scholarly journals Sarcomas of the Larynx: One Institution’s Experience and Treatment Protocol Analyses

Medicina ◽  
2021 ◽  
Vol 57 (3) ◽  
pp. 192
Author(s):  
Jaromir Astl ◽  
Richard Holy ◽  
Inna Tuckova ◽  
Tomas Belsan ◽  
Miloslav Pala ◽  
...  

Soft tissue sarcomas in the head and neck are rare malignancies. They occur in this area in less than 1% of all malignant tumors. Some authors have described the development of sarcoma from the mesenchymal tissue in the larynx. The histological diagnosis of a sarcoma depends on the immunohistochemical investigation. In particularly difficult diagnoses, electron microscopy has to be used. The treatment recommendation depends on the histological type of sarcoma. We analysed and summarized data on the diagnostic criteria and therapy for sarcoma of the larynx presented in the literature. We present three new cases of laryngeal sarcoma and describe the analyses of the published diagnostic and treatment schedules of laryngeal sarcomas. We developed a treatment protocol recommendation for laryngeal sarcoma based on an analysis of literature data and case reports. This recommendation is based on histological type, staging, grading, size, and survival data.

Cancers ◽  
2021 ◽  
Vol 13 (15) ◽  
pp. 3865
Author(s):  
Tânia Fortes-Andrade ◽  
Jani Sofia Almeida ◽  
Luana Madalena Sousa ◽  
Manuel Santos-Rosa ◽  
Paulo Freitas-Tavares ◽  
...  

Soft-tissue sarcomas (STS) represent about 80% of sarcomas, and are a heterogeneous group of rare and malignant tumors. STS arise from mesenchymal tissues and can grow into structures such as adipose tissue, muscles, nervous tissue and blood vessels. Morphological evaluation has been the standard model for the diagnosis of sarcomas, and even in samples with similar characteristics, they present a diversity in cytogenetic and genetic sequence alterations, which further increases the diversity of sarcomas. This variety is one of the main challenges for the classification and understanding of STS patterns, as well as for their respective treatments, which further decreases patient survival (<5 years). Despite some studies, little is known about the immunological profile of STS. As for the immunological profile of STS in relation to NK cells, there is also a shortage of studies. Observations made in solid tumors show that the infiltration of NK cells in tumors is associated with a good prognosis of the disease. Notwithstanding the scarcity of studies to characterize NK cells, their receptors, and ligands in STS, it is noteworthy that the progression of these malignancies is associated with altered NK phenotypes. Despite the scarcity of information on the function of NK cells, their phenotypes and their regulatory pathways in STS, the findings of this study support the additional need to explore NK cell-based immunotherapy in STS further. Some clinical trials, very tentatively, are already underway. STS clinical trials are still the basis for adoptive NK-cell and cytokine-based therapy.


2021 ◽  
Vol 22 (14) ◽  
pp. 7518
Author(s):  
Marcella Tazzari ◽  
Laura Bergamaschi ◽  
Alessandro De Vita ◽  
Paola Collini ◽  
Marta Barisella ◽  
...  

Soft tissue sarcomas (STSs) are a family of rare malignant tumors encompassing more than 80 histologies. Current therapies for metastatic STS, a condition that affects roughly half of patients, have limited efficacy, making innovative therapeutic strategies urgently needed. From a molecular point of view, STSs can be classified as translocation-related and those with a heavily rearranged genotype. Although only the latter display an increased mutational burden, molecular profiles suggestive of an “immune hot” tumor microenvironment are observed across STS histologies, and response to immunotherapy has been reported in both translocation-related and genetic complex STSs. These data reinforce the notion that immunity in STSs is multifaceted and influenced by both genetic and epigenetic determinants. Cumulative evidence indicates that a fine characterization of STSs at different levels is required to identify biomarkers predictive of immunotherapy response and to discover targetable pathways to switch on the immune sensitivity of “immune cold” tumors. In this review, we will summarize recent findings on the interplay between genetic landscape, molecular profiling and immunity in STSs. Immunological and molecular features will be discussed for their prognostic value in selected STS histologies. Finally, the local and systemic immunomodulatory effects of the targeted drugs imatinib and sunitinib will be discussed.


Author(s):  
Kunhi Mohammed K. P. ◽  
Snehasis Pradhan ◽  
Supratim Bhattacharyya ◽  
Prafulla Kumar Das ◽  
Muhammed Navas N. K.

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.


2021 ◽  
Vol 2021 ◽  
pp. 1-8
Author(s):  
Asmae Mazti ◽  
Mohamed El Idrissi ◽  
Abdelhalim El Ibrahimi ◽  
Mustapha El Maaroufi ◽  
Ghizlane El Koubaiti ◽  
...  

