scholarly journals Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia

Rare Tumors ◽  
2019 ◽  
Vol 11 ◽  
pp. 203636131882017 ◽  
Author(s):  
Sarra Mestiri ◽  
Mohamed Amine Elghali ◽  
Rym Bourigua ◽  
Nihed Abdessayed ◽  
Salsabil Nasri ◽  
...  

Background: Soft tissue leiomyosarcomas are rare, accounting for almost 5%–10% of all soft tissue sarcomas; they account for almost 1% of all sarcomas. They are aggressive tumors where location, size, and management require a multidisciplinary approach. Since there are few series published, we here analyze epidemiological pattern, clinical and pathologic features of soft tissue leiomyosarcomas. Methods: We conducted a retrospective study of 29 consecutive cases of histologically proven soft tissue leiomyosarcoma extracted from the database of the Cancer Registry of the Center of Tunisia and the Department of Pathology of Farhat Hached University Hospital of Sousse of Tunisia, during a 10-year period (from January 1996 to December 2005). Epidemiologic details, clinico-pathological features, and treatment modalities were assessed with focus on patients’ 5-year overall survival, tumor relapse, and metastases. Results: Soft tissue leiomyosarcoma accounted for 17.5% of all soft tissue sarcomas diagnosed at our pathology department. Most of patients were of advanced age (median: 52 years), with extremes ranging from 12 and 87 years. There was a slight male predominance (sex-ratio = 1.07). Tumors were located mostly in the lower limbs (45%). Deep sites as retroperitoneum was found only in two cases. Tumor size was more than 5 cm in 83% of cases (average size = 9.4 cm). Five cases had metastasis on initial staging. For 24 patients, the disease was locally limited at the moment of diagnosis. Palliative chemotherapy was indicated for four patients and surgery was performed for 20 patients. Local recurrence occurred in 11 patients (55% of operated patients) and metastasis in 6 patients. Overall, 5-year survival was about 24%. Conclusion: Our study results highlight the scarcity of soft tissue leiomyosarcoma. Unfortunately, unusual tumor sites, disease’s advanced stages, and intralesional resection made the prognosis poorer than in other series. Clinical course of soft tissue leiomyosarcoma was highly marked by local recurrence and metastasis.

Sarcoma ◽  
2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Varun K. Chowdhry ◽  
John M. Kane ◽  
Katy Wang ◽  
Daniel Joyce ◽  
Anne Grand’Maison ◽  
...  

Introduction. Paratesticular sarcomas are defined as tumors that arise within the scrotum and include the subsites of epididymis, spermatic cord, and tunica vaginalis and represent the most common type of GU sarcoma. The mainstay of treatment is often surgical resection, combined with histology specific chemotherapy and radiotherapy. Due to the rare nature of the disease, there are limited data to guide management. We present our single-institution retrospective experience regarding the management and treatment of paratesticular sarcomas. Materials and Methods. We queried our oncology registry database for patients treated for testicular, spermatic cord, and scrotal soft tissue sarcomas between 1971 and 2017. Patients in this series had pathological confirmation of a sarcoma diagnosis by a sarcoma-specialized pathologist. Only patients with localized disease were included in this analysis with the exception of patients with a diagnosis of rhabdomyosarcoma where patients with both localized and metastatic disease were included on this study. Results. A total of 34 patients were included in this retrospective analysis. The median was 24 (range, 5–78), and the median tumor size was 6.25 cm. Twenty-six patients had localized disease (76.6%) at the time of diagnosis. A predominance of patients had tumors involving the spermatic cord (45.5%), and the most common histology was rhabdomyosarcoma (35.3%), leiomyosarcoma (26.5%), and well-differentiated liposarcoma (23.5%). The median follow-up was 71.0 months (range, 2.5–534.4 months). A total of 7 patients experienced an isolated local failure (20.6%), four patients developed distant metastatic disease (11.8%), and one patient (2.9%) with synovial sarcoma of the spermatic cord experienced a regional recurrence. The median progression-free survival (PFS) was 99.6 months, 95% CI (45.8–534.3 months), with a three-year PFS rate of 71%, 95% CI (53%–83%), and a 5-year PFS rate of 64% (range, 46%–78%). We did not find any statistically significant associations based on surgery type ( p = 0.15 ), the use of chemotherapy, ( p = 0.36 ), or final margin status ( p = 0.21 ). Two patients who were treated with preoperative radiotherapy had significant wound healing complication with chronic sinus tracts, though these patients did not experience a local recurrence. Conclusions. We provide a characterization of the natural history and treatment patterns of paratesticular sarcomas. While effective at reducing a local recurrence, preoperative radiotherapy was associated with significant toxicity. As a result, we prefer the use of postoperative radiotherapy in patients as clinically indicated. We did not find any specific treatment patterns associated with an improvement in clinical outcomes.


