Primary Pulmonary Sarcoma

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Presentation transcript:

Primary Pulmonary Sarcoma Wael Batobara

History 78y Caucasian Male presented to ER 9 days SOBE , Dry Cough , Night Sweating X smoker >50 y ( 10 pack y ) Received 3 days Levofluxacin for ? CAP No Chest pain , Hemoptysis , Orthopnea or PND No Leg swelling No Change in Wt or appetite

History PMH : GERD , BPH & Knee OA No contact with similar case No recent travel No pets or occupational exposure NKA Rx : PRN NSAID , H2 Blocker

Examination Afebrile BP 150/90 HR 100 regular RR 20 Sat 93% on 5 liters O2 No Pallor or Lymphadenopathy Chest: Central Trachea Dull percussion Lt Lower 2/3 Post Absent breath sound Lt L 2/3 post CVS ,Abd & LL NAD

Investigation CBC WBC & Plt  N HB 130 MCV N PTT & INR  N Lytes ,BUN & Creat  N LFT , EKG & UA  N ABG PH 7.38 PO2 75 3L O2 PCo2 35 HCO3 26 CXR & Chest CT

Investigation Pleural Tapping  2.8 liters drained Serosanguinus exudate -ve cytology CT Guided Bx  Spindle Cell Sarcoma CT Head , Abd & Pelvis N

Hospital Course Seen By Thoracic Sx  Inoperable Seen By Med.Onc.offered Chemo & XRT Post tap CXR small effusion Not accessible By US Improved SOB Sat 94% RA Discharged Home F/U Oncology clinic for XRT +/- Chemo

Primary Lung Sarcoma Incidence Presenting Symptoms & Signs Diagnosis Treatment Prognostic Factors

Incidence Sarcomas are rare malignant tumors arising from the mesenchymal tissues at all body sites and include a wide array of histopathologic types 10,700 newly diagnosed sarcomas per year in the United States {0.80 percent of the 1,334,100 new malignant tumors } 77 percent arise in soft tissue and the remainder in bone CA Cancer J Clin 2003 Jan-Feb

Incidence Primary Lung Sarcoma is Rare { 0.1-1% of all lung cancers} From 1970 To 1999 In Hospital Louis Pardel 12 Cases Lung Cancer 38-2002 From 1982 To 1998 In Lille University Hospital  18 Cases Euro Jr Of Cardiothoracic Sx 18-2000

Patients Characteristics Male 7/12 Vs 7/18 Female 5/12 Vs 11/18 Mean age 50 Vs 53 Smoking 7/12 Vs 8/18 Asymptomatic 1/12 Vs 9/18 Lung Cancer 38-2002 Vs Euro Jr Of Cardiothoracic Sx 18-2000

Symptoms & Signs Chest pain ,SOB & Cough common Fatigue , Wt loss less common Supraclavicular LN & Pancoast Syn in 1 Pt CXR & CT  Solitary masses Upper Lobe other features : cavitations ,effusions ,atelectasis ,chest wall & ribs involvements Lung Cancer 38-2002 & Euro Jr Of Cardiothoracic Sx 18-2000

Bronchoscopy & Pathology N 8/12 Vs 8/18 Endobronchial lesion 1/12 Vs 7/18 Extrinsic compression 3/12 Vs 3/18 Most common type Leiomyosarcoma 7/12 then monophasic synovial sarcoma 2/12 While in the other study Malignant Fibrous Histiocytoma 4/18 Undifferentiated Sarcoma 4/18 Lung Cancer 38-2002 & Euro Jr Of Cardiothoracic Sx 18-2000

Diagnosis PET Scan 18 Patients with soft tissue sarcoma 4 Patients with benign soft tissue lesion 100% sensitivity for sarcoma Uptake correlate with tumor grade Only one benign lesion showed uptake Journal of Nuclear Medicine Feb 1996

Treatment & Prognosis 9/12 Surgery 6 lobectomies & 3 pneumonectomies 3/12 Inoperable : 1 Pancoast , 1 Age 1 locally advanced disease 3/12 Chemotherapy Ifosamiade containing regimens IVA or IVAD CA Cancer J Clin 2003 Jan-Feb

Treatment & Prognosis 6/12 alive disease free 6/12 died 4 from disease progression 1,3,5 y Survival was 71,50,38 % Relapse 5/12 4 intrathoracic Rx wit Chemo CA Cancer J Clin 2003 Jan-Feb

Treatment & Prognosis 15/18 Surgery 3/18 pre op Chemo to shrink tumor size  successful in 2 patients 1 free resection margins 1 microscopic invasion Euro Jr Of Cardiothoracic Sx 18-2000

Treatment & Prognosis 9/18 died 5 from sarcoma 9/18 alive till now 3 with recurrent disease which was resected in 2 Significant correlation between stage & survival 5 y survival 43% Euro Jr Of Cardiothoracic Sx 18-2000

Thank You I hope It Was Not Boring