Soft-tissue sarcomas are malignant tumors that require good management within specialized centers. Our study aims to assess the benefit of handling these kinds of tumors using the Multidisciplinary Meeting (MDM) approach. The current paper details this approach through a prospective study that has lasted for 42 months in the HASSAN II University Hospital Center, Fez, Morocco. During this research work, 116 cases were selected with an average age of 53 years. In 95.7% of the cases, it was found that the lower limb was the most frequent tumor type (78.4%). Also, ninety-two (92) patients (79.3%) have had a prior biopsy. Ninety-nine (99) patients (85.3%) have received a magnetic resonance imaging scan (MRI) before surgery. Sixty-three (63) patients were operated on, including R0 resection used for 37 patients, R1 used for 21 patients, and R2 used for five patients. As a result, liposarcomas were the most frequent type (30.1%), followed by synovial sarcomas (14.6%), leiomyosarcomas (9.5%), ewing sarcoma (8.6), and undifferentiated pleomorphic sarcomas (7.7%). In addition, neoadjuvant chemotherapy was used for 36 patients. The other 22 patients received adjuvant chemotherapy and/or radiotherapy. The overall survival rate was 60.56 months, which proves a significant improvement, thanks to the multidisciplinary meeting approach. Conclusion. The conducted investigation has shown that using MDM for managing soft-tissue sarcomas of extremities improves the patients’ survival rate. Moreover, results have proven MDM might allow optimal treatment regarding less local recurrence and metastasis.


2018 ◽  
Vol 1 (Supplement) ◽  
pp. 45
Author(s):  
A.M. Bratu ◽  
I.A. Sălcianu ◽  
C. Zaharia ◽  
G. Iana ◽  
A.N. Marinescu

Abstract Introduction. Soft tissue sarcomas (STS) are rare entities of soft tissue cancers. Their incidence is low, of only 1% of the malignant tumors. In terms of localization, most of the STS affect the extremities, and their incidence is much higher in children than in adults. Material and method. The present paper is a retrospective study that includes tumors with lower limb localizations, including the bony pelvis, over a 3-year period (2013-2016). The study group consisted of 29 patients who, following the MRI examination, were diagnosed with softtissue tumors. Of the 29 patients, 17 patients had a MRI (magnetic resonance imaging) and an anatomopathological diagnosis of leiomyosarcoma. The location of the tumor, its characteristics, and the relationship with the adjacent anatomical structures were analyzed in all cases. Results. The ages of the final group of 17 patients ranged between 28 and 84 years, with female predominance. In terms of localization, one showed a muscle tumor in the pelvis, namely left oblique muscle, other cases being located in the thigh and knee. A special importance was given to the superficial and profound location. In 5 cases, the tumor was localized in subcutaneous fatty tissue, thus superficial. In terms of the contours of the tumor, well-defined margins were present in 11 cases, and poorly defined contour in 6 cases. Regarding the size, the leiomyosarcomas in our study had dimensions between 5.2 cm and 18 cm, and their structure was inhomogeneous, with the presence of necrosis and calcifications. Necrosis was found in 14 cases, and calcifications were present in 68%, being more frequent than necrosis. Except for the necrotic areas, the contrast enhancement was intense. Conclusions. Although the diagnosis is always histopathological, the MRI plays an important role in defining a precise localization and tumor characteristics.


2006 ◽  
Vol 72 (8) ◽  
pp. 665-671
Author(s):  
Gerame Wells ◽  
Robert C.G. Martin ◽  
Kelly M. Mcmasters ◽  
Charles R. Scoggins

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.


2020 ◽  
Vol 13 (1) ◽  
pp. 113-119 ◽  
Author(s):  
Magda Cordeiro ◽  
José Manuel Casanova ◽  
Joana Rodrigues ◽  
João Freitas ◽  
Ruben Fonseca ◽  
...  

Leiomyosarcomas of the lower extremity are extremely rare disorders and account for 10–15% of limb soft tissue sarcomas. These tumours have poor prognosis and even in early stages, patients persist at high risk for local and distant relapse; consequently, the treatment of advanced leiomyosarcoma of the lower extremity embodies a substantial defy. We present the case of a 73-year-old man diagnosed with metastatic lower extremity leiomyosarcoma of the hallux soft tissue, and with bone, lung and lymph node metastasis. After core needle biopsy confirmation of high-grade fusocellular sarcoma, the patient underwent surgery of the primary tumour and received anthracycline-based chemotherapy. However, after a 7-month progression-free survival period, a CT revealed lung disease progression. Sequentially, the patient was treated with trabectedin (Yondelis®) at a dose of 1.5 mg/m2 resulting in complete remission of the lung metastasis and stable disease of the remaining lesions after 26 months of treatment. Afterwards, the patient started on maintenance therapy with trabectedin, resulting in long-lasting stable disease, as he was able to receive 94 cycles with very acceptable quality of life. Finally, in March 2019, the patient died of community-acquired pneumonia without objective progression disease. This clinical case reports the first patient ever treated with 94 cycles of trabectedin. Our results additionally confirm that trabectedin wields relevant oncostatic benefits with a manageable safety profile and without cumulative toxicities. Trabectedin properties enable a maintenance long-term therapy (until disease progression or unbearable toxicity), with a high impact on survival and with a preserved quality of life.


