scholarly journals Fourth Primary Malignant Tumor in a Patient with Possible Li-Fraumeni Syndrome: Synchronous Diagnosis of Postirradiation Sarcoma, Cutaneous Relapse of a Previous Soft Tissue Sarcoma, and Lung Adenocarcinoma

2014 ◽  
Vol 2014 ◽  
pp. 1-6
Author(s):  
Feridun Yumrukçal ◽  
Yalin Dirik ◽  
Arda Çinar ◽  
Levent Eralp
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Lung Adenocarcinoma ◽  
Young Patient ◽  
Malignant Tumor ◽  
Malignant Tumors ◽  
Rare Entity ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni ◽  
Postirradiation Sarcoma

We present a 46-year-old female patient who is diagnosed with synchronous postirradiation sarcoma, cutaneous relapse of a previous soft tissue sarcoma, and lung adenocarcinoma. More than one malignant tumor at the same time with an accompanying relapse of a previous malignant tumor is a rare entity. A relatively young patient diagnosed with adenocarcinoma of the urethra before age 40, which is an unusual tumor for that age, later three more different malignant tumors being diagnosed, two of which are synchronous, causes the suspicion of Li-Fraumeni syndrome.

EGFR-mutant lung adenocarcinoma in a patient with Li-Fraumeni syndrome

2007 ◽  
Vol 8 (6) ◽  
pp. 559-560 ◽  
Author(s):  
Lynne T Bemis ◽  
William A Robinson ◽  
Robert McFarlane ◽  
Elizabeth Buyers ◽  
Karen Kelly ◽  
...  
Keyword(s):  
Lung Adenocarcinoma ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni ◽  
Egfr Mutant

scholarly journals Clinicopathological study of soft tissue sarcoma-retrospective study of tertiary cancer institute in eastern India

2020 ◽  
Vol 8 (11) ◽  
pp. 4075
Author(s):  
Kunhi Mohammed K. P. ◽  
Snehasis Pradhan ◽  
Supratim Bhattacharyya ◽  
Prafulla Kumar Das ◽  
Muhammed Navas N. K.
Keyword(s):  
Retrospective Study ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Malignant Tumors ◽  
Soft Tissue Sarcomas ◽  
The Body ◽  
Female Ratio ◽  
Pleomorphic Sarcoma ◽  
Frequent Variety ◽  
Male To Female

Background: Soft tissue sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1 percent of all adult malignancies. Although they occur anywhere in the body, they involve most commonly in extremities, trunk, retroperitoneum and head and neck. The aim of the study was to analyze clinical and histopathological features of various soft tissue sarcomas.Methods: This was a retrospective study, conducted in tertiary cancer centre in Odisha during the period 2015 to 2018. We collected clinical parameters like age, sex, site of swelling, any associated pain and biopsy reports and these variables were correlated with final histopathology reports.Results: A total of 107 patients were included in the study, with male to female ratio of 2:1(71 and 36) and average age of 43.45 years. All of them presented with a swelling. The lower extremities were the most common sites i.e. 44.62%. Pleomorphic sarcoma was the most frequent histologic variety comprising 43% and less frequent variety were angiosarcoma, and myxoid sarcoma.Conclusions: Soft tissue sarcoma are predominant in males and middle aged population are frequently affected. Most common affected site is lower extremity and pleomorphic sarcoma is the prominent histologic type.

A pan-cancer study on characteristic of CDK4/6 amplification between Chinese and Western patients.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e15074-e15074
Author(s):  
Yamin Zhang ◽  
Zilin Cui ◽  
Rui Shi ◽  
Xiaolong Liu ◽  
Yang Li ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Liver Cancer ◽  
Soft Tissue Sarcoma ◽  
Lung Adenocarcinoma ◽  
Ovarian Carcinoma ◽  
Cancer Patients ◽  
Stomach Carcinoma ◽  
Chinese Cohort ◽  
Cancer Types ◽  
Pan Cancer

