Sebaceous carcinoma: evidence-based clinical practice guidelines.

Sebaceous carcinoma usually occurs in adults older than 60 years, on the eyelid, head and neck, and trunk. In this Review, we present clinical care recommendations for sebaceous carcinoma, which were developed as a result of an expert panel evaluation of the findings of a systematic review. Key conclusions were drawn and recommendations made for diagnosis, first-line treatment, radiotherapy, and post-treatment care. For diagnosis, we concluded that deep biopsy is often required; furthermore, differential diagnoses that mimic the condition can be excluded with special histological stains. For treatment, the recommended first-line therapy is surgical removal, followed by margin assessment of the peripheral and deep tissue edges; conjunctival mapping biopsies can facilitate surgical planning. Radiotherapy can be considered for cases with nerve or lymph node involvement, and as the primary treatment in patients who are ineligible for surgery. Post-treatment clinical examination should occur every 6 months for at least 3 years. No specific systemic therapies for advanced disease can be recommended, but targeted therapies and immunotherapies are being developed.

Case 2-2016. An 84-Year-Old Woman with Chest Pain, Dyspnea, and a Rash.

An 84-year-old woman was admitted to the hospital because of chest pain, respiratory distress, and a purpuric rash. Initial chest imaging showed bilateral patchy and confluent opacities, a finding consistent with pulmonary edema. A diagnostic procedure was performed.

Perforating Gout: Expanding the Differential for Transepidermal Elimination.

Perforating dermatoses are dermatologic disorders with transepidermal elimination (TE) of dermal substances. While TE is typically associated with collagen and elastin, it can also occur as a secondary event in other processes, and it is important to keep a broad differential. We present a case of perforating tophaceous gout, which underscores the need for a thoughtful approach to perforating disorders. An updated review of recent literature is also presented.

Cardiac amyloidosis: what are the indications for transplant?

PURPOSE OF REVIEW: We review the clinical and pathological features of the various types of amyloid that involve the heart, the diagnostic utility of endomyocardial biopsy, and the experience of patient survival and disease recurrence following cardiac transplant for amyloidosis. RECENT FINDINGS: Patient outcome is dependent on arresting the cause of amyloid as well as controlling the accumulated damage, which may involve multiple organs. As such, the stratification of patient suitability for cardiac transplant must be considered in conjunction with concurrent treatments, which may include autologous stem cell, liver or kidney transplant, and chemotherapy. SUMMARY: As the efficacy of these therapies changes, the indications for cardiac transplantation need to be re-evaluated.

Cardiac Amyloid - A Hidden Contributor to Cardiac Dysfunction Following Cardiac Surgery: Case Report and Literature Review.

We present two patients who underwent cardiac surgery followed by post-operative low cardiac output, diastolic dysfunction and resistance to inotropic support. Despite aggressive medical management, both patients died. At autopsy, the hearts were enlarged and showed previously undiagnosed myocardial and vascular amyloidosis. Occult cardiac amyloidosis is an uncommon, often occult, contributor to post-operative complications post cardiac surgery. Pre-operative or intraoperative myocardial biopsy may be useful in patients with unexplained diastolic dysfunction. Brief Summary: We present two patients who underwent cardiac surgery followed by low cardiac output, diastolic dysfunction and resistance to inotropic support. Cardiac dysfunction was due to occult amyloidosis. Pre-operative or intra-operative myocardial biopsy may be useful in patients with unexplained diastolic dysfunction. With recent therapy advances, classification and possible treatment of amyloid are possible.

Predicting OncoDX recurrence scores with immunohistochemical markers: effect of stromelysin.

Recent reports suggest that immunohistochemistry (IHC) markers can be used to give prognostic information in breast cancer that is similar to that contained in the Genomic Health Inc. OncoDX recurrence score (Onco-RS). Our own previous work confirmed that an IHC model based on estrogen receptor (ER), progesterone receptor (PR), and Ki67 predicts 62% of the Onco-RS variability. Other markers used in the Onco-RS include proteins thought to increase tumoral invasive potential, and one such marker is matrix metalloproteinase-11, also called stromelysin 3 (ST3). The goal of this study is to examine the additional value of including ST3 in an IHC-based model that also includes ER, PR, and Ki67 in predicting the Onco-RS, as compared with an IHC model based only on ER, PR, and Ki67 (IHC-RS). The patient population consists of a retrospectively identified cohort of 91 women with ER-positive, HER2neu-negative breast cancer who completed OncoDX testing. Using stepwise multiple regression incorporating Ki67 percentage and semiquantitative ER, PR, and ST3 scores, the ST3 score was not statistically significant.

