Sarcomas and rare tumors

Surgery is still the main treatment for sarcomas, often in combination with other therapies. High-level interventions require complex, multidisciplinary preparation, as the site, location, and histopathological features of the tumor vary from case to case, involving different specialties.

Unfortunately, some patients initially receive inadequate surgical treatment, which can adversely affect disease progression and quality of life. Therefore, it is essential that operations be carried out in centers with high specialized expertise, or in related facilities with the necessary requirements.

Surgery must always be radical and complete so that all cancer cells are removed, as radiation therapy cannot compensate for insufficient surgery.

In selected cases, surgery of metastases, such as lung metastases, may be considered if it is possible to completely reclaim the disease.

In our institute, surgery is supported by the Enhanced Recovery After Surgery (ERAS) protocol, which aims to promote a rapid return to independence, reduce hospitalization time and decrease postoperative complications.

The protocol involves a multidisciplinary approach involving a surgeon, anesthesiologist, dietitian, nurse, psychologist, physical therapist, and social-health worker, all of whom are engaged in coordinated management of the patient’s journey.

The key points of the program are:

• Optimal pain control;

• Minimally invasive surgical techniques;

• Preoperative counseling;

• Early rehabilitation.

The patient also benefits from:

• Nutritional support with abolition of pre-operative fasting;

• Personalized anesthesia that allows a rapid return to natural feeding;

• Limited use of probes, drains, and IVs;

• Early mobilization.

Chemotherapy uses drugs capable of destroying cancer cells by exploiting their high rate of reproduction compared to healthy cells. Because of this very mechanism, it can also damage normal cells in the body and cause side effects, which usually resolve when the treatment ends. Before starting treatment, the oncologist explains to the patient the planned medications and useful strategies to reduce their impact.

When used

In soft tissue sarcomas, bone tumors, and other rare forms, chemotherapy can be used at different points in the course of treatment:

• After surgery (adjuvant): to reduce the risk of recurrence;

• before surgery (neoadjuvant): to reduce the tumor mass and make removal easier; in some cases it can be combined with radiation therapy, after multidisciplinary evaluation;

• in metastatic disease: to slow progression, alleviate symptoms and improve quality of life.

Mode of administration

• Endovenous: the most frequent route, in day hospital or ordinary hospitalization; duration varies from a few minutes to several hours;

• Oral: in tablet form, as in the case of gastrointestinal stromal sarcomas (GISTs).

• treatments are performed in cycles, alternating days of therapy with periods of rest. Number and duration of cycles depend on tumor type, individual response, and drugs used.

Specific directions

Chemotherapy is mainly proposed in localized limb or trunk sarcomas with a high degree of malignancy and/or diameter greater than 5 cm, with the aim of reducing the risk of distant metastasis.
Clinical trials have shown that, in these cases, chemotherapy can delay the onset of metastasis, but has not demonstrated a significant benefit on overall survival.

Therefore, the treatment decision must be individualized and shared with the patient, taking into account the prognostic factors of the disease, the sensitivity of the tumor type to available drugs, and the balance between expected benefits and side effects.

Biological therapies, also called molecular targeted therapies (target therapies), are treatments that selectively target specific targets found primarily in cancer cells, such as receptors, growth factors, and enzymes.

These targets are involved in tumor growth and spread, resistance to traditional therapies, and the formation of new blood vessels (angiogenesis), which provide the tumor with the substances it needs to grow.

The drugs used are Pazopanib, Sunitinib, Regorafenib, which act against the vascular growth factor VEGF.

In gastrointestinal stromal sarcomas, tyrosine kinase inhibitors such as Imatinib, Sunitinib, and Regorafenib are a standard of treatment.

Immunotherapy uses drugs that do not directly target cancer cells, but stimulate the immune system to react against the tumor, overcoming the blocking mechanisms put in place by the disease.

It is currently a standard therapy for only a few specific diseases, such as Merkel cell carcinoma treated with Avelumab, while it is not yet an established strategy in soft tissue and bone sarcomas.

Radiation therapy uses high-energy radiation to eliminate cancer cells. It is performed on an outpatient basis, with daily sessions Monday through Friday, for cycles that can range from a few days to several weeks.

It is an important therapeutic strategy in some sarcomas and rare cancers: it can be used before surgery (neoadjuvant radiotherapy) to reduce the size of the mass, or after surgery (adjuvant radiotherapy) to decrease the risk of recurrence. In some cases it can be combined with chemotherapy after multidisciplinary evaluation.

In advanced soft tissue sarcomas, radiation therapy has primarily a palliative role, aimed at controlling symptoms and slowing disease progression.

Treatment for rare sarcoma-tumors often involves side effects that impact quality of life more or less severely. However, they can be mitigated and in some cases prevented by specific treatments and/or appropriate lifestyle.