e15074 Background: CDK4/6 kinases associate with cyclin D proteins during transition from G1 to S phase of the cell cycle. Amplification of CDK4/6 may elicit the activity of cyclin D, which hyperphosphorylates RB, ultimately leading to uncontrolled cell proliferation. Currently, three CDK4/6 inhibitors are used in breast cancer, ovarian cancer and sarcoma. Herein, we investigate the prevalence of CDK4/6 amplification in Chinese and Western cancer patients, hope to find more cancer subtypes with CDK4/6 amplification. Methods: Next-generation sequencing data and clinical data were collected from 10828 TCGA pan-cancer patients (Western cohort). A 539-gene panel targeted sequencing assay was performed on FFPE tumor samples from 4181 Chinese pan-cancer patients (Chinese cohort). CDK4 and CDK6 amplification were calculated on the two cohorts following the same criteria. Results: In total, 182 (4.4%) of the 4181 Chinese patients and 529 (4.9%) of the 10828 Western patients had CDK4 amplification, 133 (3.2%) of the 4181 Chinese patients and 475 (4.4%) of the 10828 Western patients had CDK6 amplification. In Western cohort, the top 5 CDK4 amplification-associated cancer types were sarcoma, glioblastoma multiforme, lung adenocarcinoma, ovarian carcinoma, and adrenocortical carcinoma, and the top 5 CDK6 amplification-associated cancer types were esophageal carcinoma, ovarian carcinoma, lung squamous cell carcinoma, stomach adenocarcinoma, sarcoma. In Chinese cohort, the top 5 CDK4 amplification-associated cancer types were lung adenocarcinoma, melanoma, sarcoma, stomach carcinoma, liver cancer, and the top 5 CDK6 amplification-associated cancer types were lung adenocarcinoma, stomach carcinoma, liver cancer, melanoma, glioma. In addition, CDK4 amplification in Chinese cohort, 22 (11%) of the 203 Chinese bone and soft tissue sarcoma patients had CDK4 amplification, and 4 (2%) of the 203 had CDK6 amplification. Bone and soft tissue sarcoma types with CDK4 / 6 amplification including soft tissue sarcoma, bone cancer, fibrosarcoma, chondrosarcoma, rhabdomyosarcoma, liposarcoma, synovial sarcoma. Conclusions: Our study provided a characteristic of CDK4/6 amplification in Chinese and Western pan-cancer patients. Analysis revealed frequent CDK4 / 6 amplification in lung cancer, sarcoma, stomach carcinoma, ovarian carcinoma and liver cancer. It is suggested patient with these cancer types may potentially benefit from CDK4/6 inhibitor.

Evaluation of a Soft Tissue Mass in the Extremity or Trunk

2018 ◽  
Author(s):  
Daniel C Thomas ◽  
Dale Han
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Physical Examination ◽  
Core Needle Biopsy ◽  
Needle Biopsy ◽  
Malignant Tumors ◽  
Soft Tissue Mass ◽  
Tissue Mass ◽  
Core Needle ◽  
Needle Tract

Soft tissue sarcoma (STS) represents a diverse group of malignant tumors derived from mesenchymal elements. Evaluation of a soft tissue mass of the trunk or extremity starts with a detailed history and physical examination, which will help guide further diagnostic evaluation.  Imaging should then be obtained for an STS, with MRI being the preferred modality for the trunk or extremities. Imaging can assist with diagnosis and delineate the extent of the primary lesion, including the relationship to contiguous structures, for treatment planning and assessing resectability. Pathology diagnosis is most commonly performed via examination of a specimen obtained through image-guided percutaneous core-needle biopsy. This requires multidisciplinary planning between Surgery and Radiology to place the needle within the anticipated definitive surgical incision to allow for en bloc resection of the needle tract. Once the diagnosis of an STS is made, appropriate staging studies should be performed based on the history and physical examination and tumor histology, grade, and size. Clinical and pathology data are used to stage STS patients using the American Joint Committee on Cancer staging system, which incorporates the STS site, size and grade of the primary tumor, and absence or presence of nodal and distant metastatic disease. This review contains 6 figures, 6 tables and 50 references Key words: core-needle biopsy, extremity, histology, imaging, risk factors, soft tissue mass, soft tissue sarcoma, staging evaluation, trunk, tumor grade

Article Commentary: Soft Tissue Sarcoma of the Extremity and Trunk: Issues for the General Surgeon

2006 ◽  
Vol 72 (8) ◽  
pp. 665-671
Author(s):  
Gerame Wells ◽  
Robert C.G. Martin ◽  
Kelly M. Mcmasters ◽  
Charles R. Scoggins
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Current Literature ◽  
Medical Literature ◽  
Malignant Tumors ◽  
Soft Tissue Sarcomas ◽  
Heterogeneous Group ◽  
General Surgeon ◽  
Academic Interest ◽  
General Surgeons

