Infiltrating Myxofibrosarcoma of the Forearm: An Infrequent Entity of Difficult Diagnosis and Complex Treatment, which Requires Great Collaboration

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.

 

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Keywords

myxofibrosarcoma - soft tissue sarcomas - subdermal tumor - resection limits - “tail sign”

Introduction

Soft tissue sarcomas are a heterogeneous group of lesions of mesenchymal origin. According to the latest WHO classification, they are characterized not only by their rarity but also by their often-unpredictable behavior and a high tendency to be confused with benign pathologies. In this article, we describe a type of tumor that is not so uncommon, according to the literature, with aggressive, infiltrating characteristics, a tendency to recur, and the potential for metastasis. We want to emphasize the importance of diagnosis with imaging tests and the use of contrast agents, and the surgical solutions we have had to adopt.

Myxofibrosarcoma (MFS) was described in 1977 by Enzinger and Weiss, as a subtype of malignant fibrous histiocytoma, and it was not until 2002, when the WHO reclassified it as a distinct entity characterized by myxoid stroma, great pleomorphism and a high propensity to local recurrence, although in the latest classification of 2020 it is included within the subgroup of fibroblastic or myofibroblastic tumors, as a malignant tumor.[1]

MFS is one of the most common sarcomas, usually affecting the extremities in elderly patients. Its macroscopic appearance is typically characterized by one or multiple soft, gelatinous nodules located superficially.[2] In the earliest stages, it can appear as a small, painless subdermal tumor, which can easily be mistaken for a benign skin condition. Furthermore, the diffuse pattern of the tumor contributes to the increased burden of the lesion, as another characteristic is its high recurrence rate.

In imaging studies, such as ultrasound and MRI, these tumors can be mistaken for less aggressive conditions.

Therefore, the objective of this article is to review the bibliography of this pathology, highlighting the importance of a multidisciplinary team, where diagnosis with imaging tests (particularly MRI) helps in correct preoperative planning, locating the limits of the lesion to achieve a complete resection, as well as subsequent reconstruction and adjuvant treatment to prevent recurrence.

 

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Materials and Methods

We present two cases of MFS received by our upper limb department in the last year. Both were initially diagnosed and treated as less aggressive: the first as nodular fasciitis and the second as a subdermal angioma. In both patients, initial surgery had imprecise margins, and subsequent pathological examination of the specimen confirmed the diagnosis of MFS, with affected margins.

The first case involved a 65-year-old man with a subdermal tumor located in the proximal and ulnar aspect of the forearm over the Extensor Carpi Ulnaris (ECU) muscles, which had been developing slowly for several months. He was referred to our clinic after an initial surgery consisting of an excisional biopsy and a pathological diagnosis of MFS, with affected resection margins. Clinically, only the scar from the previous surgery was observed, without any tumor being observed, since this had been exercised in the previous surgery. Skin alterations, such as inflammation or edema due to venous or lymphatic hyperpressure, were not observed.

The second case involved a 76-year-old man who had also undergone surgery for a tumor located on the dorsal aspect of the forearm, above the middle third and centered on the extensor digitorum communis (EDM). In this case, we should mention that he had a transverse scar that occupied the entire back of the forearm ([Fig. 1]). Similarly, the results of the pathological anatomy (PA) following the excision of the lesion had indicated the existence of an intermediate-grade MFS with affected surgical margins.

Fig. 1 Dorsal scar of the forearm from our second case patient. No associated tumors are observed. This type of transverse scar implies involvement and possible contamination of all extensor compartments located beneath it.

The studies prior to the initial surgery, consisting of ultrasound and MRI, were available: in both cases, the initial presumptive diagnosis leaned toward much more trivial conditions:

• In case 1, the MRI was interpreted as a solid, superficial lesion with a spindle-shaped or flat shape located in the subcutaneous tissue and dorsal fascia of the forearm, with linear projections extending to the skin ([Fig. 2]). The diagnosis was oriented towards a tumor of myofibroblastic origin, although the morphology of the lesion without the presence of a dominant nodule suggested that the process could be benign in the context of nodular fasciitis or fibromatosis. A biopsy was recommended.

• In case 2, the patient was referred from another center. Preoperative MRI, also with contrast, indicated the presence of a nodule in the deep subcutaneous tissue, without invading the muscles, which was interpreted as a hemangioma. Adjacent to the nodule, a curvilinear contrast enhancement was seen, representing the tail sign ([Fig. 3]).

Fig. 2 Preoperative MRI images from Case 1. Sagittal slice (A) and axial slice (B). The white arrows indicate a solid, fusiform, elongated lesion rather than a nodular one. It is located in the deep subcutaneous tissue, thickening the dorsal and mainly ulnar fascia of the forearm, with extension into the superficial subcutaneous tissue and dermis (arrowhead). It is considered a lesion with poorly defined or irregular borders and without muscle infiltration. MFS lesions are usually nodular in shape; this elongated or plaque-like morphology is less common. This appearance suggested nodular fasciitis as the most likely diagnosis.

