Epithelioid Sarcoma: A Puzzling Soft Tissue Neoplasm in a Child

Dermatology ◽  
2000 ◽  
Vol 200 (2) ◽  
pp. 179-180 ◽  
Author(s):  
A. Theunis ◽  
J. André ◽  
D. Larsimont ◽  
M. Song
2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S58-S58
Author(s):  
S Zaheer ◽  
K Xu ◽  
F Shakil

Abstract Introduction/Objective Epithelioid Sarcoma is a rare malignant soft tissue neoplasm that is well known for local recurrence, regional lymph node involvement, and metastasis. It accounts for less than one percent of all sarcomas. Two subtypes of epithelioid sarcoma are described: the conventional or classic subtype and the proximal subtype, with the classic type being more common than the proximal type. We present a case of proximal epithelioid sarcoma involving the inguinal region, in which the patient presented with the history of weight loss for one year and a slow- growing inguinal mass for the last few months. The biopsy was done in an outside hospital and showed carcinoma of unknown origin. Imaging also failed to reveal any clear source of this mass. Methods The mass was resected and sent to pathology, where H&E staining and immunohistochemistry was done for diagnosis. Results The H&E sections of the left inguinal mass showed proliferation of large epithelioid cells with a moderate amount of cytoplasm, vesicular nuclear chromatin and prominent nucleoli. Mitosis was also seen. Necrosis was not identified. Immunohistochemical staining was done and revealed that the tumor cells were immunoreactive for epithelial marker cytokeratin AE1/AE3 and showed loss of INI-1. Tumor cells were also negative for Myogenin, ERG, HMB-45, MART-1, Myo-D1, S100, and SMA. Considering the cytomorphology and immunophenotypic findings the diagnosis of epithelioid sarcoma, proximal type was made. Conclusion Epithelioid sarcoma is a malignant mesenchymal neoplasm with epithelioid cytomorphology and phenotype. Among the two types of epithelioid sarcoma, the proximal type is reported about 50% less than the classic type and is associated with aggressive behavior and worse prognosis. The classic cytomorphology and immunohistochemistry findings helped us in making the diagnosis in this case.


Author(s):  
Filippo Boriani ◽  
Edoardo Raposio ◽  
Costantino Errani

: Musculoskeletal tumors of the hand are a rare entity and are divided into skeletal and soft tissue tumors. Either category comprises benign and malignant or even intermediate tumors. Basic radiology allows an optimal resolution of bone and related soft tissue areas, ultrasound and more sophisticated radiologic tools such as scintigraphy, CT and MRI allow a more accurate evaluation of tumor extent. Enchondroma is the most common benign tumor affecting bone, whereas chondrosarcoma is the most commonly represented malignant neoplasm localized to hand bones. In the soft tissues ganglions are the most common benign tumors and epithelioid sarcoma is the most frequently represented malignant tumor targeting hand soft tissues. The knowledge regarding diagnostic and therapeutic management of these tumors is often deriving from small case series, retrospective studies or even case reports. Evidences from prospective studies or controlled trials are limited and for this lack of clear and supported evidences data from the medical literature on the topic are controversial, in terms of demographics, clinical presentation, diagnosis prognosis and therapy.The correct recognition of the specific subtype and extension of the tumor through first line and second line radiology is essential for the surgeon, in order to effectively direct the therapeutic decisions.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 11547-11547
Author(s):  
Jiayong Liu ◽  
Zhengfu Fan ◽  
Wei Guo ◽  
Tian Gao ◽  
Shu Li ◽  
...  

11547 Background: Anlotinib, a multitargeted tyrosine kinase inhibitor, had been prove to be effective for the treatment of advanced or metastatic soft tissue sarcoma(STS) faild anthracycline chemotherapy. With the lack of prospective data of combination of PDL-1 inhibitor and antiangiogenic agent, we designed a phase 1b study to investigated the efficacy and safety of anlotinib plus TQB2450 in patients with STS. Methods: Eligible patients (age 18-70, ECOG 0-1, with histopathologically confirmed advanced STS, at least one measurable lesion according to RECIST 1.1, and previously received front-line anthracycline chemotherapy) were included and received anlotinib (12mg qd, D1-14, 21d/cycle) plus TQB2450 (1200 mg, IV, D1, 21d/cycle) until disease progression or intolerable toxicities.The primary endpoint was objective response rate (ORR), secondary endpoints included safety, overall survival (OS), progression-free survival (PFS), disease control rate (DCR). Results: From January 2019 to January 2021, 30 pts were enrolled1, 12 alveolar soft part sarcoma and 18 others (7 synovial sarcoma, 4 leiomyosarcoma, 5 undifferentiated pleomorphic sarcoma, 1 fibrosarcoma and 1 epithelioid sarcoma). ORR by RECIST was 36.7%, DCR was 83.3%, 11/30 pts had PR, 14/30 (46.7%) had SD, 5/30 (16.7%) PD. Median PFS was 9.6 m in all pts and 4.9m. in non-ASPS, respectively. Median OS in non-ASPS was 10.27m, while mOS in all pts and both mPFS and mOS in ASPS had not been reached. Notably, to ASPS pts, ORR was 75%, and DCR was 100%. The most common 1-2 grade treatment-related adverse reaction (TRAE) was hypothyroidism (19/30,63.3%),hypercholesterolemia (16/30, 53.3%) and hypertriglyceridemia (16/30, 53.3%), the most common ≥3 grade TRAEs were hypertriglyceridemia (3/30, 10%). 6 SAE (20%) occurred, including 2 pneumothorax, 1 Immune associated hapatic injury, 1 hypotension, 1 Immune myocarditis and 1 diabetic ketoacidosis. Conclusions: The combination of anlotinib and TQB2450 showed promising activity in second-line treatment of advanced STS, especially in ASPS, with well tolerance and acceptable toxicity.