Soft tissue sarcomas represent a heterogeneous group of malignant tumors that arise from mesenchymal tissues. The majority of these tumors arise on the extremity or trunk. Despite their rarity, soft tissue sarcomas continue to generate vigorous academic interest, and as a result, the ever-expanding medical literature dealing with sarcomas continues to grow. Many general surgeons will see few of these tumors during their careers, and a review of the current literature and how it applies to patients afflicted with soft tissue sarcoma of the extremity or trunk is warranted.

Combination chemotherapy using adriamycin, DTIC, cyclophosphamide, and actinomycin D for advanced soft tissue sarcomas: a randomized comparative trial. A phase III, Southwest Oncology Group Study (7613).

1987 ◽  
Vol 5 (6) ◽  
pp. 851-861 ◽  
Author(s):  
L H Baker ◽  
J Frank ◽  
G Fine ◽  
S P Balcerzak ◽  
R L Stephens ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Actinomycin D ◽  
Malignant Tumors ◽  
Soft Tissue Sarcomas ◽  
Comparative Trial ◽  
Phase Iii ◽  
Oncology Group ◽  
Southwest Oncology Group ◽  
Significant Difference

The term soft tissue sarcoma refers to a large variety of malignant tumors arising in extraskeletal connective tissues that connect, support, and surround discrete anatomic structures. All visceral organs also contain a connective stroma that can undergo malignant transformation. Because of the histological similarities of this group of tumors and their relative rarity, treatment prescriptions for patients that have disseminated disease are most often uniform. In this study, we asked the question whether adding a third drug (cyclophosphamide or actinomycin D) to Adriamycin (Adr [Adria Laboratories, Columbus, OH])-(3,3-dimethyl-1-triazeno)- imidazole-4-carboxamide (DTIC) would improve the response rate and/or survival. A unique feature of this cooperative group clinical trial was the mandatory pathology review of the histological material. All patients of the Southwest Oncology Group between June 1, 1976, and November 17, 1979, who had a biopsy-confirmed diagnosis of a soft tissue sarcoma with convincing clinical or biopsy-documented evidence of metastatic disease were eligible for the study. Patients were randomized to receive (1) Adr, 60 mg/m2 intravenously, day 1, and DTIC, 250 mg/m2 every 3 weeks (104 patients); (2) Adr and DTIC as in (1) and cyclophosphamide, 500 mg/m2, day 1 (112 patients); or (3) Adr and DTIC as in (1) and actinomycin D, 1.2 mg/m2, day 1, (119 patients). There was no statistically significant difference in response rates (33%, 34%, and 24%) (P = .25). Median durations of response were 31 weeks in the Adr-DTIC arm, 26 weeks in the cyclophosphamide-DTIC-Adr arm, and 23 weeks in the Adr-DTIC-Actinomycin D arm (P = .78). Median durations of survival were 37, 42, and 50 weeks, respectively. Again, no statistically significant differences were observed (P = .59). Toxicities from each of these treatment arms were formidable and were equivalent. Prognostic factor analysis showed a prognosis based on bone marrow reserve, sex, and pathology subtype favorable to patients.

Atypical fibroxanthoma arising in a young patient with Li-Fraumeni syndrome

2013 ◽  
Vol 41 (3) ◽  
pp. 303-307 ◽  
Author(s):  
Sun Mi Lee ◽  
Wei Zhang ◽  
Martin P. Fernandez
Keyword(s):  
Young Patient ◽  
Li Fraumeni Syndrome ◽  
Atypical Fibroxanthoma ◽  
Li Fraumeni

scholarly journals Albumin-bound paclitaxel and gemcitabine combination therapy in soft tissue sarcoma

2020 ◽  
Author(s):  
Zhichao Tian ◽  
Fan Zhang ◽  
Po Li ◽  
Jiaqiang Wang ◽  
Jinpo Yang ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Malignant Tumors ◽  
Objective Response ◽  
Progression Free Survival ◽  
Target Lesion ◽  
Free Survival ◽  
Metastatic Soft Tissue Sarcoma ◽  
Low Toxicity ◽  
Grade 3

