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Hand Surgery and Rehabilitation 36 (2017) 233–243

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Recent advance

Soft tissue sarcomas of the forearm, wrist and hand


Tumeurs malignes des parties molles de l’avant-bras, du poignet et de la main

C. Lazerges
Chirurgie de la main et du membre supérieur, chirurgie des nerfs périphériques, hôpital Lapeyronie, CHRU de Montpellier, 191, avenue du
Doyen-Gaston-Giraud, 34295 Montpellier cedex 5, France

A R T I C L E I N F O A B S T R A C T

Article history: Soft tissue sarcoma of the forearm, wrist and hand are rare. Their benign appearance leads often to
Received 26 September 2016 primary inadequate treatment. Due to the complex anatomy of the hand and forearm, they are
Received in revised form 4 December 2016 challenging to treat. The two goals are to obtain wide resection of the primary tumor while preserving
Accepted 22 December 2016
function. Limb-sparing surgery is now the cornerstone for the treatment of most sarcomas of the
Available online 16 June 2017
forearm, hand and wrist. To achieve optimal oncological and functional outcomes, the surgical excision
should be associated with early reconstructive procedures and a multidisciplinary meeting to define the
Keywords:
treatment strategy including adjuvant medical treatments. This article outlines the current principles
Soft tissue sarcoma
Wrist
and presents the results of the treatment of soft tissue sarcomas with emphasis on to particularities
Hand related to their forearm, wrist and hand location.
C 2017 SFCM. Published by Elsevier Masson SAS. All rights reserved.

Limb-sparing surgery
Reconstructive surgery

R É S U M É

Mots clés : Les tumeurs malignes des parties molles de l’avant-bras, du poignet et de la main sont des tumeurs rares,
Sarcome tissus mous souvent d’apparence bénigne et régulièrement initialement négligées. De par la complexité de
Main l’anatomie de la main et de l’avant-bras, leur prise en charge est difficile et représente un vrai challenge
Poignet
thérapeutique. L’objectif à atteindre est de concilier à la fois une exigence carcinologique et une exigence
Chirurgie conservatrice
Chirurgie reconstructrice
fonctionnelle d’importance majeure à l’avant-bras, au poignet et à la main. La chirurgie d’exérèse
conservatrice est aujourd’hui la pierre angulaire du traitement de la plupart des sarcomes à l’avant-bras,
au poignet et à la main. Pour permettre un résultat carcinologique et fonctionnel optimal, cette exérèse
chirurgicale doit être associée à une chirurgie reconstructrice précoce et à une prise en charge
multidisciplinaire, afin de définir un projet thérapeutique global incluant les traitements médicaux
adjuvants. Cet article précise les principes actuels et les résultats de prise en charge des tumeurs
malignes des parties molles en insistant sur les aspects spécifiques des localisations à l’avant-bras, au
poignet et à la main.
C 2017 SFCM. Publié par Elsevier Masson SAS. Tous droits réservés.

1. Introduction affect the hand or wrist [5–7]. STS are histologically heteroge-
neous, with about 50 different histological kinds described [8]. In
Malignant tumors of the soft tissues, or soft tissue sarcomas the upper limb, the most common STS are synovial sarcomas,
(STS), are rare malignant tumors. They make up less than 1% of epithelioid sarcomas and malignant histiocytofibromas [5,9]
newly diagnosed cancers each year [1]. Their incidence is 2–3 cases (Table 1).
per 100,000 [2] and there are an estimated 3000 to 4000 new cases These tumors in the forearm, wrist and hand are challenging to
each year in France [3,4]. About 60% of these STS develop in the treat. Optimal treatment for the cancer should be balanced with
limbs, of which 15% to 25% affect the upper limb and only 5% to 10% minimal impact on function.
Since the end of the 1970s, radical treatment by amputation is
no longer the reference surgical treatment for these lesions
E-mail address: c-lazerges@chu-montpellier.fr [10,11]. Limb-sparing excision and reconstructive surgery are now

http://dx.doi.org/10.1016/j.hansur.2016.12.010
2468-1229/ C 2017 SFCM. Published by Elsevier Masson SAS. All rights reserved.
234 C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243

