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Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia
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...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
SAGE Publications
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348570/ https://www.ncbi.nlm.nih.gov/pubmed/30719261 http://dx.doi.org/10.1177/2036361318820171 |
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author | Mestiri, Sarra Elghali, Mohamed Amine Bourigua, Rym Abdessayed, Nihed Nasri, Salsabil Amine, Ben Abdallah Missaoui, Nabiha Ben Maitig, Mahmoud Hmissa, Sihem Sriha, Badreddine Mokni, Moncef |
author_facet | Mestiri, Sarra Elghali, Mohamed Amine Bourigua, Rym Abdessayed, Nihed Nasri, Salsabil Amine, Ben Abdallah Missaoui, Nabiha Ben Maitig, Mahmoud Hmissa, Sihem Sriha, Badreddine Mokni, Moncef |
author_sort | Mestiri, Sarra |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-6348570 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | SAGE Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-63485702019-02-04 Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia Mestiri, Sarra Elghali, Mohamed Amine Bourigua, Rym Abdessayed, Nihed Nasri, Salsabil Amine, Ben Abdallah Missaoui, Nabiha Ben Maitig, Mahmoud Hmissa, Sihem Sriha, Badreddine Mokni, Moncef Rare Tumors Original Article 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. SAGE Publications 2019-01-21 /pmc/articles/PMC6348570/ /pubmed/30719261 http://dx.doi.org/10.1177/2036361318820171 Text en © The Author(s) 2019 http://www.creativecommons.org/licenses/by-nc/4.0/ This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage). |
spellingShingle | Original Article Mestiri, Sarra Elghali, Mohamed Amine Bourigua, Rym Abdessayed, Nihed Nasri, Salsabil Amine, Ben Abdallah Missaoui, Nabiha Ben Maitig, Mahmoud Hmissa, Sihem Sriha, Badreddine Mokni, Moncef Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title | Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title_full | Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title_fullStr | Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title_full_unstemmed | Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title_short | Soft tissue leiomyosarcoma—diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia |
title_sort | soft tissue leiomyosarcoma—diagnostics, management, and prognosis: data of the registry cancer of the center of tunisia |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6348570/ https://www.ncbi.nlm.nih.gov/pubmed/30719261 http://dx.doi.org/10.1177/2036361318820171 |
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