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[PMID]:29095310
[Au] Autor:Xia C; Zhu Q; Yue C; Hu M; Li P; Li Z
[Ad] Endereço:aDepartment of Diagnostic Ultrasound, Beijing Tongren Hospital, Capital Medical University bDepartment of Pathology, Beijing Tongren Hospital, Capital Medical University cDepartment of Otorhinolaryngology Head and Neck Surgery, Beijing Tongren Hospital, Capital Medical University dDepartment of Diagnostic Ultrasound, Beijing Aerospace General Hospital, Beijing, China.
[Ti] Título:Sonography used in the infantile desmoid fibromatosis of postcricoid area: A case report.
[So] Source:Medicine (Baltimore);96(44):e8500, 2017 Nov.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Infantile desmoid fibromatosis of the postcricoid area is a rare disease and is characterized by a proliferation of fibrous tissue with non-metastasis, local infiltration, and a high rate of recurrence after surgical resection. Currently, ultrasound is scarcely used in the hypopharynx and larynx area. PATIENT CONCERNS: A 4-year-old boy presented with hoarseness, deep voice and snoring for 2∼4 years without any surgical history. On sonography, the lesion was found in the postcricoid area, and the left larynx showed impaired mobility in real time observation. Complete excision with a negative margin in this pivotal anatomic area is impossible, and necessitates a long-time surveillance. DIAGNOSES: Infantile desmoid fibromatosis of the postcricoid area was diagnosed according to surgery and histopathology. INTERVENTIONS: Local excision was carried out to relieve the upper airway narrowing. OUTCOMES: Relieved hoarseness and snoring were reported on the latest follow-up. A residual lesion was seen in the surgical bed and maintained a stable extent on ultrasound and MR imaging after a year. LESSONS: Considering the non-radiation merit and diagnostic ability, ultrasonography is advocated as a valuable supplementary imaging method to CT, MR and laryngoscopy in the juvenile larynx and hypopharynx.
[Mh] Termos MeSH primário: Cartilagem Cricoide/diagnóstico por imagem
Fibromatose Agressiva/diagnóstico por imagem
Rouquidão/diagnóstico por imagem
Neoplasias Laríngeas/diagnóstico por imagem
[Mh] Termos MeSH secundário: Pré-Escolar
Rouquidão/etiologia
Seres Humanos
Neoplasias Laríngeas/complicações
Masculino
Ultrassonografia/métodos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171109
[Lr] Data última revisão:
171109
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:171103
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008500


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[PMID]:28904708
[Au] Autor:Bourra K; El Mazouz S
[Ad] Endereço:Service de Chirurgie Plastique, Hôpital Al Farabi Oujda, Maroc.
[Ti] Título:[Latissimus dorsi flap in reconstruction following treatment of giant tumor of the abdominal wall: about a rare case].
[Ti] Título:Lambeau grand dorsal dans la reconstruction d'une tumeur géante de la paroi abdominale: à propos d'un cas rare..
[So] Source:Pan Afr Med J;27:181, 2017.
[Is] ISSN:1937-8688
[Cp] País de publicação:Uganda
[La] Idioma:fre
[Ab] Resumo:We report the case of a 16-year old patient presenting with giant, multinodular, mesenchymal tumor of the abdominal wall occupying the left abdominal region and measuring 25 cm on the vertical axis, 20 cm on the transverse axis, mobile when compared with the deep structures and gradually increasing in volume over childhood and neglected. After small biopsy, which showed desmoid tumor, the patient underwent complete surgical resection of the tumor with immediate reconstruction by free muscolo skin flap of the latissimus dorsi attached to the large blood vessels of the inguinal fold (left iliac artery and left external iliac vein), connected by termino lateral anastomosis. Flap survival was correctly performed and reconstruction was successful.
