Soft Tissue Sarcoma

Soft tissues are defined as supportive tissue of various organs and extaskeletal nonepithelial structures, excluding lymphohematopoietic tissue. Soft tissues include fibrous connective tissue, adipose tissue, skeletal muscle, lymph vessels, blood and peripheral nervous system. Embryologically mostl of them are derived from mesoderm and with input neuroectodermal in the case of the peripheral nervous system. Soft tissue tumors constitute a heterogeneous group of big neoplasms.

Epidemiology

It represents 1% of adult malignancies and 15% of malignant tumors of the child. The incidence is 2 per 100,000 inhabitants. Test strips soft tumors occur twice as frequently as primary bone sarcomas. About 45% of sarcomas occur in the lower extremities, upper extremities 15% to 10% in head-neck region, 15% in the retroperitoneum and the remaining 15% in the abdomen and thorax. Rhabdomyosarcomas occur most often in children and young adults. Synovial sarcomas occur in young adults. Malignant fibrous histiocytoma and liposarcoma usually occur in older adults.

 

Risk Factors

1.       Prior irradiation. Post-irradiation fibrosarcomas have been described.

2. Chronic lymphedema in patients with breast carcinoma (Stewart Treves syndrome)

3. Exposure to chemicals: phenoxyacetic acid, chlorophenols, torotrast, vinilclorid and arsenic.

4. Cytostatic drugs: cyclophosphamide, melphalan, procarbazine, nitrosoureas and chlorambucil.

5. Genetic Diseases: neurofibromatosis, Li Fraumeni syndrome, retinoblastoma Gardner syndrome and family.

6. Infections. Kaposi’s sarcoma can occur in patients with HIV (human immunodeficiency virus) infected with herpes virus type 8. Epstein-Barr virus infection in a patient increases the likelihood of tumor immuno-compromised soft tissues.

7. Trauma. The relationship between trauma and soft tissue tumors appears to be coincidence. Trauma draws attention to preexisting lesions.

 

Pato- phisiology

In general soft tissue tumors grow centripetal. Most soft tissues respect facial borders, remain confined to the compartment of origin until the last stages of development. Once the tumor compartment achieve anatomical limits, the tumor is more likely to make a breakthrough in the compartment boundaries. Major neuro-vascular structures are usually deployed or not surrounded invaded by tumor. Tumor that arises in extra-compartment locations, for example popliteal can expand quickly due to lack fascial borders. They may also invade neurovascular structures. Peripheral portions of the tumor compresses the surrounding tissues due to increased centripetal expansive. This causes the formation of a relatively well-defined area of fibrous tissue that contains compressed and disseminated tumor cells. This area may also contain inflammatory cells and neuro-vascularization. A thin layer of tissue called the reactive surrounds compressed area especially in high grade tumors. Together, the compressed area and the reactive area form pseudocapsule that surrounds the tumor that and is useful in defining the extension of the surgical resection. Some highly aggressive tumors with infiltrative growth pattern- child rhabdomyosarcoma may not respect anatomical boundaries and will often invade fascial planes.

 

Local Recurrence

Soft tissue sarcomas tend to recur locally. Since there are relapses is difficult to treat than the primary lesion, complete resection and radiation therapy are critical to proper use during initial treatment. The pseudocapsule provides the surgeon a dissection plan more or less evident; yet such a microscopic tumor excision and occasionally can leave macroscopic. This leads to local recurrences paote up to 80% of patients. The addition of radiotherapy reduces the risk of relapse associated with a marginal resection. The pattern of relapse is generally predictable and most tumors relapse, in the first 2-3 years. Radiation minimizes local recurrence, but its ability to increase survival chances, although likely is not safe. Adjuvant Chemotherapy may decrease risk of local recurrence of high grade tumors, probably due to the reduction in tumor size and increased reactive area, but this notion is controversial.

