Case report: A EWSR1-CREM-Rearranged Gastric Mesenchymal Tumor Accompanied by Gastritis Cystica Profunda and with Probable Benign Behavior

Background:Genomic rearrangements involving EWSR1 and the CREB family of transcription factors are increasingly detected in an array of mesenchymal neoplasms, including clear cell sarcoma-like tumors of the gastrointestinal tract (CCSLGT), a gastrointestinal malignancy. Gastritis cystica profunda (GCP) is a rare disease characterized by cystic dilatation of gastric glands into the submucosa and generally regarded as a precursor to tumor.Case presentation:Herein, we report a peculiar case in which a EWSR1-CREM-rearranged gastric mesenchymal tumor was admixed with GCP in a gastric fundic mass in a 64-year-old woman. Histologically, the mass showed readily distinguishable epithelial and mesenchymal components. All layers of the gastric wall were invaded, although no lymph node or neural invasion, or tumoral vascular emboli was noted. The epithelial component consisted of foveolar-type glands interspersed with pyloric-type ones, with glands showing metaplastic growth. Most glands were elongated with irregular contour, with some forming cystic structures containing eosinophilic secretory material. The epithelial cells showed focally atypical hyperchromatic nuclei, inconspicuous nucleoli, slightly eosinophilic cytoplasm, and infrequent mitosis. The mesenchymal component consisted of monomorphic, ovoid-shaped cells often arranged in sheets surrounding the glands. These cells displayed scanty cytoplasm, regular nuclei, and rare mitotic figures. Immunohistochemically, the epithelial cells were uniformly positive for cytokeratins and negative for markers of neuroendocrine differentiation, and the mesenchymal neoplasm showed focal positivity for CD10, CD117 and CD56 as well as negativity for cytokeratin, neuroendocrine markers, DOG-1, CD34, SMA, desmin, HMB-45, Melan A, and S-100. An EWSR1-CREM fusion was identified with genomic profiling and confirmed with fluorescence in situ hybridization in the tumor. Given the low mitotic activity, absence of nodal or distant spread and vascular or neural invasion, and the disease-free status at 28-month follow-up, both lesions were likely benign. Conclusions:To our knowledge, this is the first to report a EWSR1-CREM fusion in a gastric mesenchymal tumor with accompanying GCP. Whether this case suggests a novel entity or falls into one of proposed classes awaits report of more similar cases and insights into the relationship between GCP pathogenesis and oncogenesis.

Peri-neural Invasion in Oral Squamous Cell Carcinoma (OSCC): A Clinico-pathological Marker of OSCC Prognosis and Risk Assessment

Aims And Objectives: To assess the incidence of Peri-Neural invasion in oral squamous cell carcinoma (OSCC), and to notify its significance as potent risk factor in OSCC recurrence and metastasis. Study Design and Place of Study: A retrospective in vitro study was conducted at Indira Gandhi Institute of Medical Sciences, Patna, after approval of ethical committee Material and Methods: Clinical, histopathological and follow-up details of 86 cases of oral squamaous cell carcinoma were collected from medical records as secondary data from December 2019 till April 2021. Peri-Neural invasion was assessed in all patients and was associated with, lymph node involvement and with the different histologic grades of OSCC. Location, density and patterns of Peri-Neural invasion was checked along with extracapsular spread and survival. Chi-square test was used for analyzing statistical data. P value of < .05 was considered as significant Results: On statistical analysis it was found that, out of total 86 cases of OSCC, 59 were primary and 27 were recurrent OSCC. Male to female ratio was 3:1 and primary cases were more in age group of 35-44 years where as recurrent OSCC were more in age group of 55-64 years. Out of 59 primary cases of OSCC, Peri-Neural invasion was seen in 27 cases and out of 27 recurrent OSCC 18 had Peri-Neural invasion with a total of 45 cases with Peri-Neural invasion out of 86. Intratumor location was assessed in 22 cases and density of 1-2 nerves per section was seen. “Crescent” pattern was commonly occurring pattern in our study. Conclusion: Our study affirmed that the incidence of Peri-Neural invasion is high in OSCC. It is present in both primary and recurrent tumors, irrespective of tumor’s grading. PNI must be assessed in all cases of OSCC as it gives markable impression on recurrence, metastasis and prognosis, and modulates treatment protocols.

Grade G2 Rectal Neuroendocrine Tumor Is Much More Invasive Compared With G1 Tumor

BackgroundTo compare clinicopathologic feature of rectal neuroendocrine tumor (NET) grade G1 with G2 NET.MethodsSix hundred-one cases of rectal G1 and G2 NETs diagnosed in our center were analyzed.ResultsOf 601 cases of rectal NET, 515 cases were with grade G1 and 86 cases were with grade G2. Median tumor size was 0.7 cm. Compared with G1 NET, G2 tumors were with significantly larger tumor size (0.8 vs 2.2 cm, p &lt; 0.001), less percentages of patients with tumors confined to submucosa (92.6 vs 42.8%, p &lt; 0.001), more frequent presence of microvascular invasion (MVI) (3.6 vs 16.9%, p &lt; 0.001) or peri-neural invasion (PNI) (2.0 vs 24.1%, p &lt; 0.001). Incidence of lymph node and distant metastasis was 5.2 and 2.1% in G1 NET compared with 44.2 and 31.4% in G2 tumor, respectively (p &lt; 0.001). For tumors sized 1–2 cm and confined to submucosa, incidence of lymph node metastasis was 6.1% for G1 NET compared with 21.1% for G2 NET. Status of MVI/PNI was predictive of lymph node metastasis for G2 tumor rather than G1 NET in this subgroup.ConclusionsRectal G2 NET was much more invasive with significantly elevated prevalence of lymph node metastasis compared with G1 tumor.

Microscopic Invasion of Nerve Is Associated With Aggressive Behaviors in Pancreatic Neuroendocrine Tumors

ObjectivesThe role of neural invasion has been reported in cancers. Few studies also showed that neural invasion was related to survival rate in patients with pancreatic neuroendocrine tumor (PNET). The aim of this study is to explore the association between neural invasion and aggressive behaviors in PNET.MethodsAfter excluding those patients with biopsy and with missing histological data, a total 197 patients with PNET who underwent surgery were retrospectively analyzed. The demographic data and histological data were obtained. Aggressive behavior was defined based on extra-pancreatic extension including vascular invasion, organ invasion and lymph node metastases. Logistic regression analyses were used to identify risk factor for aggressive behavior. Receiver operating characteristic (ROC) curves were performed to show the performance of nomograms in evaluating aggressive behavior of PNET.ResultsThe prevalence of neural invasion in the cohort was 10.1% (n = 20). The prevalence of lymph node metastasis, organ invasion, and vascular invasion in PNET patients with neural invasion was higher than those in patients without neural invasion (p &lt; 0.05). Neural invasion was more common in grade 3 (G3) tumors than G1/G2 (p &lt; 0.01). Tumor size, tumor grade, and neural invasion were independent associated factors of aggressive behavior (p &lt; 0.05) after adjusting for possible cofounders in total tumors and G1/G2 tumors. Two nomograms were developed to predict the aggressive behavior. The area under the ROC curve was 0.84 (95% confidence interval (CI): 0.77–0.90) for total population and was 0.84 (95% CI: 0.78–0.89) for patients with G1/G2 PNET respectively.ConclusionsNeural invasion is associated with aggressive behavior in PNET. Nomograms based on tumor size, grade and neural invasion show acceptable performances in predicting aggressive behavior in PNET.