[Mh] Termos MeSH primário: Neoplasias Abdominais/cirurgia
Parede Abdominal/cirurgia
Fibromatose Agressiva/cirurgia
Procedimentos Cirúrgicos Reconstrutivos/métodos
[Mh] Termos MeSH secundário: Neoplasias Abdominais/patologia
Parede Abdominal/patologia
Adolescente
Biópsia
Fibromatose Agressiva/patologia
Retalhos de Tecido Biológico
Seres Humanos
Masculino
Músculos Superficiais do Dorso
Retalhos Cirúrgicos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170925
[Lr] Data última revisão:
170925
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170915
[St] Status:MEDLINE
[do] DOI:10.11604/pamj.2017.27.181.11028


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[PMID]:28819469
[Au] Autor:Nair KK; Chaudhuri K; Lingappa A; Shetty R; Vittobarao PG
[Ad] Endereço:Department of Oral Medicine and Radiology SJM Dental College and Hospital, Chitradurga, Karnataka, India.
[Ti] Título:Aggressive fibromatosis of the oral cavity in a 5 year old boy: a rare case report.
[So] Source:Pan Afr Med J;27:47, 2017.
[Is] ISSN:1937-8688
[Cp] País de publicação:Uganda
[La] Idioma:eng
[Ab] Resumo:Fibrous tissue proliferations express a wide spectrum of histologic and morphologic variation in both infants and adults. This ranges from hypertrophic scar formation at one end to malignant fibrosarcoma at the other end of the spectrum. Aggressive fibromatosis is an intermediate tumor which is in proximity to fibrosarcomas. These are locally invasive and often recur after excision, but do not metastasize. Histologically, they are characterized by proliferating fibroblasts with little mitotic activity. Aggressive fibromatosis in the head and neck region is not common, and very sporadically occurs in the oral cavity or jaw bones. Here we report a rare case of aggressive fibromatosis occurring in a 5 year old boy.
[Mh] Termos MeSH primário: Fibromatose Agressiva/diagnóstico
Neoplasias Bucais/diagnóstico
[Mh] Termos MeSH secundário: Pré-Escolar
Fibromatose Agressiva/patologia
Seres Humanos
Masculino
Neoplasias Bucais/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170904
[Lr] Data última revisão:
170904
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170819
[St] Status:MEDLINE
[do] DOI:10.11604/pamj.2017.27.47.11739


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[PMID]:28735069
[Au] Autor:Penel N; Le Cesne A; Bonvalot S; Giraud A; Bompas E; Rios M; Salas S; Isambert N; Boudou-Rouquette P; Honore C; Italiano A; Ray-Coquard I; Piperno-Neumann S; Gouin F; Bertucci F; Ryckewaert T; Kurtz JE; Ducimetiere F; Coindre JM; Blay JY
[Ad] Endereço:Medical Oncology Department, Centre Oscar Lambret, Lille, France. Electronic address: n-penel@o-lambret.fr.
[Ti] Título:Surgical versus non-surgical approach in primary desmoid-type fibromatosis patients: A nationwide prospective cohort from the French Sarcoma Group.
[So] Source:Eur J Cancer;83:125-131, 2017 Sep.
[Is] ISSN:1879-0852
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:PURPOSE: The outcome of desmoid-type fibromatosis (DTF) is unpredictable. Currently, a wait-and-see approach tends to replace large en bloc resection as the first therapeutic approach. Nevertheless, there are no validated factors to guide the treatment choice. METHOD: We conducted a prospective study of 771 confirmed cases of DTF. We analysed event-free survival (EFS) based on the occurrence of relapse after surgery, progressive disease during the wait-and-see approach, or change in therapeutic strategy. Identification of prognostic factors was performed using classical methods (log-rank test and Cox model). RESULTS: Overall, the 2-year EFS was 56%; this value did not differ between patients undergoing an operation and those managed by the wait-and-see approach (53% versus 58%, p = 0.415). In univariate analysis, two prognostic factors significantly influenced the outcome: the nature of diagnostic sampling (p = 0.466) and primary location (p = 0.0001). The 2-year EFS was only 32% after open biopsy. The 2-year EFS was 66% for favourable locations (abdominal wall, intra-abdominal, breast, digestive viscera and lower limb) and 41% for unfavourable locations. Among patients with favourable locations, the 2-year EFS was similar in patients treated by both surgery (70%) and the wait-and-see approach (63%; p = 0.413). Among patients with unfavourable locations, the 2-year EFS was significantly enhanced in patients initially managed with the wait-and-see approach (52%) compared with those who underwent initial surgery (25%; p = 0.001). CONCLUSION: The location of DTF is a major prognostic factor for EFS. If these findings are confirmed by independent analysis, personalised management of DTF must consider this easily obtained parameter.
