Teratocarcinosarcoma is a rare, highly aggressive malignancy of the head and neck, characterized by multiphenotypic and triphasic growth of epithelial, mesenchymal, and primitive neuroepithelial elements. Owing to its rarity and morphological heterogeneity, as well as the lack of experience with this neoplasm, teratocarcinosarcoma is often misdiagnosed, particularly in small biopsy samples when only some of the elements are identified, thus leading to delayed management. Aggressive clinical behavior and poor survival outcomes, necessitate an accurate diagnosis and appropriate treatment. This review describes the main demographic and clinicopathological features of teratocarcinosarcoma, with an emphasis on the recent advances that have attempted to identify the molecular signature of this neoplasm.

The relationship between PLIN2 expression and prognosis, and clinicopathological significance of various cancers has been extensively studied, but the results are not completely consistent. This review followed the guidelines for systematic reviews of prognostic factors studies and was reported under the Preferred Reporting Program for Systematic Reviews and Meta-Analysis (PRISMA). We searched PubMed, Embase, Cochrane Library, Web of Science, and Google Academia for relevant articles up to September 2, 2022, and calculated the pooled hazard ratios (HR) with 95% confidence intervals (CI) to determine the association between PLIN2 expression and the prognosis of various cancers. The meta-analysis ultimately included 17 studies. The quality of all included cohort studies was evaluated using the Quality in Prognosis Studies (QUIPS) tool, and an adaptation of Grading of Recommendations Assessment, Development and Evaluation (GRADE) method was used to assess the certainty of the results. High expression of PLIN2 was associated with poorer overall survival (HR  =  1.65; 95% CI  =  1.14, 2.38; P  =  0.008), metastasis-free survival (HR  =  1.48; 95% CI  =  1.12, 1.94; P  =  0.005), progression-free survival (HR  =  2.11; 95% CI  =  1.55, 2.87; P < 0.0005) and recurrence-free survival/relapse-free survival (HR  =  2.21; 95% CI  =  1.64, 2.98; P < 0.0005) in cancers. The clinicopathological parameters of digestive system malignancies suggested that high expression of PLIN2 was notably associated with distant metastasis ( + ) (odds ratio (OR)  =   3.37; 95% CI  =  1.31, 8.67; P  =  0.012), lymph node metastasis ( + ) (OR  =  1.61; 95% CI  =  1.01, 2.54; P  =  0.004), and tumor stage (III-IV) (OR  =   1.96; 95% CI  =   1.24, 3.09; P  =  0.006). In summary, overexpression of PLIN2 is significantly associated with a poor prognosis in various human cancers, especially in respiratory and digestive malignancies. Thus, PLIN2 expression may be a potential prognostic biomarker in cancer patients.

Carcinoma cuniculatum (CC) is a rare variant of squamous cell carcinoma (SCC) that is characterized by minimal cytologic atypia and a unique deeply infiltrative growth pattern resembling rabbit burrows (cuniculi). With less than 75 cases reported in the head and neck, the clinical and pathologic spectrum of this entity remains poorly understood. A retrospective review of the clinical and pathologic features of archival cases of oral CC was performed. A total of six cases of oral CC were identified. Age ranged from 25-77 years; the male-to-female ratio was 5:1. All patients had a long-standing history of tobacco and betel-quid consumption. The tumors were distributed in the gingivobuccal sulcus (n = 2), the tongue (n = 2), buccal mucosa (n = 1), and the palate (n = 1). Histology in all cases typically revealed a tumor composed of well-differentiated squamous epithelium, devoid of atypia, lining deeply infiltrative, large-sized, branching, keratin-filled cavities, resembling rabbit-burrows. Dense lymphocytic infiltrates and discharging micro-abscesses were regular features. Underlying bone invasion and lymph node metastasis were observed in 1 patient. One patient with a tongue tumor developed locoregional recurrence at 10 months while none developed distant metastasis. Oral CC is a rare and under-recognized variant of SCC with locally aggressive behavior. Lack of familiarity with this variant exacerbated by the absence of cytologic anaplasia makes CC susceptible to multiple negative biopsies and erroneous diagnoses. Awareness of this clinicopathologic entity is essential to allow its accurate diagnosis and optimal management.

