesion with potential for malignant transformation.

Hidrocystomas are benign, cystic lesions of eccrine and apocrine sweat glands. The literature on hidrocystomas is sparse, consisting of a handful of case reports and limited retrospective reviews. This is the first known bi-institutional, retrospective, chart review aimed to elucidate the demographics and basic clinical presentation of hidrocystomas. Medical records of adult patients with a pathological diagnosis of hidrocystoma from September 1, 2008 to August 1, 2015 in the Oculoplastic and Reconstructive Surgery Service at the Illinois Eye and Ear Infirmary (UIC) and Department of Ophthalmology of Weill Cornell Medical College (Cornell) were reviewed. Children under the age of 18 were not included. Data collection included: gender, race, age at diagnosis, laterality, location, total number of lesions, and recurrence. Results from both institutions were compared against each other and as a whole. A total of 107 patients were diagnosed with hidrocystoma on pathology. The mean age of diagnosis was 56 years (22-85). Hidrocystomas were diagnosed in 69 (64.4%) females and 38 (35.5%) males. Lesions were most commonly found in African American (37.4%), Caucasian (30.8%), and Hispanic (16.8%) patients combined across the two institutions with different patient populations. Lesions were largely unilateral (74.8%) and found on the lower lid (38.6%), lateral canthus (31.2%), upper lid (17.7%), and medial canthus (12.6%). Recurrences were seen in 2.3% of lesions. The majority of recurrences occurred in patients who identified their race as Hispanic (2/5) and Caucasian (3/5). Recurrences were seen in 2 males and 3 females. Apocrine and ecccrine hidrocystomas may be more common in female, African American, Caucasian, and Hispanic patients, presenting most commonly in adults in their mid-fifties. Lesions tend to be unilateral with lower lid lesions being the most prevalent location and medial canthus lesions being the least prevalent location for lesion growth. Recurrences may be most common in Hispanics and Caucasians and less common in African Americans. Although a precise recurrence rate cannot be determined at this time, our data suggests that the recurrence rate is low with current excisional methods.

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.

Introduction: Microcystic adnexal carcinoma (MAC) is a rare malignant cutaneous neoplasm presenting as a slow-growing, indurated nodule, papule or plaque. Clinically, the lesion can blend into the surrounding skin, obscuring borders and consequently delaying diagnosis histologically. Surgical and histologic techniques that emphasize examination of all margins may optimize management through early diagnosis and prevention of recurrences., Objective: This review aims to assess the current surgical and histology techniques that result in lower rates of tumor recurrence and, consequently, better clinical outcomes., Methods: A literature search of the PubMed database was conducted to identify studies examining wide local excision (WLE), Mohs micrographic surgery (MMS), radiotherapy (RT) and chemotherapy in the treatment of MAC., Results: WLE had a high likelihood of positive margins and local recurrence. MMS was found to have the lowest recurrence rates. Definitive RT could be considered for elderly patients or those who are poor surgical candidates, as large surgical defects may be required to obtain free margins with either WLE or MMS. Chemotherapy was found to be ineffective., Conclusion: Complete margin evaluation with MMS permits complete tumor removal with subsequently low recurrence rate.

Background: To report a rare case of eyelid eccrine porocarcinoma and compare this to previous documented cases in the literature., Main Observations: We report a case of an 86-year-old man who presented with three months' history of irritation in the right eye, who was found to have an irregular nodule on lower eyelid, which was later diagnosed as eccrine porocarcinoma (EPC). The lesion was excised and the defect repaired with Hughes flap. A computerized tomography (CT) scan of the head, neck, and chest showed no metastasis and no lymphadenopathy. There was no evidence of recurrence after 18 months of follow-up., Conclusion: To date, there have been only six cases of eyelid EPC reported in the literature. EPC has significant risk of recurrence and metastases after local excision. It is therefore important to consider it in the differential diagnosis of malignant eyelid tumour. A histological diagnosis should prompt wide margin excision, assessment of the patient for regional lymph node involvement, and imaging for metastatic disease.

Objective: To describe the clinical features, ancillary diagnostic studies, and treatment outcomes in a cohort of pediatric patients with giant orbital hydrocystomas., Design: Retrospective case series., Participants: Pediatric patients with giant orbital hydrocystomas treated in the practice of one surgeon (PDL)., Methods: A retrospective review of the clinical charts of pediatric patients with orbital hydrocystoma was performed and diagnostic information collected. Results were reviewed and compared with reported clinical data in the literature., Main Outcome Measures: Clinical presentation and histopathological findings of pediatric orbital hydrocystomas., Results: Three pediatric cases of giant orbital hydrocystoma were encountered, each with an unusual feature, including deep orbital location, occurrence following trauma, and eccrine pathology., Conclusion: Giant orbital hydrocystomas may present in the pediatric population. Ophthalmologists should be cognizant of this entity when evaluating a child with a large, cystic orbital mass.

