• In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45. (nih.gov)
  • It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus. (nih.gov)
  • A family of mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. (curehunter.com)
  • Epithelial tumors of nasal cavity, sinuses, nasopharynx: A) Squamous cell carcinoma with variants of nasal cavity, sinuses, and nasopharynx and trachea (excluding laryngeal, nasopharyngeal cancer [NPC], and squamous cell carcinoma of the head and neck [SCCHN]) B) Adenocarcinoma and variants of nasal cavity, sinuses, and nasopharynx (closed to accrual 07/27/2018) 2. (sncrf.org)
  • Rare pancreatic tumors including acinar cell carcinoma, mucinous cystadenocarcinoma or serous cystadenocarcinoma. (sncrf.org)
  • Non-epithelial tumors of the ovary: A) Germ cell tumor of ovary B) Mullerian mixed tumor and adenosarcoma (closed to accrual 03/30/2018) 14. (sncrf.org)
  • Cell tumor of the testes and extragonadal germ tumors: A) Seminoma and testicular sex cord cancer B) Non seminomatous tumor C) Teratoma with malignant transformation (closed to accrual) 17. (sncrf.org)
  • Epithelial tumors of penis - squamous adenocarcinoma cell carcinoma with variants of penis (closed to accrual) 18. (sncrf.org)
  • Malignant giant cell tumors 30. (sncrf.org)
  • Launch The perivascular epithelioid cell (PEC) is a cell type that's histologically and immunohistochemically within several tumors, including renal angiomyolipoma (AML), clear-cell glucose tumor (CCST) lymphangioleiomyomatosis (LAM), and rare clear-cell tumors of other anatomical sites. (cell-signaling-pathways.com)
  • The cell of origins and various types of obvious cell tumors of the lung (the so-called sugars tumor) have been enigmatic and controversial for the last 3 decades. (cell-signaling-pathways.com)
  • Zamboni et al[4] asserted that obvious cell tumors of the lung arise from your perivascular epithelioid cell (PEC) and mentioned that related cells have been recognized in AML and lymphangiomyomas. (cell-signaling-pathways.com)
  • There have been increasing numbers of reports of different views on and insights into obvious cell tumors in recent years. (cell-signaling-pathways.com)
  • The World Health Corporation defines PEComa as unusual mesenchymal tumors composed of histologically and immunohistochemically special PECs. (cell-signaling-pathways.com)
  • This phase II trial studies how well Ataxia telangiectasia and Rad3-related (ATR) kinase inhibitor AZD6738 works alone or in combination with olaparib or durvalumab in treating participants with renal cell carcinoma (RCC), urothelial carcinoma, all pancreatic cancers, endometrial cancer, and other solid tumors excluding clear cell ovarian cancer that have spread to nearby tissue or lymph nodes or other parts of the body. (ucbraid.org)
  • Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms characterized by the presence of histologically and immunohistochemically distinctive perivascular epithelioid cells [ 1 ], which can occur in any part of the body. (biomedcentral.com)
  • Leptomeningeal infiltration of the brain or spinal cord by neoplastic cells may occur as complication of solid or hematopoietic tumors such as non-Hodgkin lymphoma. (cnr.it)
  • Long-term follow-up for duration of response (DoR) after weekly nab-sirolimus in patients with advanced malignant perivascular epithelioid cell tumors (PEComa): results from a registrational open-label phase II trial, AMPECT. (oncdata.com)
  • One of the most important characteristics to differentiate these tumors from other renal cell neoplasms is their typical reactivity to premelanosome antigens. (biomedcentral.com)
  • These features might lead to an erroneous diagnosis of high-grade renal cell carcinoma, particularly since intra-tumoral fat can occur in these tumors [ 4 ]. (biomedcentral.com)
  • The FDA has granted priority review to the New Drug Application for nab-sirolimus for consideration as a treatment for patients with advanced malignant perivascular epithelioid cell tumors. (targetedonc.com)
  • The genes responsible for this multi-system genetic disease have also been implicated in other PEComas. (wikipedia.org)