OBJECTIVE: To systematically and meta-analytically pool the existing evidence regarding the prognostic impact of preoperative anemia (hemoglobin level <12 mg/dl) in patients with endometrial cancer.
METHODS: Four (PubMed, Embase, Scopus and Web of Science) databases were searched from inception to 20-August-2020. We assessed the risk of bias using the Newcastle-Ottawa Scale. We estimated the pooled prevalence of preoperative anemia in the included studies. We pooled odds ratios (ORs) and hazard ratios (HRs) with their 95 % confidence intervals (95 % CIs) to evaluate the correlation between preoperative anemia and its impact on clinicopathologic parameters and survival outcomes. Analyses were performed under random- or fixed-effects meta-analysis models depending on data heterogeneity.
RESULTS: Seven studies met the inclusion criteria comprising 1495 patients with endometrial cancer. Nearly all studies had low risk of bias. The pooled prevalence of preoperative anemia was 26.5 % (95 % CI: 18.6%-36.2%). Preoperative anemia significantly correlated with advanced FIGO stage III-IV (OR = 5.14, 95 % CI [3.36, 7.86], p < 0.00001), ≥50 % myometrial invasion (OR = 1.95, 95 % CI [1.36, 2.78], p = 0.0003), lymph node metastasis (OR = 4.46, 95 % CI [2.39, 8.30], p < 0.00001), non-endometrioid histology (OR = 3.25, 95 % CI [1.89, 5.60], p < 0.0001), adnexal involvement (OR = 5.88, 95 % CI [3.05, 10.23], p < 0.001), cervical involvement (OR = 2.91, 95 % CI [1.65, 5.11], p = 0.0002), positive peritoneal cytology (OR = 3.24, 95 % CI [1.41, 7.44], p = 0.006), preoperative thrombocytosis (OR = 6.66, 95 % CI [3.05, 14.52], p < 0.00001) and lymphovascular space invasion (OR = 3.50, 95 % CI [1.82, 6.74], p = 0.0002). High tumor grade II-III was increased in patients with preoperative anemia, yet this effect was not statistically significant (OR = 2.12, 95 % CI [0.97, 4.66], p = 0.06). Consistently, the five-year overall survival (OS) and disease-free survival (DFS) rates were significantly lower in patients with preoperative anemia when compared to those without preoperative anemia. Pooled HR showed that preoperative anemia was significantly associated with reduced DFS at univariate (HR = 3.22, 95 % CI [1.28, 8.11], p = 0.01) and multivariate (HR = 1.02, 95 % CI [1.00, 1.05], p = 0.03) analyses.
CONCLUSIONS: Preoperative anemia predicts poor clinicopathologic and survival outcomes in patients with endometrial cancer.

UNASSIGNED: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with dismal prognosis. Pathological and genetic markers may predict more aggressive behavior in MPNSTs but have uncommonly been investigated, and few are used in daily practice. This study reviews the prognostic value of immunohistochemical markers and genetic alterations in MPNST.
UNASSIGNED: A systematic search was performed in PubMed and Embase databases according to the PRISMA guidelines. Search terms related to \'MPNST\' and \'prognostic\' were used. Studies investigating the association of immunohistochemical markers or genetic alterations with prognosis were included. Qualitative synthesis was performed on all studies. A distinction was made between univariable and multivariable associations.
UNASSIGNED: Forty-six studies were included after full-text screening. Sixty-seven different immunohistochemical markers were investigated. Absence of S100 and H3K27me3 and high Ki67 and p53 staining was most commonly independently associated with worse survival and disease-free survival. Several genetic alterations were investigated as well with varying association to survival. TP53, CDK4, RASSF1A alterations were independently associated with worse survival, as well as changes in chromosomal length in Xp, 10q, and 16p.
UNASSIGNED: MPNSTs harbor complex and heterogeneous biology. Immunohistochemical markers and genetic alterations have variable prognostic value. Absence of S100 and H3K27me3 and increased Ki67 can be of prognostic value. Alterations in TP53 or increase in p53 staining may distinguish MPNSTs with worse outcomes. Genetic alterations and staining of other cell cycle regulatory and Ras pathway proteins may also help stratifying patients with worse outcomes. A combination of markers can increase the prognostic value.