Table 1 the diagnosis [18], it has no certitude and cannot substitute for a
The most common histological types of soft tissue sarcomas in the hand, wrist and biopsy.
forearm.
Local CT scan of the STS region is only relevant if MRI cannot be
Malignant histiocytofibroma (undifferentiated pleomorphic sarcoma)
performed, or if bone invasion is present.
Synovial sarcoma
Epithelioid sarcoma
Chest and abdomen/pelvis CT with contrast is the gold standard
Clear-cell sarcoma examination for disease staging. Metastasis typically impacts the
Fibrosarcoma lungs, infrequently the soft tissues or abdomen in certain
Leiomyosarcoma histological STS types (myxoid liposarcoma, clear-cell sarcoma)
Liposarcoma
and even more rarely the lymph nodes (clear-cell sarcoma,
Malignant peripheral nerve sheath tumor
Dermatofibrosarcoma protuberans epithelioid sarcoma, synovial sarcoma, rhabdomyosarcoma, angio-
Myxoinflammatory fibroblastic sarcoma sarcoma). In certain histological STS types, a positron emission
tomography (PET) scan, which has growing indications for STS
[19–21], can be proposed in the context of disease staging or of
used in the hand and wrist [12–14]. To achieve the best possible recurrences. However, this examination is not currently recom-
functional and cancer-related outcomes, this surgical excision mended in the context of STS care [20].
must be combined with early reconstructive surgery and Lastly, vascular imaging (angiography, CT angiography, MR
multidisciplinary care, in order to define the overall treatment angiography) can be indicated, particularly for tumors requiring
plan including adjuvant medical treatments (chemotherapy and complex reconstruction.
radiation therapy). The current principles of the various steps of Carrying out this complete diagnostic strategy will reduce the
the care of these STS are presented in succession, with specific probability of diagnostic errors and inappropriate surgical
features related to the forearm, hand and wrist. treatments. Also, a complete preoperative imaging work-up will
make surgical revisions easier to perform, if needed.
2. Diagnosis
3. Treatment
The clinical presentation of STS is often misleading and can
appear trivial [15]. In most cases, a subcutaneous mass is discovered The treatment principles for STS in the hand, wrist and forearm
by chance, sometimes during the final healing phases of an injury; it are widely described in the literature and do not differ from the
is often painless and without associated symptoms. In this location, treatment of STS in other locations [3,15,22–24]. There are
the tumors are usually small – less than 4 cm – in size [5]. The however some distinctive features in the hand and wrist.
chronicity of the mass is not evidence of its benign nature and rapid Because of their small size and more superficial nature, STS of
progression is more suggestive a high-grade tumor. It is important the hand, wrist and forearm are often operated on right away
to remember that in the hand and wrist, there are more benign than without appropriate consideration of the possibility of malignancy,
malignant tumors. However, any atypical ‘‘suspicious’’ mass must with or without an incomplete, or even inappropriate preoperative
receive comprehensive care, including a full clinical examination work-up. This was the case for 38% of patients in the Pradhan study
and imaging work-up. If malignancy is still suspected after this [25] and 68% of patients in the Bray study [26]. And yet, the impact
assessment, a primary biopsy is recommended. of the initial surgery on the risks of residual or recurrent disease it
The clinical examination must qualify the mass (size, adhesion, is widely reported in the literature [27–30], along with the
hardness, cutaneous invasion, etc.), evaluate the local consequen- importance of care in a specialized cancer center [31–33] and a
ces (functional, vascular, neurological) and in general, palpate the review of the case in a multidisciplinary meeting (MDM) before
lymph nodes (certain tumors target the lymph nodes: epithelioid treatment is initiated [22].
sarcomas, clear-cell sarcomas, synovial sarcomas, rhabdomyosar-
coma) and look for familial disease that is often associated with a 3.1. Biopsy
tumor (e.g. malignant tumor of the peripheral nerve sheaths and
type I neurofibromatosis, Li-Fraumeni syndrome, etc.) or even The first phase of care is the biopsy. It will be used to confirm
exposure to carcinogens (herbicides, pesticides, radiation) [15]. the benign or malignant nature of the mass, classify the tumor,
The imaging work-up must include standard X-ray views to rule plan the type of surgery needed in an optimal manner and in
out bone tumors or invasion of neighboring bones. advanced STS cases, to consider performing neoadjuvant preoper-
Ultrasound imaging, which is commonly available and non- ative treatments (chemotherapy, isolated limb perfusion, radio-
irradiating, now has very extensive indications for the assessment therapy) [3].
of forearm, wrist and hand masses. While ultrasonography only Not every soft tissue mass in the hand and forearm require a
amounts to a scout scan, it makes it possible to evaluate the liquid biopsy. There are many more benign than malignant tumors. If the
or solid, homogeneous or heterogeneous, superficial or deep clinical and radiological findings are typical (synovial cyst, para-
nature of the mass. When correlated with the clinical findings, it is articular mucoid cyst, superficial lipoma, giant cell tumor of the
often able to confirm the benign nature of most suspicious masses synovial sheaths, etc.), an excision biopsy can be performed right
in these locations. If the results of US are atypical, not well away. However, if there is even the slightest clinical or radiological
correlated with the clinical findings and do not allow malignancy suspicion on the potential malignancy of a mass, primary biopsy is
to be ruled out, MRI is required before any treatment is initiated. justified. There are no formal clinical or imaging criteria for
Ultrasonography is also indicated in the supplemental assessment malignancy. A long-standing mass is not always benign. Converse-
of STS to look for associated adenopathy. ly, a rapidly growing mass is not always malignant [15]. However, a
MRI is now the gold standard examination for STS. The MRI must mass that has rapidly grown, of more than 3 cm in the upper limb,
be performed before any biopsy is done [15] and consists of several adhering to the superficial or deep layers, that is poorly defined or
T1- and T2-weighted sequences, with and without contrast. In each subfascial, must be suspected for malignancy. Similarly, on
sequence, orthogonal planes [16] (ideally with an axial plane) must imaging, a heterogeneous mass that is poorly systematized, that
be visible to allow better surgical targeting of the tumor margins takes up contrast agent on MRI and is intramuscular, must be
and anatomical structures and to facilitate preoperative planning of suspected for malignancy. Under these conditions, a primary
the tumor excision margins [17]. While MRI often orients biopsy is indicated.
C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243 235

