SMARCA4-deficient central nervous system metastases: A case series and systematic review.

SMARCA4 alterations can be encountered in a variety of human neoplasms, and metastases to the central nervous system (CNS) are rare, offering a challenge to neuropathologists despite not representing a distinct diagnostic entity. To better understand the clinical and histologic presentation of such neoplasms, we report an observational case series and systematic review of 178 unique articles that yielded 15 published cases and 7 cases from institutional files. In the systematic review, the median age was 58 years, the male-to-female ratio was 2:1, and the most common diagnosis was lung adenocarcinoma; all CNS metastases were discovered within 1 year of presentation. In the case series, the median age was 58 years, the male-to-female ratio was 6:1, and all known metastases originated from the lung. Most patients had a smoking history and died of disease. GATA-3 positivity was seen in most case series examples. Concurrent TP53 mutations (83.3%) and a high tumor mutation rate (60%) were common. To our knowledge, this is the only case series and systematic review in the English literature aimed at assessing SMARCA4-altered metastases in the CNS and vertebral column. We highlight the challenges of neuropathologic evaluation of such tumors and provide observational evidence of early metastases, histologic appearances, and immunohistochemical findings, including previously unreported GATA-3 positivity.

Expression of TLE1 in a Carcinoid Tumor of the Lung With Spindle Cell Morphology-A Potential Diagnostic Pitfall.

Tumors of the lung with a spindle cell morphology require consideration of many entities in the differential diagnosis, including metastases. Ancillary immunohistochemical stains but also molecular studies are typically required to arrive at the proper diagnosis. We present a case of a 71-year-old woman with multiple lung nodules, mediastinal lymphadenopathy, and a history of uterine cancer who underwent endobronchial ultrasound-guided fine needle aspiration and biopsy of the lung and mediastinal lymph nodes. A sampling of the lung lesion showed a cytologically bland neoplasm with spindle cell morphology, lacking necrosis or brisk mitotic activity. In conjunction with the cytomorphology, strong and diffuse Transducin-like enhancer of split 1 (TLE1) reactivity in the tumor cells initially raised the diagnosis of synovial sarcoma; however, subsequent results of additional testing showed strong and diffuse expression with AE1/AE3, CK 8/18, TTF-1, synaptophysin and chromogranin and focal or negative staining with a large number of other antibodies. This warranted a diagnosis of a carcinoid tumor. This is the first report of TLE1 staining in a carcinoid tumor of the lung. Therefore, when evaluating tumors of the lung with spindle cell morphology in which the differential diagnosis may include both carcinoid tumor and synovial sarcoma, TLE1 expression should be interpreted with caution and in conjunction with an expanded immunohistochemical staining panel.

Fibroma-like PEComa: a newly recognized soft tissue neoplasm in tuberous sclerosis patients-imaging features and review of literature.

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms composed of spindled to epithelioid cells that co-express both melanocytic and myogenic markers. Recently, in 2018, a distinctive variant of PEComa has been described that arises in association with tuberous sclerosis complex (TSC) and resembles a fibroma by conventional morphology (called fibroma-like PEComa). Herein, we describe a case of a fibroma-like PEComa in a 4-year-old male child with a known diagnosis of tuberous sclerosis who presented with a firm mass along the anteromedial aspect of the right knee. The mass was excised, and microscopic examination showed bland spindled to stellate cells embedded in a dense collagenous stroma, morphologically resembling a fibroma. Immunohistochemistry analysis showed positivity for desmin (a myogenic marker) and HMB45 (a melanocytic marker), a hallmark for PEComas. To our knowledge, only six cases of fibroma-like PEComa have been described in the literature so far and this is the first report of such a tumor in the medial retinaculum of the knee joint with illustrations of conventional and diffusion imaging features. This case highlights the unique association of fibroma-like PEComa lesions with TSC. This should be considered a differential diagnosis for T2 hypointense masses in tuberous sclerosis patients. In addition, a diagnosis of fibroma-like PEComa should prompt further evaluation for associated TSC.

Primary cutaneous epithelioid mesenchymal neoplasm with ACTB-GLI1 fusion: a case report.