Distant metastasis

The invasion of regional lymph nodes is rare soft tissue sarcomas; less than 4% have lymph node metastases at presentation. Ganglionic invasion is most common in epithelioid sarcoma, rhabdomyosarcoma, synovial sarcoma and clear cell sarcoma. Carcinoma and melanoma should be included in the differential diagnosis of any tumor mass is present with lymph node metastases. Many patients with high grade sarcomas, as well as some low degrees progresses to metastatic disease even after adequate local control of the primary tumor. It is the most common lung metastasis headquarters, which occurs in 52% of patients with high grade sarcomas. Although when most patients have metastases presenting clinically evident, they may have occult micro-metastases that ultimately manifests clinically. Not certain if systemic chemotherapy may improve long-term survival for patients with high grade sarcomas.

 

 

Location: 50% in the limbs, 15% retroperitoneal sarcomas, 13% and 5% visceral sarcomas and  sarcomas of the head and neck.

 

Histological Classification is based on the cell of origin is more difficult to determine as histological differentiation decreases. Histological Classification has less prognostic value than the degree of differentiation and tumor size.

 

Soft tissue tumors are classified into the following four categories (WHO 2002)

 

1. Benign they usually do not recur locally, and if re-offend are curable by complete local excision. Morphologically benign lesions, which are extremely rare, can give rise to distant metastases, which cannot be assessed on the basis of current histological examination. This is the best documented cases of cutaneous benign fibrous histiocytoma.

2. Tumors intermediate (locally aggressive) these tumors show a pattern of destructive and infiltrative local growth. Although local recurrence cannot never metastasize. It usually requires excision with a margin of normal tissue for local control. Example – desmoid fibromatosis is in this category.

3. Intermediate tumors (rarely metastasize) these tumors are often aggressive local, but in some cases have a tendency to produce distant metastasis (usually in lymph nodes or lungs). The risk is low (<2%), but histomorfologically is not predictable. The classic example of this group is fibrohistiocitary plexiform tumor and fibrous angiomatoid histiocytoma.

4. Malignant soft tissue sarcomas are potentially destructive local relapse. The risk of distant metastasis significantly, depending on the histology and hstologic grading between 20% and 100%. Low grade sarcomas have a smaller chance of metastasis, only 2-10%. However recurrences of these tumors can advance in grading and may have an increased risk of metastasis similar to that associated with mixofibrosarcoamele and leiomyosarcomas.

 

 

See table no. 1 The classification of soft tissue sarcomas (soft tissue malignancies):

 

A). Malignant fibrous tumors

Fibrosarcoma

B). Malignant Neoplasms fibrohistiocitare

Malignant fibrous histiocytoma

C). Malignant tumors lipomatous

Liposarcoma

D). Malignant tumors of smooth muscle

1. leiomyosarcoma

2. epitheloid leiomyosarcoma

E). Malignant tumors of skeletal muscle

1. rhabdomyosarcoma

2. rhabdomyosarcoma with lymph differentiation

F). Tumors of blood and lymph vessels

A. borderline tumors

1. hemangioendothelioma

B. Malignancies

1. angiosarcoma and lymphangiosarcoma

2. Kaposi’s Sarcoma

3. follicular dendritic cell sarcomas

G). Malignant Neoplasms perivascular

1. malignant glom tumor

2. Malignant hemangiopericytomas

H). Malignant synovial

1. Synovial sarcoma

2. tumor of tendon sheath giant cell

I). Malignant mesothelial tumors

1. solitary malignant mesothelioma

2. diffuse malignant mesothelioma

J). Malignant neural tumors

1. malignant Schwannoma

2. granulation malignant tumor cells

3. Clear cell sarcoma

4. melanocytic malignant schwannoma

5.Plexosarcoma (gastrointestinal autonomic nerve tumor)

6. primitive neuroectodermal tumor

K). Malignant Neoplasms paraganglionary

Malignant paraganglioma

L). Malignant tumors of bone, extraskeletal and cartilage

1. extraskeletal chondrosarcoma

2. extraskeletal osteosarcoma

M). Mesenchymal Tumors

Malignant mezenchimoma

N). Other sarcomas

1. alveolar soft tissue sarcoma

2. epitheloid sarcoma

3. rhabdoid extraskeletal tumor

4. small cell dezmoplastic tumor

 

