Sclerosing Epithelioid Fibrosarcoma of the Kidney: First Reported Case in a Young Child

2021 ◽  
pp. 109352662097773
Author(s):  
Justin L Kurtz ◽  
Serena Y Tan ◽  
Florette K Hazard
Keyword(s):  
Clinical Course ◽  
Distant Metastases ◽  
Renal Tumors ◽  
Low Grade ◽  
Visceral Organ ◽  
Deep Soft Tissue ◽  
Sclerosing Epithelioid Fibrosarcoma ◽  
Patient Reported ◽  
Aggressive Clinical Course ◽  
Middle Aged Adults

Sclerosing epithelioid fibrosarcoma (SEF) is a rare variant of fibrosarcoma primarily arising in the deep soft tissue of the extremities and trunk. Despite having the morphologic appearance of a low-grade sarcoma, it generally has an aggressive clinical course with frequent local recurrences and distant metastases. It typically occurs in middle aged adults and is characterized by immunoexpression of MUC4 and recurrent gene fusions, most commonly EWSR1-CREB3L1. We report a primary renal SEF in a 4-year-old male. To our knowledge, this is the youngest patient reported with SEF and the second case of SEF in a pre-adolescent child. It is the eleventh reported case of primary renal SEF in the literature. While SEF arising in visceral organs is rare, the kidney is the most common primary site of any visceral organ. This case demonstrates SEF can occur in pre-adolescents, is an important consideration when evaluating sarcomas in young children, and should be considered in the differential diagnosis for primary renal tumors.

Malignant odontogenic myxoma of the maxilla: case with cytogenetic confirmation

2000 ◽  
Vol 114 (7) ◽  
pp. 533-535 ◽  
Author(s):  
Stefan Pahl ◽  
Wolfram Henn ◽  
Thomas Binger ◽  
Ute Stein ◽  
Klaus Remberger
Keyword(s):  
Cytogenetic Analysis ◽  
Clinical Course ◽  
Chromosome Complement ◽  
Low Grade ◽  
Odontogenic Myxoma ◽  
Cranial Cavity ◽  
Nuclear Pleomorphism ◽  
Histological Features ◽  
Benign Tumours ◽  
Aggressive Clinical Course

An odontogenic myxoma of the maxilla with an aggressive clinical course is presented. The tumour arose in a 53-year-old patient, recurred two times after extended maxillectomy and ultimately caused the patient’s death by uncontrollable local disease with infiltration of the cranial cavity.Microscopically, the tumour showed histological features of a low grade malignant myxosarcoma with cellular areas, enhanced mitotic activity and nuclear pleomorphism. Cytogenetic analysis revealed an unexpectedly aberrant hypertetraploid chromosome complement, that was considered as incompatible with the usual karyotypic patterns of benign tumours.

DIRECT RESULTS OF SURGICAL TREATMENT OF THREE-NEGATIVE BREAST CANCER

2020 ◽  
pp. 59-62
Author(s):  
Anna Vladimirovna Baranova
Keyword(s):  
Breast Cancer ◽  
Surgical Treatment ◽  
Lymph Nodes ◽  
Clinical Course ◽  
Radical Mastectomy ◽  
Distant Metastases ◽  
Wound Complications ◽  
Post Surgery ◽  
Aggressive Clinical Course ◽  
Direct Results

Three−negative breast cancer is characterized by aggressive clinical course, early metastasis and poor prognosis, leading to more active oncosurgical tactics and systemic treatment. Due to the aggressive clinical course of this oncopathology with increasing risk of locoregional and distant metastases, the most common surgical treatment tactics are modified variants of radical mastectomy with wide dissection of lymph nodes in areas of potential metastasis. Radicalism of the operation is the cause of the increase in the incidence of wound and lymphoenosis, the development of which depends on many factors, the determination of which is important for the development of methods of prevention of complications. In recent years, in the early stages of the disease, a radical breast resection after a course of adjuvant radiotherapy has been considered as an alternative. To study the immediate post−surgery results of three−negative breast cancer, depending on the initial clinical and pathological parameters and features of therapeutic tactics, a study was conducted in which 66 patients participated. Patients underwent radical mastectomy and breast resection, as well as lymph node dissection. Nine patients underwent aloplastic reconstruction. The results of the analysis showed that most often after radical surgeries, irrespective of their volume, there are lymphovenous complications, the development of which is affected by an increase in body mass index and lesions of lymph nodes. Aloplastic breast reconstruction contributes to a reduced incidence of lymphoid complications, and neoadjuvant chemotherapy is associated with an increased incidence of wound complications. These factors should be considered when planning the surgery protocols of patients with three negative breast cancer. Key words: three−negative breast cancer, surgical treatment, postoperative complications, risk factors.

