Subscribe to RSS

DOI: 10.1055/s-0045-1809552
Infiltrating Myxofibrosarcoma of the Forearm: An Infrequent Entity of Difficult Diagnosis and Complex Treatment, which Requires Great Collaboration
Article in several languages: español | English
Abstract
Introduction
Myxofibrosarcoma (MFS) is one of the most common sarcomas affecting the extremities in elderly patients. Clinically, it may present as a tumor with a benign appearance, but its aggressive features and tendency for easy local recurrence with increasing aggressiveness classify it as a malignant tumor within the subgroup of fibroblastic or myofibroblastic tumors.
We aim to highlight the need for an aggressive diagnostic and therapeutic consensus, with the goal of preserving limb integrity while minimizing the chances of recurrence. Therefore, we emphasize the importance of multidisciplinary work, utilizing Magnetic Resonance Imaging (MRI) for lesion mapping.
Materials and Methods
We present two cases of superficial and diffuse MFS of the forearm, previously intervened as supposedly benign lesions, but in which, later, the anatomopathological study demonstrated the presence of an aggressive lesion with infiltration of the edges. Control studies with MRI showed that the extension of the tumor was greater than preconceived due to the clinically visible nodule and allowed us to correctly perform surgical planning for the extended marginal excision of the lesion. Subsequently, the different stages for the reconstruction of the defect created are discussed.
Results
In both cases, resections were achieved with wide margins and optimal results in terms of functionality and quality of life.
Discussion
We evaluate the diagnostic characteristics of these lesions with clinical and imaging studies (MRI) and their differential diagnosis with other pathologies. We address the surgical options and how we should approach complete resection and subsequent reconstruction to avoid recurrence, as well as the need for adjuvant treatment and follow-up with serial radiological studies in case of recurrence or metastatic disease.
Conclusions
Among soft tissue sarcomas, we find a rare entity in the upper limb, but we must be aware of it, especially its radiological characteristics, in order to be able to treat it as safely as possible to avoid its progression.
Ethical Responsibilities and Protection of People and Animals
The authors declare that the procedures followed were by the ethical standards of the committee responsible for human experimentation and by the World Medical Association and the Declaration of Helsinki.
Data Confidentiality
The authors declare that they have followed their workplace protocols on the publication of patient data.
Patient Privacy and Informed Consent
The authors have obtained informed consent from the patients and/or subjects referred to in the article. This document is in the possession of the corresponding author.
Publication History
Received: 03 July 2024
Accepted: 07 April 2025
Article published online:
21 July 2025
© 2025. SECMA Foundation. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
Thieme Revinter Publicações Ltda.
Rua Rego Freitas, 175, loja 1, República, São Paulo, SP, CEP 01220-010, Brazil
-
Referencias
- 1 Sbaraglia M, Bellan E, Dei Tos AP. The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives. Pathologica 2021; 113 (02) 70-84
- 2 Vanni S, De Vita A, Gurrieri L. et al. Myxofibrosarcoma landscape: diagnostic pitfalls, clinical management and future perspectives. Ther Adv Med Oncol 2022; 14: 17 588359221093973
- 3 Sambri A, Caldari E, Fiore M. et al. Margin Assessment in Soft Tissue Sarcomas: Review of the Literature. Cancers (Basel) 2021; 13 (07) 1687
- 4 Martín D. La cirugía en el diagnóstico y tratamiento de los sarcomas de partes blandas. En Guia OncoSur de Sarcomas de partes blandas. Sociedad Española de Oncología Médica. Ed. Arán Ediciones, S.L. 2017. . ISBN: 978-84-17046-16-3
- 5 Ulla-Anes M, Palma-Huertas E, Pena-Burgos M, Matarranz-del Amo M, Torres-Macho J. Myxofibrosarcoma: a rare diagnosis. Rev Esp Casos Clin Med Intern 2023; 8 (02) 84-87 (RECCMI)