The most common histologic types encountered are:

1. malignant fibrous histiocytoma30%

2. liposarcoma 11-13%

3. leiomyosarcoma 10%

4. synovial sarcoma from 5.9 to 8.4%

5. Malignant schwannoma 5-7%

6. fibrosarcoma 5%

7. rhabdomyosarcoma 6.6%

8. Hemangiosarcomas 3.8%

9. Chondrosarcoma extrascheletal 3.8%

10. clear cell sarcoma2.6%

11. epitheloid sarcoma 1,3%

12. unclassified sarcomas 12.4%

Histological grading is the best indicator of biological aggressiveness of tumor. In determining the histological grading is envisaged: histological subtype, tumor differentiation, mitotic index, tumor necrosis amount, cellular and nuclear pleomorphism.

 

Signs and symptoms: painful lumps; usually confused with intramuscular hematoma, sebaceous cyst or lipoma. Patients with abdominal or retroperitoneal sarcomas present with vague abdominal aches, nausea, vomiting, palpable abdominal mass. Sarcomas that arise from specific viscera can produce signs and symptoms related to organ involved. Sarcomas marrow disseminate special about being the most common lung metastases. In gastrointestinal or gynecologic sarcomas occur liver metastases occur. Lymph node metastases are rare and occur in case of rhabdomyosarcoma and cells alveolar sarcoma.

Paraneoplastic syndromes:

1. Hypoglycemia

2. Hypertrophic osteoarthropathy

3. Hypocalcemia

4. Oncogenic osteomalacia

 

The diagnosis is confirmed on histology.

Biopsy is usually indicated for a tumor mass that occurs in a patient without a history of trauma, tumor mass that persists for more than 6 weeks after a local trauma. All masses of soft tissue tumor larger than 5 cm or any symptom or lesion should be biopsied increases. There are several techniques including FNAB biopsy, core biopsy, incisional biopsy and excisional biopsy. Choosing biopsy is based on the size and location of the tumor mass and the surgeon’s experience. Excisional biopsy is indicated for small lesions, superficial tumor mass <3.5 cm in diameter, which is small probability of malignancy.

Core biopsy of the tumor obtains a tumor tissue core (about 1x 10 mm). The core of the tumor may be unrepresentative for the entire tumor so it is a possibly unrepresentative grading. FNAB harvested a larger area than core biopsy of the tumor. Dissemination of tumor cells outside the limits of primary location is quite rare. Both core biopsy and open biopsy obtain a histological diagnosis and a histological grading in more than 90% of cases.

In sarcomas it is advisable to carry out incisional biopsy placed in the incision direction so as to be incorporated in subsequent excision.

Excisional biopsy is not recommended although some authors use it in small and shallow tumors (<5 cm).

By needle puncture aspiration (FNAB) or biopsy needle aspirate individual tumor cells and micro-fragments of tumor mass; establish malignancy but cannot establish the diagnosis histogenetically. By FNAB diagnostic accuracy is very high and soft tissue tumors can be set histological grading. It is recommended in the diagnosis of tumors and tumor recurrence. Tumor cell seeding along the biopsy tract is rare.

 

see table no. TNM Classification of two soft tissue sarcomas

 

Histological grading (G)

Gx – cannot be assessed

G1 – well differentiated

G2 – moderately differentiated

G3 – poorly differentiated

Primary Tumor (T)

Tx – primary tumor cannot be assessed

T0 – no evidence of primary tumor

T1 – tumor ≤ 5 cm in greatest diameter;

T1a – superficial tumor

T1b –deep  tumor

T2 – tumor> 5 cm in greatest diameter

T2a- superficial tumor

T2b – deep tumor

x – Superficial tumors are located exclusively above the superficial fascia without invasion of the fascia.

xx – the tumor is located deep under the superficial fascia or is above the fascia without invading it

Mediastinal or pelvic retroperitoneal tumors are classified as deep tumors.

