Soft tissue leiomyosarcoma-diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia.
Identifieur interne : 000288 ( PubMed/Curation ); précédent : 000287; suivant : 000289Soft tissue leiomyosarcoma-diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia.
Auteurs : Sarra Mestiri [Tunisie] ; Mohamed Amine Elghali [Tunisie] ; Rym Bourigua [Tunisie] ; Nihed Abdessayed [Tunisie] ; Salsabil Nasri [Tunisie] ; Ben Abdallah Amine [Tunisie] ; Nabiha Missaoui [Tunisie] ; Mahmoud Ben Maitig [Tunisie] ; Sihem Hmissa [Tunisie] ; Badreddine Sriha [Tunisie] ; Moncef Mokni [Tunisie]Source :
- Rare tumors [ 2036-3605 ] ; 2019.
Abstract
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.
DOI: 10.1177/2036361318820171
PubMed: 30719261
PubMed Central: PMC6348570
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uniqKey="Mestiri S" first="Sarra" last="Mestiri">Sarra Mestiri</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Cancer Registry of the Center of Tunisia, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Elghali, Mohamed Amine" sort="Elghali, Mohamed Amine" uniqKey="Elghali M" first="Mohamed Amine" last="Elghali">Mohamed Amine Elghali</name><affiliation wicri:level="1"><nlm:affiliation>Department of General Surgery, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of General Surgery, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Bourigua, Rym" sort="Bourigua, Rym" uniqKey="Bourigua R" first="Rym" last="Bourigua">Rym Bourigua</name><affiliation wicri:level="1"><nlm:affiliation>Department of Oncology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Oncology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Abdessayed, Nihed" sort="Abdessayed, Nihed" uniqKey="Abdessayed N" first="Nihed" last="Abdessayed">Nihed Abdessayed</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Department of Oncology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Oncology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Nasri, Salsabil" sort="Nasri, Salsabil" uniqKey="Nasri S" first="Salsabil" last="Nasri">Salsabil Nasri</name><affiliation wicri:level="1"><nlm:affiliation>Department of General Surgery, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of General Surgery, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Amine, Ben Abdallah" sort="Amine, Ben Abdallah" uniqKey="Amine B" first="Ben Abdallah" last="Amine">Ben Abdallah Amine</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Missaoui, Nabiha" sort="Missaoui, Nabiha" uniqKey="Missaoui N" first="Nabiha" last="Missaoui">Nabiha Missaoui</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Cancer Registry of the Center of Tunisia, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Ben Maitig, Mahmoud" sort="Ben Maitig, Mahmoud" uniqKey="Ben Maitig M" first="Mahmoud" last="Ben Maitig">Mahmoud Ben Maitig</name><affiliation wicri:level="1"><nlm:affiliation>Department of Orthopedy, Hospital Sahloul, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Orthopedy, Hospital Sahloul, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Hmissa, Sihem" sort="Hmissa, Sihem" uniqKey="Hmissa S" first="Sihem" last="Hmissa">Sihem Hmissa</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Cancer Registry of the Center of Tunisia, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Research Unit UR14ES17, Cancer Epidemiology and Cytopathology in Tunisian Center, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Research Unit UR14ES17, Cancer Epidemiology and Cytopathology in Tunisian Center, Faculty of Medicine of Sousse, University of Sousse, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Sriha, Badreddine" sort="Sriha, Badreddine" uniqKey="Sriha B" first="Badreddine" last="Sriha">Badreddine Sriha</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Cancer Registry of the Center of Tunisia, Sousse</wicri:regionArea></affiliation></author><author><name sortKey="Mokni, Moncef" sort="Mokni, Moncef" uniqKey="Mokni M" first="Moncef" last="Mokni">Moncef Mokni</name><affiliation wicri:level="1"><nlm:affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Department of Pathology, Farhat Hached University Hospital, Sousse</wicri:regionArea></affiliation><affiliation wicri:level="1"><nlm:affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</nlm:affiliation><country xml:lang="fr">Tunisie</country><wicri:regionArea>Cancer Registry of the Center of Tunisia, Sousse</wicri:regionArea></affiliation></author></analytic><series><title level="j">Rare tumors</title><idno type="ISSN">2036-3605</idno><imprint><date when="2019" type="published">2019</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p><b>Background</b></p><p>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.