scholarly journals Target therapy with celecoxib in pediatric recurrent desmoid tumors. A case report

Author(s):  
A. Jové Blanco ◽  
J. Huerta Aragonés ◽  
C. Mata Fernández
2021 ◽  
Vol 28 (5) ◽  
pp. 3659-3667
Author(s):  
David Chan ◽  
Jason Kaplan ◽  
Gary Gordon ◽  
Jayesh Desai

Desmoid tumors (aggressive fibromatosis) are soft tissue mesenchymal tumors that can be locally invasive and life-threatening. Depending on the location, these tumors are often unresectable or tend to recur after surgery. To date, there are no approved systemic therapies for desmoid tumors. These tumors typically harbor mutations in the β-catenin oncogene CTNNB1 or the tumor suppressor gene adenomatous polyposis coli, resulting in constitutive activation of the WNT pathway. The Notch pathway is part of the underlying cause for desmoid tumor development, possibly due to crosstalk with the WNT pathway, providing a rationale for Notch inhibition as a therapeutic strategy. The gamma secretase activation of the Notch receptor can be targeted with investigational gamma secretase inhibitors. In this case report, we follow the course of 2 patients with desmoid tumors treated with the highly potent, parenterally administered investigational gamma secretase inhibitor AL101, resulting in long-lasting responses. Case 1 reports on a patient with a mesenteric desmoid tumor who participated in a phase 1 trial and then transitioned into a compassionate use program; Case 2 reports on a patient with recurrent pelvic tumors receiving AL101 through a compassionate use program. After tumor progression on other systemic therapies, Cases 1 and 2 had confirmed partial responses (41% and 60% maximal tumor size decrease from baseline) recorded after 1.0 and 1.6 years of treatment with AL101, with a duration of response of 8.6+ and 2.6+ years, respectively. Also, in a phase 1 study of AL102, a potent orally administered gamma secretase inhibitor that shares structural features with AL101, a patient with a desmoid tumor was noted to have tumor shrinkage. Formal clinical testing of AL102 for the treatment of patients with desmoid tumors that are not amenable to surgery or are refractory to/recurrent from other prior therapies is currently underway.


Medwave ◽  
2020 ◽  
Vol 20 (07) ◽  
pp. e8010-e8010
Author(s):  
Víctor Manubens-Vargas ◽  
Manuel Rodríguez-Ortubia ◽  
Alejandra Salas-Gianini ◽  
Fernando Valenzuela ◽  
Laura Carreño-Toro

2011 ◽  
Vol 99 ◽  
pp. S368
Author(s):  
A. Parisi ◽  
S. Takanen ◽  
V. Graziano ◽  
B. Resuli ◽  
S. Fouraki ◽  
...  

Mastology ◽  
2020 ◽  
Vol 30 (Suppl 1) ◽  
Author(s):  
Luciana Carvalho Horta ◽  
Fabia Beraldo Silveira ◽  
Bruno Henrique Alvarenga ◽  
Anna Carolina Pereira Jácome ◽  
Juliana Cristina de Oliveira Lima

Desmoid tumors are neoplasms that originate from fascial or musculoaponeurotic structures, constituted by fibroblast proliferation. It has no metastatic or differentiation potential, but is locally aggressive and has high rates of recurrence even after complete resection. It represents 0.03% of all neoplasms. This study aimed at reporting a rare clinical case of a young patient diagnosed with a desmoid tumor of the breast after a silicone implant surgery.


2020 ◽  
Vol 7 ◽  
Author(s):  
Amine Benjelloun ◽  
Ikram Samri ◽  
Hicham Janah ◽  
Salah Belassri ◽  
Rachid Benchanna ◽  
...  

Background : Desmoid tumors are rare  and represent 0.03% of all solid tumors. Their histology is benign but they are willingly aggressive with significant locoregional invasions and frequent recurrences.Case presentation : We report the case of a 52-year-old patient who presented with a  scapular and pulmonary localization of this tumor, but stable over time. In view of potentially decaying surgery and stability of the lesions, simple monitoring was decided. The lesions are currently stable, two years after diagnosis. We discuss the clinical, radiological and therapeutic characteristics of this type of tumor, in the light of data from the literature.Conclusion : Desmoid tumors are rare, benign but agressive. Surgery is the main treatment but is often decaying. Simple monitoring can be scheduled in case of less agressive tumors.


2012 ◽  
Vol 3 (8) ◽  
pp. 158-159
Author(s):  
Dr.Mohammed Raza ◽  
◽  
Dr.Shivanand Reddy K.V

2001 ◽  
Vol 44 (1) ◽  
pp. 89 ◽  
Author(s):  
Yong Il Lee ◽  
Hae Kyung Lee ◽  
Hyun Sook Hong ◽  
Kui Hyang Kwon ◽  
Deuk Lin Choi ◽  
...  

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 25-26
Author(s):  
Juliana Santos Carvalho ◽  
Anna Flávia Silva Alves Silva ◽  
Maysa Coité Silva ◽  
Itala Gomes Santos ◽  
Laryssa Pinto Aragão ◽  
...  

