REVIEW ARTICLE Year : 2022  |  Volume : 30  |  Issue : 4  |  Page : 115-120

Soft-Tissue sarcomas: Diagnosis and treatment from the plastic reconstructive surgeon perspective

Rana Kapukaya
Departmant of Reconstructive and Aesthetic Surgery, Plastic, UHS Adana Medical Faculty, City Training and Research Hospital, Adana, Turkey

Date of Submission16-Apr-2022Date of Acceptance19-Jun-2022Date of Web Publication09-Sep-2022

Correspondence Address:
Dr. Rana Kapukaya
SBÜ Adana Medical Faculty, City Training and Research Hospital, Adana
Turkey

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/tjps.tjps_22_22

Advances in multimodal therapy and limb-salvage surgery (LSS) have led to improvements in the quality of life of sarcoma patients. Despite all the advances, controversy continues in the diagnosis and treatment of soft-tissue sarcomas (STSs). Significant structural and functional losses occur in the extremity due to both wide resections and radiotherapy combined with surgery. This situation necessitates the restructuring of extremity functions. The opinion of plastic surgery must be obtained, when planning LSS, before excision of high-volume tumors, to decide postresectional reconstruction and to manage the postsurgical complications. The need for plastic surgery in the multidisciplinary approach to STSs is increasing. The information presented in this article has been compiled from the articles obtained by searching the titles of “soft tissue sarcomas, reconstruction, diagnosis, current treatment” in Google Scholar and PubMed databases, international books written in the field, and the experiences of the authors.

Keywords: Current treatment, diagnosis, reconstruction, sarcoma

How to cite this article:
Kapukaya R. Soft-Tissue sarcomas: Diagnosis and treatment from the plastic reconstructive surgeon perspective. Turk J Plast Surg 2022;30:115-20
  Introduction 

Soft-tissue sarcomas (STSs) were previously thought of as a single disease. Today, it identifies approximately 100 different pathological lesions, each of which has a different genomic feature, different progenitor cell, different mode of spread, different prognosis, and is sensitive to different adjuvant treatments.[1] Approximately 85%–90% of these tumors of mesenchymal origin occur in elderly patients, and the remaining 10%–15% in children and young adults.[2]

In recent years, adjuvant therapy has been added to surgical treatment in sarcomas. It has been shown that there is no significant difference in terms of local recurrence or survival rates between amputation and limb-salvage surgery (LSS) in patients undergoing adjuvant therapy. Therefore, interest in LSS has increased today and we can say that it is the standard treatment.[3],[4] However, in these procedures, both wide resections and radiotherapy (RT) combined with surgery cause significant structural damage to extremity functions. This situation necessitates the restructuring of extremity functions. An extremity that has regained its functions has a positive effect on the patient's psychology and indirectly on immunity.

STSs cover a wide heterogeneous group of diseases. Since there have been important developments in diagnosis and treatment in recent years, it will now be discussed in terms of reconstructive surgery.

  Diagnosis 

It is estimated that 2–5/100,000 people are diagnosed with STS annually. It constitutes <1% of solid tumors and 50% is localized in the extremities. Liposarcoma (30%) is the most common sarcoma. In childhood, rhabdomyosarcoma is most common.[2] The most important parameters in the differential diagnosis are the age of the patient and the location of the lesion [Figure 1].

Figure 1: (a) Infantile fibrosarcoma should be considered first in a 4-year-old child presenting with a mass in the gluteal region. (b) MR image of the mass. c: Excisional tissue macroscopic image. (c) Epithelioid sarcoma should be considered in a 67-year-old patient who presented with a mass in the gluteal region. (d) MR image of the mass. (e)Epitheloid sarcoma macroscopic image. These diagnoses have the same radiological appearance and clinical findings. Age and localization are important parameters when diagnosing sarcoma

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It is difficult to generalize STS. However, there is usually no pain in malignant masses. The size of the tumor is important in the first application. Any mass >5 centimeters (cm) in diameter should be considered a malignant tumor until proven otherwise. Growth in malignant tumors is rapid and can reach large sizes within months. However, not all tumors follow this rule. Malignant tumors are usually deeply located. Malignant tumors such as dermatofibrosarcoma protuberans, epithelioid sarcoma, and myxofibrosarcoma do not follow this rule.[5]

X-ray

Bilateral direct graphy should be taken in every patient. This will give important information in the evaluation of magnetic resonance images.

Ultrasonography

It is very useful in the evaluation of superficial lesions. Deep lesions can be examined with special probes. Doppler examination may be requested in the evaluation of flow in postoperative vascular grafts or in microsurgical planning for the postresection patient. Due to technological advances, ultrasonography is indispensable in soft-tissue tumors. Its noninvasiveness and easy accessibility are its important advantages. If evaluated by an experienced radiologist, it provides a lot of information about the mass.

Computed tomography

It is important in the evaluation of the internal character of the tumor. It should definitely be requested in the evaluation of bone pathologies developing secondary to the tumor.

