Download chest wall tumors

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