scholarly journals Rare giant dermatofibrosarcoma protuberans with uncommon fibrosarcomatous transformation - case report and review of literature

2021 ◽  
Vol 8 (10) ◽  
pp. 3171
Author(s):  
Manisha Aggarwal ◽  
Dinesh Manchikanti ◽  
Sunayana Misra ◽  
Shaji Thomas ◽  
Ashish Arsia ◽  
...  
Keyword(s):  
Clinical Course ◽  
Dermatofibrosarcoma Protuberans ◽  
Distant Metastases ◽  
Metastatic Potential ◽  
Recurrence Risk ◽  
Skin Tumor ◽  
Rapid Progression ◽  
Split Skin Grafting ◽  
Aggressive Clinical Course ◽  
Slow Growing

Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue sarcoma with aggressive local behavior but with a low metastatic potential. Although slow growing and indolent, they rarely reach huge sizes. Very uncommonly, these locally invasive tumors undergo fibrosarcomatous transformation with a more aggressive clinical course, with higher rate of recurrence risk and distant metastases. A 32-years-old lady, presented with a gradually progressive lump in the upper central back for the past 6 years, with rapid progression in size during the last 6 months. On examination, she had a single lump of size 18×18 cm in the midline of the upper back, with prominent veins over its surface. Magnetic resonance imaging (MRI) showed no connection with the spinal canal and appeared flush with the paraspinal muscles. Core needle biopsy showed DFSP. The patient underwent a wide local excision with split skin grafting. The histopathology now showed a fibrosarcomatous transformation of DFSP. The patient again underwent a wide re-excision with a 3 cm margin. Histology reported no evidence of tumor cells in the specimen. The patient’s postoperative period was uneventful and she was referred for adjuvant radiotherapy. DFSP is a rare, slow-growing malignant fibroblastic mesenchymal skin tumor with low metastatic potential. However, in any patient with long standing DFSP with a recent increase in size, this fibrosarcomatous transformation must be kept in mind as it represents an uncommon form of DFSP that tends to follow a more aggressive clinical course, with higher rate of recurrence risk and distant metastases.

MYC/BCL2/BCL6 triple hit lymphoma: A study of 33 patients who had an aggressive clinical course similar to patients with double hit lymphomas.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 7559-7559
Author(s):  
Shaoying Li ◽  
Wenting Huang ◽  
Yasuhiro Oki ◽  
L Jeffrey Medeiros
Keyword(s):  
B Cell ◽  
De Novo ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Low Grade ◽  
Significant Difference ◽  
History Of ◽  
Double Hit ◽  
Aggressive Clinical Course

7559 Background: Large B cell lymphomas with MYC, BCL2, and BCL6 rearrangements, designated as triple hit lymphoma (THL), are uncommon. Large series studies of THL are scant and studies comparing THL to different types of double hit lymphoma (DHL) are lacking. Methods: We studied the clinicopathologic features and prognosis of 33 patients with THL and compared them to 83 patients with MYC/BCL2 DHL and 13 patients with MYC/BCL6DHL. Results: There were 21 men and 12 women, with a median age of 63 years (range, 34-85). Six patients had a history of low-grade B cell lymphoma and 27 had de novo lymphoma. These tumors were classified histologically as: 21 DLBCL, 10 high grade B-cell lymphoma, one concurrent DLBCL and follicular lymphoma (FL), and one concurrent DLBCL and mantle cell lymphoma. Immunohistochemical analysis showed that these tumors were positive for CD10 (94%), BCL6 (80%), BCL2 (93%), and MYC (69%, 40% as cutoff). 62% of tumors (8/13) with available data showed coexpression of MYC and BCL2. Using the Hans algorithm, 30 of 33 (91%) tumors had a germinal center B cell like (GCB) immunophenotype. All 7 cases tested by conventional cytogenetics showed a complex karyotype. Although BCL2 was always translocated with IGH, BCL6 translocated to MYC in 2of 7 cases of THL. Twenty-nine patients had treatment information available and all received immune-chemotherapy induction, including 11 with R-CHOP, 14 with R-EPOCH, 3 with R-HyperCVAD, and one with RICE (patient had a history of FL). The clinicopathological features of THL including induction chemotherapy were very similar to both the MYC/BCL2 DHL and MYC/BCL6 DHL (all P>0.05). There was no significant difference in median overall survival (OS) between patients with de novo lymphoma and those with a history of low-grade lymphoma (P=0.99). The OS in THL patients was 17.9 months, similar to the OS (17.2 months) of patients with MYC/BCL6 DHL and those with MYC/BCL2DHL (19.9 months) (P=0.60). Conclusions: MYC/BCL2/BCL6 THL is an aggressive B cell lymphoma and >90% of cases have a GCB immunophenotype. THL patients usually have an aggressive clinical course and a poor prognosis, similar to patients with double hit lymphomas.