Biopsy of hand and forearm tumors may appear simple. Table 2


Classification of the histological grade used by the French Federation of Cancer
However, incorrect positioning of the biopsy tract or an inappro-
Centers Sarcoma Group (FNCLCC).
priate biopsy technique can lead to more aggressive and mutilating
Tumor differentiation
definitive care, or even amputation [15,34]. The proximity of
1 point Sarcoma resembling normal adult tissue
functional anatomical structures magnifies this problem. 2 points Sarcoma with confirmed histological diagnosis
The gold standard technique for an STS biopsy is a percutaneous 3 points Embryonic sarcoma, synovial sarcoma,
core needle biopsy under local anesthesia that is ultrasound-guided epithelioid sarcoma, clear-cell sarcoma, soft tissue
or CT-guided [35,36], unless contraindicated by the radiologist alveolar sarcoma, undifferentiated sarcomas and
sarcomas with uncertain histological type
(most often because of accessibility reasons). The biopsy tract must Mitotic index
be incorporated into the likely surgical excision area for the tumor 1 point 0 to 9 mitotic cells in 10 fields
and must be defined beforehand by the radiologist and surgeon. Its 2 points 10 to 19 mitotic cells in 10 fields
trajectory must be marked. The advantages are the speed with 3 points More than 19 mitotic cells in 10 fields
Tumor necrosis
which it can be performed and reduced contamination of the tissues
1 point No necrosis
around the biopsy tract. The main drawback is the small volume of 2 points Less than 50% necrosis
biopsied tissue, which may limit the histopathological analysis and 3 points More than 50% necrosis
provide incomplete information. Grade 1: 2–3 points Low risk of metastasis
In the forearm, when imaging-guided biopsy cannot be Grade 2: 4–5 points Real risk of metastasis
Grade 3: 6–8 points High risk of metastasis
performed, surgical biopsy is indicated and must comply with
certain rules. It must be performed with a tourniquet without
exsanguination of the limb; only short, longitudinal incisions size and depth of the lesion. The rules for cancer-related surgical
should be made (transverse incisions must be avoided), over the care are widely published and do not differ for the hand and
potential excision path, direct and without detachment. After forearm [3,14,15,22]. In the past, the gold standard treatment for
careful hemostasis, suturing is performed by placing the points STS was amputation. Several studies have found that amputation is
laterally near the incision to limit contamination of adjacent not superior to limb-sparing surgery when the latter can be
tissues; if draining is needed, it must be done in the axis of the limb performed [10].
and as close as possible to the incision [15]. This limb-sparing (conservative) treatment must reconcile two
In the hand, a percutaneous biopsy often cannot be performed fundamental but contradictory principles, which are even more
because of the small size of the tumor. Surgical biopsy is highly strongly interrelated in the hand and forearm. The first principle is
relevant here also, with some particularities [15]. On the dorsal that of wide ‘‘radical’’ or ‘‘en bloc’’ excision of the tumor (i.e.
side, the incisions are typically direct and longitudinal. On the without seeing the tumor), without breaching the tumor walls,
palmar side, the incisions are as longitudinal as possible or short with clear circumferential histological margins. The tumor
and oblique; Bruner incisions and short broken incisions must be resection must encompass the biopsy and drain scars. Marginal
avoided as much as possible to prevent contamination of adjacent excision is not appropriate. The second principle is to limit as much
tissues and to limit the extension of tumor excision. The biopsy as possible the functional impact of this excision, without limiting
tract must not extend beyond the borders of the skin flaps needed the quality of the surgical excision [12].
for potential distal amputations. The current trend is to reduce the surgical margins
Excisional biopsy is not recommended and must be limited to [3,15,22,42,43]. The primary objective of surgical treatment is to
benign-appearing lesions or very small lesions that do not have a obtain R0 margins (Table 4) [44]. A tissue margin of 10 mm or more
great risk of local contamination. appears sufficient [3,13,22]. But the type of tissue around the
Lastly, tumors in the Guyon canal or carpal tunnel have their tumor drives it quality more than the 1 cm margins. Interposition
own particularities. Given the difficulty of surgical revision after of healthy fascia or healthy periosteum is considered as a sufficient
biopsy and the complex anatomy and its functional after-effects, surgical margin. Bone resection is generally not necessary except
an immediate wide resection without prior biopsy may be
indicated in these two locations. This scenario is rare and the
decision must be made during a MDM. Table 3
Anatomical classification and prognostic groups used by the American Joint
Before a treatment decision is made, the histological results of Committee on Cancer (AJCC) 7th edition.
the biopsy must be reviewed in a specialized center to reduce the
Stage IA
risk of interpretation errors [37], and thereby incorrect treatment. T1a N0 M0 G1, GX
T1b N0 M0 G1, GX
3.2. Classification Stage IB
T2a N0 M0 G1, GX
T2b N0 M0 G1, GX
Once the biopsy and full work-up have been completed,
Stage IIA
the lesion must be classified and graded to implement an T1a N0 M0 G2, G3
appropriate overall treatment. The histological grade is known T1b N0 M0 G2, G3
to be one of the most important prognostic factors [38]. In Stage IIB
France, it is evaluated using the FNCLCC classification [39] (Table T2a N0 M0 G2
T2b N0 M0 G2
2). The prognostic classification system of the American Joint Stage III
Committee on Cancer (AJCC) takes into account the size, depth T2a, T2b N0 M0 G3
and extension of the tumor, lymph node involvement and ANY T N1 M0 ANY G
metastasis [40] (Table 3). Stage IV
ANY T ANY N M1 ANY G

