13 research outputs found

    Morphologic evaluation of the effect of denosumab on giant cell tumors of bone and a new grading scheme

    No full text
    Giant cell tumor (GCT) is a rare, usually benign but locally aggressive neoplasm. Recent studies suggest new approaches in light of the elucidation of molecular pathways in bone. The osteolytic nature of GCT is caused by the receptor for activating nuclear factor-kB ligand (RANKL) associated osteoclasts. Denosumab is a monoclonal antibody that affects GCT through RANKL and it prevents normal and neoplastic osteolysis. The aim of this study is to evaluate the histopathologic alterations due to denosumab treatment and the efficiency of this drug in GCT therapy. Ten patients had been treated with denosumab and were included in the study. Pretreatment biopsies were interpreted as conventional GCTs and posttreatment biopsies of the ten patients’ GCTs were classified in accordance with the grading system. Only one patient had tumor remaining after treatment. There is limited data on histopathologic alterations that follow denosumab treatment. The bone pathologist should keep these changes in mind because they mimic different types of bone tumors. Furthermore, there is no widely accepted grading system to evaluate the effect of denosumab in GCT. Our study suggested a scheme that would be helpful to evaluate the efficiency of denosumab treatment in GCT

    Action Myoclonus and Renal Parenchymal Disease: A Case Report

    No full text
    WOS: 000390945000007Progressive myoclonic epilepsies are a devastating group of rare disorders. The case of a young man who presented with a late-diagnosed nephrotic syndrome, progressive myoclonic seizures with action myoclonus, is described in the present report. He was the fifth child of a consanguineous marriage, and there was no familial history of nephrotic syndrome or end-stage renal failure. Renal biopsy showed focal segmental glomerulosclerosis with collapsing glomerulopathy. The patient died at 25 years of age in a context of end-stage renal function consistent with nephrotic syndrome

    Multifocal Epitheloid Hemangioendothelioma: A Case Report

    No full text
    Epitheloid hemangioendothelioma (EHE) is a rare vascular tumor with malignant biological behavior. It arises from vascular endothelial cells, usually within soft tissues, and can occur in almost all locations, but tumor can be found in liver, lungs, bone and skin. It is considered to be a low or borderline malignant tumor with, usually, slow progression, but aggressive forms have been described. We present a 24 year-old female case of multifocal epitheloid hemangioendothelioma of the soft tissue with its clinical, radiological and histopathological findings [Cukurova Med J 2014; 39(2.000): 383-386

    Therapy-Induced Neural Differentiation in Ewing's Sarcoma: A Case Report and Review of the Literature

    No full text
    WOS: 000466572300008PubMed ID: 28272685Ewing's sarcoma (ES) is a small round cell tumor of adolescents or young adults that usually arises in the deep soft tissues of the extremities. The tumor cells have uniform round nuclei, fine powdery chromatin and indistinct nucleoli. CD99 (O13) is a product of the MIC 2 gene that is highly sensitive to ES but not specific. A panel of markers should be used for the differential diagnosis of small round cell tumors because nearly all others, on occasion, show membranous staining for CD99. One of the defining feature of ES is the presence of 22q12 gene rearrangement. The presented case is a 6 year-old boy complaining of swelling on his right leg. The biopsy was compatible with classic ES in terms of histopathological, immunohistochemical and cytogenetic criteria. Wide surgical resection was performed after chemotherapy. The posttreatment specimen was composed of uniformly small round cells mixed with areas of ganglion cells embedded in neurophil-like fibrillary background. Immunohistochemically, neoplastic cells revealed strong CD99 (O13) and NSE staining and the tumor had EWSR1 gene rearrangement. Morphologic alterations due to treatment are commonly seen in pediatric tumors. Single case reports have defined neural differentiation in ES but to the best of our knowledge this is the first report of ES in the literature with all histopathological, immunohistochemical, and cytogenetic criteria evaluated in both pretreatment and posttreatment specimens

    Evaluation of epidermal growth factor receptor in odontogenic tumors and rare soft tissue tumors by immunohistochemical and fluorescence in situ hybridization methods

    No full text
    Purpose: The aim of this study is to investigate the role of epidermal growth factor receptor (EGFR) in odontogenic tumors and rare soft tissue tumors. Material and Methods: This study includes 28 cases (11 cases of odontogenic tumors and 17 cases of soft tissue tumors) which were diagnosed at Cukurova University Medical Faculty, Department of Pathology between 2002 and 2010. They were evaluated for immunohistochemical staining of EGFR and the presence of genetic abnormality by FISH method. Results: Eleven cases were positively stained by immunohistochemistry. Eight cases had genetic abnormality of EGFR gene by FISH method. Nine of eleven odontogenic tumors (81,8%) showed overexpression of EGFR by immunohistochemical method or FISH method. Conclusion: Our findings highlight that EGFR plays an important role in chordoma pathogenesis in soft tissue tumors. In addition to odontogenic tumors; two cases of vascular neoplasia, composite and retiform hemangioendothelioma, exhibited EGFR gene amplification. Interestingly, there is no avaliable data about EGFR gene status in these tumors. EGFR can be used for diagnostic purposes. Also, it is a target for anti-EGFR therapy. Therefore, EGFR gene status is a valuable marker for therapeutic management. [Cukurova Med J 2016; 41(2.000): 316-325

    A Giant Aggressive Angiomyxoma of the Pelvis Misdiagnosed as Incarcerated Femoral Hernia: A Case Report and Review of the Literature

    No full text
    Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions. AA is a locally infiltrative slow growing tumor with a marked tendency to local recurrence. Painless swelling located around the genitofemoral region is the common symptom; thus, it is often misdiagnosed as a gynecological malignancy or a groin hernia. A 35-year-old female patient who previously underwent surgery for left femoral hernia operation resulting in surgical failure was reoperated for a giant AA located in the pelvis. The tumor was completely excised with free margins. Histopathologic examination revealed an AA. The tumor size was measured as 24 × 12 × 6 cm with a weight of 4.2 kg. Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor. S100, MUC4, CD34, and SMA were negative in the tumor cells. AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The principle treatment should be complete surgical excision with tumor-free margins. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence
    corecore