clinical aspects of benign and malignant soft tissue

7/25/2019 Clinical Aspects of Benign and Malignant Soft Tissue

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Prof .Dr. dr. Teguh Aryandono ,SpB(K)Onk

Division of Surgical Oncology

Dept of Surgery

GMU


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Epidemiology 1% of newly diagnosed adult cancer

7% of newly cancer in children

Location :- Extremity : 59%

- Trunk : 19%

- Retroperitoneum : 13%

- Head and neck : 9%


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Most common histology (STS) Malignant fibrous histiocytoma : 24%

Leiomyomyosarcoma : 21%

Liposarcoma : 19% Synovial sarcoma : 12%

Peripheral nerve sheat tumors : 6%


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Etiology Trauma (?)

Occupational chemical

Previous radiation exposure Chronic lymphedema

Genetic predisposition

Oncogene activation

Tumor supressor gene


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Benign and malignant soft

tissue tumor Connective tissue : fibroma/ fibrosarcoma

Fat tissue : lipoma/liposarcoma

Smooth muscle : leiomyoma/ leiomyosarcoma Striated muscle tissue : rhabdomyoma/

rhabdomyosarcoma

Synovial membrane: synovioma/ synoviosarcoma

Etc

Most common benign tumor : Lipoma and fibroma


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LipomaMORPHOLOGY.

The conventionallipoma, the most common subtype, is a

well-encapsulated mass ofmature adipocytes that varies

considerably in size. It arises in the subcutis of the proximal

extremities and trunk, most frequently during mid-adulthood.

Infrequently, lipomas are large, intramuscular, and

circumscribed. Histologically, they consist ofmature fat cells

with no evidence of pleomorphism or abnormal growth.

Lipomas are soft, mobile, and painless (except angiolipoma)

and are usually cured by simple excision.

conventional lipomas often show rearrangements of 12q14-

15, 6p, and 13q, and spindle cell and pleomorphic lipomas

have rearrangements of 16q and 13q .


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Intermediate group Between benign and malignant : matoses

Fibroblast : fibromatosis

Lipoblast : lipomatosis

Benign : grow non invasive, but expansive, do notmetastasize

matoses : grow invasive, do not metastasizeMalignant : expansive and invasive, frequently

metastasize


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Metastic potential of STS Low metastatic potential

Desmoid tumor

Atypical lipomatous tumor Dermato i rosarcoma protu erans

Hemangiopericytoma

Intermediate metastatic potential Myxoid liposarcoma Myxoid malignant fibrous histiocytoma

Extrasceletal chondrosarcoma


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High metastatic potential

Alveolar soft part sarcoma

Angiosarcoma

Clear cell sarcoma ( melanoma of soft parts)

Epitheloid sarcoma

Extrasceletal`Ewing sarcoma

Malignant fibrous histiocytoma

Liposarcoma ( pleomorphic and dedifferentiated)

Leiomyosarcoma

Neurogenic sarcoma ( malignant schwannoma) Rhabdomyosarcoma

Synovial sarcoma


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Dermatofibroma


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Dermatofibrosarcoma Protuberans


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Local growth pattern of the sarcomas is of

crucial clinical importance

Expansive growth compression of surroundingtissues pseudocapsule

nvas ve growt a rea y sprea m croscop ca y much further into the surrounding tissues than thepseudocapsule macroscopically suggest


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Risk of malignancy Tumor composed of fat tissue located in the subcutis is

usually lipoma

umor compose o at t ssue ocate un er t e asc ahas a much bigger risk of being a liposarcoma


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Presenting problem Identical way : painless slowly growing lump

Alert of sarcoma:

- Rapid growth of a soft tissue swelling- Sudden rapid growth of a long existing soft tissue

tumor

- Soft tissue tumor in children

Soft tissue tumor can develop everywhere in the body


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Physical examination History

- How long, change the size, how rapidly, any pain,

inflammation recently, fever, function normal?,

Inspection

- Compare with other side : symmetry?, color of

overlying skin?, atrophic muscle?. Contracturemuscle? Sceletal symmetry also during movement?Unusual posture? Functional disorder?