Metastatic Sclerosing Epithelioid Fibrosarcoma in Bone Marrow

2017 ◽  
Vol 25 (8) ◽  
pp. 702-704 ◽  
Author(s):  
Rana Asakra ◽  
Shane Zaidi ◽  
Khin Thway
Keyword(s):  
Bone Marrow ◽  
Soft Tissues ◽  
Low Grade ◽  
Diffuse Infiltration ◽  
Metastatic Site ◽  
Sclerosing Epithelioid Fibrosarcoma ◽  
Eosinophilic Cytoplasm ◽  
Fibromyxoid Sarcoma ◽  
Middle Aged Adults ◽  
Hematolymphoid Neoplasms

Sclerosing epithelioid fibrosarcoma (SEF) is an aggressive neoplasm thought to be related to low-grade fibromyxoid sarcoma, which typically occurs in middle-aged adults in the deep soft tissues of the lower extremities and trunk. It comprises nests and cords of relatively uniform epithelioid polygonal cells with clear or eosinophilic cytoplasm in densely sclerotic stroma, and it is typically associated with EWSR1 gene rearrangements, and most commonly EWSR1-CREB3L1 fusions. As primary SEF can arise in bone, and bone is also a common metastatic site for SEF, its recognition at this site is important. We illustrate bone marrow showing diffuse infiltration by SEF and highlight the potential for confusion with a range of neoplasms such as carcinoma, hematolymphoid neoplasms, and other sarcomas.

Thyroid-Like Follicular Carcinoma of the Kidney With Low-Grade Sarcomatoid Component: A Hitherto Undescribed Case

2020 ◽  
pp. 106689692094040
Author(s):  
Vidya Rao ◽  
Santosh Menon ◽  
Ganesh Bakshi ◽  
Gagan Prakash ◽  
Archi Agarwal ◽  
...  
Keyword(s):  
Unusual Case ◽  
Spindle Cell ◽  
Clinical Course ◽  
Single Case ◽  
Follicular Carcinoma ◽  
Low Grade ◽  
Cell Component ◽  
Spindle Cell Component ◽  
Regional Node ◽  
Aggressive Clinical Course

Thyroid-like follicular carcinoma of the kidney (TLFCK) is a rare subtype of renal cell carcinoma, which closely resembles follicular neoplasms of the thyroid and has a distinctive indolent clinical behavior. Until now, a single case of TLFCK with extensive sarcomatoid differentiation has been documented with aggressive clinical course. We present an unusual case of sarcomatoid TLFCK with a low-grade spindle cell component in a 34-year-old male patient, with an indolent course following radical nephrectomy and regional node dissection.

scholarly journals Proximal-type epithelioid sarcoma - Case report

2013 ◽  
Vol 88 (3) ◽  
pp. 444-447 ◽  
Author(s):  
Luciana Mendes dos Santos ◽  
Lisiane Nogueira ◽  
Christiane Yuri Matsuo ◽  
Carolina Talhari ◽  
Monica Santos
Keyword(s):  
Young Adults ◽  
Case Report ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Distant Metastasis ◽  
Clinical Course ◽  
Epithelioid Sarcoma ◽  
Aggressive Clinical Course ◽  
Middle Aged Adults ◽  
Rhabdoid Features

Epithelioid sarcoma, first described by Enzinger in 1970, is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. In 1997, Guillou et al. described a different type of epithelioid sarcoma, called proximal-type epithelioid sarcoma, which is found mostly in the pelvic and perineal regions and genital tracts of young to middle-aged adults. It is characterized by a proliferation of epithelioid-like cells with rhabdoid features and the absence of a granuloma-like pattern. In this paper we present a case of proximal-type epithelioid sarcoma with an aggressive clinical course, including distant metastasis and death nine months after diagnosis.

