Soft tissue sarcomas and rare tumors

Soft tissue sarcomas are rare diseases with a severe prognosis. They have a low incidence, high histopathological heterogeneity and the possibility of appearing in any area of the body.

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Soft tissue sarcomas are rare diseases with a severe prognosis. They have a low incidence, high histopathological heterogeneity and the possibility of appearing in any area of the body.

Given their rarity, patients affected with these cancers encounter greater difficulties in obtaining a suitable diagnosis and consequently, the appropriate treatment.

These growths often appear in young people, although they have no age restriction, and they affect the connective tissue found throughout the body. More frequently they are located in the limbs (60%) and retroperitoneum (15%). Those of the superficial trunk (10%) and the head/neck area (5%) are more rare. Visceral sarcomas (oesophagus, stomach, intestine, uterus, et cetera) form the remaining 10%. Among these are also the subgroup of gastrointestinal stromal tumours (GIST). The numerous isotypes include liposarcoma, leiomyosarcoma, synovial sarcoma, malignant peripheral nerve sheath tumour, haemangioendothelioma, angiosarcoma, solitary fibrous tumour, epithelioid sarcoma, alveolar soft part sarcoma, pleomorphic sarcoma, rhabdomyosarcoma, desmoplastic round cell tumour, dermatofibrosarcoma protuberans, Ewing's sarcoma, aggressive Desmoid-type fibromatosis

More than 70 different isotypes of soft tissue sarcoma are known, classified according to morphology and in relation to the tissue of origin, and include tumours of the soft musculature (leiomyosarcoma) gastrointestinal tract (gastrointestinal stromal tumours, or GIST), the adipose tissue (liposarcoma) and the peripheral nervous system (malignant schwannoma or malignant peripheral nerve sheath tumour).


Soft tissue sarcomas also include:

Tumours of vascular structures

•             Angiosarcoma

•             Haemangioendothelioma

•             Haemangiopericytoma (solitary fibrous tumour)

Fibrous tissue tumours

•             Fibromatosis (desmoid tumours)

•             Fibrosarcoma

•             Dermatofibrosarcoma

•          Low grade fibromyxoid sarcoma

Tumours of more than one type of tissue

•             Malignant mesenchymoma

•             Malignant triton tumour

Tumours of unknown origin

•             Alveolar soft part sarcoma (ASPS)

•             Clear cell sarcoma (malignant melanoma of the soft parts)

•             Epithelioid sarcoma

•             Synovial sarcoma

•             Undifferentiated soft tissue sarcoma


For the majority of these isotypes, the therapeutic strategy always involves a multidisciplinary group, comprising surgeons, radiotherapists, oncologists, pathologists, radiologists, physiotherapists.

Through the need for multidisciplinary management and through the rarity of the disease, there is therefore the need to create a transversal network of collaboration in order to use the various competences, as well as the major requirement of having few specialist centres of reference for soft tissue sarcomas.


Risk factors

The majority of soft tissue sarcomas have no identifiable risk factors. However, some subtypes of sarcoma are more common in specific age groups. For example, rhabdomyosarcoma is more common in children than in adults, and synovial sarcomas are more common in adolescents.

Some familial syndromes can predispose the person to sarcoma, such as neurofibromatosis, Gardner syndrome, Li-Fraumeni syndrome and retinoblastoma.


Soft tissue sarcomas often have no symptoms in the initial phases and there is still no routine screening test available for identifying these tumours. The first evident symptom is usually a painless nodule.



The multidisciplinary approach is essential for designing custom treatment plans. The first important result is an adequate anatomical-pathological diagnosis, which involves molecular and genetic testing, as well as determining the aggressiveness and potentially, the response to a treatment.



Each year our surgical team operates around 600 people with soft tissue sarcoma. In many cases we perform procedures on patients who are not eligible for treatment elsewhere, using techniques which preserve limbs and tissues.


For patients who require radiotherapy before and after surgery, intensity modulated radiotherapy (IMRT) is now the standard of care at Memorial Sloan Kettering. IMRT offers a greater degree of precision compared to traditional radiotherapy techniques. We also have experience in sophisticated radiotherapy techniques such as proton beam therapy for the treatment of sarcoma which has extended to the spinal column and brain.


We are at the forefront in the development of new therapies for soft tissue sarcoma. If you have just been diagnosed, we may be able to help you enrol in a clinical study for the treatment of the specific subtype. If you have already undergone standard treatments and the sarcoma has returned, we could be able to offer clinical studies testing new therapies, and also testing new combinations of existing therapies.

