Chest Wall Tumors and Congenital Chest Wall Malformations.

Chest Wall Tumors and Congenital Chest Wall Malformations

CHEST WALL TUMORS

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 and fibrous

dysplasia. Most common malignant tumors are chondrosarcoma, Ewing’s sarcoma

and osteosarcoma. Most common site of involvement for all chest wall lesions is rib cage. 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)

Margins vary depending on the type of neoplasm.

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.

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) 1st and 2nd ribs and manubrium are in normal position 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 Chondrogladiolar type: protrusion of inferior costal

cartilages Chondromanubrial type: protrusion of manubrium and

superiror costal cartilages 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:

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

Absence of sternocostal part of pectoralis major muscle

Brachysyndactyly

STERNAL DEFECTS Cleft Sternum Thoracic ectopia cordis Thoracoabdominal ectopia cordis (Cantrell Pentalogy) Cervical ectopia cordis

Heart is in a normal position in the chest in cleft sternum but is displaced in other three.

In thoracic ectopia cordis, heart protrudes anteriorly and is free of any covering tissue.

In cervical ectopia cordis, protrusion is more pronounced and the heart is often fused with the head.

In thoracoabdominal ectopia cordis, heart is covered but displaced into the abdomen through a defect in the diaphragm

Cervical ectopia cordis Thoracic ectopia cordis

CANTRELL PENTALOGY Distal sternal cleft Ventral diaphragmatic defect Omphalocele or ventral abdominal wall defect Pericardial defect and pericardio-peritoneal Cardiac anomaly (VSD, Fallot, Ventricular diverticula)

ASPHYXIATING THORACIC DYSTROPHY

(JEUNE’S SYNDROME) AR, no chromosomal abnormality Narrow, bell shaped throax and protuberant abdomen Ribs are short, wide Pulmonary impairement

JARCHO-LEVIN SYNDROME

(SPONDYLOTHORACIC DYSPLASIA) AR Multipl vertebral and rib deformities Crab like appearance of ribs Shortened thorax, moderate to severe scoliosis and

kyphosis

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