Clinical management of pediatric aggressive fibromatosis involving the mandible

Zhou, Yong; Zhang, Zhiyuan; Fu, Honghai; Qiu, Weimin; Wang, Lizhen; He, Yue

doi:10.1002/pbc.24145

Cited by 7 publications

(5 citation statements)

References 10 publications

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“…Current literature indicates total resection (negative surgical margins) as the best intervention and recurrence prevention. 3 , 5 Still, desmoid head and neck tumors are not always resectable, and when they are, surgical procedures that functionally or aesthetically compromise a child’s facial structure are not advisable. 5 , 6 Additionally, because recurrence is not exclusive to those who possess residual tumor, and no prospective studies have been conducted establishing predictive patterns of recurrence, current recommendations are to closely monitor patients postoperatively no matter the margins achieved.…”

Section: Discussionmentioning

confidence: 99%

“… 1 Pediatric fibromatoses are often locally aggressive and invade into adjacent tissue walls and bone, making them difficult to fully expunge. 2 , 3 Still, some studies advocate for a “wait-and-see” approach that favors surveillance and conservative intervention dependent on tumor location, size, and growth rate. 2 Fibromatoses exhibit a tendency toward recurrence, with reports suggesting higher rates in patients who do not undergo complete excision, or for those for whom negative margins are impossible.…”

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confidence: 99%

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Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning

Brown,

Torres,

Nuñez

et al. 2024

Plastic and Reconstructive Surgery - Global Open

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Summary: A 13-year-old girl with a painful left neck mass was referred to our institution due to suspicions of malignancy. The patient reported pain that accompanied her frequent neck spasms. Computed tomography revealed a large, soft-tissue mass in the left neck, deep to the sternocleidomastoid. The lesion anteriorly displaced the internal carotid artery and both displaced and crushed the internal left jugular vein. Uniquely, a three-dimensional virtual reality model combining magnetic resonance imaging and computed tomography data was used to determine the lesion’s resectability and visualize which structures would be encountered or require protection while ensuring total resection. During operation, we confirmed that the mass also laterally displaced the brachial plexus, cranial nerves X and XI, and spinal nerves C3–C5 (including the phrenic) of the cervical plexus. Postsurgical pathological analysis confirmed a diagnosis of desmoid tumor, also known as aggressive fibromatosis, whereas DNA sequencing revealed a CTNNB1 mutation, a somatic genetic marker found in approximately 90% of desmoid tumor cases. When possible, the most widely used method for the treatment of desmoid tumors has been gross resection. Chemotherapy, radiotherapy, and local excision are also used in the treatment of fibromatoses when complete resection is judged infeasible. In this case, a complete surgical resection with tumor-free surgical margins was performed. A standard cervical approach with a modified posterolateral incision site was implemented to avoid a conspicuous anterior neck scar. No flap, nerve repair, or reconstruction was warranted. At 1 year of postsurgical follow-up, the patient showed minimal scarring and no signs of recurrence.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning

Brown,

Torres,

Nuñez

et al. 2024

Plastic and Reconstructive Surgery - Global Open

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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 fibromatosis also called desmoid tumors (DTs) are soft tissue malignancies originating from fascial planes, connective tissues, and musculoaponeurotic structures of the muscles [1]. DTs are rare and constitute less than 3% of all soft tissue tumors and 0.03% of all neoplasms [2].…”

Section: Introductionmentioning

confidence: 99%

The Management of Desmoid Tumors: A Retrospective Study of 30 Cases

Zenzri

Yahyaoui

Charfi

et al. 2020

International Journal of Surgical Oncology

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Objectives. Desmoid tumor also called aggressive fibromatosis is a rare type of benign tumor. It is a mesenchymal malignancy without metastatic potential. The standard management is resection, but other options including observation may be discussed. Desmoid-type fibromatosis may occur throughout the body, but the abdominal wall is the most common site. The aim of our study was to assess the clinicoepidemiological profile, prognostic factors, and treatment outcome of desmoid tumors. Methods. A monocentric retrospective study was conducted over a period of 19 years between February 2000 and November 2019 at the oncology department of Salah Azaïz Institute. Our study concerns 30 patients with desmoid tumor. All data regarding patients were obtained from the medical record. Results. Thirty patients were included. The median age was 35 years with a female predominance (sex ratio = 0.07). A palpable mass was the most common complaint (n = 27). Median tumor size was 5 cm. The principal site of involvement was the abdominal wall (n = 14). Surgery was performed in 27 patients. The histopathology reports listed 14 (52%) cases with negative margins and 13 (48%) cases with positive margins. Radiation therapy was performed in 2 patients. One patient received tamoxifen. Local recurrence occurred in 11 patients. Two patients died of their desmoid tumor. Abdominal wall tumors have less risk of recurrence compared with other sites (p=0.047). Macroscopic margin involvement (R2) was the only prognostic factor influencing disease-free-survival (p=0.034). Conclusion. Desmoid tumors are aggressive tumors with a tendency for local recurrence. Abdominal wall tumors have less risk of recurrence. Macroscopic margin involvement was the only prognostic factor that affects disease-free-survival.

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Clinical management of pediatric aggressive fibromatosis involving the mandible

Abstract: For the treatment of pediatric AF, we recommend complete tumor resection. As for the involved mandible, preserving the mandible contour and continuity as much as possible and providing adjunctive therapy, such as cryotherapy or cauterization, are vital. RT is not recommended.

Cited by 7 publications

References 10 publications

Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning

Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning

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

The Management of Desmoid Tumors: A Retrospective Study of 30 Cases

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