Author(s):  
Paolo Spinnato ◽  
Andrea Sambri ◽  
Tomohiro Fujiwara ◽  
Luca Ceccarelli ◽  
Roberta Clinca ◽  
...  

: 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


2018 ◽  
Vol 1 (Supplement) ◽  
pp. 58
Author(s):  
V. Georgeanu ◽  
T. Atasiei ◽  
D. Gartonea ◽  
B. Shazam ◽  
G. Goleşteanu ◽  
...  

Abstract Introduction. Soft Tissue Sarcomas (STS) is a group of rare malignant tumors with mesenchymal tissue origin. At present, over 50 histopathological types with typical chromosome changes are described. Treatment is multidisciplinary, centered on the surgical approach. Method. Between 2014 and 2016, 12 STS cases were treated in our clinic: 3 liposarcomas, 2 synovial sarcomas, 1 angiosarcoma, 2 rhabdomyosarcomas, 2 myxofibrosarcomas, 1 fibroblast sarcoma and 1 clear cell sarcoma. With the exception of angiosarcoma that benefited only from chemotherapy, the other cases were surgically approached, followed by radiotherapy in 7 cases, and chemotherapy in one case. Results. Patient follow-up was for a minimum of 2 years. In 3 cases (fibroblast sarcoma, clear-cell sarcoma, synovial sarcoma), a local recurrence occurred between 3 and 9 months. In 2 cases (fibroblastic sarcoma, synovial sarcoma), pulmonary metastases occurred between 6 and 18 months and the patients died at 8 months and 2 years respectively. The rest of the cases were clinically and imagistically assessed (local MRI, lung CT) at 6 months for at least 2 years and showed no signs of local recurrence or metastasis. Conclusions. The evolution of STS depends on the histological type and to the stage of diagnosis (local extension, degree of differentiation and presence of metastases). Therapeutic approach should be centered on an accurate resection, within the limits of oncological safety even with re-resection. Postoperative radiotherapy is used in most of the cases, and chemotherapy is reserved for special cases with unfavorable local evolution or metastasis.


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.


1970 ◽  
Vol 32 (2) ◽  
pp. 26-29
Author(s):  
R Jha

Introduction: Soft tissue tumors represent diagnostic challenge to general practicing pathologists because of overlap in morphologic features. The usual approach is to diagnose by presumed cell lineage. A major utility of immunohistochemistry is to identify a tumor being mesenchymal or nonmesenchymal and once mesenchymal lineage has been confirmed, histologic sub typing according to specific lineage may be achieved with help of lineage specific markers. Methods: The objective of this study was to analyse different types of soft tissue malignancies according to their type, site and age distribution, discuss the difficulties faced during morphologic examination and review the literature for role of ancillary techniques, particularly immunohistochemistry in diagnosing soft tissue sarcomas.All soft tissue malignancies reported from Tribhuvan University Teaching Hospital in three years period from April 2008 to March 2010 were included in the study. Results: Total 87 soft tissue malignancies were reported during study period. Most of these patients (58.6%) were male and the rest were female. These malignancies were found in all age groups. Youngest patient was newborn who was born with tongue mass and biopsy was performed on 13th day of life. However maximum numbers of tumors (41.4%) were seen in first two decades of life. Head and neck was the most common site .Rhabdomyosarcoma was the most common diagnosis. In 20 cases (23%) a definite opinion was not possible and vague terms like sarcoma, small round cell tumor, spindle cell malignancy, vascular tumor “likely” were used. Immunohistochemistry was advised in these cases. Conclusions: Only light microscopic examination of hematoxylin and eosin stained slide not sufficient at all times because there is morphologic overlap of soft tissue sarcomas with each other and with carcinomas and melanomas. Though immunohistochemistry is not a substitute for skilled interpretation of routinely stained slides but prove helpful to reach a more definite diagnosis. Keywords: Immunohistichemistry; sarcoma; soft tissue DOI: http://dx.doi.org/10.3126/joim.v32i2.4941 Journal of Institute of Medicine, August, 2010; 32: 26-29