BACKGROUND: Extramammary Paget\'s disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis.
OBJECTIVE: To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution.
METHODS: Retrospective observation data analysis of 19 EMPD cases was performed. Demographic information, clinical management records, and histopathologic data of individual cases were obtained from the inpatient hospital data registry.
RESULTS: The mean age (years) at time of diagnosis was 62.4 with equal gender distribution. Synchronous tumors were detected in 6 cases (31.5%). 18 out of 19 patients underwent definitive surgical management in the form of wide local excision (WLE) and reconstructive surgery. Positive margins were found in 11 (68.8%) cases and 7 out of these 11 cases underwent second look surgical intervention to achieve oncological clearance or adjuvant oncology treatment. Follow-up period for living patients varied depending on time of diagnosis and definitive treatment. 10 out 19 cases (52.7%) were alive at the time of the study. Among the 7 cases of mortality from cancer, 5 cases died from progression of underlying associated malignancy and only 2 cases died with advanced stage of EMPD.
CONCLUSIONS: EMPD can be quite aggressive, especially in the secondary form, and surgical management is challenging with a high rate of residual tumor at the surgical margin. EMPD can easily mislead the clinician and patient, leading to unnecessary delay prior to definitive effective management.

Background: Programmed cell death ligand 1 (PD-L1) expression has been shown to correlate with poor prognosis in diverse human cancers. However, limited data exist on the prognostic and clinicopathologic significance of PD-L1 expression in prostate cancers (PCa), and the curative effect of anti-PD-1/PD-L1 therapy remains controversial. In this systematic review and meta-analysis, we aimed to evaluate the prognostic and clinicopathologic value of PD-L1 in PCa. Methods: We performed a systematic literature search in the PubMed, Cochrane Library, EMBASE, Web of Science, and SCOPUS databases up to July 21st, 2018. Pooled prevalence of PD-L1 in PCa was calculated using Freeman-Tukey double arcsine transformation by R software version 3.5.0. The data from the studies were examined by a meta-analysis using Review Manager software 5.3 to calculate pooled hazard ratios (HRs) and pooled odds ratios (ORs) with 95% confidence intervals (CIs) to estimate the prognostic and clinicopathologic value of PD-L1 in PCa. Heterogeneity was tested by the Chi-squared test and I 2 statistic. Results: Five studies with 2,272 patients were included in this meta-analysis. The pooled prevalence of PD-L1 in PCa was 35% (95% CI 0.32 to 0.37). Both PD-L1 expression (HR = 1.78; 95% CI 1.39 to 2.27; p < 0.00001) and PD-L1 DNA methylation (HR = 2.23; 95% CI 1.51 to 3.29; p < 0.0001) were significantly associated with poor biochemical recurrence-free survival (BCR-FS). PD-L1 tended to have high expression levels in high Gleason score cases (OR = 1.54; 95% CI, 1.17 to 2.03; P = 0.002) and androgen receptor-positive cases (OR = 2.42, 95% CI 1.31 to 4.50; P = 0.005). However, PD-L1 had relatively weak correlation with age, pathologic stage, lymph node metastasis and preoperative PSA level. Conclusions: This meta-analysis confirms the negative prognostic significance of PD-L1 expression and mPD-L1 in PCa patients. Additionally, PD-L1 has a statistically significant correlation with Gleason score and androgen receptor status, while the correlations with age, pathologic stage, lymph node metastasis, and preoperative PSA level were not statistically significant. However, the number of included studies is too small to make the conclusions more convincing, so more retrospective large-cohort studies are expected for the further confirmation of these findings.