  • abdominopelvic sarcoma of perivascular epitheloid cells primary extrapulmonary sugar tumour Thus, it has been advocated that the above could be classified PEComas. (wikipedia.org)
  • Background: Perivascular epithelioid cell tumours (PEComas) are mesenchymal neoplasms with variable biological behaviour, ranging from benign to extremely aggressive diseases able to metastasize. (fortuneonline.org)
  • Perivascular epithelioid cell tumours (PEComas) belongs to the family of mesenchymal neoplasms that can occur in many organs, but rarely found in liver. (medpharmres.com)
  • Karska K, Kozioł I, Leśniewska M, Budzyńska J, Woźniak M. Radiological features of perivascular epithelioid cell tumours (PEComas) in a paediatric patient - Case report. (jpccr.eu)
  • Perivascular epithelioid cell tumor (PEComa) 39. (sncrf.org)
  • Perivascular epithelioid cell tumor (PEComa) occurring in the female genital tract are rare, and typically found in the uterine corpus. (biomedcentral.com)
  • The FDA has approved sirolimus protein-bound particles for injectable suspension (albumin-bound) (Fyarro™, Aadi Bioscience) for patients with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor. (oncdata.com)
  • Malignant perivascular epithelioid cell tumor is a rare, aggressive sarcoma," wrote Andrew J. Wagner, MD, PhD, Medical Director of Ambulatory Oncology at Dana-Farber Cancer Institute and Associate Professor of Medicine at Harvard Medical School, and colleagues, in their publication of the AMPECT trial (NCT02494570), on which the approval was based. (oncdata.com)
  • The multicenter, single-arm clinical trial enrolled 31 patients with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor. (oncdata.com)
  • Diestelkamp T, Mikes Z, Wilson-Smith R, Germaine P. Radiological findings of two neoplasms with perivascular epithelioid cell differentiation. (jpccr.eu)
  • Zhao J, Teng H, Zhao R, Ding W, Yu K, Zhu L, Zhang J, Han Y. Malignant perivascular epithelioid cell tumour of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature. (jpccr.eu)
  • Imaging features of primary and metastatic malignant perivascular epithelioid cell tumours. (jpccr.eu)
  • Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). (wikipedia.org)
  • PECs bear significant histologic and immunohistochemical similarity to: angiomyolipoma, clear-cell sugar tumour (CCST), lymphangioleiomyomatosis, and, clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament. (wikipedia.org)
  • Perivascular epithelioid cell tumour (PEComa) is a rare family of mesenchymal tumours composed of epithelioid cells. (jpccr.eu)
  • On histopathological examination, a PEComa tumour was diagnosed. (jpccr.eu)
  • Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics-analysis of 32 cases and review of the literature. (jpccr.eu)
  • Handa A, Fujita K, Kono T, Komori K, Hirobe S, Fukuzawa R. Radiological findings of perivascular epithelioid cell tumour (PEComa) of the falciform ligament. (jpccr.eu)
  • Xuesong D, Hong G, Weiguo Z. Bladder Perivascular Epithelioid Cell Tumour: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature. (jpccr.eu)
  • Imaging (CT scan and MRI) revealed a 3.9 cm hypervascular mass centered on the pancreatic uncinate process with no evidence of metastatic disease. (pbpath.org)
  • According to findings presented at the Connective Tissue Oncology Society Annual Meeting, INT230-6 enhanced overall survival and disease control rates in relapsed, refractory, and metastatic sarcomas. (targetedonc.com)
  • Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2, although this link is stronger for angiomyolipoma and lymphangioleiomyomatosis than for other members of the PEComa family. (wikipedia.org)
  • Renal epithelioid angiomyolipoma: a study of six cases and a meta-analytic study. (karger.com)
  • The rare variant of renal epithelioid/pleomorphic angiomyolipoma has been reported in approximately 120 cases. (biomedcentral.com)
  • If such a tumor does not stain for HMB-45 or Melan-A, a specific diagnosis of epithelioid pleomorphic angiomyolipoma cannot be made with certainty. (biomedcentral.com)
  • We present here what is, to the best of our knowledge, the first case of epithelioid/pleomorphic angiomyolipoma of the kidney in a 50-year-old Caucasian man with no history of tuberous sclerosis, and with a tumor marker profile negative for several premelanosome antigens. (biomedcentral.com)