[Mh] Termos MeSH primário: Fibromatose Agressiva/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Criança
Pré-Escolar
Terapia Combinada
Intervalo Livre de Doença
Feminino
Fibromatose Agressiva/terapia
Seres Humanos
Masculino
Meia-Idade
Recidiva Local de Neoplasia
Prognóstico
Estudos Prospectivos
Conduta Expectante
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170911
[Lr] Data última revisão:
170911
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170724
[St] Status:MEDLINE


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[PMID]:28668823
[Au] Autor:DE Marchis ML; Tonelli F; Quaresmini D; Lovero D; Della-Morte D; Silvestris F; Guadagni F; Palmirotta R
[Ad] Endereço:Interinstitutional Multidisciplinary Biobank (BioBIM), IRCCS San Raffaele Pisana, Rome, Italy.
[Ti] Título:Desmoid Tumors in Familial Adenomatous Polyposis.
[So] Source:Anticancer Res;37(7):3357-3366, 2017 07.
[Is] ISSN:1791-7530
[Cp] País de publicação:Greece
[La] Idioma:eng
[Ab] Resumo:Familial adenomatous polyposis (FAP) is a cancer syndrome caused by a germline mutation in the adenomatous polyposis coli (APC) gene. It is characterized by the presence of hundreds of colonic polyps, which have a high tendency to undergo malignant transformation. Among associated lesions in FAP, desmoid tumors represent a common possible life-threatening condition that requires special attention. They are rare tumors occurring with a particularly high incidence in FAP, especially after surgery. In agreement with Knudson's 'two-hit' theory, the inactivation of the residual APC gene in FAP is a critical step in the development of both colorectal cancer and desmoids. Several lines of evidence show that germline mutations affect the functional domains of the APC gene that are responsible for interactions of the transcript with ß-catenin, whereas somatic second mutations involve the downstream region of the gene. Hence, an understanding of the molecular pathways underlying desmoid progression in FAP could be important for research and a valid resource for the early prevention and tailored treatment of this disease. In this review, we provide an updated insight into desmoids in FAP syndrome, from molecular pathogenesis to the main issues in management, with special attention given to genetic and molecular features of these tumors.
[Mh] Termos MeSH primário: Polipose Adenomatosa do Colo/patologia
Fibromatose Agressiva/patologia
Neoplasias/patologia
[Mh] Termos MeSH secundário: Polipose Adenomatosa do Colo/genética
Animais
Fibromatose Agressiva/genética
Genes APC/fisiologia
Mutação em Linhagem Germinativa/genética
Seres Humanos
Incidência
Neoplasias/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170906
[Lr] Data última revisão:
170906
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170703
[St] Status:MEDLINE


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[PMID]:28657872
[Au] Autor:Benech N; Walter T; Saurin JC
[Ad] Endereço:Hospices Civils de Lyon, Lyon, France jean-christophe.saurin@chu-lyon.fr.
[Ti] Título:Desmoid Tumors and Celecoxib with Sorafenib.
[So] Source:N Engl J Med;376(26):2595-2597, 2017 06 29.
