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

2021 ◽  
Author(s):  
Qian Cui ◽  
Hongmei Wu ◽  
Yu Chen ◽  
Zebin Xiao ◽  
Jie Chen ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Gastric Wall ◽  
Neuroendocrine Differentiation ◽  
Clear Cell Sarcoma ◽  
Mesenchymal Tumor ◽  
Gastric Glands ◽  
Neural Invasion ◽  
Mesenchymal Neoplasm ◽  
Eosinophilic Cytoplasm ◽  
Gastritis Cystica Profunda

Abstract 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.

Clear Cell Sarcoma of Tendons and Aponeuroses: A Review

2007 ◽  
Vol 131 (1) ◽  
pp. 152-156 ◽  
Author(s):  
Daniel C. Dim ◽  
Linda D. Cooley ◽  
Roberto N. Miranda
Keyword(s):  
Clear Cell ◽  
Current Knowledge ◽  
S100 Protein ◽  
Clear Cell Sarcoma ◽  
Prognostic Indicators ◽  
Gene Transcript ◽  
Poor Overall Survival ◽  
Regional Lymph Nodes ◽  
Cell Sarcoma ◽  
Eosinophilic Cytoplasm

Abstract Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

scholarly journals P3.02-084 FGF9-FGFR Pathway Induce Neuroendocrine Differentiation in Lung Epithelial Cells

2017 ◽  
Vol 12 (11) ◽  
pp. S2268
Author(s):  
K. Ishioka ◽  
H. Yasuda ◽  
K. Soejima ◽  
D. Arai ◽  
O. Keiko ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Neuroendocrine Differentiation ◽  
Lung Epithelial Cells ◽  
Lung Epithelial

scholarly journals Expression of claudins in the normal canine gastric mucosa

2014 ◽  
Vol 62 (1) ◽  
pp. 13-21 ◽  
Author(s):  
Roland Psáder ◽  
Csaba Jakab ◽  
Ákos Máthé ◽  
Gyula Balka ◽  
Kinga Pápa ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Gastric Mucosa ◽  
Epithelial Cells ◽  
Apical Part ◽  
Basal Part ◽  
Gastric Epithelial Cells ◽  
Gastric Glands ◽  
Gastric Cells ◽  
Pyloric Stomach ◽  
Mucous Neck Cells

The aim of the present study was to investigate the expression pattern of claudin-1, -2, -3, -4, -5, -7, -8, -10 and -18 in the intact fundic and pyloric gastric mucosa of dogs. Intense, linear, membranous claudin-18 positivity was detected in the surface gastric cells and in the epithelial cells of the gastric glands both in the fundic and pyloric stomach regions. The mucous neck cells in the apical part of the glands, furthermore the parietal cells and chief cells of the basal part of the gland were all positive for claudin-18, in the same way as the enteroendocrine cells. Cells of the basal part of the pyloric glands showed intense, linear, membranous claudin-2 positivity, but cells of the superficial portion of these glands and the surface gastric cells in this region were claudin-2 negative. Fibroblasts, endothelial cells, lymphocytes of the propria layer, smooth muscle cells and vegetative neurons were all negative for claudin-2. All gastric epithelial cells were negative for claudin-1, -3, -4, -5, -6, -7, -8 and -10. The endothelial cells of the propria layer had intense claudin-5 positivity. We assume that claudin-18 forms a paracellular barrier against gastric acid in the healthy canine stomach, in the same way as in mice.