3.3. Surgical excision treatment T: tumor features; T1: small (< 5 cm); T2: large (> 5 cm); a: superficial location
above fascia; b: deep to fascia; N: lymph node characteristics; N0: no evidence of
lymph node metastases; N1: presence of lymph node metastases; M: distant
Surgical excision is the cornerstone of STS treatment. The metastases status; M0: no evidence of distant metastases; M1: presence of distant
surgical resection margins are the primary prognostic factor for metastases; G: histologic grade of tumor; G1: low grade; G2: intermediate grade;
the local and general control of the tumor [13,41], along with the G3: high grade; GX: indeterminate grade.
236 C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243

Table 4 3.4.1. Planning


Classification used by the Union for International Cancer Control (UICC).
When possible, reconstructive surgery must be performed
R0: no tumor remnant, clear microscopic margins (this is the minimum margin simultaneously with the tumor excision surgery [12,15,43]. This
in millimeters, with description of the type of tissue making it up)
will allow any adjuvant treatments (radiation therapy and/or
R1: presence of microscopic tumor remnant on a surgical margin
R2: presence of macroscopic tumor remnant during surgery chemotherapy) to be initiated as soon possible and to optimize the
functional recovery. In the ideal case, reconstructive surgery will
only be delayed if there is a doubt about the excision margins.
for cases of bone invasion or a tumor location that requires Because of STS polymorphism, there is no systematic treatment,
complex excision without bone resection, with risk of breaching only treatment on a case-by-case basis. The full range of
the tumor (e.g. tumor surrounding more than half of a bone’s reconstructive techniques must be used. This reconstructive
circumference). A similar approach is used for juxta-articular STS. surgery is even more critical with distal STS.
Cutaneous detachment should be limited as much as possible. There are several reconstructive techniques. They can combine
In case of skin invasion, or to avoid having to do large skin skin coverage procedures with nerve, vascular, bone and joint
detachments, procedures to cover the skin with flaps must be reconstruction. Lastly, when functional reconstruction is needed,
planned. all the tissues needed for the reconstruction must to be harvested
Lastly, when the tumors have developed in contact with major before the tumor excision surgery if possible. In the least,
nerve structures (repressed and not invaded), epineural microsur- dedicated surgical instruments are used for the harvesting that
gical dissection is valid from a cancer treatment point of view have not been used for tumor excision, so as to limit the risks of
[45,46]. It must however be planned preoperatively [47] and an contamination in the surgical sites.
adjuvant treatment is often necessary. This is even more important
in the hand and forearm, when sacrificing a major nerve would 3.4.2. Skin and nail coverage
lead to major loss of function. To obtain fast, high-quality healing, skin coverage is an essential
Despite all this, larger excision margins must be planned for component of the reconstruction.
certain STS histological types with generally poorly defined Recent studies have highlighted the potential contribution of
anatomical limits (clear-cell sarcomas, epithelioid sarcomas, negative pressure wound therapy (NPWT), either to delay the
myxofibrosarcomas). reconstruction technique or to contribute to healing after STS
In cases of STS that were not operated adequately initially or excision [51,52]. NPWT stimulates angiogenesis, thus it can only be
were discovered accidentally during the excision of a tumor used if there are no tumor remnants (R0). If this is not the case, it is
thought to be benign, it is essential to refer these patients to a formally contraindicated and is controversial while waiting for the
specialized cancer treatment center. Surgical revision is often histopathology results.