  • Based on the characteristic microscopic appearance of this tumor, and its overall tumor marker profile, we concluded this was a renal epithelioid/pleomorphic angiomyolipoma with a negative premelanosome antigen phenotype. (biomedcentral.com)
  • Rare cases of AML, composed of a prominent epithelioid component, with spindle and giant cells, containing none or a minimal amount of adipose tissue, have been reported as the epithelioid variant of angiomyolipoma (EAML) [ 2 ]. (biomedcentral.com)
  • Upon immunohistochemistry (IHC), the tumor cells were positive for HMB45 and TFE3, focally positive for MelanA, while negative for muscle marker. (biomedcentral.com)
  • A few spindled nuclei and melanin pigments were also identified (Fig. 2 A). IHC demonstrated that the epithelioid cells were positive for HMB45 andTFE3 (Fig. 2 B&C), focally positive for Melan-A, while negative for S-100, SOX-10, AE1/AE3, EMA, Desmin, SMA, H-caldesmon. (biomedcentral.com)
  • Tumor cells are diffusely immunoreactive with HMB45. (medpharmres.com)
  • There was no significant past medical history of recent infection, inflammatory bowel disease, bleeding disorders, changes in bowel practices, significant weight loss, or tuberous sclerosis complex. (cell-signaling-pathways.com)
  • The high disease control rate with manageable toxicities suggest that sirolimus [protein-bound] is effective and represents an important new treatment option for these patients," concluded Dr. Wagner and colleagues in their abstract presented at the 2020 American Society of Clinical Oncology (ASCO) Annual Meeting and published in the Journal of Clinical Oncology . (oncdata.com)
  • No abstract avDendritic cells (DC) are the most important antigen presenting cells and play a pivotal role in host immunity to infectious agents by acting as a bridge between the innate and adaptive immune systems. (uni-wuerzburg.de)
  • Soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. (dana-farber.org)
  • For patients with advanced synovial sarcoma or myxoid/ round cell liposarcoma, afamitresgene autoleucel is a promising novel therapy. (targetedonc.com)
  • Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread. (sncrf.org)
  • Radiation therapy uses high-energy x-rays to kill tumor cells. (ucbraid.org)
  • Drugs used in chemotherapy, such as temozolomide, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. (ucbraid.org)
  • BAY 1895344 may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth. (ucbraid.org)
  • Cisplatin and gemcitabine are chemotherapy drugs that stop the growth of tumor cells by killing the cells. (ucbraid.org)
  • The tumor cells demonstrated clear/foamy vacuolated cytoplasm and small uniform nuclei with finely dispersed chromatin [Figure 4]. (pbpath.org)
  • tumor cells are characterized by round to pyknotic nuclei with fine chromatin and inconspicuous nucleoli. (pbpath.org)
  • Electron microscopic examination of tumor cells confirmed the presence of premelanosome-like granules. (biomedcentral.com)
  • In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas. (nih.gov)
  • Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45. (nih.gov)
  • From 12 epithelioid leiomyosarcomas, we selected 5 that had: 1) clear cell areas and 2) spindle cell areas that were at least focally positive for desmin and caldesmon. (nih.gov)
  • Spindle cell carcinoma of kidney, pelvis, ureter 20. (sncrf.org)
  • Representative example is the type A spindle cell uveal melanoma. (nih.gov)
  • Spindle cell melanoma: a comparison of the cytomorphological features with the epithelioid variant. (nih.gov)
  • PECs consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round nucleus without prominent nucleoli. (wikipedia.org)
  • 3] The name identifies the apparent cytoplasm from the cells, which is abundant with glycogen. (cell-signaling-pathways.com)
  • Microscopically, some loosely cohesive epithelioid cells were uniform with abundant clear cytoplasm, showing predominantly round or oval nuclei with finely stippled chromatin. (biomedcentral.com)
  • Distinct round nucleoli were visible in some cells, notably with numerous melanin pigments in the cytoplasm. (biomedcentral.com)
