2Table 45-1 Classification of Chest Wall Tumors __________________________________________Primary neoplasm of chest wall: malignant, benignMetastatic neoplasms to chest wall: sarcoma, carcinomaAdjacent neoplasms with local invasion:lung, breast, pleuraNonneoplastic disaease: cyst, inflammation
3INCIDENCE Primary chest wall tumors are uncommon. 50-80 % of these tumors are malignant.Soft tissues are major sources of chest wall tumors.The most common primary malignant chest wall tumors are malignant fibrous histiocytoma, rhabdomyosarcoma and chondrsarcoma.The most common primary benign chest wall tumors cartilaginous tumors, desmoids and fibrous dysplasia.
5BASIC PRINCUPLES Signs and Symptoms 1. Chest wall tumors grow slowly. 2. Most patients have no symptoms initially.3. Pain may occurs in nearly all malignanttumors and 2/3 of benign tumors.4. Fever, leukocytosis and eosinophilia mayaccompany some chest wall tumors
6Diagnosis History, PE and lab exams Chest plain film and CT scan MRI can distinguish tumor from vessels and nerves, but does not assess lung nodules and calcification in the lung.Chest wall tumors must be diagnosed with incisional biopsy( tumor> 5 cm ) or excisional( tumor 3 –5 cm ) biopsy.Needle aspiration biopsy should be only for patients with a known primary tumor elsewhere.
7Treatment(1)Wide resection is essential for malignant chest wall tumors.The margin of normal tissue is 4 cm.For tumors of rib cage, involved ribs, partial ribs above and below the tumor must be removed.
8Treatment(2)For tumors of the sternum, manubrium, resection of the involved bone and corresponding costal arches is indicated.Any attached structures, such as lung, thymus, chest wall muscle or pericardium must be removed.The role of resection of chest wall metastasis and recurrent breast cancer is controversial
9SPECIFIC TUMORS A. Primary Bone Tumors * Primary bone neoplasms involving chest wallare uncommon.* The most common benign bone tumors arecartilaginous in origin-osteochondroma andchondroma.* The most common malignant bone tumors aremyeloma, chondrosarcoma, malignantlymphoma and Ewing’s sarcoma.
10A-1 Benign Rib Tumors A-1-1. Osteochondroma (1) It is the most common benign bonetumor( 50% of benign rib tumors ).(2) It arises from the metaphyseal region ofthe rib and present a bony protuberancewith a cartilaginous cap.(3) Rarely, it can involves the diaphragm andinduce hemothorax.
11A-1 Benign Rib Tumors A-1-2. Chondroma (1) It occurs anteriorly at costochondraljunction.(2) It presents thinning of the cortexradiographically.(3) Differentiation of chondroma andchondrosarcoma is difficult.(4) It should be treated as malignancy.
12A-1 Benign Rib Tumors A-1-3. Fibrous dysplasia (1) It is a cystic, nonneoplastic lesion withfibrous replacement of medullay cavityof the rib.(2) Albright’s syndrome( multiple bonecysts, skin pigmentation and precocioussex maturity in girls ) should be suspected ifmultiple lesions occurs.
13A-1 Benign Rib Tumors A-1-3 Fibrous dysplasia (3) Treatment should be conservative.(4) Many lesions stop growing at puberty.(5) Resection is indicated if pain andenlarging lesions occurs.
14A-1 Benign Rib Tumors A-1-4. Histiocytosis X (1) It is not a neoplasm. (2) It is a part of the spectrum of diseaseinvolving the reticuloendothelial system, including eosinophilic granuloma,Letterer-Siwe disease and Hand-Schuller-Christian disease.(3) Histiocytosis X occurs in patientsyounger than 50 years.
15A-1 Benign Rib Tumors A-1- 4. Histiocytosis X (4) Eosinophilic granuloma is limited onlybone involvement.(5) Bone lesions may occurs in all types ofhistiocytosis X and skull is the most common.(6) For patients of solitary eosinophilicgranuloma , excision can result in cure.(7) For patients of multiple eosinophilicgranulomas, radiation is helpful.
16A-2 Malignant Rib Tumors A-2-1 Myeloma(1) It is the most common malignant ribtumor.(2) Most myelomas involving chest wallare systemic myeloma.(3) Most myeloma occurs in people of 40-60years and rare in people< 30 years.(4) Punched-out lesion with cortical thinningpresents radiographically.
17A-2 Malignant Rib Tumors A-2-1 Myeloma(5) Pathologic fracture is common.(6) Local excision is for diagnosis.(7) Radiation is for a solitary lesion andboth radiation and chemotherapy formultiple lesion.(8) 5-year survival is 20 %.
