Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated (neuroendocrine-type) MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as “EMPSGC” in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was ~21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

I ntraophthalmic artery chemotherapy (IAC) for treatment of retinoblastoma has become popular; it was used initially to salvage eyes with extensive intraocular retinoblastoma. 1 With the success of this treatment, radiotherapy or enucleation has been avoided in many eyes and patients have experienced minimal systemic toxic effects and acceptable local ophthalmic toxic effects. 2 With direct catheterization of the proximal portion of the ophthalmic artery, impressive tumor response was observed even in eyes with advanced retinoblastoma. 1,3 Initial enthusiasm for the use of IAC has been tempered by reports of ocular complications 4,5 and globe retention rates of 50% to 67%, 6 depending on many factors. One of the high-risk characteristics in retinoblastoma is the involvement of the anterior chamber by viable tumor. In most cases enucleation becomes necessary in this scenario, and if seeding is confirmed histopathologically, systemic adjuvant chemotherapy is added. In the present study we describe our experience with anterior segment tumor invasion in enucleated eyes that failed to respond to IAC. Methods A retrospective clinicopathologic review of enucleated eyes with retinoblastoma that had failed to respond to IAC was assembled from the pathology departments at University Col

Slit lamp biomicroscopy is sensitive for detecting anterior ESE. Most posterior ESE is microscopic, but macroscopic posterior ESE may also be missed by B-scan ocular ultrasound. Orbital surgeons should be suspicious of clinically undetected posterior ESE, and consider adjuvant orbital radiotherapy in cases with macroscopic ESE.

The aim of this study is to report a case of bilateral primary mucinous carcinoma of the eyelids. This is a case report and literature review. A 71 year-old-female presented with primary mucinous carcinoma of the left upper eyelid, which was excised with Mohs surgery. One year later she developed primary mucinous carcinoma of the right upper eyelid, which was also treated Mohs surgery. Extensive workup was negative for evidence of an unknown primary carcinoma or metastasis. Primary mucinous carcinoma of the eyelids may occur as multifocal tumors, and bilateral disease is not necessarily indicative of metastatic disease.

PurposeThe aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD).ObservationsA 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity. The IgG and IgG4 stained slides showed increased IgG4 positive plasma cells but did not meet the criteria for IgG4-related orbital disease. His IgG4 serology was elevated, and IgG4 staining of his tissue previously diagnosed as autoimmune sclerosing pancreatitis was found to meet the criteria for IgG4-RD.Conclusion and importanceAAPOX can be associated with systemic IgG4-RD.