Malignant Gastrointestinal Neuroectodermal Tumor: a case report and a review of the literature

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S66-S67
Author(s):  
B Youssef ◽  
D Asberry ◽  
R Mohamed
Keyword(s):  
Case Report ◽  
Gastrointestinal Tract ◽  
Clear Cell ◽  
Muscularis Propria ◽  
Clear Cell Sarcoma ◽  
Neuroectodermal Tumor ◽  
High Rate ◽  
Cell Sarcoma ◽  
Molecular Features ◽  
Eosinophilic Cytoplasm

Abstract Introduction/Objective Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare soft tissue tumor arising in the wall of the gastrointestinal tract. The GNET was first described as an osteoclast rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma, with only few cases reported in the literature. Methods/Case Report We report a case of a 71-year-old man with a past medical history of hypertension, hyperlipidemia, and benign prostatic hyperplasia presented with complaints of dyspnea, dizziness, fatigue, black stools, and a recent syncopal episode. Laboratory testing revealed anemia (HB 5.4 g/dL). Esophagogastroduodenoscopy demonstrated a submucosal gastric mass. An abdominal CT scan confirmed a 7.8 cm mass along the gastric cardia and fundus. Results (if a Case Study enter NA) Biopsy rendered a gastrointestinal neuroectodermal tumor (GNET). Microscopically, the tumor cells were spindle with eosinophilic cytoplasm and arranged in fascicules. They were positive for CD56, CD99, and Fli-1, synaptophysin, and negative for chromogranin, TTF-1, SMA, desmin, CD34, EMA, pan-cytokeratin, and lymphoid markers. Two months later, further imaging confirmed metastasis to the liver and spleen. GNETs typically arise within the muscularis propria of the gastrointestinal tract and often extend into the submucosa and subserosa. Conclusion The most important differential of GNET is the clear cell sarcoma of the gastrointestinal tract (CCS-GI). Both share similar morphological as well as molecular features and show S100 positivity; however, the lack of melanocytic differentiation in GNET distinguishes it from CCS-GI. Both typically show rearrangements of the EWSR1 gene, with t(12;22) (q13;q12) EWSR1-ATF1 or t(2;22)(q34;q12) EWSR1-CREB1 fusions. Pathologists should be aware of GNET diagnostic entity due to its aggressive behavior and high rate of recurrence and mortality even after complete resection.

Primary Clear Cell Sarcoma of the Breast: A Case Report

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S23-S23
Author(s):  
R Aldrees ◽  
S Wei ◽  
C Prieto-Granada ◽  
C Patel
Keyword(s):  
English Language ◽  
Clear Cell ◽  
Malignant Neoplasm ◽  
Clear Cell Sarcoma ◽  
Spindle Cell Carcinoma ◽  
Left Breast ◽  
Soft Tissue Neoplasm ◽  
Cell Sarcoma ◽  
First Case ◽  
Eosinophilic Cytoplasm

Abstract Casestudy: Clear cell sarcoma (CCS), also known as malignant melanoma of soft parts, is a primary soft tissue neoplasm exhibiting evidence of melanocytic differentiation. It is an uncommon aggressive tumor that arises in tendons and aponeuroses of the distal extremities. Here, we report the first case of primary CCS of the breast. The patient was a 43-year-old female who presented with a left breast mass and underwent surgical resection at an outside hospital. No history of melanoma or any other malignancies was reported. Grossly, it was described as a 2 x 1.5 x 1.5 cm, well-demarcated, white nodular mass. Microscopic examination showed a malignant neoplasm composed of short spindle cells with ill-defined, eosinophilic cytoplasm and ovoid nuclei with finely stippled chromatin and exhibiting moderate pleomorphism. The lesional cells were arranged in short interlacing fascicles with abundant collagen, with brisk mitotic activity (>15/10 HPF). The differential diagnosis included spindle cell carcinoma, myoepithelial carcinoma and melanocytic neoplasm. The tumor cells were immunoreactive for S-100 protein, SOX10, Mart-1, HMB45 and MiTF, but negative for multiple cytokeratins (including high and low molecular weight keratins), p63, EMA, CEA, Caldesmon, smooth muscle myosin, calponin, desmin, ERG, and CD31, thus confirming melanocytic origin. EWSR1 gene rearrangement was detected by fluorescence in situ hybridization analysis using break-apart probes. Overall, the histomorphology combined with the immunophenotype and cytogenetic characteristics, was most consistent with a CCS. To our knowledge, no primary CCS of the breast has been previously reported in the English language literature.