At the Candiolo Institute, the doctors and nurses of the multidisciplinary team are available to provide the patient with all the support he or she needs to manage the various side effects he or she will face in the course of treatment.

To ensure timely and direct support and receive timely answers to concerns and questions, a dedicated support service is in place at the Candiolo Institute for all patients.

From Monday to Friday, from 8 a.m. to 5 p.m., you can contact the secretariat of the oncology day hospital at 011.993.3775, reporting the need for urgent consultation.

The patient will be quickly put in touch with his or her medical specialist, to receive clear answers and immediate support.

The oncology patient is a complex patient who needs multidisciplinary support for the management, not only of his or her pathology, but also of all associated situations involving both physical symptoms, such as pain or weight loss, and the psychological sphere.

At the Candiolo Institute, specialists from different disciplines are available at Candiolo for patients who need or request them to offer:

• • nutritional support,

• • psychological support,

• • physical therapy,

• • Dressing of venous access devices,

• • pain therapy,

• • Management of other coexisting conditions.

In most cases, sarcomas arise in the absence of family history and known risk factors.

However, there is an association between some genetic syndromes and sarcomas, such as: neurofibromatosis (in which the risk of developing peripheral nerve sheath malignancies persists), Gardner syndrome (with high incidence of desmoids), Li-Fraumeni syndrome , tuberous sclerosis , hereditary retinoblastoma, and Werner syndrome.

The Candiolo Institute has an outpatient genetic counseling clinic where a geneticist, an expert in hereditary-familial cancers, offers cancer risk assessment counseling and genetic testing to patients who need it.

People at high genetic risk of developing sarcoma can be evaluated for inclusion in a specific diagnostic surveillance program.

The Social Service Department of the Candiolo Institute conducts information and orientation interviews to patients and their families on how to access services in the area and how to obtain welfare and social security benefits provided by law (disability, benefits for aids and prostheses, work leave, etc.).

The service operates on Wednesdays and Fridays from 9 a.m. to 1 p.m. (phone: 011 9933059).

With the conclusion of the course of treatment, the follow-up period begins during which, through a series of examinations and visits, the side effects of the therapies performed and their effectiveness are monitored and the patient’s functional recovery is assessed.

Follow-up examinations are especially important to intercept any recurrences early so that appropriate therapy can be intervened. For the patient, they are also a valuable opportunity for dialogue with their medical specialist.

It is the same specialist doctor who schedules follow-up visits, in which the patient’s health condition is assessed and the required test reports are viewed.

Checkups are carried out at scheduled intervals for the duration of 5-10 years.

They have a shorter cadence at first (three to six months), then gradually thin out over time (once a year). The frequency and type of examinations provided depend on the type of disease, the stage of the tumor, and the treatments given.

Typically, follow-up checks for sarcomas are:

• • Medical examination and blood tests every 3-4 months for the first 2 years, every 6 months between 3rd and 5th year, then yearly,

• • Chest CT and rm/ecography/rx of the operated district in localized and operated soft tissue sarcomas every 3-4 months for the first 2 years, every 6 months between 3rd and 5th years, then annually,

• • CT scan of the chest and abdomen every 3-4 months in the case of advanced disease,

• • Possible endoscopic controlin case of operated gastrointestinal stromal sarcomas.

Due to the high number of cases treated each year, the Candiolo Institute is a national reference for taking care of esophageal cancer. Our experience enables us to deal with even the most complex situations, always with a personalized approach built on the clinical and personal profile of each patient.

Establishing the treatment plan always starts with an accurate and timely diagnosis. Patients have access to state-of-the-art imaging technologies that allow accurate assessment of the extent of the disease.

In addition, the Institute offers advanced and sophisticated laboratory investigations, including molecular and genomic analyses, which are critical for identifying biological features of cancer and guiding therapeutic decisions.

When indicated, surgery is performed with minimally invasive techniques (laparoscopic or thoracoscopic), which reduce operative trauma, promote faster recovery, and improve postoperative quality of life. Every treatment choice is defined within the GIC, ensuring a consistent and integrated approach.

As an IRCCS, the Candiolo Institute combines clinical practice with a strong vocation for scientific research. Patients can be evaluated for inclusion in active clinical trials, which represent a real chance to access innovative therapies not yet available in standard practice. Collaboration between care and research is a distinctive value that translates into concrete opportunities for the patient.

The Interdisciplinary Care Group takes care of the person at every stage: from diagnosis to treatment to follow-up, with attention to nutritional support, psychological health, and reintegration into daily life. The organization of checkups, visits and treatment is designed to ensure continuity and serenity, always valuing the human dimension of care.