2 Table 45-1 Classification of Chest Wall Tumors __________________________________________ Primary neoplasm of chest wall: malignant, benign Metastatic neoplasms to chest wall: sarcoma, carcinoma Adjacent neoplasms with local invasion: lung, breast, pleura Nonneoplastic disaease: cyst, inflammation __________________________________________
3 INCIDENCE 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.
5 BASIC PRINCUPLES Signs and Symptoms 1. Chest wall tumors grow slowly. 2. Most patients have no symptoms initially. 3. Pain may occurs in nearly all malignant tumors and 2/3 of benign tumors. 4. Fever, leukocytosis and eosinophilia may accompany some chest wall tumors
6 Diagnosis 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.
7 Treatment(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.
8 Treatment(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
9 SPECIFIC TUMORS A. Primary Bone Tumors * Primary bone neoplasms involving chest wall are uncommon. * The most common benign bone tumors are cartilaginous in origin-osteochondroma and chondroma. * The most common malignant bone tumors are myeloma, chondrosarcoma, malignant lymphoma and Ewing’s sarcoma.
10 A-1 Benign Rib Tumors A-1-1. Osteochondroma (1) It is the most common benign bone tumor( 50% of benign rib tumors ). (2) It arises from the metaphyseal region of the rib and present a bony protuberance with a cartilaginous cap. (3) Rarely, it can involves the diaphragm and induce hemothorax.
11 A-1 Benign Rib Tumors A-1-2. Chondroma (1) It occurs anteriorly at costochondral junction. (2) It presents thinning of the cortex radiographically. (3) Differentiation of chondroma and chondrosarcoma is difficult. (4) It should be treated as malignancy.
12 A-1 Benign Rib Tumors A-1-3. Fibrous dysplasia (1) It is a cystic, nonneoplastic lesion with fibrous replacement of medullay cavity of the rib. (2) Albright’s syndrome( multiple bone cysts, skin pigmentation and precocious sex maturity in girls ) should be suspected if multiple lesions occurs.
13 A-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 and enlarging lesions occurs.
14 A-1 Benign Rib Tumors A-1-4. Histiocytosis X (1) It is not a neoplasm. (2) It is a part of the spectrum of disease involving the reticuloendothelial system, including eosinophilic granuloma, Letterer-Siwe disease and Hand- Schuller-Christian disease. (3) Histiocytosis X occurs in patients younger than 50 years.
15 A-1 Benign Rib Tumors A-1- 4. Histiocytosis X (4) Eosinophilic granuloma is limited only bone involvement. (5) Bone lesions may occurs in all types of histiocytosis X and skull is the most common. (6) For patients of solitary eosinophilic granuloma, excision can result in cure. (7) For patients of multiple eosinophilic granulomas, radiation is helpful.
16 A-2 Malignant Rib Tumors A-2-1 Myeloma (1) It is the most common malignant rib tumor. (2) Most myelomas involving chest wall are systemic myeloma. (3) Most myeloma occurs in people of 40-60 years and rare in people< 30 years. (4) Punched-out lesion with cortical thinning presents radiographically.
17 A-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 and both radiation and chemotherapy for multiple lesion. (8) 5-year survival is 20 %.
18 A-2 Malignant Rib Tumors A-2-2 Chondrosarcoma (1) It is almost a tumor of the anterior chest wall. (2) Most chondrosarcoma occurs in people of 20-30 years and male. (3) All tumors from costal cartilages should be considered malignancy. (4) Pathological fracture is uncommon. (5) Chest wall chondrosarcoma grows slowly and metastases occur late if it is left.
19 A-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 surface of the bone may be seen. (3) Pathological fracture is rare. (4) Radiation is the treatment of choice and adjuvant chemotherapy is also used.
20 A-2 Malignant Rib Tumors A-2-4. Osteogenic sarcoma (1) It is more malignant and less common than chondrosarcoma. (2) It is more common in teenagers and young adults. The male is more common than the female. (3) Serum ALP is frequently elevated.
21 A-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 and chemotherapy is controversial. (7) 5-year survival is 20%.
22 A-2 Malignant Rib Tumors A-2-5 Radiation-Associated Malignant Tumors 1. Those tumors are uncommon. 2. Osteosarcoma and soft tissue sarcoma are the most common. 3. The initial radiation is most common for breast cancer and lymphoma. 4. The treatment is similar to tumors arising de novo.
23 A-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 the breast, 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..
24 SPECIFIC TUMORS B. Primary Soft Tissue Tumors B-1 Benign Soft Tumors * Predominant tumors are fibroma, lipoma, giant cell tumors, vascular tumors and neurogenic tumors. * Malignant degeneration is uncommon. * Local excision is the choice.
25 B-1 Benign Soft Tissue Tumor B-1-1 Desmoid (1) 40 % of all demoids occur in the chest wall and the shoulder. (2) Encapsulation of vessels and brachial plexus in arms and neck is common. (3) The tumor may extend into the pleural cavity and displace mediastinal structure.
26 B-1 Benign Soft Tissue Tumor B-1-1 Desmoid (4) Desmoid is most common in people of between puberty and 40 years of age. Men and women was affected equally. (5) The tumor originates in muscle and fascia. (6) The tumor must be treated with wide excision. Recurrence may occur if inadequate excision.
27 B-2 Malignant Soft Tissue Tumors B-2-1 Malignant Fibrous Histiocytoma (1) It is the most common chest wall tumor that the thoracic surgeon was asked. (2) It is most common in people of 50-70 years of age. 2/3 of patients are male. (3) The tumor was unresponsive to radiation and chemotherapy, so wide resection is the choice. (4) 5-year survival is 38 %.
28 B-2 Malignant Soft Tissue Tumors B-2-2 Rhabdomyosarcoma (1) It is the most second common malignant soft tissue 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 postoperative chemotherapy and radiotherapy results in 5-year survival of 70%.
29 B-2 Malignant Soft Tissue Tumors B-2-3 Liposarcoma (1) It is most common in people of 40-60 years of age. (2) Most patients are men. (3) Treatment is wide excision. 5-year survival is 60 %. (4) Chemotherapy and radiotherapy have little to offer.
30 B-2 Malignant Soft Tissue Tumors B-2-4 Neurofibrosarcoma (1) Chest wall neurofibrosarcoma occurs along the intercostal nerve. (2) It is most common in people of 20-50 years of age. Most patients are male. (3) Half of patients are associated with von Recklinghausen’s disease. (4) Treatment is wide excision.
31 B-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 but painful. (4) Treatment is wide excision.