Furthermore, clinical studies have been designed to evaluate the role of vaccines and innovative immunotherapy approaches in the treatment of soft tissue sarcomas.


Surgery is the standard treatment for localised soft tissue sarcoma. Specialist therapeutic approaches allow excellent local control of the disease to be achieved, while preserving the limbs. In addition, new reconstructive techniques allow nerves and blood vessels to be repaired and muscles and soft tissues to be transferred to achieve functional and aesthetic outcomes.

By referring to specific nomograms, made available through international collaboration, a risk prediction can be made both for local relapse and for systemic proliferation. The most effective treatment can be found via this guide.

In combination with surgery, radiotherapy can reduce the risk of local relapse.

Brachytherapy and intensity modulated radiotherapy (IMRT) are sophisticated techniques which allow toxicity and its outcomes to be reduced remotely.

Thanks to the collaboration with CNAO [National Centre of Oncological Hadrontherapy], it's possible to use new energy sources in selected cases, such as protons or carbon ions.

On completion of the course of therapy, patients are scheduled for a follow up with clinic visits and instrumental exams.




-              surgery of sarcomas of the soft parts of the limbs

-              reconstructive surgery

o             local flaps

o             microsurgery of free flaps

o             motor unit (with reconstruction of the nerve component)

-              vascular reconstruction

-              bone resection

-              post-operative movement-based physiotherapy



-              surgery of sarcomas of the soft parts of the trunk

-              Thoracotomy

-              Reconstruction of the thoracic wall (metacrilate prosthesis)

-              Post-operative movement-based physiotherapy



-              Surgery of retroperitoneal sarcomas

o             preoperative treatments

compartmental surgery

o             Post-operative movement-based physiotherapy



-              GIST surgery

o             laparoscopy v/s open surgery

o             preoperative treatments v/s adjuvants target molecular therapies





-              Multidisciplinary clinics

-              Genetic counselling

-              Acknowledgement as centre of region of Lombardy

-              Thymoma: EORTC registration, genetic study in collaboration with USA, sponsored clinical research target (regorafenib with ICH)


Rare tumours

•             Promoting European or global programme of NGS and molecular screening in rare cancers steering drug development and drug discovery (Dr de Pas, Dr Cocorocchio)

•             Neurofibromatosis in the adult: creation of a national centre of reference (Dr Pennacchioli, Dr Bonanni)

•             Tumours of the thymus (in collaboration with thoracic surgery colleagues) (Dr de Pas, Dr Spaggiari)

•             Kaposi Sarcoma (in collaboration with the Dermatology Clinic of the University of Milan – Prof. Brambilla)

All clinical aspects are managed as part of the Multidisciplinary Group, consisting of surgeons, oncologists, radiotherapists, pathologists, radiologists, interventional radiologists, physiotherapists, the psycho-oncologist, which includes potential cross-collaboration with the reconstructive plastic surgeon, thoracic surgeon, neurologist, gynaecologist.

In managing the therapeutic strategy, which is often based on combination of surgery and radiotherapy or surgery/radiotherapy/chemotherapy, particular attention is paid to preservative surgery of the limbs, which uses loco-regional treatments, functional reconstruction techniques and plastic reconstruction and pre- and post-operative treatments. Patients with these diseases are offered a vast range of treatment options, in line with international standards, and the option for innovative treatments as part of clinical studies.


Primary aims


•      To promote a European or global programme of NGS and molecular screening in sarcomas, driving new drug development and discovery

•      Combined therapy studies:

             Immunotherapy with conventional x-ray or heavy particle radiotherapy (Dr Pennacchioli, Dr de Pas, Dr Fossati-CNAO, Dr Vavassori)

             Immunotherapy with target-therapy (Dr de Pas, Dr Cioffi, Dr Conforti)

•       Preoperative treatments in high grade sarcomas (Dr de Pas, Dr Cioffi, Dr Vavassori, Dr Jereczek)

•        Immunosignature of sarcomas of soft parts (Dr Pennacchioli, Dr de Pas, Dr G. Pelicci, in collaboration with INT and ICH)

•        Ablative radiotherapy (radio surgery and stereotactic radiotherapy) in the oligometastatic patient (Dr Pennacchioli, Dr de Pas, Dr Jereczek, Dr Vavassori)

Active participation of IEO in the activities of the Italian Sarcoma Group (ISG), CTOS (Connective Tissue Oncology Society) and EORTC.


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