needed, along with adjuvant radiation therapy [29]. Unfortunately, Skin grafts are only indicated if there is healthy muscle,
the excision ends up being wider, more complex and often more periosteum or synovial tissue after excision that can protect the
mutilating that it could have been initially. underlying anatomical structures. If there is a resulting defect with
The freshly excised tumor piece must be sent for histopatho- exposure, particularly of tendons in the hand and wrist, flap
logical analysis with appropriate landmarks in place to confirm the coverage is required. In rare cases (precarious general health,
diagnosis, tumor grade and to determine the excision margins. comorbidities), the need for a coverage flap can be avoided by
In the forearm and wrist, thanks to the large number of using artificial skin.
reconstruction techniques and the progress in adjuvant treat- Local pedicled or perforating flaps are often the first option
ments, there is practically no place for treatment by amputation considered. In the hand and forearm, most defects can be covered
[15]. In the hand, the treatment must be as conservative as by a radial antebrachial flap (and its perforating variations that do
possible. This is possible for superficial STS or those on the dorsal not sacrifice the radial artery) and the posterior interosseous flap.
side. Unfortunately for deep or palmar STS, the most appropriate The pedicled lateral brachial flap has a role in reconstructing
surgical treatment is often partial amputation of the hand [48] or defects in the proximal portion of the forearm. However, pedicled
amputation of one or more rays [49,50]. flaps can only be used with small defects. In addition, these local
Proximal STS in the fingers require that one or more rays be flaps increase the local wound healing area and bring the tumor
amputated, while distal STS can be treated by proximal or distal excision site and harvest site into contact, thereby increase the risk
interphalangeal amputation [14]. Only the most superficial STS can of cross-contamination [14].
be treated more conservatively. Distal amputation may require It is almost always possible to use a free flap for skin coverage.
skin coverage procedures with a flap to limit the extent of the Several authors have reported that the results are at least as good
amputation and to reduce the functional impact. as with local flaps [53,54]. The choice of flap types is even larger. In
STS that are very advanced locally and difficult to operate the hand and forearm, thin fasciocutaneous flaps are preferred as
immediately in a tissue-sparing manner can benefit in some cases they can recreate tendon gliding planes and provide the best
from neoadjuvant treatments (chemotherapy, isolated limb esthetics possible, with low functional and cosmetic sequelae.
perfusion, radiation therapy), in order to facilitate the surgical Perforator flaps have a role here, such as the anterolateral thigh
treatment and to avoid or limit amputation, if possible. Lastly, in (ALT) flap (Fig. 1) or the thoracodorsal artery perforator (TAP) flap
the cases of metastatic STS at the onset, it is reasonable to reject a for the volar side of the hand, as it is typically harvested from
surgical procedure that would be functionally very disabling. hairless skin. Other options are possible, such as lateral brachial,
scapular and parascapular, abdominal or inguinal flaps. Muscle
3.4. Functional reconstruction flaps are not used in the hand and forearm, except in cases of very
large defects.
To limit the functional impact of surgical STS treatment in the Skin coverage flap procedures can also be indicated to limit the
hand and forearm, all the surgical reconstruction procedures must amputation level.
be anticipated and planned preoperatively, particularly during the
MDM. Depending on the health care facility in question, the 3.4.3. Bone and joint reconstruction
treatment team must include an oncology surgeon, hand surgeon Excision of STS in the hand and forearm may also require that
and plastic surgeon. bone or joint be removed.
C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243 237