Fig. 3 Preoperative MRI images from Case 2. (A) Sagittal slice with a line marking the level of the axial slices. (B) Axial slice enhanced with STIR, and (C) axial T1-weighted slice with fat suppression after contrast administration. In all three images, the white arrows point to the main tumor nodule located in the deep subcutaneous tissue in contact with the fascia. The laminar or curvilinear perifascial and fascial extension is indicated with dashed arrows. The lesion does not invade deep planes, and in this case, there is no or very minimal subdermal infiltration. It was initially diagnosed as a vascular malformation, likely an angioma.

Once referred to our Unit with a diagnosis of MFS, updated MRI imaging studies were requested, including diffusion and perfusion analysis after contrast administration. These tests primarily revealed the presence of a "superficial" solid and flat tumor affecting the dermis, hypodermis, and both the superficial and deep subcutaneous tissues, located beneath the previously created scar. The lesions exhibited multidirectional extension with circumferential growth following the fascial plane of the extensors, along with an infiltrative pattern that could suggest lymphatic and vascular dissemination. In neither case was subfascial infiltration observed, although there were concerns about possible contact with the underlying musculature.

The second surgery was planned preoperatively, based on the references obtained from MRI studies, which were able to indicate both the depth of the involvement and the margins in the coronal and sagittal planes of the forearm. We should add a safety margin of at least 1.5 cm to achieve an extended marginal resection. In both cases, the surgery was performed with an ischemia cuff, created by elevation, to avoid problems arising from compression by the Smarch bandage.

As references, it is always a good idea to rely on structures that may be involved, so in the first case, which had a more ulnar location, the Basilica Vein was affected close to the extension edge, and therefore it served as a reference to maintain an extended safety margin when resecting it ([Fig. 4]).

Fig. 4 Corresponds to patient Case 1: A: Image of the forearm. Label 1 marks the excisional biopsy scar, which showed contaminated margins in the pathology report. It is located over the extensor carpi ulnaris muscle. Nearby, the relationship with the basilic vein is marked as 2, and label 3 indicates the expanded resection margins measured by MRI, with a 1.5 cm safety margin. B: Sagittal MRI image showing the flat, hyperintense tumor located 5.35 cm from the distal edge of the ulna (in this plane) and extending 6.54 cm. The margins identified on MRI are fundamental for surgical margin planning. C: Axial forearm image at the mid-third level showing a remnant of the solid lesion at the dorsal and ulnar fascia of the left forearm, with infiltration into the subcutaneous tissue and dermis, and proximity to the basilic vein (2). The lesion does not cross the superficial forearm fascia and does not invade muscle groups.

Once the mapping of the area to be excised has been performed, we must also be clear about the deep limit; in both cases, they were located subdermally, and the fascia always behaves as a "natural barrier" against the spread of tumors.[3] [4] Even so, having performed a previous surgery with incorrect margins, it was assumed that this one could be affected, so that it was best to increase the deep resection plane to the muscles immediately in contact with it, which were the ECU and the extensor proprio of the 5th finger in the first case ([Fig. 5]), and the EDM partially (only the most dorsal fascicles), to avoid further iatrogenesis possibly unnecessary due to the loss of digital extension, and without the need to perform subsequent transfers, given his age, in the second patient.

Fig. 5 Extracompartmental tumor resection (case 1) according to the margins marked by MRI. As the deep limit of the previously biopsied area, the muscle in intimate contact. Here, the extensor carpi ulnaris (1) was taken, exposing the ulna (2). Marked as 3, the basilic vein can be seen included within the resection margin.

As the previously mapped area was excised, it was decided to send small quadrants from medial to lateral and from distal to proximal of the excised tissue margins to Pathology as an extemporaneous biopsy, since macroscopically, the tissue we removed had a completely normal appearance. Of all the margins that were sent intraoperatively, we were informed of histological normality pending histochemical confirmation, except for the distal medial margin of case 2, in which nonspecific myxoid tissue was detected, so we increased that margin by 1.5 cm more ([Fig. 6]).

Fig. 6 Anatomical specimen from case 2. Margins are marked with sutures of different colors: Proximal (P), Distal (D), Ulnar or Medial (C), and Radial or Lateral (R). "Punches" (S) are visible, which are fragments of the specimen sent for intraoperative frozen section analysis. The previous surgery scar and the deep muscle bed, corresponding in this case to a muscular margin of the extensor digitorum communis, are also visible

Once the complete excision was performed, greased gauze with Nitrofural ointment was applied to prevent drying and over-infection of the exposed tissue. This bandage was kept with the limb elevated to prevent edema and was changed after 48 hours using a dressing performed under sedation using a negative pressure system (VAC). This dressing was maintained with successive changes every 5 or 6 days until the results of the pathological anatomy were obtained. These were obtained after three weeks, at which time the defect was covered with a partial skin mesh graft ([Fig. 7]). Before this and as added security, laminar samples were taken from the exposure surface, just below the area where the primary lesion was located, so that PA could confirm (although not immediately) the existence of the free margin in depth.