Regional lymph nodes (N)

Nx – regional lymph nodes cannot be assessed

N0 – no regional lymph node metastases

N1 – regional lymph node metastases

This positive nodes (N1) in M0 tumors is considered stage III

Distant metastasis (M)

Mx – the presence of distant metastases cannot be assessed

M0 – no distant metastases

M1 – distant metastases

 

Stages:

Stage I

IA (Low grade, small, G1, GX-1b T1a N0 M0

IB (Low grade, high-G1 GX T2a, T2b N0 M0

 

 

Stage II

G2-G3 II T1a N0 M0 -T1b

II B G2 N0 M0 T2a -T2b

Stage T2b N0 M0 III G3 T2a-

 

N1 M0 Any G Any T

 

Stage IV Any T Any N M1 Any G

Grading Histology and staging are essential components. Ganglionic invasion is characteristic of most sarcomas except rhabdomyosarcomas, synovial sarcomas, clear cell, vascular and epithelioid. This classification does not apply gastrointestinal stromal tumors, rhabdomyosarcoma, Ewing sarcoma, desmoplastic tumors and round cells and primitive neuroectodermal tumors.

For staging is recommended:

· History, clinical examination,

· Blood counts, liver and renal biochemical tests,

· X-rays of the area, chest x-ray, abdominal ultrasound,

· Bone scintigraphy

· C. T. abdominal and lung and affected segment,

· MRI of the affected segment.

PET · CT

 

TREATMENT

Therapeutic Indication

Soft tissue sarcomas low grade I.

Stage I

Surgery is the primary treatment for stage I sarcomas; It is considered definitive when margins are> 1 cm or fascia is still intact. Resection or adjuvant external radiation therapy is recommended to prevent local recurrences or if margins are <1 cm or when the location makes it impossible resection relapse.

Radiation therapy is associated with a decrease in local recurrence but does not alter overall survival. Definitive radiotherapy is recommended for patients who are not surgical candidates. Radiotherapy doses range between 70-80 Gy.

Re-resection is associated with improvement in survival at 5 years- disease free (DFS).

 

II. High grade soft tissue sarcomas

Stages II and III

Tumors in these stages can be> 8-10 cm and have an increased risk of relapse and metastasis.

For patients with resectable disease, it is recommended surgical treatment followed by radiotherapy with or without adjuvant chemotherapy. As alternatives are preoperative radiotherapy, chemotherapy or chemoradiation regimens based on doxorubicin.

 

The role of locoregional radiation therapy in the treatment of soft tissue sarcomas

 

Benefits of using preoperative radiotherapy are large retroperitoneal tumors or groin tumors may become resectable; treatment requires a smaller field; during tumor resection may be lower seeding. The usual dose is 50 Gy external radiotherapy. The negative aspects of using this therapy include wound healing complications. If the margins are close (<1cm) or positive is recommended brachytherapy, intraoperative radiation therapy (IORT) or intensity modulation radiation therapy (IMRT) in doses ranging from 10 to 26 Gy.

Brachytherapy postoperative

The usual doses for brachytherapy or external beam radiotherapy are 45Gy dose of 50 Gy in.

Consider re-resection if the margins are positive.

Consider external radiation even when the margins are negative after re-resection and the patient is elderly and / or have stage III disease. When re-resection is not an option recommended postoperative radiotherapy. Benefits include the fact that wound healing complications are minimized.

 

A. limb sarcomas and sarcomas of the trunk surface

 

Stg.IA (T <5 cm) is recommended: Complete surgical resection with tumor free margins

Stg.IB, II A, II B, II C Recommended treatment is complete surgical resection of tumor free margins.

Postoperative radiotherapy is associated in total dose of 60-66 Gy.

In patients with high grade tumors (G3-G4) indicated brachytherapy dose of 45 Gy.

In patients with low grade (G1-G2) external radiotherapy is indicated.