[Is] ISSN:1533-4406
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Celecoxib/uso terapêutico
Fibromatose Agressiva/tratamento farmacológico
Niacinamida/análogos & derivados
Compostos de Fenilureia/uso terapêutico
[Mh] Termos MeSH secundário: Polipose Adenomatosa do Colo/complicações
Adulto
Feminino
Fibromatose Agressiva/diagnóstico por imagem
Fibromatose Agressiva/etiologia
Seres Humanos
Imagem por Ressonância Magnética
Meia-Idade
Recidiva Local de Neoplasia/tratamento farmacológico
Niacinamida/uso terapêutico
Tomografia Computadorizada por Raios X
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Nm] Nome de substância:
0 (Phenylurea Compounds); 25X51I8RD4 (Niacinamide); 9ZOQ3TZI87 (sorafenib); JCX84Q7J1L (Celecoxib)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170718
[Lr] Data última revisão:
170718
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170629
[St] Status:MEDLINE
[do] DOI:10.1056/NEJMc1702562


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[PMID]:28489620
[Au] Autor:Penel N; Chibon F; Salas S
[Ad] Endereço:aDepartment of Medical Oncology, Centre Oscar Lambret, Lille bDepartment of Biopathology, Institut Bergonié, Comprehensive Cancer Centre, Institut National de la Santé et de la Recherche Medicale (INSERM) U916, Bordeaux cDivision of Adult Oncology, Department of Medicine, Aix-Marseille Université, INSERM U910 and Timone Hospital, Marseille, France.
[Ti] Título:Adult desmoid tumors: biology, management and ongoing trials.
[So] Source:Curr Opin Oncol;29(4):268-274, 2017 Jul.
[Is] ISSN:1531-703X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE OF REVIEW: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors. RECENT FINDINGS: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/ß-catenin pathway, sporadic desmoid tumors are associated with somatic mutations of CTNNB1, and germline mutations of APC and somatic mutations of CTNNB1 are probably mutually exclusive. One-third of desmoid tumors are misdiagnosed; a second pathological opinion is therefore of major importance for desmoid tumor. Surgery is no longer regarded as the cornerstone of desmoid tumors; several retrospective studies have demonstrated the safety of a 'wait and see' policy in sporadic abdominal wall desmoid tumor. Desmoid tumors is no longer regarded as an absolute contraindication for pregnancy. At least two new investigational drugs targeting the Wnt/APC/ß-catenin pathway are currently being developed. SUMMARY: The management of desmoid tumors requires multidisciplinary expertise by an experienced team. We must fully understand the physiopathology of the disease (factors influencing the natural history of the disease) and learn how to avoid desmoid tumors occurrence in patients with APC germline mutations, identify reliable prognostic/predictive factors and better assess the efficacy of systemic treatment.
[Mh] Termos MeSH primário: Fibromatose Agressiva/patologia
Fibromatose Agressiva/terapia
[Mh] Termos MeSH secundário: Adulto
Seres Humanos
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170920
[Lr] Data última revisão:
170920
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170511
[St] Status:MEDLINE
[do] DOI:10.1097/CCO.0000000000000374


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[PMID]:28439797
[Au] Autor:Martínez Trufero J; Pajares Bernad I; Torres Ramón I; Hernando Cubero J; Pazo Cid R
[Ad] Endereço:Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain. jmtrufero@seom.org.
[Ti] Título:Desmoid-Type Fibromatosis: Who, When, and How to Treat.
[So] Source:Curr Treat Options Oncol;18(5):29, 2017 May.