Mesenchymal tumors harboring GLI1 gene abnormalities are a rare but distinctive group of neoplasms whose clinicopathologic features are currently evolving. In particular, examples of this tumor with ACTB-GLI1 gene fusion, tentatively termed ACTB-GLI1 epithelioid mesenchymal neoplasm (EMN), show a distinctive monomorphic round-to-epithelioid morphology, nested to trabecular pattern of growth, and S100+/SOX10-/SMA- immunophenotype. We report the first case of this entity arising exclusively in the skin. A 69-year-old man with no prior history of neoplasia presented with a 1.5-cm raised lesion on the left buttock. Histopathologic examination revealed a diffuse dermal proliferation of small, monomorphic, round-to-ovoid cells with hyperchromatic nuclei, focally enlarged nucleoli, and minimal eosinophilic to clear-staining cytoplasm. These cells were arranged in confluent nests and trabeculae in a background of fibrocollagenous to focally myxoid stroma. Immunohistochemical analysis revealed strong positivity for S100 and CD56, and negativity for SOX-10, SMA, Melan-A, HMB-45, and a variety of other markers. Based on the morphology and immunophenotype, molecular studies were performed, which revealed the presence of an ACTB-GLI1 fusion transcript, confirming the diagnosis. Given the morphologic overlap of this tumor with other cutaneous round cell neoplasms and its potential for malignant behavior, ACTB-GLI1 EMN is an important entity for pathologists to recognize.

Cutaneous pleomorphic fibromas arising in patients with germline TP53 mutations.

Pleomorphic fibromas are rare benign cutaneous neoplasms associated with deletion/loss of chromosomes 13q and 17p, where RB1 and TP53 are located, respectively. Herein, we report five cases of pleomorphic fibroma arising in patients with germline TP53 mutations, suggesting a potential link with Li-Fraumeni syndrome. All three patients were female and young (mean age 27) with a strong personal and/or family oncologic history and confirmed pathogenic germline TP53 mutations. In two patients, multiple pleomorphic fibromas were diagnosed. Clinically, the lesions arose at various cutaneous sites and were small (≤2 cm) and raised (4/5). Histopathologically, the tumors were paucicellular, composed of atypical spindled to stellate cells with hyperchromatic and variably pleomorphic nuclei. Mitotic activity was exceedingly low, although rare atypical mitotic figures were seen in one case. Immunohistochemically, the tumor cells were diffusely positive for p16 (3/3) and showed loss of Rb expression (5/5). All cases showed aberrant p53 expression (overexpression in 4, complete loss in 1). The tumors have followed a benign clinical course with no evidence of progression or recurrence. In conclusion, the development of multiple pleomorphic fibromas in a young patient may be a clue to an underlying genetic cancer syndrome involving TP53.

Internet Pornography Use, Perceived Addiction, and Religious/Spiritual Struggles.

Prior work has demonstrated that religious beliefs and moral attitudes are often related to sexual functioning. The present work sought to examine another possibility: Do sexual attitudes and behaviors have a relationship with religious and spiritual functioning? More specifically, do pornography use and perceived addiction to Internet pornography predict the experience of religious and spiritual struggle? It was expected that feelings of perceived addiction to Internet pornography would indeed predict such struggles, both cross-sectionally and over time, but that actual pornography use would not. To test these ideas, two studies were conducted using a sample of undergraduate students (N = 1519) and a sample of adult Internet users in the U.S. (N = 713). Cross-sectional analyses in both samples found that elements of perceived addiction were related to the experience of religious and spiritual struggle. Additionally, longitudinal analyses over a 1-year time span with a subset of undergraduates (N = 156) and a subset of adult web users (N = 366) revealed that perceived addiction to Internet pornography predicted unique variance in struggle over time, even when baseline levels of struggle and other related variables were held constant. Collectively, these findings identify perceived addiction to Internet pornography as a reliable predictor of religious and spiritual struggle.

How Long Is Too Long? Trials and Tribulations of an Indolent Tumor.

A 31-year-old African American man presented for workup of a right hilar and tracheal mass. Stability of the mass when compared with a computed tomographic scan performed 3 years prior suggested an indolent process. On bronchoscopy, there were 2 separate although morphologically similar endobronchial lesions, one in the distal trachea and the second at the level of the right upper lobe bronchus. Biopsies of both lesions demonstrated granular cell tumors. Subsequent rigid bronchoscopy with ablation led to resolution of wheeze, decrease in dyspnea, and documented improvements in both ventilation and perfusion to the right lung. This case illustrates both a rare disease (multifocal endobronchial granular cell tumor) and the physiological impact of reducing large airway obstruction.

Fine-needle aspiration cytology of epithelioid malignant peripheral nerve sheath tumor: A case report and review of the literature.

The epithelioid variant of malignant peripheral nerve sheath tumor (eMPNST) is an extremely rare soft tissue neoplasm comprising less than 5% of all MPNSTs. It is distinguished cytomorphologically from a conventional MPNST by the presence of polymorphous round epithelioid cells arranged in loose clusters with or without spindled tumor cells. These features pose a diagnostic challenge because the differential diagnosis involves a variety of mesenchymal and non-mesenchymal tumors including epithelioid sarcoma, sclerosing epithelioid fibrosarcoma, malignant rhabdoid tumor, chordoma, metastatic carcinomas, and melanoma. Thus, it may become imperative to perform immunochemical stains on cell blocks of FNA aspirates to arrive at definitive diagnosis. Reports describing the cytologic features of eMPNST are rare. Herein, we report a case of eMPNST with focus on cytomorphologic and cytoimmunochemical features and differential diagnosis.