Stg.III complete surgical resection is recommended followed by radiotherapy and chemotherapy.

Local recurrences:

a) Complete surgical resection if the patient has been previously irradiated

b) surgical resection followed by radiation therapy unless the patient has been irradiated anteriorRezecţie surgery followed by radiotherapy and chemotherapy if the tumor relapse is high-grade

 

Retroperitoneal sarcomas, or visceral B.

Of the treatment is complete surgical resection is possible to 50-55% of the cases. Tumor recurrence occurs in 40-50% of cases completely resected tumor grading matter.

 

a) If completely resected tumor was recommended postoperative external radiotherapy of 55-60 Gy total dose intraoperative radiation dose or combination of 15-20 Gy external beam radiation therapy at a dose of 35-40 Gy.

b) If incompletely resected tumor was recommended chemotherapy with or without radiotherapy.

 

C. Head and neck sarcomas

We recommend multimodal treatment consisting of at least macroscopic surgical resection of the tumor associated with postoperative radiotherapy.

 

Stg. IV chemotherapy is recommended

In metastatic disease it is important to differentiate between limited metastases and disseminated metastases.

Limited metastases comprise a single body and should be considered for surgical resection to improve survival without disease and overall survival.

Disseminated metastases can be treated by observation, palliative care (palliative radiotherapy, chemotherapy or palliative surgery). Initiating palliative chemotherapy should be based on histology, tumor growth rate, chemosensitivity and associated symptoms. Adjuvant Chemotherapy is not considered as first-line therapy.

In metastatic disease- use monochemotherapy and polichemotherapy. Polichemotherapy compared to monochemotherapy gets even higher response rates are associated with higher toxicity and have no survival advantage. The decision to use multi-agent based on patient age, performance status and organ function. Polichemotherapy may require support with growth factor.

Always bear in mind placing the patient in a trial. If the patient is asymptomatic and has a slower tumor growth rate then observation with careful monitoring is a reasonable option. Patients with metastatic disease limited or recurrent disease after primary therapy should be considered for surgical treatment, radiofrequency ablation (RFA), embolization or radiotherapy with curative intent.

There are no clear guidelines for metastatic or recurrent disease because it is dependent on disease-free interval, performance status, and histology.

Sarcomas in stage IV disease should be considered for limited resection or other intervention definitively because it is associated with improved disease free survival.

For positive or close margins consider re-resection.

Prevention of local recurrence may require additional radiotherapy and / or chemotherapy.

If surgical resection of pulmonary metastases is recommended if the following conditions are met:

1. primary tumor is controlled or controllable.

2. There is no extrathoracic disease.

3. medical condition allows thoracotomy and surgical resection.

4. Complete resection of the disease is possible.

Survival at 3 years for patients with pulmonary metastases surgically removed was 23-40%.

 

 

Therapeutic Modalities

 

Surgery is the mainstay of treatment for localized disease. Local resection involves resection of the primary tumor satisfactory with a margin of normal tissue around the lesion. Amputation is reserved for tumors that cannot be resected (less than 10% of cases).

 

Radiotherapy

a). External beam radiation therapy Postoperative total dose of 60-66 Gy over a period of 6-7 weeks. The volume includes irradiated tumor bed and 5-7 cm around him.

b). Postoperative brachytherapy. It is given 45 Gy in 4-6 days. It is recommended in patients with high grade tumors where it causes a better local control than external radiotherapy.

c). Intraoperative irradiation of 20 Gy dose of external radiation dose associated with 35-40 Gy is indicated in retroperitoneal sarcomas.

Radiation therapy is associated adjuvant purposes after complete tumor resection or microscopic positive margins.

 

Definitive radiotherapy is indicated in patients with unresectable disease or medical condition that precludes surgery.

The total dose of 64-66 Gy and is the 5-year survival was 25%.

 

Chemotherapy

Adjuvant chemotherapy is recommended in stg.III sarcomas [large tumors (> 10 cm) deep, high grade (G3-G4)]

 

Preoperative chemotherapy used in retroperitoneal inoperable tumors and large high grade tumors.

