Soft tissue sarcoma

Case,etiology,epidemiology,diagnosis,treatment

1. Case Presentation Dr Badal khan PGR Ortho Unit III BMCH Quetta

2. PATIENT PARTICULARS Name . Karam khan Age .60 years Sex . Male Occupation: Labourer Address. Killa saifullah Date of admission 1st July 2018

3. H istory Chief complains Painless mass on right proximal arm for 7 years.

4. History of present illness According to patient he was alright until 7 years back then he noticed a swelling on proximal arm, initially it was small in size, gradually it increased in size and became huge within 4 months. There is no hx of trauma, fever, weight loss and pain.

5. Systemic Inquiries GPE, CVS, CNS, Respiratory, GIT & Urinary systems are normal. Systemic symptoms such as fever, weight loss, and night sweat are absent.

6. Past medical & Surgical History He has operated three times for same mass. He has received 3 dose of chemotherapy and radiation in 2017. Family History Married 5 sons & 2 daughter

7. Personal History Non smoker Sleep Normal Appetite normal Bowel habits normal

8. Socioeconomic History He belongs to a poor family. There are 9 members of family and 3 rooms for them.

9. General Physical & Systemic Examination No anemia, pallor and weight loss.

10. Focused Examination LOOK Round shaped mass on anterolateral aspect of right proximal arm , skin over the swelling is lost and necrosed . Margins of mass is red and there is no dilated vein around . Previous surgical scar is present. No visible muscle wasting of arm, forearm & wrist.

11. Focused Examination….. FEEL Local skin temperature is warm as compared to normal side. Tenderness –ve Distal pulses normal. Sensations intact

12. Focused Examination….. Movement Shoulder Joint All movements Normal Elbow Joint All movements Normal Wrist Joint All movements normal

13. Neurological Examination Sensation Normal Reflexes Normal Motor Normal

14. Labs Blood CP Hb..13mg/dl ESR Normal CRP 0.4 mg/dl (normal)

15. Radiographs On x-ray there is soft tissue opacity and no any bony involvement.

16. MRI

17. BIOPSY Histopathology show low grade soft tissue sarcoma.

18. Biopsy report

19. Diagnosis Recurrent soft tissue sarcoma

20. Management Plan Wide excision with reconstruction of defect.

21. Resection of mass Done

22. Soft tissue sarcoma Introduction Soft tissue sarcomas are malignant tumor that originate in soft tissues of body. Common sites. Extremity 43 % Visceral 19 % Retroperitoneal 15 % Trunk or thoracic 10 % Others 13 %

23. Etiologies Radiation exposure. Chronic lymphedema. Trauma . Chemical exposure e.g. arsenic, polyvinyl chloride. Infections such as herpes human virus.

24. Presentation Mostly asymptomatic mass. Pain later may occur due to destruction of surrounding tissues.

25. D iagnosis Labs. CBC ESR CRP To rule out infection

26. Imaging MRI For extremity mass. Give good delineation between muscles, tumor and blood vessels. Pet scan May help to determine high vs low grade May be helpful in recurrence.

27. Ct abdomen and pelvis To look for any metastasis

28. Biopsy FNAC Core needle biopsy Incisional biopsy Excisional biopsy

29. Metastatic workup Evaluation for site of potential metastasis Lymph nodes metastasis occur in less than 3 % For extremity lesion lung is the principle site for metastasis.

30. Staging AJCC/UICC staging system for soft tissue sarcomas T1: <5 cm T1a: Superficial to muscular fascia T1b: Deep to muscular fascia T2: >5 cm T2a: Superficial to muscular fascia T2b: Deep to muscular fascia N1: Regional lymph node involvement

31. Grading G1: Well differentiated G2: Moderately differentiated G3: Poorly differentiated G4: Undifferentiated

32. Prognostic factors Increased risk of local recurrence Age >50 Recurrent disease Positive surgical margins Increased risk of distant metastasis Size >5cm High grade Deep location Recurrent disease

33. Management Surgical resection Adjuvant radiotherapy Chemotherapy

34. Surgical resection Intralesional resection Marginal resection Wide resection Radical resection

35. Adjuvant radiotherapy Small low grade tumor <5 cm resected with 2 cm margin may not require radiation Adjuvant radiotherapy should be added to surgical resection . 1. if excission margin is close 2. if extra muscular involvement is present 3. if local recurrence would result in sacrifice of major neurovascular bundle or amputation. It improves local recurrence but not survival

36. Radiotherapy Can be given as brachytheraphy or intraoperative radiotherapy Brachytheraphy for high grade lesion External beam radiation therapy for large >5cm high or low grade leission Intraoperative radiotherapy can be given in case of retroperitoneal sarcoma Can be given as preoperative and post operative

37. Chemotherapy Can improve local control but not survival Doxorubicin and Ifosfamide have response rate of 20% Used only in advance disease Combination with radiation or neoadjuvant therapy are controversial

38. Metastatic disease Lung is most common site of mets. Median survival after metastatic disease is 8 to 12 months Resection of pulmonary mets can give 5 year survival of 32 % if all mets can be removed >3 mets poor prognosis