[Is] ISSN:1534-6277
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OPINION STATEMENT: Desmoid-type fibromatosis is a sarcoma subtype that gathers some singular characteristics, making it a difficult challenge to face in clinical practice. Despite its excellent survival prognosis, these tumors may be unpredictable, ranging from an asymptomatic indolent course to persistent, local, and extended recurrences that significantly impair quality of life. Although surgery was initially considered the first elective treatment, collected published data during the past few years are now pointing to the "wait and see" approach as a reasonable initial strategy because many patients can live a long life with the disease without having symptoms. When symptoms appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) can be useful in improving symptoms and controlling the disease. Because of the low incidence of desmoid-type fibromatosis, there is scarce scientific evidence supporting any specific treatment. Nonetheless, if volumetric responses are needed, chemotherapy may be a reasonable early option. However, if long-term control of disease is desirable, hormonal therapy, NSAIDs, and TKIs are the likely treatments of choice. Recent new findings in the biologic development of these tumors, such as the role of Wnt/ß-catenin dependent pathway, have shown that the prognostic information provided by specific CTNNB1 gene mutations and other genetic profiles can lead to better methods of selecting patients as candidates for other approaches. Based on recent research, the Notch pathway inhibition in DF is one of the most promising potential targets to explore. As an orphan disease, it is mandatory that as many patients as possible be included in clinical trials.
[Mh] Termos MeSH primário: Fibromatose Agressiva/terapia
Sarcoma/terapia
[Mh] Termos MeSH secundário: Biomarcadores Tumorais
Tomada de Decisão Clínica
Terapia Combinada/efeitos adversos
Terapia Combinada/métodos
Gerenciamento Clínico
Fibromatose Agressiva/diagnóstico
Fibromatose Agressiva/etiologia
Fibromatose Agressiva/mortalidade
Hormônios/metabolismo
Seres Humanos
Mutação
Prognóstico
Sarcoma/diagnóstico
Sarcoma/etiologia
Sarcoma/mortalidade
Transdução de Sinais
Resultado do Tratamento
Conduta Expectante
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Biomarkers, Tumor); 0 (Hormones)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171105
[Lr] Data última revisão:
171105
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170426
[St] Status:MEDLINE
[do] DOI:10.1007/s11864-017-0474-0


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[PMID]:28420393
[Au] Autor:Scheer L; Lodi M; Molière S; Kurtz JE; Mathelin C
[Ad] Endereço:Unité de Sénologie, Hôpital Hautepierre, Hôpitaux Universitaires de Strasbourg, CHRU, 1 Avenue Molière, 67200, Strasbourg, France.
[Ti] Título:Medical treatment of mammary desmoid-type fibromatosis: which benefit?
[So] Source:World J Surg Oncol;15(1):86, 2017 Apr 18.
[Is] ISSN:1477-7819
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Breast fibromatosis is a rare disease characterized by monoclonal fibroblast proliferation. It has no ability to metastasize but has a high local recurrence rate and often infiltrates surrounding tissues. Surgical treatment is the reference, but recently, new targeted therapies have emerged. We report an original case of a patient with breast fibromatosis who received exclusive medical treatment. Our aim was to analyze these treatments based on the clinical and radiological outcome, iatrogenic effects, and pharmacological action. CASE PRESENTATION: We report the case of a 19-year-old woman who developed a desmoid-type fibromatosis of the lower inner quadrant of the right breast, measuring 50 × 25 mm (i.e., a volume of 27.4 cm ). Initial surgery was not possible because of potential esthetic and functional prejudice. Thus, she had an exclusive medical treatment including several lines: NSAIDs with tamoxifen and triptorelin, followed by sorafenib, then interferon α2b, and finally sunitinib. With tyrosine-kinase inhibitors (TKIs) (sunitinib), a significant partial response was observed (57% reduction of the maximal tumoral volume). For each treatment, we provided the clinical and radiological outcome in association with known pharmacological action. CONCLUSIONS: TKI had been an interesting alternative option to initial surgery, providing at least a partial response and potentially allowing less mutilating surgery. However, no pharmacological mechanism can unequivocally explain TKI efficacy. In general, breast fibromatosis should be treated along with oncologist and interventional radiologists in a trans-disciplinary modality, thus offering an adapted treatment for this particular desmoid-type fibromatosis localization.
