Eosinophilic angiocentric fibrosis and immunoglobulin 4-related disease revisited.

Eosinophilic angiocentric fibrosis (EAF) has been postulated to lie on the spectrum of IgG4-related disease (IgG4-RD) in a study reported in 2011. However, the aetiology of EAF remains controversial, despite increasing literature reporting IgG4-RD targeted investigations in those studies. This review aims to formally evaluate the relationship between EAF and IgG4-RD using the latest 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification scheme for IgG4-RD. A literature search was performed on the PubMed database for all studies describing patients diagnosed with EAF. Additionally, two EAF cases were retrospectively identified from our institution and presented. The demographics, clinical presentation, histological and serological data of each patient were analysed. A total of 20 cases were evaluated, including 18 patients from 14 articles in the English literature and two cases from our institution. Six of 20 (30%) patients with EAF met the criteria for IgG4-RD with the 2019 ACR/EULAR classification scheme. This represents the first attempt at formally evaluating the relationship between EAF and IgG4-RD using a validated set of classification criteria. Using the currently available data, a small proportion of patients with EAF met the criteria for IgG4-RD. However, various limitations of this study suggest that further efforts at disease characterization of EAF may be required.

Myopericytoma: A Review of Twenty-Three Cases Over Twelve Years and a Case Report of a Rare Neoplasm.

ABSTRACT: Myopericytoma is a rare soft tissue tumor with a predilection for the distal extremities. It is commonly found in the skin and subcutaneous tissues and usually takes a benign course. Current knowledge is limited to isolated case series and reports; hence, this study aims to report our tertiary institution's experience with this uncommon entity. A review of our institution's pathology records for cases of myopericytoma was performed. From January 2009 to September 2020, 23 cases of myopericytoma were identified and their clinicopathologic features were reported. A unique case of myopericytoma of the ankle from the series was also highlighted as a case report. Among the 22 cutaneous cases, 18 were in the extremities and 4 in the head and neck. One patient had an intracranial lesion. Most patients developed asymptomatic nodules (72.2%), but 1 patient had a locally aggressive tumor on presentation. None recurred despite marginal excision in some patients (80.0%). In conclusion, pathologists and surgeons who encounter this rare neoplasm can reassure patients of its benign tendency.

Dermoscopy in cutaneous sarcoidosis.

Cutaneous sarcoidosis has a wide variety of manifestations and can be challenging to diagnose clinically. Dermoscopy is a useful tool to support the clinical diagnosis. Herein, we report an elderly woman with pruritic facial plaques. Dermoscopy showed translucent orange globules with shiny white lines, and microscopic examination showed non-necrotizing granulomas with perigranulomatous fibrosis. Shiny white structures on dermoscopy are conventionally associated with basal cell carcinoma, melanoma, and dermatofibroma, and have not yet been reported in sarcoidosis. Current descriptions of dermoscopy findings of sarcoidosis in the literature are summarized. Further differential diagnostic entities for this presentation are described and treatment options for cutaneous sarcoidosis are discussed.

Detecting residents at risk of attrition - A Singapore pathology residency's experience.

The SingHealth Pathology Residency Program (SHPRP) is a 5-year postgraduate training program in Singapore. We face the problem of resident attrition, which has a significant impact on the individual, program and healthcare providers. Our residents are regularly evaluated, using in-house evaluations as well as assessments required in our partnership with the Accreditation Council for Graduate Medical Education International (ACGME-I). We hence sought to determine if these assessments were able to distinguish residents who would attrite from residents who would graduate successfully. Retrospective analysis of existing residency assessments was performed on all residents who have separated from SHPRP and compared with residents currently in senior residency or graduated from the program. Statistical analysis was performed on quantitative assessment methods of Resident In-Service Examination (RISE), 360-degree feedback, faculty assessment, Milestones and our own annual departmental mock examination. Word frequency analysis of narrative feedback from faculty assessment was used to generate themes. Since 2011, 10 out of 34 residents have separated from the program. RISE, Milestone data and the departmental mock examination showed statistical significance in discriminating residents at risk of attrition for specialty-related reasons from successful residents. Analysis of narrative feedback showed that successful residents performed better in areas of organization, preparation with clinical history, application of knowledge, interpersonal communication and achieving sustained progress. Existing assessment methods used in our pathology residency program are effective in detecting residents at risk of attrition. This also suggests applications in the way that we select, assess and teach residents.

Pazopanib elicits remarkable response in metastatic porocarcinoma: a functional precision medicine approach.

Metastatic porocarcinomas (PCs) are vanishingly rare, highly aggressive skin adnexal tumors with mortality rates exceeding 70%. Their rarity has precluded the understanding of their disease pathogenesis, let alone the conduct of clinical trials to evaluate treatment strategies. There are no effective agents for unresectable PCs. Here, we successfully demonstrate how functional precision medicine was implemented in the clinic for a metastatic PC with no known systemic treatment options. Comprehensive genomic profiling of the tumor specimen did not yield any actionable genomic aberrations. However, ex vivo drug testing predicted pazopanib efficacy, and indeed, administration of pazopanib elicited remarkable clinicoradiological response. Pazopanib and its class of drugs should be evaluated for efficacy in other cases of PC, and the rationale for efficacy should be determined when PC tumor models become available. A functional precision medicine approach could be useful to derive effective treatment options for rare cancers.