Primary Renal Hybrid Low-grade Fibromyxoid Sarcoma-Sclerosing Epithelioid Fibrosarcoma: An Unusual Pediatric Case With EWSR1-CREB3L1 Fusion

2018 ◽  
Vol 21 (6) ◽  
pp. 574-579 ◽  
Author(s):  
Yingting Mok ◽  
Yin Huei Pang ◽  
Jain Sudhanshi Sanjeev ◽  
Chik Hong Kuick ◽  
Kenneth Tou-En Chang
Keyword(s):  
Fusion Gene ◽  
Genetic Profile ◽  
Low Grade ◽  
Gene Fusions ◽  
Gene Detection ◽  
Sclerosing Epithelioid Fibrosarcoma ◽  
Fibromyxoid Sarcoma ◽  
Exon 11 ◽  
Aggressive Clinical Course ◽  
Generation Sequencing

Low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF) are rare tumors with distinct sets of morphological features, both characterized by MUC4 immunoreactivity. Tumors exhibiting features of both entities are considered hybrid LGFMS-SEF lesions. While the majority of LGFMS cases are characterized by FUS-CREB3L2 gene fusions, most cases of pure SEF show EWSR1 gene rearrangements. In the largest study of hybrid LGFMS-SEF tumors to date, all cases exhibited FUS rearrangements, a similar genetic profile to LGFMS. We herein describe the clinicopathological features and genetic findings of a case of primary renal hybrid LGFMS-SEF occurring in a 10-year-old child, with disseminated metastases. Fusion gene detection using a next-generation sequencing-based anchored multiplex PCR technique (Archer FusionPlex Sarcoma Panel) was performed on both the primary renal tumor that showed the morphology of a LGFMS, and a cervical metastasis that showed the morphology of SEF. An EWSR1-CREB3L1 gene fusion occurring between exon 11 of EWSR1 and exon 6 of CREB3L1 was present in both the LGFMS and SEF components. This unusual case provides evidence that a subset of hybrid LGFMS-SEF harbor EWSR1-CREB3L1 gene fusions. In this case, these features were associated with an aggressive clinical course, with disease-associated mortality occurring within 12 months of diagnosis.

scholarly journals The DEAD/DEAH Box Helicase, DDX11, Is Essential for the Survival of Advanced Clear Cell Renal Cell Carcinoma and Is a Determinant of PARP Inhibitor Sensitivity

Cancers ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 2574
Author(s):  
Jee Soo Park ◽  
Myung Eun Lee ◽  
Won Sik Jang ◽  
Koon Ho Rha ◽  
Seung Hwan Lee ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Quantitative Polymerase Chain Reaction ◽  
Parp Inhibitor ◽  
Renal Tumors ◽  
Low Grade ◽  
Normal Kidney ◽  
The Dead ◽  
The Impact

Genes associated with the DEAD-box helicase DDX11 are significant biomarkers of aggressive renal cell carcinoma (RCC), but their molecular function is poorly understood. We analyzed the molecular pathways through which DDX11 is involved in RCC cell survival and poly (ADP-ribose) polymerase (PARP) inhibitor sensitivity. Immunohistochemistry and immunoblotting determined DDX11 expression in normal kidney tissues, benign renal tumors, and RCC tissues and cell lines. Quantitative polymerase chain reaction validated the downregulation of DDX11 in response to transfection with DDX11-specific small interfering RNA. Proliferation analysis and apoptosis assays were performed to determine the impact of DDX11 knockdown on RCC cells, and the relevant effects of sunitinib, olaparib, and sunitinib plus olaparib were evaluated. DDX11 was upregulated in high-grade, advanced RCC compared to low-grade, localized RCC, and DDX11 was not expressed in normal kidney tissues or benign renal tumors. DDX11 knockdown resulted in the inhibition of RCC cell proliferation, segregation defects, and rapid apoptosis. DDX11-deficient RCC cells exhibited significantly increased sensitivity to olaparib compared to sunitinib alone or sunitinib plus olaparib combination treatments. Moreover, DDX11 could determine PARP inhibitor sensitivity in RCC. DDX11 could serve as a novel therapeutic biomarker for RCC patients who are refractory to conventional targeted therapies and immunotherapies.

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