Fig. 7 Partial meshed skin graft in patient from Case 2, one week after placement under a negative pressure wound therapy system (VAC) to better conform to the bed created by the surgical defect.

Subsequently, once the graft had taken root, the patients underwent adjuvant radiotherapy on the forearm bed and compartmental margin of up to 50 Gy during 25 sessions at 2 Gy per session.

 

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Results

In both cases, the PA studies reported the presence of a diffuse myxofibrosarcoma, with wide free margins greater than 1 cm, and close to the resected muscle at the deep level.

Both patients healed from the defects created by surgical excision, and after radiation therapy, there were no coverage defects or scarring changes in the grafted area.

Regarding hand function, this was partially altered by the resection of part of the extensor muscle tissue in both cases, but also by scar adhesions of the muscles exposed to the skin graft area ([Fig. 8]).

Fig. 8 Functional outcome of case 1 after adjuvant radiotherapy. Loss of extension of the fifth finger's extensor tendon and loss of ulnar extension capacity can be observed due to their removal along with the tumor specimen. The remaining extensors, despite scar adhesions to the graft, are functional. However, there is a noticeable loss of wrist flexion, although finger flexion is preserved.

Post-treatment MRI studies showed postoperative changes with no pathology, and extension studies with a thoracic-abdominal CT scan for possible metastases indicated no distant disease.

Both patients are currently undergoing clinical follow-up and imaging studies for possible recurrence. These studies will be continued for at least 5 years to determine disease remission.

 

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Discussion

MFS is not a tumor as rare as some clinical articles describe;[5] in fact, its frequency is quite high in elderly patients, its incidence being more frequent in the lower limb than in the upper.[6] Its problem lies in three determining factors. The first is its benign appearance, which often leads to inadequate primary treatment. The second, its great capacity to simulate other processes, with a diffuse pattern of subcutaneous distribution, which makes diagnosis difficult, and therefore difficult to know what the origin of our patient's problem may be. Thus, in the literature, we can see cases diagnosed a posteriori and with a discouraging outcome, having been confused with an entrapment of the Median Nerve in the context of an acute Carpal Tunnel Syndrome, or with an inflammatory condition such as Erosive Arthritis.[7] [8] Thirdly, we know that these tumors lack a pseudocapsule and may show non-palpable dissemination along superficial tissues, as well as muscle bundles and fascial planes.[9] Therefore, their diagnosis will be based on clinical suspicion but fundamentally on imaging tests; however, the difficulty in interpreting them lends itself to confusion and, therefore, can lead to important errors in their surgical management.[2]

Undoubtedly, the lesion may go unnoticed on clinical examination, as it appears as subtle soft tissue nodules that are not considered problematic. MRI with intravenous contrast (gadolinium) and perfusion studies are key to a correct diagnosis. The presence of a high myxoid matrix content provides a high signal on T2-weighted sequences, and the morphology and growth of the lesion, with an infiltrative pattern, are appreciated as a multidirectional curvilinear projection that extends along the fascial plane, showing a signal intensity similar to that of the main mass and the same enhancement after contrast injection ([Fig. 9]). This finding is known as the "tail sign"[10], and although it is not exclusive to MFS, it should make us think about it when dealing with superficial dissemination, and is related to a higher risk of local recurrence after surgical excision and the appearance of distant metastases.[11] Therefore, the detection of this pattern is of great relevance for the complete and effective excision of the lesion, dictating the need for wider resection margins to include the entire "tail".

Fig. 9 Axial MRI images corresponding to case 1 (A) and 2 (B), taken before compartmental resection surgery performed after the excisional biopsy in patient No. 1 (black arrow) and after the resection of the main nodule in patient No. 2 (black arrow). In both cases, a curvilinear projection (white arrows) extending dorsally along the fascial plane is still present, representing residual tumor tissue, showing a signal intensity similar to the original mass, as seen in [figures 2] (case 1) and 3 (case 2). Linear projections and signal alterations extending into the superficial subcutaneous tissue and dermis (white arrowheads) are also observed.

There are many published articles on MFS, but few of these addresses the surgical aspect, and even fewer address the disseminated form, located in the topography of the hand and forearm, where they can be more difficult to treat. Most works refer to nodular tumors and sequenced surgeries according to their recurrence,[12] which in most cases are considered benign tumors with incorrect marginal resection, and subsequently referred to reference units such as the study by Sabri,[13] on 129 cases treated at the Rizzoli Institute over 25 years and with an estimated survival rate of 73%.

