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General Consideration
Brian Blades, 1949
IncidencePrimary chest wall tumors are rare. Statistical data remains inaccurate. Primary malignancy ranges from 13 to 50%. They should be considered malignant until proved otherwise by detailed analysis by and experienced surgical pathologist. Chest wall tumors are 5 % of all thoracic malignant.
OriginThey originates in bone, cartilage, soft tissue of chest wall. Most arise in ribs (85%).
Clinical ManifestationMost often clinical manifestations are either pain, palpable mass or an abnormality detected on a CXR. Pain occurs when normal struture are compressed or periosteal invasion (more common with malignant tumors). Patients may be only relatively mild discomfort.
Differential Diagnosis
InvestigationsChest x-rays, CT, bone scan to rule out multiple lesion. CT : Used to map the local extent of invasion and to plan resection and reconstruction Biopsy the mass is necessary for tissue diagnosis. Tumor size < 5 cm. à excisional biopsy. Inadequate biopsies are untrustworthy. Pulmonary function and Pt’s ability to tolerate physiologic deficit must be included. Chest wall reconstruction must be concerned. Accurate diagnosis required for proper treatment : multimodality or resection Requires adequate tissue FNA should be avoided Frozen sections should be performed
Classification
Primary Chest Wall Tumor
Benign Tumor of Chest Wall
Fibrous Dysplasia
Incidence : 30 % of benign chest wall tumors. Ribs are the most common site of solitary fibrous dysplasia especially at posterior or lateral portion of a rib. Age : 20 - 40 years old, Male = Female It usually presents as slowly enlarging nonpainful mass Pain can occur when large and pathologic fracture can develop. Chest x-rays : Soap bubble or ground glass appearance. Expansion and thinning of the bony cortex with a central trabeculated Fibrous dysplasia forms part of Albright syndrome (precocious puberty & skin pigmentation in girls).
ChondromaIncidence : 15 - 20 % of benign chest wall tumors. Age : 10 - 30 years old., Male = Female It usually occurs at costochondral junction anteriorly. It’s usually symptomatic & slow growing tumor It’s divided into 2 type
Chest X-Ray : Lytic lesions with sclerotic margins Expansion of bone with thinned but intact cortex Difficult to distinguish from chondrosarcoma Excisional biopsy is always recommended.
OsteochondromaOsteochondroma always present as a painless mass in young males ( Male : Female = 3:1 ) It origins from cortex of rib Chest x-rays : A pedunculated bony mass capped with viable cartilage
Resection is only for symptomatic & enlarging lesion. The recurrence is rare.
Eosinophilic granuloma
Multiple lesions of rib are common. It may be a part of Histiocytosis X or eosinophilic granuloma of lung. It’s associated with pain & localized tenderness. Pathologic fracture can developed. Chest x-rays : punched-out osteolytic lesion Microscopic : consists of chronic granuloma. Treatment
Osteoid OsteomaOsteoid Osteoma is a rare tumor. It arises from bony cortex of rib or vertebral arches. Symptoms occur especially at night Chest x-rays : small, radiolucent nidus encircled by sclerotic margin Treatment : Resection of entire rib for relief of symptom.
Desmoid TumorsTumors are a form of benign fibromatosis or low-grade fibrosarcoma. They arise from deep fascia & connective tissue of muscles. They’re slowly enlarging masses that invade locally. Pain is caused by pressure (common presenting symptom). Local recurrence is very high. Treatment : wide local excision Additional radiotherapy (external beam or brachytherapy) after resection is recommended.
LipomaLipoma is well circumscribed, thin wall and very soft. It composes of mature adipose tissue. Deeper lipomas may infiltrate muscle. Recurrence is common. Wide excision is required. Resection is for cosmetic reason or if true nature of tumor is in doubt.
Malignant Tumors of Chest Wall
Prognosis is ralated to the histologic grade rather than cell classification. Factors that influence prognosis include age, size of tumor, histologic grade and stage. Staging plays a central role in planning the therapeutic approach.
TNM Definitions
In all but stage I : Preoperative and/or postoperative radiotherapy is increase recommended Stage III & IV : Adjuvant chemotherapy
Chondrosarcoma
Chondrosarcoma is the most common chest wall malignant tumors (20%) Age : >40 years old. It arises after local trauma to chest or secondary to malignant degeneration of benign chondromas or osteochondromas It usually involves anterior costochondral junction of sternum. Chest x-rays : Similar appearance to benign chondroma Treatment : Resection with wide margins of greater than 4 cm. 5 year survival rate is 70 %.
Osteogenic SarcomaIncidence : 10-15 % of malignant tumors. Chest x-rays : Sunburst pattern It enlarges rapidly. Metastasis is often present. Investigation : Chest and abdominal CT scan & bone scan for assessment metastasis Treatment : Surgery and adjuvant chemotherapy 5-year survival rate is 60%.
Ewing’s SarcomaEwing’s sarcoma is the third most common (5-10 %). It frequently occurs in children & young man Male :Female = 2:1 Intermittent pain & inflammatory response with fever and leukocytosis may be found. Chest x-rays : Onion peel appearance Metastasis is common (Lung, CNS). Treatment : Multimodality therapy 5-year survival rate is 50%.
Metastatic TumorsHematologic dissemination is most common : Thyroid, Breast, Kidney. Radiation therapy is used for palliation. Breast and lung cancers are direct extension. 5 % of non small cell lung cancers invade the chest wall. 5 years survival rate is about 60% for pt. with chest wall invasion without LN involvement, but with N1 ~ 35% and N2 ~ 7-16%.
Chest Wall Reconstruction
Surgical guideline includes a 2-3 cm. soft tissue clear margin & removal of one normal rib above & below the involved ribs, a 2 cm. margin on the sternum is sufficient. Principle goal : “Protection of the intrathoracic organ, support of respiration by preventing paradoxical movement, and an acceptable cosmetic result without compromising an indicated cancer operation” No reconstruction in the defect size < 5 cm.
Ideal chest wall replacement
Prosthetic materials ;
Myocutaneous flaps
Summarized By Thirayost Nimmanon ÊÃØ»â´Â ¸ÕÃÂÊ¶ì ¹ÔÁÁÒ¹¹·ì
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