Aggressive Intra-Abdominal Fibromatosis in Children and Response to Chemotherapy

Alebouyeh, Mardawig; Moussavi, Farideh; Tabari, Ahmad Kahlegh-Nejad; Vossough, Parvaneh

doi:10.1080/08880010591002215

Cited by 8 publications

(7 citation statements)

References 15 publications

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“…This treatment modality should therefore be used as sparingly as possible in children [12]. Radiation therapy must be considered in life-threatening conditions as the last resort in a growing child and may be proposed as an alternative to mutilating surgery [26]. …”

Section: Discussionmentioning

confidence: 99%

Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Oudot

Orbach

Minard‐Colin

et al. 2012

Sarcoma

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Background. Only limited data are available concerning desmoid tumor in children. Methods. Fifty-nine children and adolescents with desmoid tumor treated in 2 French cancer centers with a very long followup were retrospectively reviewed. Results. Median age was 6 years (range, 0–15). Tumors mainly involved the limbs (42%). Five cases occurred in a context of genetic disorder. Surgery was first-line treatment in 80% of cases. Resection was microscopically complete in 3 patients (pts), with a microscopic residue in 19 pts and a macroscopic residue in 35 cases. Various adjuvant therapies were used. Overall response to all systemic therapies was 33%. Thirty-eight patients developed one or more recurrences or progressions. After a median followup of 8.5 years, 34 patients were alive in complete remission (CR), including 16 first CR. Seven patients died, 6 from refractory disease and 1 from colorectal carcinoma in a genetic context. Ten-year progression-free survival (PFS) and overall survival were 31% and 88%, respectively. In univariate analysis, age less than 10 years and head-neck site were favorable prognostic factors for PFS. Conclusions. When surgery is required, surgical margins must be negative. Low-dose chemotherapy can be proposed as adjuvant therapy. Prospective trials must be developed to evaluate long-term response and side effects.

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Section: Discussionmentioning

confidence: 99%

Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Oudot

Orbach

Minard‐Colin

et al. 2012

Sarcoma

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“…Surgery is the primary treatment, with the goal of complete resection with negative margins, but mutilating surgery is avoided [366][367][368]. Surgery combined with chemotherapy has reduced the recurrence rate [333,364,369,370]. Irradiation may not be effective in children [371].…”

Section: Desmoid Fibromatosismentioning

confidence: 99%

Fibroblastic and Myofibroblastic Tumors in Children and Adolescents

2012

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Fibroblastic and myofibroblastic tumors in children and adolescents are a relatively common group of soft tissue proliferations that range from reactive to hamartomatous to neoplastic, with a full spectrum of benign, intermediate, and malignant neoplasms. These lesions are diagnostically challenging because of morphologic and immunohistochemical overlap, despite significant clinical, genetic, and prognostic differences. The fibromatoses are a major subgroup, and all types of fibromatoses can occur in the 1st 2 decades of life. Intermediate and malignant fibroblastic-myofibroblastic tumors are an important group that includes variants of fibrosarcoma and other tumors with recurrent cytogenetic or molecular genetic abnormalities and low metastatic potential. Pathologic examination is enhanced by adjunct techniques, such as immunohistochemistry, cytogenetics, and molecular genetics, although morphology provides the ultimate criteria for a specific diagnosis. This article reviews the clinicopathologic features of fibroblastic and myofibroblastic tumors with an emphasis on the unique aspects of these neoplasms in children and adolescents, the use of diagnostic adjuncts, and differential diagnoses.

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“…We identified 141 patients with DF in the head and neck [ 7 , 8 , 13 – 72 ]. Age at diagnosis ranged from birth to 18 years, and the mean age was 4 years 3 months.…”

Section: Discussionmentioning

confidence: 99%

Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature

et al. 2016

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BackgroundDesmoid-type fibromatosis is defined as an intermediate tumor that rarely occurs in the head and neck of children. There is no doubt as to the value of complete surgical excision for desmoid-type fibromatosis. However, in pediatric patients, surgeons may often be concerned about making a wide excision because of the potential for functional morbidity. Some studies have reported a lack of correlation between margin status and recurrence. Therefore, we discussed our findings with a focus on the state of surgical margins.Case presentationWe report an unusual case of a 9-month-old Japanese girl who prior to presenting at our hospital underwent debulking surgery twice with chemotherapy for desmoid-type fibromatosis of the tongue at another hospital. We performed a partial glossectomy and simultaneous reconstruction with local flap and achieved microscopic complete resection. We also reviewed available literature of pediatric desmoid-type fibromatosis in the head and neck.ConclusionsWe described successful treatment for the refractory case of pediatric desmoid-type fibromatosis. The review results showed that some microscopic incomplete resections of tumors in pediatric patients with desmoid-type fibromatosis tended to be acceptable with surgical treatment.

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Aggressive Intra-Abdominal Fibromatosis in Children and Response to Chemotherapy

Cited by 8 publications

References 15 publications

Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Desmoid Fibromatosis in Pediatric Patients: Management Based on a Retrospective Analysis of 59 Patients and a Review of the Literature

Fibroblastic and Myofibroblastic Tumors in Children and Adolescents

Desmoid-type fibromatosis of the head and neck in children: a case report and review of the literature

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