Increased gastric expression of MMP-7 in hypergastrinemia and significance for epithelial-mesenchymal signaling

2007 ◽  
Vol 292 (4) ◽  
pp. G1133-G1140 ◽  
Author(s):  
Andrea Varro ◽  
Susan Kenny ◽  
Elaine Hemers ◽  
Catherine McCaig ◽  
Sabine Przemeck ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
Neutralizing Antibodies ◽  
Smooth Muscle Actin ◽  
Carcinoid Tumors ◽  
Gastric Carcinoid ◽  
Gastric Epithelial Cells ◽  
Cell Hyperplasia ◽  
Gastric Glands ◽  
Ecl Cell ◽  
Gastric Corpus

Chronic hypergastrinemia is associated with enterochromaffin-like (ECL) cell hyperplasia, which may progress to gastric carcinoid tumors. The latter consists of epithelial cells and stroma, and both compartments usually regress after normalization of hypergastrinemia. We previously showed that matrix metalloproteinase (MMP)-7 in gastric epithelial cells was upregulated by Heliobacter pylori and described MMP-7-dependent reciprocal signaling between the epithelium and a key stromal cell type, the myofibroblast. Here, we describe the regulation of gastric MMP-7 by gastrin and the potential significance for recruiting and maintaining myofibroblast populations. Biopsies of the gastric corpus and ECL cell carcinoid tumors were obtained from hypergastrinemic patients. Western blot analysis, ELISA, immunohistochemistry, and promoter-luciferase (luc) reporter assays were used to study MMP-7 expression. Gastric myofibroblasts were identified by α-smooth muscle actin (α-SMA) expression, and the effects of MMP-7 on myofibroblast proliferation were investigated. In hypergastrinemic patients, there was an increased abundance of MMP-7 and α-SMA in gastric corpus biopsies and ECL cell carcinoid tumors. In the latter, MMP-7 was localized to ECL cells but not stromal cells, which were nevertheless well represented. Gastrin stimulated MMP-7-luc expression in both AGS-GR and primary human gastric epithelial cells. Conditioned medium from gastrin-treated human gastric glands stimulated myofibroblast proliferation, which was inhibited by neutralizing antibodies to MMP-7. MMP-7 increased the proliferation of myofibroblasts via the MAPK and phosphatidylinositol 3-kinase (PI3K) pathways. In conclusion, stimulation of gastric MMP-7 by elevated plasma gastrin may activate epithelial-mesenchymal signaling pathways regulating myofibroblast function via MAPK and PI3K pathways and contribute to stromal deposition in ECL cell carcinoid tumors.

scholarly journals G-protein αolf subunit promotes cellular invasion, survival, and neuroendocrine differentiation in digestive and urogenital epithelial cells

Oncogene ◽  
2002 ◽  
Vol 21 (25) ◽  
pp. 4020-4031 ◽  
Author(s):  
Karine Régnauld ◽  
Quang-Dé Nguyen ◽  
Luc Vakaet ◽  
Erik Bruyneel ◽  
Jean-Marie Launay ◽  
...  
Keyword(s):  
Epithelial Cells ◽  
G Protein ◽  
Neuroendocrine Differentiation ◽  
Cellular Invasion

scholarly journals Characterization of A Rare Case of Vulvar Epithelioid Sarcoma with local recurrence and metastases

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S81-S81
Author(s):  
E Shkolnik ◽  
D Cai
Keyword(s):  
Lymph Node ◽  
Local Recurrence ◽  
Tumor Cells ◽  
Epithelioid Sarcoma ◽  
Molecular Testing ◽  
Adjuvant Radiation ◽  
Inguinal Lymph Node Dissection ◽  
Mesenchymal Neoplasm ◽  
Eosinophilic Cytoplasm ◽  
Abundant Eosinophilic Cytoplasm

