A High-Grade Primary Leiomyosarcoma of the Bladder in a Survivor of Retinoblastoma

2001 ◽  
Vol 125 (9) ◽  
pp. 1231-1234
Author(s):  
Sharon X. Liang ◽  
Yegappan Lakshmanan ◽  
Bruce A. Woda ◽  
Zhong Jiang
Keyword(s):  
Soft Tissue ◽  
Urinary Bladder ◽  
Soft Tissue Sarcomas ◽  
Common Type ◽  
Prior History ◽  
Adjuvant Radiation ◽  
High Grade ◽  
Cyclophosphamide Therapy ◽  
The Past ◽  
History Of

Abstract Second nonocular malignancies develop with increased incidence in patients with hereditary retinoblastoma. Osteosarcoma is by far the most common type with an incidence of up to 50%, followed by soft tissue sarcomas. Visceral leiomyosarcoma is extremely rare and only 2 cases have been reported in the past 2 decades, one in the liver and another one in the urinary bladder, both of which developed after cyclophosphamide therapy. Here we report a case of vesical leiomyosarcoma that was diagnosed in a 49-year-old woman 47 years after the diagnosis of a hereditary retinoblastoma. The patient's retinoblastoma was treated with unilateral enucleation without adjuvant radiation or chemotherapy. We believe that this is the first report of vesical leiomyosarcoma occurring in a patient with retinoblastoma without a prior history of radiation or chemotherapy. This report is significant not only because of the rarity of vesical leiomyosarcoma as a second nonocular tumor in retinoblastoma patients, but also because of the infrequency of vesical leiomyosarcoma in general. We also investigated the potential molecular pathogenesis of the leiomyosarcoma.

scholarly journals Smoking and Family Cancer History Are Associated With Sarcomas in A Japanese Population Study

2020 ◽  
Author(s):  
Yoshihiro Araki ◽  
Norio Yamamoto ◽  
Yoshikazu Tanzawa ◽  
Takahiro Higashi ◽  
Katsuhiro Hayashi ◽  
...  
Keyword(s):  
Family History ◽  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Smoking Habit ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
Cancer History ◽  
Family Cancer History ◽  
History Of ◽  
History Of Cancer

Abstract Background: Sarcoma is a rare cancer, and it is also the cause of the development of various kinds of sarcomas, such as gene abnormalities, which has recently becoming evident due to advances of genetic testing. The approach to solve the origin of diseases is essential to elucidate both the external environmental factors and the internal genetic factors. However, the lifestyle habits, lifestyle-related diseases, personal and family cancer history of sarcoma patients remain unclear.Methods: A total of 1320 sarcoma patients were enrolled in this study. A questionnaire on lifestyle habits, life-style diseases, and the patient’s personal and family cancer history was completed at presentation. A total of 1320 controls were selected by propensity score matching for age and gender. Smoking, drinking, obesity, hypertension, dyslipidemia and diabetes mellitus were compared. In addition, we investigated the incidence of a personal and family cancer history in sarcoma patients. Results: A smoking habit was the only independent risk factor for high-grade soft tissue sarcoma development in adults ≥20 years old (n=952), excluding low-grade and intermediate malignant soft tissue tumors (Odds ratio [OR], 2.45; 95% confidence interval [CI] 1.88-3.20, p<0.001). The ORs of high-grade liposarcoma and undifferentiated pleomorphic sarcoma (UPS) were 2.56 and 3.00, respectively. Eight percent of sarcoma patients had a personal history of another cancer. Thirty percent of soft tissue sarcoma patients had a family history of cancer in a first-degree relatives (malignant peripheral nerve sheath tumor, 52%; leiomyosarcoma, 46%). Conclusions: We confirmed that a smoking habit were associated with the development of high-grade soft tissue sarcomas. A family history of cancer might be associated with certain soft tissue sarcomas, but a further investigation will be necessary.

scholarly journals Analysis of trends in race and gender disparities in incidence-based mortality in patients diagnosed with soft tissue sarcomas from 2000 to 2016.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23549-e23549
Author(s):  
Lakshmi Priyanka Pappoppula ◽  
Germame Hailegiorgis Ajebo ◽  
Justin Yeh ◽  
Picon Hector ◽  
Allan N. Krutchik ◽  
...  
Keyword(s):  
African American ◽  
African Americans ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Mortality Rates ◽  
Low Grade ◽  
High Grade ◽  
The Past ◽  
Significant Change ◽  
And Gender

e23549 Background: The clinical course of soft tissue sarcomas is often dependent on the grade of the tumor. The incidence of soft tissue sarcomas have been known to be higher in males compared to females and more in African Americans compared to Caucasians (1995 to 2008 SEER data). The variability of incidence-based mortality in low grade and high grade soft tissue sarcomas with respect to gender and race over the past decade has not been well studied. This study analyzes the rates of incidence-based mortality from the years 2000 to 2016 amongst for both the grades, genders and racial groups. Methods: The Surveillance, Epidemiology, and End Results (SEER) Database was queried to conduct a nation-wide analysis for the years 2000 to 2016. Incidence-based mortality for all stages of low grade and high grade soft tissue sarcomas was queried and the results were grouped by race (Caucasian/White vs African American/Black) and gender. All stages and ages were included in the analysis and trend from 2000 to 2016 was analyzed. Results: Incidence-based mortality rates (per 10000) for low grade and high grade soft tissue sarcomas for both races and genders are shown in the table below. The incidence-based mortality rates for Caucasians are similar to African American in both grades and genders. Rates were not analyzed for American Indian and Asian/Pacific Islanders due to small sample size. Mortality rates of high grade soft tissue sarcomas were significantly higher compared to low grade tumors. A higher rate of mortality is noted in Caucasian males compared to African Americans males despite past observations of higher incidence in African Americans. There was no significant change in the rate when trended over the past decade (2007 to 2016). Conclusions: This study highlights the higher rate of incidence-based mortality in Caucasian males compared to African American males in the past 15 years despite a lower incidence reported in the 1995 to 2008 period. With no significant change in mortality rates/year noted during this time period, this study implies that soft tissue sarcomas in Caucasian males have worse outcomes. Further research is needed to understand the mechanism underlying this disparity. [Table: see text]