 

Palliative chemotherapy is recommended in stage IV sarcomas.

Most sensitive to chemotherapy are fibrosarcomas and synovial sarcomas. Leiomyosarcomas respond to chemotherapy in 20%. Uterine leiomyosarcomas respond better than those of gastrointestinal.

 

In leiomyosarcomas Doxorubicin is the most active. In synovial sarcoma, ifosfamide seems more active.

a) For patients with bad performance status monochimioterapie with Doxorubicin is recommended 60-70 mg / m2 / day repeated every 3 weeks (Line I). As treatment Line II is recommended:

Ifosfamide 5 g / m2 / day x 5 days every 3-4 weeks iv bolus or

Dacarbazine 250 mg / m2 IV x 5 days every 3-4 weeks.

b) For patients with good performance status polichemotherapy is recommended although no combination with doxorubicin has proved superior to monochemotherapy in terms of overall survival.

c) potentially resectable metastases in patients with recommended multi-agent that produces a response rate higher than monochemotherapy.

Monochemotherapy. Most used cytostatics were Doxorubicin, ifosfamide, Dacarbazine.

 

 

 

see table no. 3 Monochemotherapy in soft tissue sarcomas

Doxorubicin 60 mg / m2 / day i.v., every 3 weeks. 20% response rate. The maximum dose = 475 mg life / m2

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Doxorubicin 25 mg / m2 / day IV x 3 days to 3 weeks. 20% response rate.

……………………………………………………………………………………………..

Epirubicin 160 mg / m2 / day IV, to 3 weeks.

G- CSF 5mg / kg / day 2-8 days

………………………………………………………………………………………………

Pegylated liposomal doxorubicin 50 mg / m2 to 4 weeks (prefer 30-35 mg / m20 Since there dose of 50 mg / m2 is associated with high toxicity); no lifetime maximum dose.

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Ifosfamide 2 g / m2 / day IV pefuzie continuous 1-4 days

Mesna 400 mg / m2 / day iv bolus and then prior to 2g / m2 / day on days 1-5 IV continuous infusion. Repeat every 3 weeks response rate of 18%.

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Ifosfamide 5 g / m2 (maximum total dose of 8g / m2) iv infusion for 24 hours.

Mesna 5g / m2 / day continuous iv infusion for 24 hours and an additional dose of 400-600 mg / m2 in 2 hours after the ifosfamide

Repeat every 3 weeks.

————————————————– —————————————

Dacarbazine 250 mg / m2 / day IV on days 1-5 repeated every 3 weeks with a 15-18% response rate. or

Dacarbazine 800-1000 mg / m2 / day IV on day 1 every 3 weeks repeated response rate of 15-18%.

………………………………………………………………………………………………….

Gemcitabine 1200mg / m2 IV in 90-120 minutes on days 1 and 8 repeat to 3 weeks

………………………………………………………………………………………………………

Pazopanib 800 mg PO (not recommended in liposarcomas) FDA recommends that second line chemotherapy; to start at a dose of 200 mg for 2 weeks and gradually increases.

……………………………………………………………………………………………………….

ET-743 (trabetidine) is available in US clinical trials

…………………………………………………………………………………………………………

 

 

 

 

 

Polichemotherapy:

 

 

AD: doxorubicin 15 mg / m2 / day, continuous infusion iv 1-4 days

Dacarbazine 250 mg / m2 / day continuous iv infusion days 1-4

Repeat every 3 weeks response rate of 17%.

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AI: Doxorubicin 20-25 mg / m2 continuous infusion days 1-3

Ifosfamide 2-3g / m2 / day by continuous infusion, days 1-3

Mesna 2-3g / m2 / day continuous infusion days 1-4

It is repeated every 3-4 weeks a 25% response rate.