Apocrine hidrocystomas are benign cystic lesions derived from the sweat glands of Moll and seldom found in the orbit. The authors present a case of a 41-year-old healthy man, with no prior medical history, referred for a painless enlarging mass, medial to his right upper eyelid for the past 3 months. Computed tomography showed a well-defined cystic lesion localized in the supero-medial anterior orbit. Following complete excision of the lesion, histopathology revealed an apocrine hidrocystoma. Although rare, apocrine hidrocystomas should be considered in the differential diagnosis for cystic mass of the orbit at any age group.

Apocrine hidrocystomas are cysts resulting from obstruction of the apocrine sweat gland ducts. They are usually solitary and seen in the head and neck areas. Apocrine hidrocystomas are rarely seen in the orbit with very few adult cases published in literature until now.

Purpose: Hidradenoma papilliferum (HP) is a cystic lesion of apocrine gland origin that occurs most commonly in the perineal region. Although there are scattered reports of HP involving the eyelid, to our knowledge, we present the first case of HP of the orbit., Methods: A 63 year-old female presented with progressive left upper eyelid fullness over an 18 month period., Results: The lesion was excised via transconjunctival anterior orbitotomy without incident., Conclusions: The pathologic features of HP are presented.

A 52-year-old man presented with a 5-year history of multiple bilateral apocrine hidrocystomas of the eyelids. For the past 3 years, the patient had developed a mechanical ectropion of the right inferior eyelid secondary to progressive enlargement of the lesions. Different therapeutic options were discussed with the patient. Surgical excision of all lesions was performed under local anesthesia. There was no recurrence after 15 months of follow-up.

Apocrine hidrocystomas are benign cysts of sweat duct origin, originating mainly from the apocrine secretory glands of Moll. They are typically encountered in the head and neck, particularly around the inner canthus of the eyelid. An intraorbital location of this lesion is extremely rare but should be considered in the differential diagnosis of a painless cystic lesion in the ocular adnexa at any age.

Purpose: To review the medical literature on mucinous sweat gland adenocarcinoma of the eyelid (MSA) and present two new cases., Methods: Details of published case reports and small series (between 1971-2010) were evaluated and summarized including two patients diagnosed and treated at our institution. Data regarding age, gender, ethnicity, precise location, clinical presentation, treatment and follow up of each patient were collected., Results: 25 reports describing 55 patients were found in the medical literature. The mean age was 61.3 years (30-87), 22(59%) were male and 28(80%) were Caucasian. In 23(44.2%) patients the lesion was in the lower lid, in 20(38.5%) in the upper lid, in 3(5.7%) involving both lids and in 6(11.6%) in a canthus. In 12(21.8%) patients a lesion with a benign diagnosis was previously excised from the same location. In 2 of them histological re-examination resulted in a diagnosis of MSA. Intraorbital involvement was found in 2(3.6%) patients. Regional lymph node metastasis was found in 2(4.4%) patients. Surgical excision was the treatment of choice using Mohs' micrographic-controlled excision technique in recent years. Radiotherapy was applied to 2 patients with clinical resolution in 1. Recurrence of the tumor was reported in 14(30%) patients., Conclusions: MSA is a rare tumor of the eyelid with no clinically distinguishing features. It should be suspected particularly with recurrent eyelid lesions and must be differentiated from metastatic disease. The tumor may extend into the orbit and metastasize regionally. Surgical removal with continued regular follow-up examination is the treatment of choice.

We describe a patient with a benign chondroid syringoma of the little finger of the right hand. She was a 56-year-old pianist who had had the swelling for 25 years without it causing any symptoms. The tumour was excised with an excellent result. Although chondroid syringoma is rare, it should be included in the differential diagnosis of the soft tumours of the hand.

Background: Apocrine hidrocystoma is a benign cystic tumour with apocrine differentiation. Although most cases are solitary tumours, multiple tumours may occur. Surgical removal is the usual treatment for apocrine hidrocystoma, but it may be troublesome and disfiguring in cases with multiple tumours., Objective: To determine the efficacy and cosmetic outcome of carbon dioxide laser vaporization in the treatment of multiple apocrine hidrocystomas., Patients and Methods: A total of 11 lesions in three adult patients were treated with carbon dioxide laser vaporization using a continuous and defocused mode, with a power density of 5 J/cm(2). The lesions were localized lateral to the outer canthus, on the free edge of the eyelids, and on the ear. Only a single session of treatment was performed for each lesion. Photographic controls were taken before and after treatment., Results: The lesions cleared after laser treatment without residual changes, and a successful cosmetic result was obtained., Conclusion: Carbon dioxide laser is an efficient method of the treatment for multiple apocrine hidrocystomas.