Myxoid mesenchymal neoplasm presenting as massive arm and chest wall oedema with pleural effusion.

Myxoid mesenchymal tumours are a heterogeneous group of neoplasms characterised histologically by their abundant mucoid and myxoid extracellular matrix (ECM). Encompassing a broad spectrum of clinical behaviour ranging from benign to malignant, there are more than 60 reactive and neoplastic entities currently classified under its domain. Its varied clinical and histopathologic features continue to pose a diagnostic challenge to clinicians and pathologists. Here, we describe a rare case of myxoid mesenchymal tumour presenting as oedema of the upper extremity with pleural metastasis and partial response to chemotherapy, which to the best of our knowledge has not yet been described in the literature.

Perceived addiction to Internet pornography and psychological distress: Examining relationships concurrently and over time.

In the United States, Internet pornography use is a common behavior that has risen in popularity in recent years. The present study sought to examine potential relationships between pornography use and well-being, with a particular focus on individual perceptions of pornography use and feelings of addiction. Using a large cross-sectional sample of adults (N = 713), perceived addiction to Internet pornography predicted psychological distress above and beyond pornography use itself and other relevant variables (e.g., socially desirable responding, neuroticism). This model was replicated using a large cross-sectional sample of undergraduates (N = 1,215). Furthermore, a 1-year, longitudinal follow-up with a subset of this sample (N = 106) revealed a relationship between perceived addiction to Internet pornography and psychological distress over time, even when controlling for baseline psychological distress and pornography use. Collectively, these findings suggest that perceived addiction to Internet pornography, but not pornography use itself, is uniquely related to the experience of psychological distress.

Diffuse infiltrative neurofibroma: a clinical, radiological, and histological conundrum.

Diffuse infiltrative neurofibroma is a rare clinical entity that can pose a diagnostic challenge not only due to its rarity but also due to its varied clinical, radiological, and histological features. Our case illustrates how this entity may be misdiagnosed on clinical and pathological examination. Radiological imaging plays a critical and collaborative role in guiding clinicians and pathologists when faced with this challenging diagnosis.

Leiomyosarcoma of the urinary bladder: a clinicopathological study of 34 cases.

BACKGROUND: Leiomyosarcomas of the urinary bladder (LMS-UB) are rare, usually aggressive neoplasms. Owing to their rarity, only a limited number of cases with clinical follow-up information have been published. There is no current consensus on LMS-UB grading, and it is unknown whether the widely accepted Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) and National Cancer Institute (NCI) grading systems of soft-tissue sarcomas are applicable to LMS-UB. METHODS: The authors studied 34 well-characterised LMS-UB and compared the prognostic power of the FNCLCC and NCI systems with that of one published grading scheme for LMS-UB (Mayo). All available slides from 34 LMS-UB were retrieved and evaluated with regards to degree of differentiation, mitotic rate/10 high-powered fields (HPF), and % necrosis. Cases were graded using published criteria for the FNCLCC, NCI and Mayo schemes. Follow-up information was obtained. RESULTS: The tumours occurred in 17 females and 17 males, ranging from 31 to 91 years (median 65), and measured 2-12 cm in size. One tumour was well differentiated, 17 tumours were moderately differentiated, and 16 tumours were poorly differentiated. Mitotic rates ranged from 1 to >30/10 HPF (median 12/10 HPF), and tumours showed 0-60% necrosis (median 25%). FNCLCC grades were 1 (3), 2 (12) and 3 (19). NCI grades were 1 (2), 2 (11) and 3 (21). Mayo grades were low (7) and high (27). FNCLCC and NCI grades were identical in 23/34 cases (68%). Four cases were FNCLCC/NCI grade 2 or 3 and Mayo low-grade. Clinical follow-up was available for 25 of 34 patients (74%). Clinical follow-up of > or =12 months was available for 17 of these 25 cases (68%) with a median follow-up duration of 52 months (range 12-120 months). Adverse outcome was seen in nine of these 17 patients (53%). Seven of the eight cases (88%) with a clinical follow-up duration of <12 months died of their disease. Overall, adverse outcome was documented in 16 of 25 (64%) cases. Metastatic disease was seen in 13 of 25 (52%) cases, with the lungs being the most common site of metastasis (62%). Adverse outcome was noted in 15 of 23 (65%) of FNCLCC grade 2 or 3 LMS-UB, as compared with zero of two (0%) FNCLCC grade 1 tumours (p=0.15), in 15 of 23 (65%) NCI grade 2 or 3 LMS-UB, versus zero of two (0%) NCI grade 1 sarcomas (p=0.17) and in 13 of 20 (65%) Mayo high grade LMS-UB, as opposed to two of five (40%) low-grade lesions (all results not statistically significant). CONCLUSIONS: The authors conclude that LMS-UB occurs in older adults of either sex and is characterised by aggressive behaviour, with adverse outcome in >60% of cases. Certain advantages of the FNCLCC system may support its more widespread adoption for future studies.