The importance of maintaining a wide margin, combined with a possible natural barrier such as the fascia within the excised specimen, is essential to prevent recurrence,[14] [15] as the recurrence rate is the highest of all soft tissue sarcomas, ranging from 20% to 60% after five years. It is also important to highlight that between 15% and 38% of local recurrences of MFS evolve into a higher histological grade of disease with greater metastatic potential, which ranges between 20% and 25%.[6] [16] [17]

Although the prognosis of MFS is indeed relatively better than that of other types of sarcomas, it depends on the histological grade of the primary tumor, therefore, the state of the surgical margins will act as a predictor of future survival, since local recurrences can be predicted by their quality, as well as distant metastases since these depend on histological grade, and as has been described, this increases as the recurrences occur.[18]

The description of an extensive resection of skin, subcutaneous tissue, and fascia is very well described by Batista,[19] although the idea of performing primary coverage at the same time as the excision, without knowing the margins obtained, forced him to extend them three weeks later and perform a new graft with abdominal skin. For this reason, we prefer to take extemporaneous samples, which, while not free from error, at least provide information that gives us a good expectation or confidence in the correct marginality obtained. We believe it is better to wait with a negative pressure system (VAC), capable of maintaining good endothelial perfusion of the exposed tissues, until we have the final results of the margins obtained, also taking into account that there is no demonstrated increased rate of local recurrence with the use of these systems in tumor surgery.[20] [21] [22] [23] [24]

The use of meshed skin grafts, instead of vascularized coverage flaps, which could potentially result in a smaller aesthetic defect and better gliding of the extensor tendons underneath, thereby avoiding functional loss of wrist flexion, was considered and carried out for several reasons. These included the patients' age, to avoid creating a significant morbidity zone at the donor site, and to allow for radiotherapy to be applied based on the healing or vascularization of the grafts with the shortest possible waiting time. Additionally, a major advantage of using meshed grafts is that, if recurrences occur, they can be quickly diagnosed through clinical examination, thus preventing further delays. It has also been demonstrated that meshed grafts are well tolerated in these cases, with minimal comorbidity and dysfunction. Indeed, this approach would not be viable in the more distal areas of the hand, where tendon exposure would require coverage with fatty flaps to allow exposed tendons to glide properly. In such cases, negative pressure therapy systems could still be used, but would require moisture control to prevent tendon tissue desiccation while awaiting pathology results. Temporary skin substitute dressings could be an option, but they carry the risk of contamination and margin extension if a safe margin is not confirmed, potentially triggering recurrences and thereby worsening the prognosis.[25]

Regarding the application of radiotherapy in cases of MFS, it remains an unknown, since in most of the consulted texts we can observe that, although it is considered especially in cases of recurrence or unsafe margins, it does not seem to considerably reduce the recurrence rate,[17] [26] just as when a "tail sign" appears, in which case radiotherapy, whether neoadjuvant or postsurgical, may have an imprecise prognosis,[27] making control of safe margins essential. Cases have been described in which, considering the age of the patient and the difficulty of surgical excision, due to involvement of noble anatomical structures, which could endanger the viability of the upper limb, were treated solely with radiotherapy associated with brachytherapy.[28]

 

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Conclusions

MFS is a tumor with low prevalence in the upper limb, but among the rare soft tissue sarcomas affecting this region, it is one of the most frequent. Clinically, it is difficult to differentiate from more benign processes, and for this reason, it is sometimes misdiagnosed and incorrectly treated, even when imaging studies are performed, as these can be quite nonspecific and lead to confusion unless the possibility of this entity is specifically considered. Key features to raise suspicion include subcutaneous spread around the nodule and the presence of the "tail sign," which indicates fascial extension and suggests a worse prognosis in cases of insufficient resection margins. Even when adjuvant measures such as radiotherapy are applied, inadequate margins can lead to recurrences, metastasis, and a considerable mortality rate, ranging between 25% and 40% of patients at five years.

Therefore, surgery is the fundamental part of the treatment, and we must plan it so that it is sufficiently extensive to resect the possible "tail" of the tumor. To achieve this, the subcutaneous and fascial excision must be extensive. This planning must be carried out in conjunction with radiologists, who can map and provide us with the most precise limits of the resection we will need to complete.

Coverage can be deferred until PA results confirm clear margins, at which point skin coverage can be safely performed using a procedure tailored to the patient's circumstances and the area of the limb to be treated.

 

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Conflicto de Intereses

Los autores declaran no tener ningún conflicto de intereses.

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.

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Publication History

Received: 03 July 2024

Accepted: 07 April 2025

Article published online:
21 July 2025