Conjunctival inverted squamous papilloma: a case report with immunohistochemical analysis and review of the literature.

A 63-year-old man presented with an asymptomatic papillary, sessile lesion of the juxtalimbal bulbar conjunctiva that was surgically excised with cryotherapy. Histopathologically, the lesion created some diagnostic confusion as it displayed an endophytic, or inverted, growth pattern-with squamous cells pushing into the substantia propria around fibrovascular cores, but without significant cytologic atypia, consistent with a conjunctival inverted papilloma (IP). Unlike previously reported cases of conjunctival IP, there were no goblet cells or cysts within the tumor. Immunostaining was diffusely positive for cytokeratin (CK) 7, and CK14 stained the basilar and suprabasilar cells, as in normal conjunctiva. CK17 weakly and non-uniformly stained the tumor, ruling out a dysplasia, which is usually strongly positive. The lesion's cytokeratin profile therefore paralleled that of normal conjunctiva. The proliferation index with Ki67 nuclear staining was extremely low (<1%), as was p53 nuclear staining (10-20%), both in contrast to squamous cell dysplasias or carcinomas that have a much higher percentage of positive cells. The lesion was negative for human papillomavirus subtypes associated with squamous neoplasias including carcinomas. We review the previous literature devoted to this comparatively rare condition and contrast its benign clinical course with that of inverted papillomas of the sinonasal, lacrimal drainage, and genitourinary systems and provide a set of criteria for establishing the diagnosis.

Predicting OncoDx recurrence scores with immunohistochemical markers.

Recent reports suggest that immunohistochemistry (IHC) markers can be used to give prognostic information in breast cancer that is similar to that contained in the Genomic Health Inc. OncoDx recurrence score (Onco-RS). The goal of this study is to examine the potential prognostic value of a score derived from results of a simple set of IHC tests in the prediction of the Onco-RS. A score (IHC-RS) was derived to predict the Onco-RS using IHC-based quantitative and semiquantitative results from a subset of markers selected from those used in the generation of an Onco-RS score. The patient population consists of a retrospectively identified cohort of 158 women with ER-positive, HER2neu-negative breast cancer who completed OncoDx testing. A predictive model was developed to generate the IHC-RS using stepwise multiple regression incorporating Ki67 percentage and semiquantitative ER and PR scores. Using only these 3 IHC markers, the IHC-RS predicted 62% of the Onco-RS variability (adjusted R=0.624, P=0.004). In addition, analysis of outliers in the correlation between the IHC-RS and the Onco-RS reveals the possibility of sampling error as a drawback of the Onco-RS. This is contrasted against potential interlab and intralab variability and preanalytic issues that may negatively impact the implementation of an IHC-RS.

Left ventricular mesenchymal hamartoma, a new hamartoma of the heart.

Primary neoplasms of the heart are rare. Benign entities in adult populations include myxoma, fibroma, lipoma, hamartoma of mature myocytes, and vascular hamartoma. The most common pediatric cardiac neoplasm is the rhabdomyoma. We report a 22-year-old female with a primary left ventricle tumor composed of multiple mature mesenchymal tissues, including mature cardiac myocytes, smooth muscle, fibroblasts, fat, blood vessels including a hemangioma like area, and nerve fibers. The various elements were disorganized, but well differentiated, and showed little mitotic activity, which are features suggestive of a hamartoma. Unlike a cardiac fibroma, which may entrap myocardium at the periphery of the lesion, the present case demonstrated all tissue elements throughout the tumor mass. We suggest that this lesion is sufficiently different from those hamartomas previously described to warrant a new designation, for which we propose the title cardiac mesenchymal hamartoma.

A practical method to rapidly dissolve metallic stents.

Metallic stents are commonly used in many clinical applications including peripheral vascular disease intervention, biliary obstruction, endovascular repair of aneurysms, and percutaneous coronary interventions. In the examination of vascular stent placement, it is important to determine if the stent is open or has become obstructed. This is increasingly important in the era of drug-eluting stent usage in coronary arteries. We describe a practical, rapid and cost-effective method to dissolve most metallic stents leaving the vascular and luminal tissues intact. This practical method may replace the laborious and expensive plastic embedding methods currently utilized.