Treatment of infantile fibrosarcoma associated to an abdominal aortic aneurysm with larotrectinib: a case report

Sánchez, María Dolores Corral; Gómez, Víctor Galán; Urgelles, Ana Sastre; Sabando, Diego Plaza López de; Aparicio, P; Martínez, Leopoldo; Gamarra, Eduardo A.; Kreilinger, José Juan Pozo; Zapata, Rita María Regojo; Gutiérrez, Juan Carlos López; Alvarado, Eugenia Antolín; Martín, Felipe Gómez; Torres, Ana Sánchez; Valle, Luis González del; Pérez‐Martínez, Antonio

doi:10.1080/08880018.2021.1889730

Cited by 6 publications

(4 citation statements)

References 20 publications

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“…The study demonstrated an overall response rate of 94% in paediatric patients with solid tumors containing fusions of the NTRK gene who received larotrectinib ( 20 ). Increasing evidence also supports the clinical benefits associated with larotrectinib ( 3 , 4 , 21 , 22 ). However, caution should be exercised due to the potential presence of publication bias in the available reports.…”

Section: Discussionmentioning

confidence: 90%

Larotrectinib treatment for infantile fibrosarcoma in newborns: a case report and literature review

Wang¹,

Zhang²,

Wang³

et al. 2023

Front. Oncol.

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Infantile fibrosarcoma (IFS) is a rare tumor in childhood characterized by a single, localized, painless mass that grows rapidly but has a relatively indolent biological behavior and a favorable prognosis. Eighty-five percent of infantile fibrosarcomas are associated with t (12;15) (p13;25) chromosomal translocation resulting in ETV6-NTRK3 gene fusion, which provides the target for targeted therapy. Here, we report a case of IFS in a newborn with a mass in the left lower extremity confirmed by imaging, histopathological examination, tissue FISH testing, and high-throughput sequencing to detect gene rearrangement. Based on gene fusion targeted drug testing results, the patient was treated with standard doses of larotrectinib, resulting in significant mass shrinkage with no adverse effects, demonstrating the treatment effect of targeted therapy. This case provides a reference for using larotrectinib in newborns with IFS.

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Section: Discussionmentioning

confidence: 90%

Larotrectinib treatment for infantile fibrosarcoma in newborns: a case report and literature review

Wang¹,

Zhang²,

Wang³

et al. 2023

Front. Oncol.

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“…When radical resection is unfeasible, neo-adjuvant chemotherapy using vincristine, actinomycin, and cyclophosphamide shows responses, allowing the posterior excision of the tumor. 1,4 Other reported cases where resistant to the treatment or had serious complications as neutropenia. 7,8 Recently, genomic studies have been conducted allowing to know the molecular origin for these tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Surgery remains the gold standard treatment but when it is not feasible, neo-adjuvant chemotherapy maybe effective. 1 3 4 Recently targeted therapies had been used to treat locally advanced and chemotherapy-resistant sarcomas with good results. 5 6 7 We report two cases of non-surgical treatment in two neonates.…”

Section: Introductionmentioning

confidence: 99%

Larotrectinib as an Effective Therapy in Congenital Infantile Fibrosarcoma: Report of Two Cases

Moratilla

Caldas

Ramirez

et al. 2022

European J Pediatr Surg Rep.

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Congenital infantile fibrosarcoma (CIF) is a rare tumor in children that occurs in the first years of life. It usually arises in the extremities but some cases affect the trunk, neck, abdomen, or retroperitoneum. Surgical resection has been traditionally the treatment of choice but the development of genomic analysis and targeted therapies has shed light on new therapeutic options.We present two patients with a congenital mass, one in the abdominal cavity (1-month-old) and the second in the left lower extremity respectively (2-months-old). In both cases, the clinical and radiological findings showed heterogeneous masses with rapidly progressive growth. MRI in the first patient exhibited an abdominal mass surrounding the aorta and inferior vena cava associated with a giant infrarenal aortic aneurysm. CT-guided biopsy was performed with pathological findings of fibrosarcoma and ETV6-NTRK3 gene fusion. The second patient underwent open biopsy also with histopathological diagnosis of fibrosarcoma and the same mutation in the TRK gene (NTRK3). Targeted therapy with a specific TRK inhibitor, larotrectinib, was started in both patients. Periodical controls were made by ultrasound or MRI, and after a few weeks of treatment, both children showed significant decrease in the mass. By the second and third months after starting the treatment, both tumors disappeared. The first patient is now 15-months-old and the second one is 8-months-old.Larotrectinib is a novel targeted therapy with excellent results in CIF but long-term outcomes are limited to establish it as a gold standard treatment.

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“…Malignant neoplasias in children and adolescents are uncommon but represent an important impact on health in these age groups [ 1 ]. Despite the gradual reduction in mortality due to neoplasia, cancer still represents the primary cause of death by disease among children and adolescents [ 2 ]. Soft tissue sarcomas make up 7%–15% of paediatric solid tumours.…”

Section: Introductionmentioning

confidence: 99%

Paediatric fibrosarcoma treatment

Cano-Padilla,

Ramírez,

Cervantes-Rivera

et al. 2023

ecancer

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Introduction Soft tissue sarcomas make up 7%–15% of childhood solid tumours. The aetiology of this disease is unknown. It is a fast-growing, painless tumour; histologically similar to adult fibrosarcoma, but having a lesser risk of metastasis and a better prognosis. The treatment is aimed towards localised intervention; complete surgical resection is the appropriate treatment as long as it can be performed. Case report An 11 years old female was referred for resection of a soft tissue tumour on the right elbow with significant peripheral vascularisation. Tumour resection was scheduled, with the placement of a partial thickness skin graft, and a piece was sent to pathology; a histological type consistent with paediatric fibrosarcoma was obtained with margins less than 1 mm from the lesion. Therefore, the patient was referred to the paediatric oncology unit. Further studies with positron emission tomography were requested, in which no evidence of macroscopic anatomy-metabolic tumour activity was found. Subsequently, treatment was started by paediatric oncology with 2 sessions of chemotherapy and 20 sessions of radiotherapy with sufficient progress; finally, assessment by plastic and reconstructive surgery was performed and an adequate quality of graft was observed, without the need for any other intervention by their service. Conclusion The involvement of the vascular surgeon in performing the tumour resection permitted the preservation of the best circulation to the extremity, thereby, avoiding amputation. The difficult decision made by the reconstructive surgeon to place a partial thickness graft over the surgical site, and to start radiotherapy/chemotherapy by paediatric oncology, were key to the success in achieving the patient’s satisfactory progress.

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Treatment of infantile fibrosarcoma associated to an abdominal aortic aneurysm with larotrectinib: a case report

Cited by 6 publications

References 20 publications

Larotrectinib treatment for infantile fibrosarcoma in newborns: a case report and literature review

Larotrectinib treatment for infantile fibrosarcoma in newborns: a case report and literature review

Larotrectinib as an Effective Therapy in Congenital Infantile Fibrosarcoma: Report of Two Cases

Paediatric fibrosarcoma treatment

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