  • The cytologic characteristics of the tumor can provide sufficient clues for PEComa diagnosis, which includes loosely cohesive, epithelioid morphology with abundant clear or eosinophilic cytoplasm, low-grade nuclear atypia, cytoplasmic melanin pigments. (biomedcentral.com)
  • Microscopically, we observed some loosely cohesive atypical cells arranged in single or clusters and sheets, which exhibited epithelioid morphology with abundant clear cytoplasm. (biomedcentral.com)
  • Distinct round nucleoli were visible in some cells (Fig. 1 C), notably with numerous melanin pigments in the cytoplasm (Fig. 1 D). The primary diagnosis was atypical cells which were suspected to be melanoma. (biomedcentral.com)
  • The cell block section showed single or clusters of medium-sized epithelioid cells in a background of fibrinoid fluid, with abundant clear or granular eosinophilic cytoplasm. (biomedcentral.com)
  • The biopsy showed infiltrating neoplastic cells characterized by cytologically bland nuclei and abundant finely vacuolated, clear cytoplasm embedded in a collagenized/fibrotic stroma [Figure 1A/B]. No necrosis or mitotic figures were identified. (pbpath.org)
  • The tumor was composed of sheets of pleomorphic, round to polygonal epithelioid cells with prominent eosinophilic cytoplasm, large nuclei, many multinucleated, and very prominent nucleoli. (biomedcentral.com)
  • This report aims to announce a case of hepatic PEComa with extramedullary hematopoiesis. (medpharmres.com)
  • The data included exploratory biomarker results reported from the final analysis of mTOR inhibitor-naïve advanced malignant PEComa patients treated with nab -sirolimus in the Advanced Malignant PEComa Trial (AMPECT) trial as well as an analysis of prior mTOR inhibitor exposed advanced malignant PEComa patients treated with nab -sirolimus in the Expanded Access Program (EAP) through June 2021 . (aerpio.com)
  • Case presentation: We present a case report of a 53-year-old woman affected by advanced retroperitoneal PEComa who developed an abdominal disease progression after 40 months of therapy with mTOR inhibitor. (fortuneonline.org)
  • that recommended mTOR inhibitors as first-line treatment in this rare disease [6]. (fortuneonline.org)
  • A poster presentation entitled, " nab -Sirolimus for patients with advanced malignant PEComa with or without prior mTOR inhibitors: Biomarker results from AMPECT and an expanded access program" was presented at the 2022 American Society of Clinical Oncology (ASCO) Annual Meeting. (aerpio.com)
  • The final pathological diagnosis was PEComa identified by the liquid-based cytology, cell block, cervical biopsy, IHC and FISH result. (biomedcentral.com)
  • We report a case of cervix PEComa in a 55-year-old postmenopausal woman, initially identified by the liquid-based cytology test and subsequently confirmed by cell block, cervical biopsy, IHC and FISH result. (biomedcentral.com)
  • This will help cytopathologists to recognize this rare tumor that occurred in the cervix, and the combination of predictive morphology evaluation, immunophenotype, and molecular testing can achieve the definitive diagnosis of PEComa. (biomedcentral.com)
  • A dysbalance between FoxP3+ regulatory CD4+ T cells (Tregs) and inflammatory T-helper (Th) 17 cells has been suggested. (uni-wuerzburg.de)
  • 1] In 1963, Liebow and Castleman[2] initial identified a definite kind of Ruxolitinib distributor benign crystal clear cell tumor known as CCST in the lung. (cell-signaling-pathways.com)
  • The same writers coined the word benign apparent cell (glucose) tumor from the lung in 1971. (cell-signaling-pathways.com)
  • Patients with platinum-resistant ovarian cancer and non-small cell lung cancer (adenocarcinoma subtype) were enrolled in the expansion segment of this study. (uci.edu)
  • They are made up of cells from the lung lining, fat and cartilage. (cancer.ca)
  • Squamous cell carcinoma with variants of GI tract (stomach small intestine, colon, rectum, pancreas) (closed to accrual 10/17/2018) 7. (sncrf.org)
  • Many PEComa types shows a female predominance in the sex ratio. (wikipedia.org)
  • The neoplastic cells routinely express pan-cytokeratin and neuroendocrine markers chromogranin and synaptophysin (focal to diffuse staining). (pbpath.org)