</p></div><div type="abstract" xml:lang="en"><p><b>Methods</b></p><p>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.</p></div><div type="abstract" xml:lang="en"><p><b>Results</b></p><p>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%.</p></div><div type="abstract" xml:lang="en"><p><b>Conclusion</b></p><p>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.</p></div></front></TEI><pubmed><MedlineCitation Status="PubMed-not-MEDLINE" Owner="NLM"><PMID Version="1">30719261</PMID><DateRevised><Year>2020</Year><Month>10</Month><Day>01</Day></DateRevised><Article PubModel="Electronic-eCollection"><Journal><ISSN IssnType="Print">2036-3605</ISSN><JournalIssue CitedMedium="Print"><Volume>11</Volume><PubDate><Year>2019</Year></PubDate></JournalIssue><Title>Rare tumors</Title><ISOAbbreviation>Rare Tumors</ISOAbbreviation></Journal><ArticleTitle>Soft tissue leiomyosarcoma-diagnostics, management, and prognosis: Data of the registry cancer of the center of Tunisia.</ArticleTitle><Pagination><MedlinePgn>2036361318820171</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.1177/2036361318820171</ELocationID><Abstract><AbstractText Label="Background" NlmCategory="UNASSIGNED">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.</AbstractText><AbstractText Label="Methods" NlmCategory="UNASSIGNED">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.</AbstractText><AbstractText Label="Results" NlmCategory="UNASSIGNED">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%.</AbstractText><AbstractText Label="Conclusion" NlmCategory="UNASSIGNED">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.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Mestiri</LastName><ForeName>Sarra</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Elghali</LastName><ForeName>Mohamed Amine</ForeName><Initials>MA</Initials><AffiliationInfo><Affiliation>Department of General Surgery, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Bourigua</LastName><ForeName>Rym</ForeName><Initials>R</Initials><AffiliationInfo><Affiliation>Department of Oncology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Abdessayed</LastName><ForeName>Nihed</ForeName><Initials>N</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Department of Oncology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Nasri</LastName><ForeName>Salsabil</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of General Surgery, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Amine</LastName><ForeName>Ben Abdallah</ForeName><Initials>BA</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Missaoui</LastName><ForeName>Nabiha</ForeName><Initials>N</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Ben Maitig</LastName><ForeName>Mahmoud</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>Department of Orthopedy, Hospital Sahloul, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Hmissa</LastName><ForeName>Sihem</ForeName><Initials>S</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Research Unit UR14ES17, Cancer Epidemiology and Cytopathology in Tunisian Center, Faculty of Medicine of Sousse, University of Sousse, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Sriha</LastName><ForeName>Badreddine</ForeName><Initials>B</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Mokni</LastName><ForeName>Moncef</ForeName><Initials>M</Initials><AffiliationInfo><Affiliation>Department of Pathology, Farhat Hached University Hospital, Sousse, Tunisia.</Affiliation></AffiliationInfo><AffiliationInfo><Affiliation>Cancer Registry of the Center of Tunisia, Sousse, Tunisia.</Affiliation></AffiliationInfo></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList><ArticleDate DateType="Electronic"><Year>2019</Year><Month>01</Month><Day>21</Day></ArticleDate></Article><MedlineJournalInfo><Country>England</Country><MedlineTA>Rare Tumors</MedlineTA><NlmUniqueID>101526926</NlmUniqueID><ISSNLinking>2036-3605</ISSNLinking></MedlineJournalInfo><KeywordList Owner="NOTNLM"><Keyword MajorTopicYN="N">Leiomyosarcoma</Keyword><Keyword MajorTopicYN="N">prognosis</Keyword><Keyword MajorTopicYN="N">soft tissue</Keyword><Keyword MajorTopicYN="N">treatment</Keyword></KeywordList><CoiStatement>Conflict of interest: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.</CoiStatement></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="received"><Year>2018</Year><Month>02</Month><Day>26</Day></PubMedPubDate><PubMedPubDate PubStatus="accepted"><Year>2018</Year><Month>11</Month><Day>27</Day></PubMedPubDate><PubMedPubDate 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