Magnetic resonance imaging

It is a very important imaging method in the diagnosis, staging, planning of biopsy and surgical resection in soft-tissue tumors, postsurgical reconstruction, and evaluation of recurrence and metastases. It offers multiplan evaluation and has high soft-tissue sensitivity. Tissues are characterized according to their signal intensities in magnetic resonance imaging (MRI). STS usually shows heterogeneous hypointense pattern in T1-A sequence and heterogeneous hyperintense pattern in T2-A sequence. Sometimes, signals can be misleading in malignant tumors. Diagnosis and treatment cannot be decided only with MRI [Figure 2]. A hyperintense signal in the T2-A fat-suppressed sequence around the tumor should be interpreted as peritumoral edema. This finding is in favor of malignancy. Heterogeneous signals on T2-A images in soft-tissue tumors indicate necrosis and bleeding in solid tumor tissue. It is necessary to distinguish necrosis, bleeding, and living tumor tissue within the mass. To make this distinction, it is necessary to examine with intravenous contrast material.[6],[7],[8],[9]

Figure 2: Histopathological diagnosis is well-differentiated liposarcoma in both cases (a and b). a1: T2 A In the first case, tumoral tissue was not suppressed in fat-suppressed sequences. Heterogeneous and hyperintense signals were received. a2: Excisional tissue macroscopically resembles adipose tissue. b1: In the second case, the mass is the same as the adipose tissue in T1 A MR images. Hyperintense signal received. b2: However, the macroscopic appearance of the excisional tissue does not resemble the adipose tissue

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Malignancy findings are deep localization, >5 cm in diameter, and presence of necrosis and peritumoral edema on MRI.[9],[10] Heterogeneity of the signal in T1-A and T2-A sequences and invasion of the vascular nerve bundle are very important findings in terms of malignancy. T1 and T2 turbo spin-echo imaging should be performed to determine the basic structural features of the mass, to define its anatomical plan, and to define the tissue of origin. Images taken with contrast material are controversial.[11] However, we recommend performing contrast-enhanced examination in all patients diagnosed with sarcoma, in the follow-up, and in the detection of local recurrences or residual materials. Three-dimensional examination of MR and computed tomography images gives important clues in surgical planning [Figure 3].

Figure 3: Synovial sarcoma. (a) Contrast T1 A MR image (b) Three-dimensional MR image shows that the tumor tissue has completely covered the proximal thigh, invaded the pelvis, and circularly engulfed the neurovascular structure proximal to the inguinal ligament. Especially in frontal (c) and caudal (d) three-dimensional MR images, the borders of the tumor tissue and the relationship of this structure to vital structures are clearly seen

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Positron emission tomography–computed tomography

It has been developed to measure the anatomical details of the mass and the metabolic activity of the tissue. Fluorodeoxyglucose (F18-FDG) is used by marking F18. This substance is a glucose analog and is an indicator of the glucose metabolism of the cell. As it is known, in sarcomas, there is an increase in glucose metabolism at the cellular level. Therefore, there is an increase in uptake in cancerous cells. This is defined as the “Standardized Uptake Value.”[12] It is very important in diagnosis, in the presence of local recurrence and distant metastasis, and in evaluating the response to treatment.[13]

Histopathological examination

The patient's history, examination findings, and radiological visual characteristics of the mass should also be considered in the diagnosis. Neurovascular structures should be preserved when performing a biopsy. In deeply located tumors, the specimen should be obtained by reaching from a single muscle or compartment. Compression should be applied to the area after the biopsy. Bleeding within the tissue should be minimal. Radiological imaging is mandatory before biopsy. Areas that do not contain contrast material in MRI, should be avoided.[14]

  Treatment 

Treatment is managed according to the patient's initial presentation. Because each case is a separate clinical entity, it requires different treatment combinations. The main treatment for sarcoma is surgical excision. The gold standard in surgery is to provide a safe surgical margin. The “International Committee for Cancer Classification” accepts the definition of wide tumor-free microscopic margin (R0) as the removal of at least 1 mm (mm) of healthy tumor-free tissue together with tumor tissue.[15] In isolated tumors, surgical treatment alone is usually sufficient. Surgical resection is essential in these tumors and the R0 limit should be achieved.[16]

Radiotherapy

In all high-grade tumors that are deep and larger than 5 cm, RT should be added to surgery.[17] If the lesion is primary, local, and large, there may be differences in the treatment management.[18] In local tumors that are too large to be resected with a negative margin, multimodal therapeutic modalities (RT, chemotherapy, or radiochemotherapy) should be considered.[7]

Chemotherapy

It can be applied in patients who have a high risk of metastatic disease or metastases, and sarcomas that are thought to be chemosensitive.[19] In patients with metastasis, surgical resection of the primary tumor is a palliative procedure. RT or chemotherapy is a more suitable option in these patients.[20]

Amputation

It is applied in very few cases if diagnosed early. It should be preferred in tumors that have completely invaded the extremity, where a negative margin cannot be obtained, and in the presence of extremities that cannot have better functions than the prosthesis.[18]

Limb-Salvage Surgery

It is applied in patients who can be repaired with microsurgery if the sarcoma has invaded neurovascular tissues and large muscle groups. In these patients, the orthoplastic approach not only reduces amputation rates but also provides the recovery of extremity functions and improves the expected quality of life in the short and long term.

