Soft Tissue Tumors Ali AlGhamdi

case scenario 38-year-old male presents with right knee pain that is progressively worsening over the past 5 months The patient had an effusion that was drained by his primary care provider 2 months ago

out lines Definition Etiology Classifications Clinical features and treatment Summary

Defintion Mesenchymal proliferations that occur in the extraskeletal, nonepithelial tissues of the body, excluding the viscera, coverings of the brain, and lymphoreticular system.

Etiology Unknown ! Radiation therapy chemical burns Trauma Heat Burns Disease related (Ex neurofibromatosis)

Classification Benign Malignant

Histologic type Benign Malignant Adipose Tissue Lipoma Liposarcoma Fibrous tissue Fibromatosis Nodular fasciitis Fibrosarcoma Fibrohistiocytic tumours Fibrous histiocytoma Dermatofibroma Malignant Fibrous Histiocytoma????? Skeletal Muscle Rhabdomyoma Rhabdomyosarcoma Smooth Muscle Leiomyoma Leiomyosarcoma Vascular Haemangioma Lymphangioma Angiosarcoma Peripheral nerve Neurofibroma Schwannoma Malignant peripheral nerve sheath tumour Uncertain histogenesis Granular cell tumour Synovial Sarcoma Alveolar soft part sarcoma Epithelioid sarcoma

Benign Soft Tissue Tumours Lipoma Hemangiomas Fibroma Myxoma Schwannoma PVNS

Lipoma Most common Soft tissue tutor Subcutaneous (common) VS Deep Male = female Slowly growing painless mass Radiolucent MRI , T1 High signal and T2 Intermediate signal

Treatment Benign and Do not metastasize No Rule for Chemotherapy of Radiotherapy Observation (small) Marginal Excision (Large and Symptomatic) Recurrence rate : rare (subcutanous) , up to 20% (deep)

Hemangiomas Benign proliferation of mature blood vessels Skin, subcutaneous or intramuscular male = female Painful mass X-rays appears as phleboliths (calcification) MRI with gadolinium increase signal in T1 and T2

Treatment Observation NSAID , Vascular stockings and activity modification Sclerotherapy or embolization (painful and large) Marginal Excision

Fibromas Fibrocytes, Fibroblast and myofibroblasts Female > male shoulder (most common), upper arm, buttocks and trunk painful or painless mass Radiolucent MRI ,T1 intermediate signal T2 High signal

Treatment Wide surgical excision (high recurrence rate ) Radiotherapy is controversial Chemotherapy is controversial

Schwannoma arising from schwann cell of peripheral nerve 5% of benign soft tissue tumors affects major peripheral nerves of upper and lower extremities painful slowly growing mass (<5cm ) +ve Tinel's sign Radiolucent MRI T1 intermediate signal T2 High (Taget sign)

Treatment Marginal Excision (spare the nerve ) Recurrence rate low (<1%)

PVNS Monoarticular neoplastic synovial disease exuberant proliferation of synovial villi affect large joints (knee most common ) male = female Painful swelling (Hx of trauma) X-rays cystic erosion with sclerotic margins on both sides of the joint MRI low signal in both T1 and T2

Treatment Observation ( Mild ) Total Synovectomy Radiation therapy

Malignant soft Tissue Tumors MFH Liposarcoma Leiomyosarcoma Fibrosarcoma Epitheliod sarcoma

Sarcoma in General Presents in All ages painless mass MRI ,T1 low signal T2 high signal Metastasize to lung >bone> liver Any part of Body (>5cm) Biopsy is the gold standard in Dx

Malignant Fibrous Histocytosis pleomorphic , High grade , anaplastic sarcoma Most common sarcoma 70 % in extremities painless mass MRI low signal T1 and High signal in T2

Treatment Wide Surgical Excision PostOP radiation Amputation (unresectable) 65% 5 year survival

Liposarcoma 2nd most common Sarcoma Adult tumor (40-70) Extremeties (thigh) painfull large mass (firm) MRI high signal in T1 and T2

Treatment Wide Surgical Excision Does not metastasize , 100 % 5 year survival high recurrence rate (20%)

Synovial sarcoma 5-10 % of all soft tissue sarcoma young adults and teenagers (15-35) In proximity to large Joints lower extremities > upper extremities Male > Female Painful slowly going mass

Radiology X -ray (calcification) CT (lobular mass close to joint) X rays calcification MRI T1 intermediate signal T2 hight signal

Treatment Wide surgical excision post Op radiation Amputation (unresectable ) Metastasize to Lungs >LN>BM 65% 5 year survival

Leiomyosarcoma 9% of soft tissue sarcomas Retroperitoneum of Thigh painless mass MRI intermediate signal in T1 and T2

Treatment Wide surgical Resection Post OP radiation metastasise to Lung >liver>bone 50% 5 year survival

Take Home message soft tissue benign tumors outnumbered malignant tumors (100:1) present with enlarging painless or painful mass mass >5 cm growing,deep to the superficial facia is soft tissue sarcoma until proven otherwise MRI is the best imaging modality for defining the anatomy and helping characratize the lesion

Take home message CT chest is required to evaluated for metastasis Exicional biopsy shouldn't performed if the clinician does not know the origin of the tumor most soft tissue sarcoma are sensitive to radiation

References www.tumorsurgery.org paediatric soft tissue tumors, Karman aflatoon, 2011 Miller’s review of orthopaedics , 7th edition www.orthobullets.com