Fig. 1. High-grade undifferentiated pleomorphic sarcoma on the dorsal side of the left forearm. Clinical appearance (A) and MRI appearance in T1 FS DIXON axial slice with
contrast with suspicion of periosteal invasion of the distal ulna (B). One-piece excision that takes a 10  15 cm skin pallet with the finger extensors, extensor carpi ulnaris and
distal ulna (C). Excised tumor (D). Design of made-to-measure contralateral free anterolateral thigh (ALT) perforator flap. Skin reconstruction with ALT without immediate
reconstruction of the extensor tendons or bone reconstruction of the distal ulna (F). Postoperative chemotherapy and radiation therapy.

For bone defects under 5 cm, reconstruction is typically done and/or ulna (split fibula), to performing radiocarpal or radio-
using an autologous corticocancellous bone graft (iliac crest, scapholunate fusion, or even to performing a one-bone forearm
proximal ulna, radius, proximal tibia). In the context of STS, reconstruction. Masquelet’s induced membrane technique has a
contrary to benign hand tumors, corticocancellous allografts and role here also [56] in the diaphyseal reconstruction of the forearm.
bone substitutes are rarely used. In the hand, in most cases, In STS of the hand and wrist, structural articular allografts of the
autologous corticocancellous grafts allow reconstruction of the distal radius or metacarpals, described in the context of giant cell
metacarpals and fusion of the carpometacarpal and/or metatarso- tumors and arthroplasty procedures, have little role in current
phalangeal joints. In more rare cases, these standard corticocan- practice.
cellous grafts can be used to perform radiocarpal or And in the wrist, wide resection (more than 6 cm) of the distal
radiometacarpal fusion (Fig. 2), but this increases the risk of ulna can be reconstructed with a vascularized fibula graft,
nonunion when the graft exceeds 5 cm. These grafts are done in lengthening [57] or a distal radio-ulnar prosthesis, even though
one or two stages using the induced membrane technique, some authors have found no functional consequences to wide
according to the preoperative plan and while waiting for the resection of the distal ulna [58].
histopathology results.
With bone defects larger than 5 cm, it is classically recom- 3.4.4. Tendon reconstruction
mended to use vascularized bone grafts [55]. In the forearm and In the ideal case, tendon reconstruction will be performed right
wrist, a vascularized fibular graft is preferred [15]. Its shape and always if skin coverage is appropriate [15]. In the context of tendon
diameter are suitable to diaphyseal reconstruction of the radius defects with the muscle belly intact, reconstruction is done with
238 C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243

Fig. 2. Grade 2 myxofibrosarcoma on the dorsal side of the right wrist that has invaded the radiocarpal and distal radio-ulnar joints. Frontal T2 STIR MRI slice (A) and axial T2
STIR MRI slice (B). Frontal MRI showing the bone invasion (C). Clinical presentation (D). One-piece radiocarpal and distal radio-ulnar arthrectomy with musculotendinous
resection of the wrist and finger extensors (E). Surgical resection piece (F). Reconstruction by tenodesis of the finger extensors, radiometacarpal fusion, and M1-M2 fusion
with corticocancellous iliac crest graft and fixation by locking plate (G). Radiograph taken at the latest follow-up (H). Neoadjuvant chemotherapy and postoperative radiation
therapy. Functional results: MSTS 76%, DASH 45, VAS for pain 3.