[Mh] Termos MeSH primário: Neoplasias da Mama/tratamento farmacológico
Fibromatose Agressiva/tratamento farmacológico
Inibidores de Proteínas Quinases/uso terapêutico
[Mh] Termos MeSH secundário: Adulto
Neoplasias da Mama/patologia
Feminino
Fibromatose Agressiva/patologia
Seres Humanos
Prognóstico
Proteínas Tirosina Quinases/antagonistas & inibidores
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Protein Kinase Inhibitors); EC 2.7.10.1 (Protein-Tyrosine Kinases)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171031
[Lr] Data última revisão:
171031
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170420
[St] Status:MEDLINE
[do] DOI:10.1186/s12957-017-1148-x


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[PMID]:28410293
[Au] Autor:Palassini E; Frezza AM; Mariani L; Lalli L; Colombo C; Fiore M; Messina A; Casale A; Morosi C; Collini P; Stacchiotti S; Casali PG; Gronchi A
[Ad] Endereço:From the Departments of *Medical Oncology, †Statistics, and ‡Surgery, IRCCS Fondazione Istituto Nazionale Tumori; §Department of Medical Oncology, Humanitas Cancer Center, IRCCS; and Departments of ∥Radiology and ¶Pathology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy.
[Ti] Título:Long-term Efficacy of Methotrexate Plus Vinblastine/Vinorelbine in a Large Series of Patients Affected by Desmoid-Type Fibromatosis.
[So] Source:Cancer J;23(2):86-91, 2017 Mar/Apr.
[Is] ISSN:1540-336X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: Today, surgery and radiation therapy have a limited role in desmoid-type fibromatosis. Different systemic treatments were shown to be effective. Herein, we report on our institutional experience with low-dose methotrexate (MTX) + vinca alkaloids in this disease over the last 25 years. METHODS: We retrospectively reviewed data from all adult patients with sporadic desmoid-type fibromatosis treated with MTX and vinca alkaloids at our institution between 1989 and 2014. RESULTS: We identified 75 patients treated with MTX + vinblastine (40%), MTX + vinorelbine (57%), and vinorelbine alone (3%). All patients had progressive disease before chemotherapy; 72%, 10%, and 48% of patients had received previous surgery, radiation therapy, and/or systemic treatments, respectively. Chemotherapy was administered for a median duration of 14 months and a median number of 37.5 cycles. Eight patients interrupted chemotherapy because of toxicity. According to RECIST (Response Evaluation Criteria in Solid Tumors) complete response, partial response, stable disease, and progressive disease were observed in 1%, 47%, 51%, and 1% of patients, respectively. Symptomatic relief was obtained in 80% of symptomatic cases. The median progression-free survival (PFS) was 75 months; it was 136 months in responding patients. Upon progression, after chemotherapy withdrawal, MTX plus vinblastine/vinorelbine was offered to 11 patients with partial response, stable disease, and progressive disease in 4, 6, and 1 cases, resulting in a median PFS of 53 months. CONCLUSIONS: In this series, chemotherapy with MTX and vinca alkaloids is confirmed to be active and effective, with a remarkable PFS, higher in responding patients, and limited toxicity. Even progression can be successfully rechallenged.
[Mh] Termos MeSH primário: Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
Fibromatose Agressiva/tratamento farmacológico
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Feminino
Seres Humanos
Masculino
Metotrexato/administração & dosagem
Meia-Idade
Estudos Retrospectivos
Vimblastina/administração & dosagem
Vimblastina/análogos & derivados
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
5V9KLZ54CY (Vinblastine); Q6C979R91Y (vinorelbine); YL5FZ2Y5U1 (Methotrexate)
[Em] Mês de entrada:1705
[Cu] Atualização por classe:170526
[Lr] Data última revisão:
170526
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170415
[St] Status:MEDLINE
[do] DOI:10.1097/PPO.0000000000000254



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