1. Chest Wall Tumors and Congenital Chest Wall Malformations
Doç Dr Çağatay TEZEL

2. CHEST WALL TUMORS

3. Primary chest wall tumors are 5% of all thoracic neoplasms.
Majority are the result of invasion of adjacent malignancies (Most common lung cancer, sarcoma and breast cancer) or metastatic lesions
Primary chest wall tumors are 5% of all thoracic neoplasms.
Most frequent benign lesions are
osteochondroma,
chondroma
fibrous dysplasia.
Most common malignant tumors are
chondrosarcoma,
Ewing’s sarcoma
osteosarcoma.
Previous irradiation may result in malignant chest wall tumors.

4. Symptoms and signs and Diagnosis
First nonpainful mass, with continued growth pain occurs.
All malignant tumors are painful, only 2/3 of benign tumors produce pain.
Chest X-ray, CT, MRI
Needle biopsy, excisional biopsy, incisional biopsy

5. Surgical Management Primary chest wall tumors
Selected metastatic lesions
Locally recurrent breast cancer
Tumors unresponsive to nonsurgical therapies
Tumors causing local complications (wound ulceration, infection or intractable pain)

6. BENIGN RIB LESIONS
Osteochondroma : most common benign bone neoplasm, 50% of all benign rib tumors. Arises from bony cortex in the metaphyseal region of a rib. Tumor begins in childhood. M/F: 3/1 Malignant degeneration reported. Should be resected. No recurrence.
Chondroma: 15% of all benign rib neoplasms, most commonly in 2nd or 3rd decade of life, M=F, at the costochondral junction, painless mass. Wide excision to prevent local recurrence.
Fibrous Dysplasia: Benign, cystic lesion, characterized by fibrous replacement of the medullary cavity of the rib. Presents as a solitary mass in the lateral or posterior rib cage. 30% of all benign chest wall tumors. M=F. Resection is curative.

7. MALIGNANT RIB LESIONS
Chondrosarcoma: most common primary chest wall bone neoplasm, 33% of all primary malignant bone lesions. Arises in the costochondral arches of rib or sternum. M>F. 3rd or 4th decade of life. Complete resection.
Ewing’s sarcoma: small round cell sarcoma, primarily in flat bones and midshaft of long bones. 17% of all malignant chest wall tumors. M/F: 2/1. Mottled destruction containing lytic and blastic areas, onion skin appearance. First systemic chemotheraphy, then primary site either irradiated or resected.
Osteosarcoma: 10% of all malignant primary chest wall tumors. Poor prognosis. Teenagers and young adults. Induction chemotherapy , then wide excision.
Solitary Plasmacytoma: 6% of all malignant primary chest wall tumors. 5th-7th decades of life. 2/3 of patients are male. Abnormal protein electrophoresis, urinary Bence Jones protein and hypercalcemia. Osteolytic lesion. Primary chemotheraphy then resection or radiation.

8. PRIMARY SOFT TISSUE TUMORS
Predominant benign tumors are fibromas, lipomas, giant cell tumors, neurogenic tumors, vascular tumors (hemangiomas) and connective tissue tumors.
Neurogenic tumors include neurilemomas nad neurofibromas.
Neurofibromas: isolated or asso with von Recklinghausen’s disease (neurofibromatosis).
Local excision

9. MALIGNANT SOFT TISSUE TUMORS
Desmoid tumors: locally invasive tumors, propensity to recur, M=F, between adolescence and 40 yeras of age, originates in muscleand fascia, Wide surgical excision, local recurrence is common.
Soft tissue sarcoma: M/F= 2:1, seen in adult life except rhabdomyosarcoma which is seen most frequently in children and young adults <45 years of age.

11. C

14. CHEST WALL DEFORMITIES

15. Pectus Excavatum
Pectus Carinatum
Poland’s Syndrome
Sternal defects
Miscellaneous conditions (vertebral and rib anomalies, asphyxating thoracic dystrophy (Jeune’s disease), rib dysplasia

16. PECTUS EXCAVATUM
Posterior depression of the sternum and costal cartilages
Funnel chest
Most common congenital chest wall deformity
1/ , boys>girls (4:1)
Most common associated deformity is scoliosis (25%), cardiac anomalies may be seen
Familial
Asymptomatic; seldom dyspnea, easy fatique, palpitation
Cosmetic defect and psychosocial problem
Surgical repair: Classic: Ravitch sternoplasty, MIRPE: NUSS repair

21. PECTUS CARINATUM
Anterior protrusion of the sternum
Boys>girls (4:1)
Trisomy 18, Marfan syndrome, homocystinuria, Morquio syndrome, Ehler-Danlos syndrome, scoliosis (15%)
Surgery: Open surgery, Abramson method

23. POLAND’S SYNDROME Major components: Minor components: 1/32000 births
Congenital absence of pectoralis major and minor muscles
Hand anomaly (Syndactyly, brachydactyly, acromely)
Breast agenesis
Minor components:
Hypoplasia of subcutaneous tissue
Agenesis of II-IV costal cartilages
Absence of axillary hair
Scapula deformity (scapula alata)
1/32000 births
Conservative, Surgery

24. Brachysyndactyly
Absence of sternocostal part of pectoralis major muscle

25. Tietze’s syndrome (chondrodynia)
Painful, nonsuppurative swelling of costal cartilages without abnormal histologic change
Chest pain and swelling of costochondral junction
2nd costochondral junction is tender to deep palpation
İbuprofen