Reconstruction

With careful preoperative planning, primary repair is often possible after resection. Mostly fasciocutaneous flaps are used in the repair [Figure 4].

Figure 4: Infantile fibrosarcoma (a) Before neoadjuvant chemotherapy, (b)After neoadjuvant chemotherapy and skin necrosis (c) Skin reconstruction after the operation (d) Late follow-up view. Although the tumor tissue is very large, the skin is necrotic and ulcerated, the defect was repaired with fasciocutaneous flaps with careful preoperative planning

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Reconstruction options are determined according to the patient [Figure 5]a, [Figure 5]b, [Figure 5]c. Even if all negative factors are excluded in STS, the local recurrence rate varies between 15% and 30%.[21] Difficulties may be experienced in the diagnosis of local recurrence due to postoperative tissue changes. Therefore, it is necessary to be careful in the follow-up of these patients. At this point, it should be emphasized that experience is very important in the treatment of sarcoma.

Figure 5: (a) A 32-year-old male patient, diagnosed with angiosarcoma. Initial presentation, plain X-ray, and MRI images. Although the tumor created a soft-tissue defect, it invaded the bone cortex and caused edema in the bone medulla. (b) The patient underwent wide resection of soft and bone tissue. The resected bone tissue of the patient was autoclaved. (c) After resection, the bone defect was repaired with sliding fibula and autoclaved autogenous bone graft. Osteosynthesis was performed with a plate screw. Soft-tissue repair was achieved with medial gastrocnemius flap and split-thickness skin graft. MRI: Magnetic resonance imaging

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The most important parameter affecting the planning of defect repair after resection is RT.[22] Free flaps may be needed in the repair of defects that occur in large-volume tumoral masses and recurrent cases, especially in cases of RT.[23] In cases requiring large skin defect and functional repair, latissimus dorsi and rectus abdominis free musculocutaneous flaps are quite safe.[24] In addition, contralateral anterolateral thigh flap and parascapular flap are frequently preferred fasciocutaneous flaps.[25]

Especially in the thigh and gluteal regions, large cavitary defects may occur after resection of the tumor. To mobilize the patient as soon as possible, flaps that will be transferred from the contralateral healthy extremity for repair should be avoided as much as possible.

Complications

Especially in the extremities, wide resection and adjuvant RT can cause both loss of function and development of chronic ulcers. Complication rates were found to be high in patients who underwent complex reconstruction. In particular, RT, tobacco use, diabetes, tumor size (>10 cm), proximity to the skin surface (<3 mm), and failure to protect major neurovascular structures are important factors affecting the rate of complication development.[26]

  Discussion 

If a negative surgical margin was achieved in isolated tumors, adding RT to the LSS had no effect on overall survival. However, it has been shown to reduce the risk of local recurrence by 20%–25%.[27]

Adjuvant treatment is RT in STS. Chemotherapy can be applied as an adjuvant in some selected cases. However, its benefit in STSs is controversial.[28]

There is no common consensus on the application of adjuvant RT in superficially located tumors larger than 5 cm in diameter, low and high grade.[19]

Controversy continues regarding adjuvant therapy in deep-seated, low-grade tumors.[20]

RT can be applied before or after surgery. Phase 3 studies comparing these two methods give similar clinical results in terms of local control and survival.[28],[29]

There is no consensus on when to perform the reconstruction after resection.[30] We mostly prefer to do it in the same session. We believe that this approach has many important advantages. First of all, the patient's postoperative RT applications are not highly compromised. Since the wound surface does not remain open, infection rates remain at a minimal level and allow early initiation of extremity rehabilitation.[31] We prefer delayed reconstructions in the presence of systemic infection and in cases where R0 cannot be achieved. Some authors argue that vacuum-assisted closure (VAC) is beneficial in wound bed preparation in delayed reconstruction.[32],[33] However, we do not and do not recommend the VAC system in malignant tumor beds due to its angiogenetic effect.

  Conclusion 

The main treatment for sarcoma is surgery. The basic principle in surgical treatment is wide resection. Despite everything in some cases, amputation may be the only option to obtain a safe margin. There is an increasing need for plastic surgeons in the multidisciplinary approach in the treatment of sarcoma. Especially in LSS, plastic surgeon assistance should be sought to decide on the reconstruction to be performed after the resection and to manage the postsurgical complications while planning the surgery for the excision of high-volume tumors.

Others

Written and verbal consent was obtained from all patients. Pictures in the article are taken from the archives of the authors. Approval was obtained from the local ethics committee.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]