Soft tissue Sarcoma

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CASE HISTORY:

CASE HISTORY Patient (XYZ), 29/M, Married, Driver by profession, Resident of Mianwali, admitted through OPD on 30/11/09. Presenting complaints: Swelling in left calf ------- 3years

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History of present illness: Noticed 3y ago Initially small Increased in size gradually Accompanied by pain on touch No discharge No H/O trauma, fever, cough, walking difficulty loss of sensation, or weight loss.

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PAST HISTORY: Operated a year ago for same swelling Excision done (according to patient) No medical record available FAMILY HISTORY: Not significant

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PERSONAL HISTORY: Non smoker Non addiction SOCIO-ECNOMIC STATUS: Single earning member Poor s/e

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SYSTEMIC REVIEW: unremarkable No H/O bone pains, shortness of breath, yellowness of eyes or any urinary complaints.

GENERAL PHYSICAL EXAMINATION:

GENERAL PHYSICAL EXAMINATION A young man sitting comfortably on bed, well oriented in time place and person, with the vitals of Blood Pressure = 110/70 mmHg Pulse Rate = 90/min regular Temp = 98.6 °F Resp. Rate = 17/min No pallor, jaundice, cyanosis, clubbing, odema No lymph node palpable

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RESPIRATORY SYSTEM: NVB with no added sound CARDIOVASCULAR SYSTEM: NAD GASTRO-INTESTINAL SYSTEM: Abdomen soft non tender, no visceromegaly Bowel sounds normal P/R normal

LOCAL EXAMINATION:

LOCAL EXAMINATION SITE : ------------- Left calf 15 cm from medial malleolus SIZE : ------------- 10 cm × 15 cm SHAPE: ------------- Globular SURFACE: ------------- Smooth Skin: ------------- Shiny with sparse hair, Scar mark & - ve skin pinch TEMP: ------------- Raised TENDERNESS: ------------- + ve MOBILITY: ------------- Non mobile fixed to underlying tissue REDUCIBILITY: ------------- - ve COMPRESSIBILITY: --------- - ve TRANSILLUMINATION: ----- - ve FLUCTUATION: ----------- Positive in two areas over the swelling PEREPHERAL PULSES: ----- Weak posterior tibial LYMPH NODES: ------------ Not palpable NEUROLOGICAL EXAM:----- No neurological deficit

INVESTIGATIONS:

INVESTIGATIONS CBC: Hb : 13.6 mg/dl WBC: 4300 Platelets: 216000 LFTS: Bilirubin : 1.6 mg/dl Alk . Phosph : 104 IU / L AlT : 35 mg/dl AST: 32 IU / L RFTS: Urea: 24 mg/dl Creatinine : 0.7 mg/dl S.E: Na⁺: 138 mmol /L K ⁺ : 3.7 mmol /L

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Ultrasound abdomen: Normal FNAC: Abundant cellular smear Highly atypical spindle shaped cells Pleomorphic hyperchromatic nuclei Scanty to moderate cytoplasm

CHEST X-RAY:

CHEST X-RAY Multiple rounded well defined radio opaque opacities in both hemi thoraces of variable sizes. The largest one overlying left dome of diaphragm. C.P angles normal. Cardio- mediastinum normal Impression: metastaic disease

MRI scan of LEG :

MRI scan of LEG Multiple axial, coronal, saggital T1 T2 wt images with and without contrast. A large expansile lesion , hypointense on T1 wt image and heterogeneous intensity on T2 wt image. Middle third part of the posterolateral - and postero medial compartment of left leg Mass effect by displacing muscle Not involving tibia/fibula No periosteal thickening No adjacent joint or muscle involvement No comment on vascular involvement given in MRI. Impression: Soft tissue sarcoma

Mri scan:

Mri scan

PLAN:

PLAN Above knee amputation Followed by ajuvant chemo-radiotherapy for metastatic disease. Rehabitilation

Soft tissue SARCOMAS:

Soft tissue SARCOMAS

definition:

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

Background:

Background Haematogenous spread (LN rare!) Extremity vs. visceral/ RP sarcomas Large, painless, rapidly growing mass May present with GI bleed, bowel obstruction, neurological deficit <1% of all malignancies 50% in extremity (thigh), 50% in children Painless mass (but 33% have pain!) Lungs are #1 mets Liposarcoma is most common histopathology

Aetiology:

Aetiology Unknown Radiation therapy Chemical burns , Heat burns Chemical Exposure Phenoxyherbicides Chlorophenols Arsenic Asbestos Kaposi sarcoma ( AIDS and in immunosuppression ) genetic syndromes neurofibromatosis type 1 ( neurofibroma , malignant schwannoma ), Gardner syndrome ( fibromatosis ), Li- Fraumeni syndrome (soft tissue sarcoma), Osler-Weber- Rendu syndrome ( telangiectasia ). Trauma does not increase risk of sarcoma

Types:

Types 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

Age distribution:

Age distribution

Lipoma:

Lipoma Most common Mature adipocytes Soft, mobile, painless Simple excision

Liposarcoma:

Liposarcoma M ost common sarcomas of adulthood F orties to sixties uncommon in children D eep soft tissues proximal extremities retroperitoneum Notorious for developing into large tumors. Types well-differentiated Myxoid round cell pleomorphic variants Lipoblasts , (which mimic fetal fat cells)

Fibrosarcoma:

Fibrosarcoma Rare Occur anywhere in the body , Retroperitoneum Thigh Knee Distal extremities. unencapsulated , infiltrative, soft, fish-flesh masses Areas of hemorrhage and necrosis. All degrees of differentiation , Slowly growing tumors (cellular fibromas ) Spindled cells (herringbone fashion) Highly cellular neoplasm architectural disarray Pleomorphism frequent mitoses areas of necrosis.