BACKGROUND: Primary intestinal NK/T cell lymphoma is rare but aggressive and exhibits a poor prognosis. Little is known about its clinical characteristics because few studies with small sample sizes have been reported.
OBJECTIVE: To provide clinicopathological features and endoscopic findings and to summarize the treatment outcomes of primary intestinal NK/T cell lymphoma to improve our understanding of this disease.
METHODS: Between January 2011 to December 2016, 13 patients with confirmed primary gastrointestinal NK/T cell lymphoma at our center were described, and an updated literature review was provided.
RESULTS: In this series of 13 cases, 69.23% were men, the median age was 39 years, and the median survival was 6 months. The common clinical manifestations included abdominal pain (76.92%) and gastrointestinal bleeding (46.15%). Lymphomas were common in the large intestine (69.23%). In 76.92% of patients, the clinical staging was stage I, and all 13 patients manifested ulcerative lesions and no tumor mass on endoscopy. The clinical characteristics of primary intestinal NK/T cell lymphomas were similar to results in existing literature.
CONCLUSIONS: Intestinal NK/T cell lymphoma shows nonspecific clinical features and poor prognosis, which is mainly expressed as ulcers on endoscopy. Emergency surgery may be an adverse prognostic factor of lymphoma, since it is prone to progress toward gastrointestinal perforation.

Primary bronchopulmonary mucoepidermoid carcinoma (BPMEC) is a rare tumor. The fusion protein MECT1-MAML2 has been implicated as a causative genetic event in salivary and BPMECs. Several studies have shown the impact of MECT1-MAML2 on the diagnosis and prognosis of salivary gland mucoepidermoid carcinoma; however, few studies have been published regarding MECT1-MAML2 in the context of primary BPMEC. We describe the clinicopathologic, genetic, and outcome data of 16 patients with BPMEC. Clinicopathologic features were recorded from the electronic medical records. All tumors were reviewed by two expert pulmonary pathologists and graded according to previously established criteria. The presence of MECT1-MAML2 was evaluated with reverse transcription polymerase chain reaction using RNA extracted from formalin-fixed paraffin-embedded tumor tissue. Patients included 9 women and 7 men with a median age of 50 years (range, 7 to 82 years). Tumors exhibited low (n = 14, 88%), and high (n = 2, 12%) grade histologic features. Eight of nine tested tumors (89%) were positive for MECT1-MAML2. The median follow-up time was 40.8 months (range, 1.8-120). Median overall survival for patients with high-grade tumors was 12 months, which was significantly (p = 0.002) shorter than that for patients with low-grade tumors (survival undefined). We also provide a comprehensive review of literature of cases of primary bronchopulmonary mucoepidermoid carcinoma and summarize our findings in this context. MECT1-MAML2 fusion transcript is a driver genetic event in the pathogenesis of primary BPMEC. Histologic grade continues to play a pivotal role in the survival of patients with primary bronchopulmonary mucoepidermoid carcinoma.

BACKGROUND: Although it is a well recognized premalignant lesion of the stomach, there is a dearth of information on the clinicopathologic features of gastric intestinal metaplasia in Nigerians. It is, therefore, necessary to study these features and their possible contribution to the development of gastric carcinoma in Nigerians.
METHODS: All gastric biopsies with the histo-morphologic features of intestinal metaplasia diagnosed at the department of morbid anatomy and forensic medicine, Obafemi Awolowo university teaching hospitals complex, Ile-Ife, Nigeria between January 2006 and December 2010 were used for the study.
RESULTS: A total of 165 biopsies (21.3% of all gastric biopsies within the study period) with background chronic gastritis and intestinal metaplasia were reviewed. The mean age of patients with intestinal metaplasia was 50.3 years ± 17 standard deviation (SD) while the ages of the patients ranged from 10-100 years. There were 83 males (50.3%) with a mean age of 48.1 ± 18.2 SD years and 95% confidence interval (CI) of 44.1-52.1 years. There were, however, 82 females (49.6%) with a mean age of 52.5 (± 15.8 SD) years and a 95% CI of 49.0-56.0 years. There was no significant association between the histologic type of intestinal metaplasia and the patients\' sex, age groups, severity of chronic gastritis, disease activity or degree of gastric glandular atrophy.
CONCLUSIONS: There are no statistically significant differences in the clinicopathologic characteristics of the subtypes of intestinal metaplasia. In majority of patients, progression from intestinal metaplasia to gastric adenocarcinoma probably takes an average of about 7 years.