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Gemcitabine 900 mg / m2 on days 1 and 8 30-90 min

Docetaxel 75-100mg / m2 in 60 min on day 8

G-CSF 5mcg / kg / day, days 2-8

Repeat in 21 days

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Gemcitabine 900 mg / m2 on days 1 and 8 30-90 min

Docetaxel 35 mg / m2 on days 1 and 8 min 60

Repeat in 21 days

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MAID: Doxorubicin 20-25 mg / m2 / day IV or continuous infusion days 1-3

Ifosfamide 2-2.5 g / m2 / day i.v. injection or continuous infusion days 1-3

Mesna 2.5 g / m2 / day i.v. continuous infusion days 1-4

Dacarbazine 300 mg / m2 / day IV bolus injection or continuous infusion days 1-3

Repeat 3-4 weeks with response rates of 32-49%.

————————————————– —————————-

For children with rhabdomyosarcoma COW procedure is used with a response rate of 90%.

COW: Cyclophosphamide 1200 mg / m2 / day i.v. Day 1

Doxorubicin 20 mg / m2 / day i.v. infusion for 4 hours, days 1-3

Vincristine 1.5 mg / m2 / day i.v. (Maximum 2 mg) on ​​Day 1

Alternate 3 weeks:

Cyclophosphamide 1200 mg / m2 / day i.v. Day 1

Vincristine 1.5 mg / m2 / day i.v. (Maximum 2 mg) on ​​Day 1

Actinomycin D 0.5 mg / m2 / day i.v. Days 1-3

3 weeks Pause and resume cycle.

 

 

 

Special histology treatment of sarcomas

 

1. Leiomyosarcoame

………………………………………….. ……………………….

1. Gemcitabine 900 mg / m2 on days 1 and 8 30-90 min

Docetaxel 75-100mg / m2 in 60 min on day 8

G-CSF 5mcg / kg / day, days 2-8

Repeat in 21 days

————————————————– —————————-

2. Gemcitabine 900 mg / m2 on days 1 and 8 30-90 min

Docetaxel 35 mg / m2 on days 1 and 8 min 60

Repeat in 21 days

………………………………………….. ………………………

 

3. Temozolomide 150 mg / m2 (75mg / m2 X 2 / day) on days 1-5 repeated 4 weeks

 

2. Angiosarcomas have a 5-year survival rate of 10-30%. Angiosarcomas associated with radiation therapy have a poorer prognosis. Treatment consists of wide excision and radiotherapy.

Systemic chemotherapy useful includes:

1.Doxorubicin and ifosfamide,  response rates of 10% -20%

2. Paclitaxel 80 mg / m 2 IV days 1, 8 and 15; Repeat 4 weeks. The response rate of 19%

3. Docetraxel

4. Sorafenib with an overall response rate of 14%.

5. Bevacizumab obtained a response rate of 12% and 62% tumor stabilizing.

 

3. Synovial sarcoma

It is particularly sensitive to ifosfamide. Supravieetuirea average patient treated with either doxorubicin or ifosfamide was 22 months, with a response rate of 25%, but the combination of two chemotherapy achieved a response rate of 58%.

Prognostic

1. The size of the tumor. Tumors> 5 cm were poor prognosis.

2. The histology. Poorly differentiated tumors G3, G4 , have a bad prognosis.

3. The depth of the lesion. The tumors have a worse prognosis deeper than superficial tumors.

4. The location of the tumor. Retroperitoneal sarcomas have a poor prognosis.

5. Local recurrence is a factor predisposing to subsequent recurrence.

6. The stage disease. Survival at 5 years depending on disease stage was becoming smaller as the stage was more advanced.

The survival to 5 years depending on the stage of the patients was 70-90% for std.I,

55-70% for stg.II,

20-50% for stg.III

4-20% for stg.IV

Tracking patients by clinical examination is 3 months during the first 2 years and 6 months for the next 3 years. CXR is recommended 6 months in the first 2 years and annually thereafter. C. T. chest indicated to the presence of respiratory symptoms. In patients with retroperitoneal sarcomas recommended abdominal CT every 6 months in the first 2 years and annually over the next three years.