standard tendon autografts (palmaris longus, hemi-flexor carpi 3.5. Adjuvant treatments
radialis, gracilis, semitendinosus, fascia lata, plantaris) (Fig. 3). The
advantage of gracilis tendon, semitendinosus tendon and fascia 3.5.1. Isolated limb perfusion [61]
lata autografts is that they can be used to reconstruct multiple This consists of perfusing a limb (with tourniquet) with tumor
tendon slips. necrosis factor a (TNF-a) or Melphalan, and is mainly indicated for
When the muscle bellies are sacrificed during the tumor STS in the extremities. It can be performed in the same way as
excision, reconstruction makes use of the standard tendon transfer neoadjuvant radiation therapy, in the context of locally very
techniques for radial palsy or brachial plexus injuries. Functional advanced STS, in which immediate excision is likely to induce
free flaps are rarely used (free re-innervated transfer of gracilis to major functional sequelae or does not allow for limb-sparing
reconstruct finger flexors and extensors [59], free tendon transfer surgery.
(dorsalis pedis flap) to reconstruct the finger extensors [60]).
3.5.2. Radiation therapy
3.4.5. Nerve reconstruction Radiation therapy is standard in STS treatment. It is known to
Nerve reconstruction also typically uses interposition grafts improve the local control of STS [62]. The effectiveness in terms of
(sural nerves), or even distal nerve transfers and in rare cases, local control is the same whether it is done as a neoadjuvant
vascularized nerve grafts. A vascular graft may also be needed. (before surgical excision) or adjuvant (after surgical excision)
The complexity of the treatment at the hand and wrist stems treatment. However, there is a greater risk of postoperative
from the combination of defects in multiple tissues and the need complications with neoadjuvant treatment and a greater risk of
for combined functional reconstruction. Consequently, reconstruc- delayed complications with adjuvant treatment [63]. In the hand
tion procedures must not be considered individually, but as a and forearm, despite the thin tissues and nearby functional
whole (Fig. 4). For example, bone and skin defects can be treated anatomical structures, Bray et al. [26] have found that toxicity is
with an osteofasciocutaneous flap (free osteocutaneous flap of rarely a problem and should not preclude radiation therapy from
vascularized fibula); skin, bone and vascular defects can be treated being performed. Nevertheless, radiation therapy will not negate
with an osteocutaneous vessel-holder flap (free contralateral the consequences of positive surgical margins [64]. Also, neoadju-
radial antebrachial flap). vant or adjuvant radiation therapy does not appear to hinder the
C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243 239

Fig. 3. Epitheloid sarcoma on the volar side of the right hand that has extended to the thenar eminence and encompasses the trapeziometacarpal joint. T2-weighted FS axial
MRI slice: hyperintense infiltrate that is poorly limited on the superficial side of the flexor retinaculum, extending to the 1st metacarpal (A). T1-weighted GADO frontal MRI
slice: poorly limited hyperintense infiltrate (B). Preoperative clinical presentation (C). En bloc skin and bone excision taking the flexor retinaculum, 1st metacarpal and
trapezium (D). Temporary fixation while waiting for the histopathology results (E). Delayed reconstruction after the surgical excision margins were confirmed. Skin coverage
using a pedicled posterior interosseous flap (F). Bone reconstruction of the 1st metacarpal with a corticocancellous graft with thumb metacarpophalangeal fusion and M1-M2
fusion (G). Functional outcomes at latest follow-up: MSTS 70%, DASH 49, VAS pain 4. Postoperative radiation therapy (H).

microsurgical reconstruction [65] that is often needed in the hand development of targeted therapies could modify this care in
and forearm. the future.

3.5.3. Chemotherapy 4. Disease monitoring


Most STS are not very sensitive to chemotherapy. There is
currently no formal recommendation for its use [66,67]. The There is no true monitoring standard. At most hospitals, follow-
indication for chemotherapy (neoadjuvant, adjuvant or isolated) up visits are scheduled every 6 months (every 4 months for
is made on a case-by-case basis at the MDM based on the tumor medium and high-grade tumors) for the first 3 years, then twice a
grade, metastatic potential and its operability; based on certain year for up to 5 years and once per year for up to 10 years. It
criteria, it can be proposed in the context of clinical trial consists of a well-defined, comprehensive clinical examination,
protocols. Neoadjuvant chemotherapy is typically reserved for including adenopathy detection, particularly for tumors that target
initially metastatic STS. Adjuvant chemotherapy is an option for the lymph nodes. MRI of the local tissues and a chest and abdomen/
STS with poor prognosis (deep, large and high-grade tumors) and pelvis CT with contrast along with a PET scan are also done if there
in the case of lymph node invasion or metastasis. The is any doubt.
240 C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243