A Systematic Review and Meta-Analysis of Mapping Biopsy for Primary Extramammary Paget's Disease in Reducing Recurrence Following Surgical Excision.

Objective: To examine the association between the performance of mapping biopsies and surgical outcomes postexcision of extramammary Paget's disease (EMPD). Background: Primary EMPD is a rare entity associated with poorly defined surgical margins and difficult-to-access sites of lesions. Surgical resection with clear margins remains the preferred management method. The use of mapping biopsies might be beneficial, particularly in lowering disease recurrence. Methods: Available literature was reviewed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses methodology before a fixed-effect meta-analysis was performed to identify the presence of a correlation between performing mapping biopsies and positive margins on permanent sections as well as disease-free survival. Additional study results not included in the quantitative assessment were qualitatively assessed and reported. Results: A total of 12 studies were shortlisted for final analysis. 294 patients who underwent mapping biopsies and 48 patients who did not undergo mapping biopsies were included in the assessment. Forest plot analysis revealed a pooled rate ratio of 0.50 (95% CI, 0.32-0.77) in the prevalence of positive margins in patients with mapping biopsies performed as compared to patients without. The pooled rate ratio of the prevalence of disease-free survival in patients with mapping biopsies performed as compared to patients without was 1.38 (95% CI, 1.03-1.84). Qualitative assessment of the remaining selected studies revealed equivocal results. Conclusions: Mapping biopsies are able to improve EMPD surgical excision outcomes but given the rarity of the disease and heterogeneity of mapping biopsy procedures, further confirmation with randomized controlled trials or a larger patient pool is necessary.

Lichen planus pemphigoides after body tattooing.

Decorative tattooing is very popular worldwide and is associated with cutaneous complications, ranging from infections to localized or generalized skin reactions. We report a case of a patient presenting with generalized violaceous pruritic papular lesions 1 month after obtaining a black ink tattoo. Histological examination of a papular lesion distal to the tattoo site showed focal band-like lymphocytic infiltrate. He subsequently developed bullae over the papular lesions, with elevated serum BP180 antibody levels. A diagnosis of generalized lichen planus and lichen planus pemphigoides was made. He responded to treatment with potent topical corticosteroids and ciclosporin. We also reviewed the presentation and treatment of published cases of lichenoid reactions in the literature. With the increasing popularity of tattoos, awareness of this potential complication and possible treatments is important.

Biologically relevant laminin as chemically defined and fully human platform for human epidermal keratinocyte culture.

The current expansion of autologous human keratinocytes to resurface severe wound defects still relies on murine feeder layer and calf serum in the cell culture system. Through our characterization efforts of the human skin basement membrane and murine feeder layer 3T3-J2, we identified two biologically relevant recombinant laminins-LN-511 and LN-421- as potential candidates to replace the murine feeder. Herein, we report a completely xeno-free and defined culture system utilizing these laminins which enables robust expansion of adult human skin keratinocytes. We demonstrate that our laminin system is comparable to the 3T3-J2 co-culture system in terms of basal markers' profile, colony-forming efficiency and the ability to form normal stratified epidermal structure in both in vitro and in vivo models. These results show that the proposed system may not only provide safer keratinocyte use in the clinics, but also facilitate the broader use of other cultured human epithelial cells in regenerative medicine.

Diagnostic pitfall: Adenoid cystic carcinoma of the tongue presenting as an isolated hypoglossal nerve palsy, case report and literature review.

INTRODUCTION: We describe a patient with an unusual presentation of an isolated hypoglossal nerve palsy as a result of perineural invasion (PI) from adenoid cystic carcinoma (ACC). We will also discuss the diagnostic pitfalls as well as present a short literature review of adenoid cystic carcinomas and suggest improvement to the current diagnostic algorithm for isolated hypoglossal nerve palsies. PRESENTATION OF CASE: A 63year old Malay female presented with progressive dysphagia and slurred speech for one year. Physical examination showed unilateral right tongue wasting, fasciculation and deviation to the right. An MRI showed atrophy of the tongue due to denervation and subsequently she was treated in a neurology clinic for 8 months. Due to lack of improvement, she was referred to our surgical unit and underwent examination under anaesthesia (EUA) and biopsy. Histology showed adenoid cystic carcinoma with perineural involvement, resulting in lower motor neuron signs. She underwent radiotherapy to the base of her tongue (70Gy/35#). A PET-CT one month post treatment showed complete response. DISCUSSION: Adenoid cystic carcinoma (ACC) is a salivary gland neoplasm. It is characterized by local invasiveness with frequent recurrence and indolent growth. It affects major salivary glands more than minor salivary glands. In malignancies that have a propensity for PI such as ACC, patients may present atypically with nerve palsies. In infiltrative lesions, the primary tumour may not be evident on magnetic resonance imaging. Therefore, to achieve a diagnosis, a high index of suspicion is required. When the diagnosis is in question, deep biopsy and positron emission tomography may be useful.