Feeling validated versus being correct: a meta-analysis of selective exposure to information.

A meta-analysis assessed whether exposure to information is guided by defense or accuracy motives. The studies examined information preferences in relation to attitudes, beliefs, and behaviors in situations that provided choices between congenial information, which supported participants' pre-existing attitudes, beliefs, or behaviors, and uncongenial information, which challenged these tendencies. Analyses indicated a moderate preference for congenial over uncongenial information (d=0.36). As predicted, this congeniality bias was moderated by variables that affect the strength of participants' defense motivation and accuracy motivation. In support of the importance of defense motivation, the congeniality bias was weaker when participants' attitudes, beliefs, or behaviors were supported prior to information selection; when participants' attitudes, beliefs, or behaviors were not relevant to their values or not held with conviction; when the available information was low in quality; when participants' closed-mindedness was low; and when their confidence in the attitude, belief, or behavior was high. In support of the importance of accuracy motivation, an uncongeniality bias emerged when uncongenial information was relevant to accomplishing a current goal.

The deliberation-without-attention effect: evidence for an artifactual interpretation.

Proponents of unconscious-thought theory assert that letting the unconscious "mull it over" can enhance decisions. In a series of recent studies, researchers demonstrated that participants whose attention was focused on solving a complex problem (i.e., those using conscious thought) made poorer choices, decisions, and judgments than participants whose attention was distracted from the problem (i.e., those purportedly using unconscious thought). We argue that this finding, rather than establishing the existence of a deliberation-without-attention effect, is explained more compellingly in terms of the well-established distinction between on-line and memory-based judgments. In Experiment 1, we reversed the recent finding by simply changing participants' on-line processing goal from impression formation to memorization. Experiment 2 provided a replication and further established that some cognitive effort appears necessary to produce both the original pattern of results and its reversal, suggesting that such judgments are ultimately a product of conscious, rather than unconscious, thinking.

Videotaped interrogations and confessions: does a dual-camera approach yield unbiased and accurate evaluations?

Although an equal-focus camera perspective-suspect and interrogator each displayed in profile-produces relatively unbiased assessments of videotaped interrogations-confessions, many in law enforcement may consider it less than satisfactory because a full-face view of the suspect is precluded and thus potentially important information revealed in his or her expressions may be unavailable for fact finders' consideration. The present research investigated whether a dual-camera approach, wherein the full faces of both the suspect and interrogator are presented in a split-screen format simultaneously, is a viable alternative to an equal-focus format. Experiment 1 in fact demonstrated that the dual-camera approach does produce relatively unbiased assessments of voluntariness and guilt. However, Experiment 2 revealed that the dual-camera approach was one of the poorest presentation formats in terms of its impact on the ability to accurately distinguish between true and false confessions. Policy recommendations based on the present and previous research are presented.

Alcohol, wine, and cardiovascular health.

Studies evaluating the health benefits of alcohol and wine have demonstrated that moderate consumption is associated with a decrease in all-cause and cardiovascular mortality. Various populations and alcoholic beverages exhibit this effect to different degrees. Alcoholic beverages exhibit multiple mechanisms that may favorably influence cardiac risk potential actions on platelets, antioxidants, fibrinolysis, and lipids. However, other data suggest that the perceived benefit of alcoholic beverages in general, and wine in particular, are the result of socioeconomic confounders. In the absence of more rigorous evidence, it is not currently possible to define the role of wine in human health.

Implications of counterfactual structure for creative generation and analytical problem solving.

In the present research, the authors hypothesized that additive counterfactual thinking mind-sets, activated by adding new antecedent elements to reconstruct reality, promote an expansive processing style that broadens conceptual attention and facilitates performance on creative generation tasks, whereas subtractive counter-factual thinking mind-sets, activated by removing antecedent elements to reconstruct reality, promote a relational processing style that enhances tendencies to consider relationships and associations and facilitates performance on analytical problem-solving tasks. A reanalysis of a published data set suggested that the counterfactual mind-set primes previously used in the literature tend to evoke subtractive counterfactuals. Studies 1 and 2 then demonstrated that subtractive counterfactual mind-sets enhanced performance on analytical problem-solving tasks relative to additive counterfactual mind-sets, whereas Studies 3 and 4 found that additive counterfactual mind-sets enhanced performance on creative generation tasks relative to subtractive counterfactual mind-sets.