Fig. 4. Synovial sarcoma on the dorsal side of the 1st web space of the left hand discovered by chance after cyst excision. Secondary treatment with no imaging done before the
first excision. Clinical presentation with drawing of the initial surgical scar and the excision margins (A). ‘‘En bloc’’ skin and tendon excision with resection of the abductor
pollicis longus, extensor pollicis longus and brevis, and extensor carpus brevis (B). Surgical excision piece (C). Locating the abductor longus, extensor pollicis longus and
extensor carpus tendons before the tendon reconstruction (D). Tendon reconstruction by transfer of the extensor indicis proprius on the extensor pollicis longus and palmaris
longus interposition graft on the abductor pollicis longus and the carpal extensors (E). Skin coverage using a pedicled posterior interosseous flap (F). Functional outcomes:
MSTS 93%, DASH 0, VAS pain 0 (G). Neoadjuvant and adjuvant chemotherapy, no radiation therapy.

5. Results of the treatment of hand and forearm STS well-differentiated tumors, or to preserve important anatomical
structures, the local recurrence does not seem to be higher when
5.1. Cancer-related outcomes marginal resection is planned [47]. Kemp et al. found similar
results in the context of liposarcoma that make contact with major
There are only a few studies on survival specifically for STS in nerves in the forearm and were treated by epineurectomy without
the hand and forearm. Many studies are small case reports or nerve sacrifice [45].
clinical case-series. STS in the hand or wrist appears to have a STS that are initially operated in an inadequate manner have a
better prognosis than in other locations [68]. Yamada et al. worse prognosis [27–30,41]. However, after re-excision and radia-
reported overall 5-year survival of 89% for distal STS in the upper tion therapy, some studies, particularly ones in the hand [25], have
limb relative to 66% for proximal STS in the upper limb; the found similar results with STS patients operated correctly right away
recurrence-free 5-year survival was 87% versus 74% and the [72]. Any microscopic residual found during the re-excision is a
survival without metastasis was 84% versus 66% [69]. Most studies prognostic factor for worse overall survival, recurrence-free survival
of hand and wrist STS report overall 5-year survival between 80% and metastasis [41,72]. And despite appropriate treatment, certain
and 90% [12,25,70,71]. However, these studies emphasize the need histological STS types, particularly clear-cell sarcomas and epitheli-
to obtain clear surgical margins to limit the risk of local recurrence oid sarcomas appear to have a worse prognosis [25].
or metastasis. According to Pradhan et al., inappropriate surgical
margins lead to a 12-times higher risk of local recurrence [25]. The 5.2. Functional outcomes
local recurrence risk was 37% at 5 years and 52% at 10 years. Overall
survival went from 87% at 5 years to 71% at 10 years. They Most authors now defend the importance of microsurgical
concluded that limb-sparing surgery must not be done at the reconstructive surgery for STS of the hand and forearm to optimize
expense of good surgical margins. the functional outcomes [12,73]. In fact, surgery, even limb-
Gerrand et al. introduced the concept of ‘‘planned positive sparing, is often mutilating given the proximity of functional
margins’’ followed by radiation therapy. In certain cases of anatomical structures. It is however difficult to accurately define
C. Lazerges / Hand Surgery and Rehabilitation 36 (2017) 233–243 241

Fig. 5. Treatment algorithm for the care of soft tissue tumors in the hand, wrist and forearm.

the functional outcomes, given the polymorphism of STS in the patient to a specialized cancer treatment center. A biopsy is
forearm and hand, with extremely variable surgical excision and essential before any surgical treatment and drives the treatment.
surgical reconstruction. Surgical excision is the primary component of treatment. It
Most clinical studies evaluate the functional outcomes using must be as conservative as possible to limit the functional impact,
the Enneking score (Musculoskeletal Tumor Society rating Scale but must not be minimized at the expense of good tumor resection.
[MSTS]) [74] and report good or excellent scores, with average Other than for STS in the fingers, multiple surgical reconstruction
scores of 83% or better [12,26,71,73]. Lehnhardt et al. found a mean techniques can be used to avoid radical surgical excision by
DASH score of 37; Mirous et al., who emphasized the need for early amputation. These techniques, when planned preoperatively,
functional reconstruction, found a mean DASH score of 18. In the make it possible to reconcile both the cancer-related and
Bray et al. study, the functional outcomes were better for STS in the functional requirements.
forearm relative to STS in the hand and wrist, with MSTS scores of
31 versus 27 (out of 35) and Toronto Extremity Salvage Score Disclosure of interest
(TESS) of 94 versus 88 [75]. In their study, 88% of non-amputated
STS patients were able to resume their previous activities [26]. The author declares that he has no competing interest.
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