1989 ◽  
Vol 20 (4) ◽  
pp. 347-351 ◽  
Author(s):  
W.M. Molenaar ◽  
B. DeJong ◽  
A. Dam-Meiring ◽  
A. Postma ◽  
J. DeVries ◽  
...  

2020 ◽  
Vol 7 (4) ◽  
pp. 179-184
Author(s):  
Sam Hajialiloo Sami ◽  
◽  
Farshad Zandrahimi ◽  
Mohamadreza Heidarikhoo ◽  
Mahsa Zahmatkesh ◽  
...  

Infantile fibrosarcoma is a rare soft-tissue neoplasm, which may render a diagnostic challenge leading to misdiagnosis and consequently an inappropriate treatment of patients. This study reports a case of infantile fibrosarcoma that mimicked a hemangioma in an 11-month-old girl. As the lesion signal in the MRI was not consistent with the diagnosis of hemangiomas, we performed a core needle biopsy, which its result was consistent with the diagnosis of infantile fibrosarcoma. The lesion was initially treated with surgical resection. However, the lesion recurred one year after the surgery. The recurrence was managed with debulking surgery. The fifth finger was necrotized during the hospitalization after the relapse surgery. Finally, the necrotic finger was amputated. Also, adjuvant chemotherapy was used to prevent further relapses. The 1-year follow-up of the patient was recurrence-free. These findings highlight the importance of considering infantile fibrosarcoma when an infant presents with a lesion that clinically mimics a vascular lesion.


Medicina ◽  
2021 ◽  
Vol 57 (8) ◽  
pp. 830
Author(s):  
Wei-Che Lin ◽  
Yi-Fan Tai ◽  
Meng-Hsiang Chen ◽  
Sheng-Dean Luo ◽  
Faye Huang ◽  
...  

Background and Objective: To evaluate the effectiveness of radiofrequency ablation (RFA) using the moving-shot technique for benign soft tissue neoplasm. Materials and Methods: This retrospective study reviewed eight patients with benign soft tissue neoplasm presenting with cosmetic concerns and/or symptomatic issues who refused surgery. Six patients had vascular malformation, including four with venous malformation and two with congenital hemangioma. The other two patients had neurofibroma. All patients underwent RFA using the moving-shot technique. Imaging and clinical follow-up were performed in all patients. Follow-up image modalities included ultrasound (US), computed tomography (CT), and magnetic resonance (MR) imaging. The volume reduction ratio (VRR), cosmetic scale (CS), and complications were evaluated. Results: Among the seven patients having received single-stage RFA, there were significant volume reductions between baseline (33.3 ± 21.2 cm3), midterm follow-up (5.1 ± 3.8 cm3, p = 0.020), and final follow-up (3.6 ± 1.4 cm3, p = 0.022) volumes. The VRR was 84.5 ± 9.2% at final follow-up. There were also significant improvements in the CS (from 3.71 to 1.57, p = 0.017). The remaining patient, in the process of a scheduled two-stage RFA, had a 33.8% VRR after the first RFA. The overall VRR among the eight patients was 77.5%. No complications or re-growth of the targeted lesions were noted during the follow-up period. Of the eight patients, two received RFA under local anesthesia, while the other six patients were under general anesthesia. Conclusions: RFA using the moving-shot technique is an effective, safe, and minimally invasive treatment for benign soft tissue neoplasms, achieving mass volume reduction within 6 months and significant esthetic improvement, either with local anesthesia or with general anesthesia under certain conditions.


2021 ◽  
Vol 33 (3) ◽  
pp. 113-118
Author(s):  
Chiharu Ogawa ◽  
Shin Usuda ◽  
Hidetaka Miyashita ◽  
Seiji Asoda ◽  
Ikuhiro Uchida ◽  
...  

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