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

CHEST WALL TUMORS

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.

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

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)

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.

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.

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

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.

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CHEST WALL DEFORMITIES

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

PECTUS EXCAVATUM Posterior depression of the sternum and costal cartilages Funnel chest Most common congenital chest wall deformity 1/300-400, 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

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

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

Brachysyndactyly Absence of sternocostal part of pectoralis major muscle

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