TUMORS OF SKELETAL MUSCLE:

TUMORS OF SKELETAL MUSCLE Almost all malignant . The benign variant, rhabdomyoma , is distinctly rare. Rhabdomyosarcoma Most common soft tissue sarcomas of childhood and adolescence, Appear before age 20. Head and neck or genitourinary tract. MORPHOLOGY. Embryonal Alveolar Pleomorphic Rhabdomyoblast The diagnostic cell in all types- eccentric eosinophilic granular cytoplasm rich in thick and thin filaments. round or elongate; the latter are known as tadpole or strap cells

TUMORS OF SMOOTH MUSCLE:

TUMORS OF SMOOTH MUSCLE Leiomyomas Benign Uterus (most common neoplasm in women) Skin Subcutis from the arrector pili muscles Less frequently in deep soft tissues. Not larger than 1 to 2 cm Composed of fascicles of spindle cells Blunt-ended elongated nuclei Minimal atypia Few mitotic figures.

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Leiomyosarcoma 10% to 20% of soft tissue sarcomas. Skin Deep soft tissues of Extremities Retroperitoneum . Pleomorphic , spindle cells with blunt-ended nuclei and eosinophilic cytoplasm

Rare soft tissue sarcomas:

Rare soft tissue sarcomas Fibrous Tumours and Fibrohistiocytic tumours Dupuytren contracture plantar fibromatosis Deep-seated Fibromatosis Desmoid Tumors Synovial Sarcoma

Mass – most common presentation:

Mass – most common presentation Delays diagnosis- Often treated for “hematoma” or “pulled muscle” Biopsy mass if No H/O of trauma 6 wk post trauma Mass >5 cm Any new, enlarging, or symptomatic mass Head, neck, trunk, extremity- Often painless mass Retroperitoneal mass- Palpable abdominal mass with abdominal pain, fullness, early satiety

Diagnosis:

Diagnosis Excisional biopsy- smaller masses (<5cm) Risk of malignancy is low Take tumor pseudocapsule Incisional biopsy- > 5cm masses Allows definitive therapy later Scar *must be* incorporated into next surgery CT-guided FNA For abdominal & Retroperitoneal masses- minimizes tumor spillage

Pathologic Classification:

Pathologic Classification LOW-GRADE: little cellular atypia, few mitoses, no tumor necrosis INTERMEDIATE GRADE: atypia, some mitoses, none/little necrosis HIGH GRADE: atypia, mitoses, necrosis GRADE is most important for prognosis & Rx

Staging:

Staging 1. Tumor grade 2. Tumor size (increase risk of mets/ death) 3. Tumor depth (below fascia worse prognosis) CT scan or MRI May use X-Ray for bone tumors Sarcomas have prominent neovascularity and displace normal vessels LUNGS are #1 met (get CXR and CT)

AJCC – Stage & Grade:

AJCC – Stage & Grade Primary tumor (T) T0 T1 T2 No evidence of primary tumor Tumor <5 cm Tumor >5 cm Lymph nodes (N) N0 N1 No regional metastasis Regional node metastasis Distant metastasis (M) M0 M1 No distinct metastasis Distant metastasis Histopathalogic grading (G) G1 G2 G3 G4 Well differentiated (low grade) Moderately differentiated (intermediate grade) Poorly differentiated (high grade) Undifferentiated Stage IA G1 T1 N0 M0 IB G1 T2 N0 M0 IIA G2 T1 N0 M0 IIB G2 T2 N0 M0 IIIA G3 T1 N0 M0 G4 T1 N0 M0 IIIB G3 T2 N0 M0 G4 T2 N0 M0 IVA Any G Any T N1 M0 IVB Any G Any T Any N M1

Patterns of metastasis:

Patterns of metastasis

Treatment:

Treatment

Localized tumors:

Localized tumors Complete local excision adequate treatment for benign soft tissue tumors surgery alone or combined with radiation therapy or chemotherapy may be considered for treatment of localized primary and recurrent sarcomas.

Extremity Tumors:

Extremity Tumors Wide-local excision - definitive therapy Take 3-5 cm margins around tumor Deep margin- try for 1 uninvolved fascial plane Take nerves/vessels unless low-grade XRT (occasionally effective alone for small sarcomas) Tumors >10cm, preop XRT or chemo Amputation

Non - extremity Tumors:

Non - extremity Tumors Surgery Radiation Chemotherapy Sarcomas arising in the head and neck, thoracic or abdominal wall, mediastinum, or retroperitoneum are difficult to treat Risk of local recurrence is high For retroperitoneal tumors, the patient usually succumbs as a result of local complications, before metastases are evident.

Treatment Algorithm:

Treatment Algorithm

Postoperative Care:

Postoperative Care Compressive bandages Suction drains To minimize seroma formation that can delay administration of chemotherapy or radiation therapy Physical therapy and rehabilitation support may be required.

Follow up:

Follow up includes surveillance studies to evaluate Local recurrence Distant metastasis The precise interval - not well defined Surveillance continues for 3-5 years after treatment Benign tumors generally do not require such surveillance.

Thank you:

Thank you Dr. Imdad Ahmad Zahid