Author(s):  
Khrystyna Zhurakivska ◽  
Giuseppe Troiano ◽  
Marco Montella ◽  
Lorenzo Lo Muzio ◽  
Luca Fiorillo ◽  
...  

Malignant fibrous histiocytoma is one of the most common soft tissue sarcomas in adults. It occurs only occasionally in oral soft tissues, and knowledge about its characteristics is based on a limited number of cases reported in the literature. Malignant fibrous histiocytoma belongs to the group of soft tissue sarcomas and makes up less than 10% of soft tissue sarcomas. For therapeutic purposes, complete exeresis of the lesion (macroscopic and microscopic) is performed because they have frequent recurrences. As for complementary therapy in addition to surgery, neither radiotherapy nor chemotherapy have been shown to reduce the risk of death related to the disease. Often patients complain of a swelling that grows in a short period of time. It is quite common for patients to report trauma in the area, which is not the cause, but rather the event that allows diagnosis. The mass usually does not cause pain unless it compresses an adjacent nerve structure. The aim of this study is to systematically review the scientific literature in order to identify the most recent studies concerning malignant fibrous histiocytomas localized in oral soft tissues and report their main data. The main outcomes of this study concern the immunohistochemical, molecular, and clinical aspects of this pathology. A systematic review of articles in the electronic databases pubmed, Scopus, and Web of Science was performed. After the selection process, 11 studies met the inclusion criteria and were included in the review. The mean age of the patients was 50.8 years old. The lesions affected various parts of the oral cavity, showing predominantly storiform–pleomorphic patterns. All cases except one were treated with surgical resection and radiation therapy. Although some data emerged from this review, they remain limited to a few case reports. Further studies are necessary in order to standardize the approach to patients affected by oral malignant fibrous histiocytoma (MFH).


1987 ◽  
Vol 5 (6) ◽  
pp. 851-861 ◽  
Author(s):  
L H Baker ◽  
J Frank ◽  
G Fine ◽  
S P Balcerzak ◽  
R L Stephens ◽  
...  

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.


Author(s):  
A.L. Zubarev ◽  
◽  
A.A. Kurilchik ◽  
V.E. Ivanov ◽  
A.L. Starodubtsev ◽  
...  

Soft tissue sarcomas (STS) are rare malignant tumors of mesenchymal origin. They account for about 1% of all malignant neoplasms. Haematogenous spread is the most common route of me-tastasis for STS and bone metastases occur in 9.4% of cases. When creating treatment plan for STS, it is necessary to consider a multimodal approach. Combination treatment can include pre-operative or postoperative radiation therapy (RT), chemotherapy (CT), and local hyperthermia (LHT). Surgery for STS should be radical. This paper presents 9 STS clinical cases and treatment outcomes in patients with secondary bone disease. Four patients underwent chemotherapy and surgery. Two patients received chemoradiotherapy (CRT) and surgery. Three patients received thermo-chemo-radiotherapy (TCHRT) followed by surgery. Accelerated fractionation radiotherapy was given using a single tumor dose of 3 Gy, twice a day with interval of 4 hours between ses-sions, 3 times a week to a total tumor dose (TTD) of 30 Gy (isoeffective TTD – 42 Gy, TDF – 69 Gy). Local hyperthermia for soft tissue tumor treatment was performed over 6 sessions: 2 ses-sions were combined with CHT courses before and after RT, and 4 sessions were combined with RT. The follow up period for 6 patients varied from 12 to 1.5 months, for 3 patients it varied from 6 to 8 months, for 1 patient it lasted 3 months and for 1 patient – 2 years. According to the RECIST criteria, more than half of the patients had tumor stabilization and 22% of patients had a partial response. Grade III-IV the rapeutic pathomorphosis was observed in 70% of patients after pre-operative combination treatment. The use of CT, CRT or TCHRT in combination treatment of STS with secondary bone disease enabled us to achieve a pronounced therapeutic pathomorphosis of tumors and to perform organ preservation surgery with endoprosthetic replacement.


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