Abstract Introduction/Objective Epithelioid sarcoma (ES) is a rare, malignant mesenchymal neoplasm that has a known tendency for local recurrence, regional lymph node involvement, and distant metastases. Two histologic variants have been recognized: classic ES also known as the distal type, and proximal-type ES (PES). The classic ES is common in young adults. It occurs more frequently in the distal upper extremities followed by the distal lower limbs and has a male prevalence of 2:1. Conversely, PES commonly involves deep tissues in the pelvic region, including the genital area. It tends to occur in older patients and follows a more aggressive clinical course. In the female genital tract, PES occurs most frequently in the vulva. The incidence of primary sarcoma of the vulva accounts for 1.5-5% of all malignant tumors, making PES a very rare incidence. Methods/Case Report Here we report a 60-year-old female diagnosed with vulvar epithelioid sarcoma treated with a right radical vulvectomy and bilateral inguinal lymph node dissection in 2008. In 2017, further surgery and adjuvant radiation were given for local recurrence. In 2020, the patient developed left hip pain and was found to have an expansile lytic lesion in the left proximal femur. Extensive resection was performed. Grossly the vulvar lesion was nodular with diffuse hemorrhage, degeneration, and necrosis. Microscopically, the tumor cells had large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Histologically, the morphology of the tumor cells are similar for the primary vulvar specimen and the bone metastases. Immunohistochemically, the tumor cells are positive for vimentin, GATA, FLI-1, SMA, SMHC, partially positive for CAM5.2, AE1/AE3, CD31, and CD163. Immunohistochemistry was negative for CDX2, CD56, S-100, TTF-1, CK5/6, CK20, P40, mammoglobin, MOC31, ER, CK7, CK903, HMB45, PAX8. A Ki-67 proliferative index was around 30-40%. NGS molecular testing detected a SMARCB1 mutation with loss of exons 1-3 and exons 7-9 supporting the diagnosis of epithelioid sarcoma. Results (if a Case Study enter NA) NA Conclusion In summary, we report a case of PES of the vulva in a 60 year old female. Grossly, the lesion was nodular with histology showing large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It showed loss of INI1/SMARCB1 nuclear expression. The patient is receiving further adjuvant treatment and shows no new metastases.

scholarly journals Mechanisms of castration resistant prostate cancer formation and progression through neuroendocrine differentiation

2021 ◽  
Keyword(s):  
Prostate Cancer ◽  
Smooth Muscle ◽  
Epithelial Cells ◽  
Neuroendocrine Differentiation ◽  
Underlying Mechanism ◽  
Neuroendocrine Cells ◽  
Basal Cells ◽  
Castration Resistant Prostate Cancer ◽  
Normal Prostate ◽  
Castration Resistant

Normal prostate tissues consist mainly of epithelial cells, including secretory epithelial cells, basal cells, and neuroendocrine cells, and of mesenchymal cells, including smooth muscle cells and fibroblasts. The mechanisms leading to castration resistant prostate cancer (CRPC) are complex and diverse, but most involve neuroendocrine differentiation. In fact, during the development of prostate cancer, some of the tumor cells transform into neuroendocrine-like cells. This transition is a main underlying mechanism of CRPC formation.

Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament

2004 ◽  
Vol 14 (5) ◽  
pp. 1036-1039 ◽  
Author(s):  
D. Fink ◽  
D. E. Marsden ◽  
L. Edwards ◽  
C. Camaris ◽  
N. F. Hacker
Keyword(s):  
Broad Ligament ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasm ◽  
Eosinophilic Cytoplasm

Malignant perivascular epithelioid cell tumor (PEComa) is an extremely rare mesenchymal neoplasm mostly composed of HMB-45-positive epithelioid cells with clear-to-eosinophilic cytoplasm, a propensity for perivascular distribution and a coexpression of smooth muscle markers. The uterus seems to be one of the most prevalent sites of involvement, although only 14 cases of uterine PEComa have been described. We report the case of a 51-year-old woman with a PEComa arising in the broad ligament. She was treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic radiation, and remains without evidence of disease 15 months after diagnosis. This is, to the best of our knowledge, the first report of a malignant PEComa arising in the broad ligament. To correctly diagnose PEComa, an extensive immunohistochemical panel is essential. As PEComas can behave in an aggressive manner, careful follow-up is warranted.

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