scholarly journals Systemic Treatment for Soft Tissue Sarcoma: What is Standard, What is New

2018 ◽  
Vol 1 (Supplement) ◽  
pp. 64
Author(s):  
D.C. Jinga ◽  
D. Chetroiu
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Adjuvant Treatment ◽  
Kinase Inhibitors ◽  
Systemic Treatment ◽  
Growth Factor Receptor ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
History Of ◽  
New Treatment

Abstract Soft tissue sarcoma (STS) is a biologically heterogeneous malignancy with over 50 subtypes. This solid tumor is one of the most challenging diseases to treat for the medical oncologist. STS often forms in the body’s muscles, joints, fat, nerves, deep skin tissues, and blood vessels. The natural history of high-grade STS is characterized by a strong tendency toward local recurrence and metastatic spreading, despite optimal initial strategies. The lung is the most common site of metastases, with poor prognosis. We present the current international guidelines for the adjuvant treatment and systemic treatment for advanced STS and the new discoveries. Many new molecular targeting drugs have been tried in the last ten years, and some were approved for soft tissue sarcoma. The first approved was Imatinib, as a treatment for gastrointestinal stromal tumors (GISTs). Following Imatinib, other tyrosine kinase inhibitors (TKIs) received the approval for GISTs such as Sunitinib and Regorafenib, and Pazopanib for non-GIST soft tissue sarcomas. In 2016, FDA approved the first monoclonal antibody that targets platelet-derived growth factor receptor (PDGFR)-α, Olaratumab. The new treatment demonstrates an overall survival advantage. In this review, we aimed to summarize the results from the most recent studies on adjuvant treatment for high-grade STS and systemic strategies for advanced STS.

Myxofibrosarcoma: clinical and prognostic value of MRI features

2020 ◽  
Vol 16 ◽  
Author(s):  
Paolo Spinnato ◽  
Andrea Sambri ◽  
Tomohiro Fujiwara ◽  
Luca Ceccarelli ◽  
Roberta Clinca ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Contrast Enhancement ◽  
Imaging Modality ◽  
Soft Tissue Sarcomas ◽  
The Elderly ◽  
High Rate ◽  
Imaging Features ◽  
High Grade ◽  
Mri Features

: Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma and plays a key role in the preoperative setting of these patients.MRI features associated with high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement, presence of an infiltrative pattern (“tail sign”). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful to stratify the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features

scholarly journals Translating Molecular Profiling of Soft Tissue Sarcomas into Daily Clinical Practice

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 512
Author(s):  
Celine Jacobs ◽  
Lore Lapeire
Keyword(s):  
Soft Tissue ◽  
Unmet Need ◽  
Soft Tissue Sarcomas ◽  
Molecular Profiling ◽  
Point Of View ◽  
Molecular Techniques ◽  
Mesenchymal Tumors ◽  
The Past ◽  
Cancer Types ◽  
Sarcoma Treatment

Soft tissue sarcomas are a group of rare mesenchymal tumors with more than 70 subtypes described. Treatment of these subtypes in an advanced setting is mainly according to a one-size-fits-all strategy indicating a high unmet need of new and more targeted therapeutic options in order to optimize survival. The introduction of advanced molecular techniques in cancer has led to better diagnostics and identification of new therapeutic targets, leading to more personalized treatment and improved prognosis for several cancer types. In sarcoma, a likewise evolution is seen, albeit at a slower pace. This manuscript describes how in the past years advanced molecular profiling in soft tissue sarcomas was able to identify specific and often pathognomonic aberrations, deferring standard sarcoma treatment in favor of more targeted treatment from an oncologist’s point of view.

Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas.

1989 ◽  
Vol 7 (9) ◽  
pp. 1217-1228 ◽  
Author(s):  
A E Chang ◽  
S M Steinberg ◽  
M Culnane ◽  
M H Lampert ◽  
A J Reggia ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Soft Tissue ◽  
Sexual Activity ◽  
Standardized Test ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
Postoperative Radiation ◽  
Limb Function ◽  
Postoperative Radiation Therapy ◽  
Global Quality Of Life

We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone.

scholarly journals High-Grade Extremity Soft Tissue Sarcomas

2003 ◽  
Vol 237 (2) ◽  
pp. 218-226 ◽  
Author(s):  
Fritz C. Eilber ◽  
Gerald Rosen ◽  
Scott D. Nelson ◽  
Michael Selch ◽  
Frederick Dorey ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
High Grade
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