INTRODUCTION: The occurrence of acute myeloid leukemia (AML) and chronic lymphoid leukemia (B-CLL) simultaneously is rarely described. We describe a case report of AML and B-CLL, diagnosed simultaneously, without any previous treatment for any of the hematological neoplasms [1]. The patient received low-dose cytarabine (ARA-C) and Venetoclax, which is medically indicated on label for both hematological neoplasms. CASE REPORT: A 75-year-old male, presented with edema and joint pain one month before hospital admission, showing pancytopenia on a complete blood count and presence of blasts in peripheral blood. The patient was then referred to the hematology service. The morphological analysis of the bone marrow aspirate showed 67.7% of myeloblasts, compatible with AML. Bone marrow immunophenotyping was performed, which identified 34.10% of myeloblasts, compatible with AML and 50.48% of monoclonal B lymphocytes (chronic B-cell lymphoproliferative disease). In flow cytometry there were two distinct populations of myeloblasts. Type 1 myeloblasts labeling CD7 +, CD13 +, CD34 ++, CD38 ++, CD45 ++, CD56 ++, CD117 ++, CD123 +, HLA-DR +++ and MPO + / ++. The second population marked CD13 + / ++, CD34 + / ++, CD38 ++, CD45 +, CD117 ++, CD123 +, HLA-DR ++ / +++ and MPO + (30%). Monoclonal lymphocytes showed CD11c + / ++ (70%), CD19 ++, CD20 + / ++ (84%), CD22 + (39%), CD23 + / ++, CD25 +, CD31 +/-, CD43 ++, CD45 ++ / +++, CD81 + (38%), CD200 + (85%) and Lambda +. The molecular study was negative for genetic abnormalities: FLT3, KIT and NPM, configuring the patient as an intermediate risk for AML. In the cytogenetic analysis there was no growth of metaphases. Patient received simultaneous diagnosis of AML and B-CLL. As he was ineligle to intensive chemotherapy (IC), we started original protocol Subcutaneous Cytarabin+venetoclax(VIALE C). The patient had grade 2-3 AE(neutropenia managed with GCSF) ending the fourth cycle in July 2020. The evolution of hematimetric parameters and diseases are described in graphics. DISCUSSION: This is the first described case in our knowledge treated upfront with bcl2-inh target therapy for two absolutely different hematological neoplasms: AML and BCLL. Nowadays we are experiencing a new therapeutic model in oncohematology, in which the targeted therapy is gaining ground in relation to IC with excellent results. In this way, the importance of comprehension of the pathophysiological mechanism of the neoplasms and the way we can stop the disease proliferation is progressively guiding the new protocols. Elderly patients are more likely to have early treatment-related death and exhibit therapeutic resistance, limiting alternatives. We decided to start first-line treatment with ARA-C and Venetoclax [2]. Venetoclax associated with ARA-C has a manageable safety profile, producing quick and durable remissions in elderly people with AML ineligible for IC, as well as in B CLL, being the best therapeutic alternative for the case, in our opinion. Venetoclax belongs to a group of drugs called Bcl-2 inhibitors, an anti-apoptotic protein, which works by blocking this protein in the body, causing apoptosis of both neoplastic cells. The high rate of remission and low early mortality, combined with fast and durable remission, make Venetoclax and ARA-C a new and attractive treatment for the elderly [2]. In our case, the intention of the product in the first line was not B-CLL, but it would certainly be a good option for this profile of elderly patients. CONCLUSION: We report the first description of simultaneous diagnosis of AML and B CLL treated with a Bcl-2 inhibitor, demonstrating that antitumor mechanisms can be extremely effective in completely different diseases. We have a long way to go in the search for full knowledge of oncohematological diseases and targeted therapies. However, this case report shows that we are on the right track. References: 1. MUSSAED, Eman Al; OSMAN, Hani; ELYAMANY, Ghaleb. Simultaneous existence of acute myeloid leukemia and chronic lymphocytic leukemia: a case report.Bmc Cancer.Springer Science and Business Media LLC. http://dx.doi.org/10.1186/s12885-016-2780-5. 2. WEI, Andrew H.; et al. Venetoclax Combined With Low-Dose Cytarabine for Previously Untreated Patients With Acute Myeloid Leukemia: results from a phase ib/ii study.Journal Of Clinical Oncology, [S.L.], 20 maio 2019. American Society of Clinical Oncology (ASCO). http://dx.doi.org/10.1200/jco.18.01600. Figure Disclosures De Queiroz Crusoe: Janssen:Research Funding.


2011 ◽  
Vol 3 (2) ◽  
pp. 100-102
Author(s):  
Neelamma Patil ◽  
GR Sajjan ◽  
Tejaswini Vallabh ◽  
Jyoti Korbu ◽  
Shilpi Aggarwal

ABSTRACT Postoperative desmoid tumors in postmenopausal women is a rare condition. Desmoid tumors are unusual soft tissue benign tumors that arise from musculoaponeurotic tissues. They also have ingrowing tendencies to intestines causing unusual clinical presentation. Recent surgical intervention is known in such cases. FNAC is not contributory due to hypocellularity. CT pelvis, abdomen is helpful. Surgical treatment is usually required. Case report is of this unusual condition in our practice.


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