Abstract Background: The evidence that albumin-bound paclitaxel (nab-paclitaxel) is safe and efficacious for the treatment of many types of malignant tumors is continuously increasing. However, the clinical data and evidence of nab-paclitaxel and gemcitabine in metastatic soft tissue sarcoma (STS) treatment are rare.Methods: The data of 17 patients with metastatic STS who received nab-paclitaxel/ gemcitabine chemotherapy between January 2019 and February 2020 were retrospectively reviewed. All patients were treated with nab-paclitaxel/ gemcitabine only after doxorubicin-based chemotherapy had failed. We evaluated the median progression-free survival (m-PFS), disease control rate (DCR), objective response rate (ORR) and adverse events (AEs) in these patients.Results: The m-PFS was 6 months (95% CI, 2–9 months), ORR was 41.2% and DCR was 70.6%. The average change in target lesion diameter from baseline was -19.06±45.74%. While the majority of the treatment patients experienced grade 1 or 2 AEs, grade 3 or 4 AEs were not common, but included neutropenia (17.6%), fatigue (11.8%), anemia (11.8%), leukopenia (11.8%), nausea (5.9%), peripheral neuropathy (5.9%), diarrhea (5.9%), and thrombocytopenia (5.9%). No treatment-related deaths occurred. Conclusion: Nab-paclitaxel/ gemcitabine combination chemotherapy is comparatively effective in the treatment of STS, demonstrates low toxicity, and is worthy of further study.

scholarly journals Lung adenocarcinoma in a patient with Li–Fraumeni syndrome bearing a novel germ-line mutation, TP53R333Vfs*12

2020 ◽  
Vol 50 (10) ◽  
pp. 1214-1217 ◽  
Author(s):  
Shodai Takahashi ◽  
Kazuhiro Shimazu ◽  
Koya Kodama ◽  
Koji Fukuda ◽  
Taichi Yoshida ◽  
...  
Keyword(s):  
Bone Metastasis ◽  
Lung Adenocarcinoma ◽  
Egfr Mutation ◽  
Kinase Inhibitor ◽  
Germ Line ◽  
Growth Factor Receptor ◽  
Triple Mutant ◽  
Li Fraumeni Syndrome ◽  
Li Fraumeni ◽  
Egfr Tki

Abstract Germline mutations of TP53 are responsible for Li–Fraumeni syndrome in its 60–80%. We found a novel germline mutation, TP53: c.997del:p.R333Vfs*12 (NM_000546.6, GRCh, 17:7670713..7670713). The proband is a 40-year-old female, who was suffered from osteosarcoma in her right forearm at her age of 11. She was also suffered from lung adenocarcinoma in her right upper lobe and bone metastasis in her right scapula at her age of 37. She was treated with gefitinib, an epidermal growth factor receptor-tyrosine kinase inhibitor (EGFR-TKI) because of EGFR mutation (L747-S752 del). Her bone metastasis became resistant after 1-year treatment. Bone metastasis had an additional EGFR mutation (T790M). The secondary treatment with osimertinib, an another EGFR-TKI, can successfully control the tumors for over 2 years. This TP53 mutation (R333Vfs*12) was first found in lung adenocarcinomas. The therapeutic effect of osimertinib for this triple mutant lung adenocarcinoma is better than the previous report.

scholarly journals A case of marantic endocarditis with systemic emboli in a patient with metastatic soft tissue sarcoma

2019 ◽  
Vol 6 (3) ◽  
pp. 6
Author(s):  
Daniel Alexander Reikher ◽  
Mark Feldman
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Malignant Tumors ◽  
Tissue Injury ◽  
Clinical Manifestations ◽  
Soft Tissue Sarcomas ◽  
Mass Effect ◽  
Metastatic Soft Tissue Sarcoma ◽  
Rare Group ◽  
Marantic Endocarditis

Clinical manifestations of cancer can be categorized as resulting from direct tissue injury from the primary tumor, distant metastatic spread, or aberrant biological activity, also known as a paraneoplastic syndrome. Soft tissue sarcomas are a rare group of malignant tumors of mesenchymal origin which typically present with direct tissue injury, exerting their harmful potential by compression and mass effect. We describe a rare case of an occult retroperitoneal soft tissue sarcoma presenting with marantic endocarditis. To date, there is a paucity of available medical literature relating sarcoma to marantic endocarditis.

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