  • Selinexor blocks a protein called CRM1, which may help keep cancer cells from growing and may kill them. (ucbraid.org)
  • A tumor marker profile showed tumor cell reactivity for CD68, calponin and focally for CD10. (biomedcentral.com)
  • The cytopathological features were well correlated with cell block and histopathological findings. (biomedcentral.com)
  • Covering all aspects of skin disease from basic science through pathology and epidemiology to clinical practice, the text is recognized for its unparalleled coverage of diagnosis. (rooksdermatology.com)
  • Currently the patient is still in follow up after 18 months from the interruption of therapy, without a disease progression. (fortuneonline.org)
  • Cytological diagnosis of cervical PEComa is even rarer. (biomedcentral.com)
  • Our finding indicated that a definitive diagnosis of PEComa can be rendered based on cytologic examination alone. (biomedcentral.com)
  • Finally, a tentative diagnosis of PEComa was made by combining cell block and IHC, and with the statement that the final diagnosis will require more representative material. (biomedcentral.com)
  • Clinical and pathologic diagnosis, staging and prognostic factors of melanoma and management of primary disease. (nih.gov)
  • Perirenal PEComa: Computed Tomography Findings and Differential Diagnosis. (jpccr.eu)
  • Methods: The study aimed (1) to investigate the phenotypical and functional characteristics of Th17 and Tregs in SSc patients depending on disease manifestation (limited vs. diffuse cutaneous SSc, dcSSc) and activity, and (2) the transcriptional level and methylation status of Th17- and Treg-specific transcription factors. (uni-wuerzburg.de)
  • Squamous cell carcinoma variants of the genitourinary (GU) system 19. (sncrf.org)
  • Background: There is much evidence that T cells are strongly involved in the pathogenesis of localized and systemic forms of scleroderma (SSc). (uni-wuerzburg.de)
  • Two other small PEComa case series have since been published confirming the activity of sirolimus in this disease [9, 11]. (fortuneonline.org)
  • Patients received 100 mg/m 2 sirolimus protein-bound particles on Day 1 and Day 8 of each 21-day cycle until disease progression or unacceptable toxicity. (oncdata.com)
  • The recommended dose of sirolimus protein-bound particles is 100 mg/m 2 via intravenous infusion over 30 minutes on Day 1 and Day 8 of each 21-day cycle until disease progression or unacceptable toxicity. (oncdata.com)
  • The epithelioid cells were uniform and approximately the same size as the parabasal cells (Fig. 1 A&B), showing predominantly round or oval nuclei with finely stippled chromatin. (biomedcentral.com)
  • The term PEComa was launched by Zamboni et al[4] in 1996 to describe this rare family of morphological, immunohistochemical, and ultrastructural features. (cell-signaling-pathways.com)
  • PEComa occurring in the cervix is extremely rare, and very few cases have been reported till now. (biomedcentral.com)
  • PEComa of the gynecological tract is very rare. (biomedcentral.com)
  • PEComa is a rare disease in liver, pathologists need to recognize three components of this tumor to avoid misdiagnosing with malignant conditions. (medpharmres.com)
  • The neoplastic cells were positive for pan-CK (strong and diffuse), synaptophysin (strong and diffuse) [Figure 2], chromogranin (strong but focal) and inhibin (strong and diffuse) while negative for CK7, RCC, PAX-8, S-100, Melan-A (MART-1), and beta catenin. (pbpath.org)
  • Sections showed neoplastic cells arranged in nests/clusters and cords, surrounded by marked collagenized/fibrotic stroma [Figure 3]. (pbpath.org)
  • We present a case of rectum PEComa in a young male who in the beginning presented with an area of intratumoral hemorrhage. (cell-signaling-pathways.com)
  • Discussion and Conclusion: In our PEComa clinical case, we highlight how a multidisciplinary approach can lead to disease control and a long clinical benefit. (fortuneonline.org)
  • in 2010 reporting a series of 3 patients with advanced disease [10]. (fortuneonline.org)
  • Cytotoxic chemotherapies have limited benefit for patients with advanced disease. (oncdata.com)