2001 ◽  
Vol 19 (13) ◽  
pp. 3203-3209 ◽  
Author(s):  
Fritz C. Eilber ◽  
Gerald Rosen ◽  
Jeffery Eckardt ◽  
Charles Forscher ◽  
Scott D. Nelson ◽  
...  

PURPOSE: To determine whether treatment-induced pathologic necrosis correlates with local recurrence and overall survival in patients who receive neoadjuvant therapy for high-grade extremity soft tissue sarcomas. PATIENTS AND METHODS: Four hundred ninety-six patients with intermediate- to high-grade extremity soft tissue sarcomas received protocol neoadjuvant therapy. All patients underwent surgical resection after neoadjuvant therapy and had pathologic assessment of tumor necrosis in the resected specimens. RESULTS: The 5- and 10-year local recurrence rates for patients with ≥ 95% pathologic necrosis were significantly lower (6% and 11%, respectively) than the local recurrence rates for patients with less than 95% pathologic necrosis (17% and 23%, respectively). The 5- and 10-year survival rates for the patients with ≥ 95% pathologic necrosis were significantly higher (80% and 71%, respectively) than the survival rates for the patients with less than 95% pathologic necrosis (62% and 55%, respectively). Patients with less than 95% pathologic necrosis were 2.51 times more likely to develop a local recurrence and 1.86 times more likely to die of their disease as compared with patients with ≥ 95% pathologic necrosis. The percentage of patients who achieved ≥ 95% pathologic necrosis increased to 48% with the addition of ifosfamide as compared with 13% of the patients in all the other protocols combined. CONCLUSION: Treatment-induced pathologic necrosis is an independent predictor of both local recurrence and overall survival in patients who receive neoadjuvant therapy for high-grade extremity soft tissue sarcomas. A complete pathologic response (≥ 95% pathologic necrosis) correlated with a significantly lower rate of local recurrence and improved overall survival.


1970 ◽  
Vol 56 (5) ◽  
pp. 297-310
Author(s):  
Giovanni Crema ◽  
Bruno Damascelli ◽  
Renato Musumeci ◽  
Carlo Uslenghi

This review series comprises 245 patients with soft tissue sarcomas of various sites and histologic types observed at this Institute between 1928 and 1966. 132 patients were men (54.2%) and 113 women (45.8%). Fibrosarcomas accounted for the largest number of cases (161 cases - 65.5%), followed by undifferentiated sarcomas (36 cases). Other histologic types accounted for a smaller number of cases. As to the site of the primary tumor, the lower limbs accounted for 32.2% and the abdomen for 26.5% of the cases. 152 of the 245 patients attended for the first time and 93 presented recurrences of the tumor sometime after receiving surgical treatment and/or radiotherapy elsewhere. 159/245 cases were subjected at this Institute to designedly radical surgery followed by postoperative radiotherapy by various techniques. In the remaining 86 cases, in which surgery was either not indicated or not possible because of the patients’ local or general condition, radiotherapy only was given, where possible curative. Radium was used in 97 cases almost always as complementary treatment in superficial tumors that had developed mainly towards the surface and been removed surgically either radically or as radically as conditions allowed. The therapeutic techniques and results are discussed in relation to patient survival by histologic type and to failures and recurrences.


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