- 6 Zumárraga JP, Batista FAR, Baptista AM, Caiero MT, Martino LPR, de Camargo OP. Prognostic factors in patients with appendicular myxofibrosarcoma. Acta Ortop Bras 2018; 26 (05) 320-324 [online]
- 7 Batra S, Batra M, Sakamuri R, Sinha AK, Kanvinde R. High-Grade Infiltrative Myxofibrosarcoma in the ForearmPresenting as Acute Carpal Tunnel Syndrome. PubMed
- 8 Colak C, Kilpatrick SE, Mesko NW, Winalski CS. Upper extremity myxofibrosarcoma mimicking an erosive inflammatory arthritis: a case report. Skeletal Radiol 2019; 48 (10) 1643-1649
- 9 Jagannathan JP, Tirumani SH, Ramaiya NH. Imaging in soft tissue sarcomas: current updates. Surg Oncol Clin N Am 2016; 25 (04) 645-675
- 10 Lefkowitz RA, Landa J, Hwang S. et al. Myxofibrosarcoma: prevalence and diagnostic value of the “tail sign” on magnetic resonance imaging. Skeletal Radiol 2013; 42 (06) 809-818
- 11 Spinnato P, Clinca R, Vara G. et al. MRI features as prognostic factors in myxofibrosarcoma: Proposal of MRI grading system. Acad Radiol 2021; 28 (11) 1524-1529
- 12 Lucarelli E, De Vita A, Bellotti C. et al. Modeling Myxofibrosarcoma: Where Do We Stand and What Is Missing?. Cancers (Basel) 2023; 15 (21) 5132
- 13 Sambri A, Tuzzato G, Spinnato P, De Paolis M, Donati DM, Bianchi G. La clasificación del mixofibrosarcoma de las extremidades puede predecir la supervivencia y el control local. Tratamiento Oncol Res. 2020; 43: 189-195
- 14 Gronchi A, Colombo C, Raut PC. Manejo quirúrgico de tumores localizados de tejidos blandos. Cancer 2004; 120 (17) 2638-2648
- 15 Yurtbay A, Coşkun HS, Say F, Dabak N. Is the Thickness of the Margin Associated With Local Recurrence and Survival in Patients With Myxofibrosarcoma?. Clin Orthop Relat Res 2023; 481 (11) 2125-2136
- 16 Willems SM, Debiec-Rychter M, Szuhai K, Hogendoorn PCW, Sciot R. Local recurrence of myxofibrosarcoma is associated with increase in tumour grade and cytogenetic aberrations, suggesting a multistep tumour progression model. Mod Pathol 2006; 19 (03) 407-416
- 17 Manoso MW, Pratt J, Healey JH, Boland PJ, Athanasian EA. Infiltrative MRI pattern and incomplete initial surgery compromise local control of myxofibrosarcoma. Clin Orthop Relat Res 2006; 450 (450) 89-94
- 18 Sanfilippo R, Miceli R, Grosso F. et al. Myxofibrosarcoma: prognostic factors and survival in a series of patients treated at a single institution. Ann Surg Oncol 2011; 18 (03) 720-725
- 19 Batista KT, Martins VCS, Schwartzman UPY, Ferreira TL. Surgical Reconstruction after Resection of a Large Myxofibrosarcoma of the Upper Extremity. Rev Bras Ortop 2019; 54 (03) 353-356
- 20 Bickels J, Kollender Y, Wittig JC, Cohen N, Meller I, Malawer MM. Vacuum-assisted wound closure after resection of musculoskeletal tumors. Clin Orthop Relat Res 2005; 441: 346-350
- 21 Sakellariou VI, Mavrogenis AF, Papagelopoulos PJ. Negative-pressure wound therapy for musculoskeletal tumor surgery. Adv Skin Wound Care 2011; 24 (01) 25-30
- 22 Chen YU, Xu S-F, Xu M, Yu X-C. Use of negative pressure wound therapy as an adjunct to the treatment of extremity soft-tissue sarcoma with ulceration or impending ulceration. Oncol Lett 2016; 12 (01) 757-763
- 23 Lazerges C. Sarcomas de tejidos blandos del antebrazo, muñeca y mano. Rehabilitación Quirúrgica De La Mano 2017; 36 (04) 233-243
- 24 Gusho C, Phillips R, Cook J, Evenski A. Phillips.; Cook, J.; Evenski, A. A systematic review and meta-analysis of negative wound pressure therapy use in soft tissue sarcoma resection. Iowa Orthop J 2023; 43 (02) 52-59
- 25 Dobke M, Mackert GA. Upper extremity sarcoma: impact of current practice guidelines and controversies on reconstructive approaches. SICOT J 2017; 3: 15-15
- 26 Teurneau H, Engellau J, Ghanei I. et al. Alta tasa de recurrencia del mixofibrosarcoma: el efecto de la radioterapia no está claro. Sarcoma 2019; 2019: 8517371
- 27 van Ravensteijn SG, Nederkoorn MJL, Wal TCP. et al. The Prognostic Relevance of MRI Characteristics in Myxofibrosarcoma Patients Treated with Neoadjuvant Radiotherapy. Cancers (Basel) 2023; 15 (10) 2843
- 28 Murakami N, Nakatani F, Takahashi K, Nakamura S, Igaki H, Shikama N. Salvage high-dose rate brachytherapy for myxofibrosarcoma of the brachium: a technical report. J Radiat Res 2023; 64 (04) 746-749