18A-2 Malignant Rib Tumors A-2-2 Chondrosarcoma(1) It is almost a tumor of the anterior chestwall.(2) Most chondrosarcoma occurs in peopleof years and male.(3) All tumors from costal cartilages should beconsidered malignancy.(4) Pathological fracture is uncommon.(5) Chest wall chondrosarcoma grows slowly andmetastases occur late if it is left.
19A-2 Malignant Rib Tumors A-2-3. Ewing’s sarcoma(1) 2/3 patients is younger than 20 years.(2) An onion-skin appearance of the surfaceof the bone may be seen.(3) Pathological fracture is rare.(4) Radiation is the treatment of choice andadjuvant chemotherapy is also used.
20A-2 Malignant Rib Tumors A-2-4. Osteogenic sarcoma(1) It is more malignant and less commonthan chondrosarcoma.(2) It is more common in teenagers andyoung adults. The male is more commonthan the female.(3) Serum ALP is frequently elevated.
21A-2 Malignant Rib Tumors A-2-4 Osteogenic sarcoma(4) Pathologic fracture is rare.(5) The treatment is wide excision.(6) Radiation is not valuable andchemotherapy is controversial.(7) 5-year survival is 20%.
22A-2 Malignant Rib Tumors A-2-5 Radiation-Associated Malignant Tumors1. Those tumors are uncommon.2. Osteosarcoma and soft tissue sarcoma arethe most common.3. The initial radiation is most common forbreast cancer and lymphoma.4. The treatment is similar to tumors arisingde novo.
23A-3 Tumors of the manubrium , Sternum , Scapula and Clavicle 1. Primary tumors of the manubrium and the sternum constitute 15 % of the chest wall tumors, but nearly all are malignant.2. The sternum is a frequent site metastasis from thebreast, thyroid and kidney.3. The scapula is not a frequent site of metastasis.4. Primary tumors of the clavicle are uncommon, but 90 % are malignant.5. The clavicle is more a site of a metastasis than a primary tumor..
24SPECIFIC TUMORS B. Primary Soft Tissue Tumors B-1 Benign Soft Tumors * Predominant tumors are fibroma,lipoma, giant cell tumors, vasculartumors and neurogenic tumors.* Malignant degeneration is uncommon.* Local excision is the choice.
25B-1 Benign Soft Tissue Tumor B-1-1 Desmoid(1) 40 % of all demoids occur in the chest walland the shoulder.(2) Encapsulation of vessels and brachialplexus in arms and neck is common.(3) The tumor may extend into the pleuralcavity and displace mediastinal structure.
26B-1 Benign Soft Tissue Tumor B-1-1 Desmoid(4) Desmoid is most common in people of betweenpuberty and 40 years of age. Men and womenwas affected equally.(5) The tumor originates in muscleand fascia.(6) The tumor must be treated with wideexcision. Recurrence may occur ifinadequate excision.
27B-2 Malignant Soft Tissue Tumors B-2-1 Malignant Fibrous Histiocytoma(1) It is the most common chest wall tumorthat the thoracic surgeon was asked.(2) It is most common in people of 50-70years of age. 2/3 of patients are male.(3) The tumor was unresponsive to radiation andchemotherapy, so wide resection is the choice.(4) 5-year survival is 38 %.
28B-2 Malignant Soft Tissue Tumors B-2-2 Rhabdomyosarcoma(1) It is the most second common malignant softtissue tumor.(2) It is most common in children and young adults.(3) The tumor is neither pain nor tender,despite rapid growth.(4) Wide excision with postoperativechemotherapy and radiotherapy results in 5-yearsurvival of 70%.
29B-2 Malignant Soft Tissue Tumors B-2-3 Liposarcoma(1) It is most common in people of 40-60years of age.(2) Most patients are men.(3) Treatment is wide excision. 5-year survival is60 %.(4) Chemotherapy and radiotherapy havelittle to offer.
30B-2 Malignant Soft Tissue Tumors B-2-4 Neurofibrosarcoma(1) Chest wall neurofibrosarcoma occursalong the intercostal nerve.(2) It is most common in people of 20-50years of age. Most patients are male.(3) Half of patients are associated with vonRecklinghausen’s disease.(4) Treatment is wide excision.
31B-2 Malignant Soft Tissue Tumors B-2-5 Leimyosarcoma(1) It is most common in adults.(2) Most patients are female.(3) Most tumors present enlarging slowly butpainful.(4) Treatment is wide excision.