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Palpation

- Temperature of overlying skin

- Swelling sharply demarcated or ill- defined

- size?

- Fixation to skin and surrounding tissue?

- Relation to vessels and nerves

- Tenderness when touhed?


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Imaging diagnostic MRI : necrosis, vascular and bone involvement

CT Scan

CT scan : retroperitoneal sarcomathorax : high grade sarcoma

Angiography (if indicated)

Chest x-ray ( two directions)


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Staging Work-Up

What are we looking for?

CT/MRI (primary) Helpful to delineate soft

tissue planes; pre-surgical

evaluation

Bone Scan Look for metastases to bone

CT/PET May give helpful information

Look for metastatic disease

in the lungs (common site ofmetastases)

CT (body) Look for lymph node

involvement

about tumor activity andresponse to therapy

Bone Marrow Evaluation Look for metastatic disease


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Biopsy FNAB : benign or malignant

Core Biopsy, incisional biopsy

Smaller than 5 cm, superficial excisional biopsy

Biopsy

- Site and direction of the incisional biopsy

- Tumor spill

- The biopsy should be part of the primary surgicalapproach


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STAGE (GTNM) Histopathologic grade (G)

Tumor size and depth (T)

Metastasis - nodal (N)- distant (M)


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Treatment Tumor factor that favourable for curative intent

- tumor < 5 cm

- Low grade of malignancy- No distant metastases

- Localized at a site which allows radical excision


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Surgical aspects

Surgery holds a central position :wide local excision,limb sparing procedure, amputation

The direction : skin incisionlengthway with the

group of muscleopsy scar remove en oc

Sometimes extensive excision needed

If possible at least 2 cm of normal tissue surrounding

the tumor should be removed - radicalMore than 2 cm : no radiation therapy (except

intermediate and high grade)

Less than 2cm : adjuvant radiotherapy


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Limb- sparing surgery Neurovascular bundles : important

Cannot be saved : amputation

Isolated regional perfusion for limb sparing procedure


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During removal benign soft tissue

tumor Gross appearance strange !

- alert of malignancy

- Consider excision to incision for histopatologic

agnos s

- Inform the patient


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Radiotherapy Primary goal : to optimize local tumor control as

adjunct to surgery, for tumor with intermediate to highgrade of any size

Preoperative radiotherapy


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Chemotherapy Doxorubicin, dacarbacine, ifosfamide : response rate

20% for advanced STS

Adjuvant chemotherapy : not proven to increase

survivalarge e erapy : ma n or

Preoperative/ isolation perfusion


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Prognosis Type

Grade of malignancy

Location

Metastasis

Age of patient

Recurrence

Rare, but difficult for curative intent

refer tocenter


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Extremity sarcomas

MFH

Synovial sarcoma


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Case #1

64 y/o male with increasing abdominal girth


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Liposarcoma


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Sarcoma after mastectomy


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SUMMARY General recommendations for management of

extremity STS (Delman and Cormier, 2006).

Soft tissue tumor that are enlarging or greater than 3imaging ( ultrasonography or CT) and tissuesdiagnosis on the basis of fine needle or core needlebiopsy.

Evaluate for metastatic disease : chest radiography forlow or intermediate grade lesions and T1 tumor; andchest CT for high grade or T2 tumor.


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A wide local excision with 2 cm margins is adequatetherapy for low grade lesions and T1 tumors.

Radiation therapy plays a critical role in themanagement of T2 tumors.

a en s w recurren g gra e sarcomas or s anmetastatic disease should be considered forpreoperative ( neoadjuvant) or postoperative (adjuvant) chemotherapy.

An aggressive surgical approach should be taken in thetreatment of patients with an isolated local recurrenceor resectable distant metastases.


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Thank You

clinical aspects of benign and malignant soft tissue

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