Chest Wall Tumor
 

General Consideration

  available statistical data concerning the exact incidence of thoracic wall tumor are incomplete and probably unimportant. Moreover accurate histological classification of the tumors is often confusing   

Brian Blades, 1949

 

Incidence

Primary 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.

 

Origin

They originates in bone, cartilage, soft tissue of chest wall.

Most arise in ribs (85%).

 

Clinical Manifestation

Most 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

  • Pulmonary infection : actinomycosis, nocardiosis
  • TB chondritis
  • Costochondral separation
  • Tietzes syndrome (non specific chondritis)

 

Investigations

Chest 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 Pts 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

  • Nonneoplastic disease
    • Cyst
    • Inflammation
  • Primary neoplasm of chest wall
    • Benign
    • Malignant
  • Metastatic neoplasm to chest wall
    • Sarcoma
    • carcinoma

 

Primary Chest Wall Tumor

  • Malignant
    • Myeloma
    • MFH
    • Chondrosarcoma
    • Rhabdomyosarcoma
    • Ewings sarcoma
    • Liposarcoma
    • Neurofibrosarcoma
    • Osteogenic sarcoma
    • Hemangiosarcoma
    • Leiomyosarcoma
    • Lymphoma
  • Benign
    • Osteochondroma
    • Chondroma
    • Desmoid tumor
    • Fibrous dysplasia
    • Lipoma
    • Fibroma
    • Neurilemoma

 

 

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).

 

Chondroma

Incidence : 15 - 20 % of benign chest wall tumors.

Age : 10 - 30 years old., Male = Female

It usually occurs at costochondral junction anteriorly.

Its usually symptomatic & slow growing tumor

Its divided into 2 type

  • Enchondroma : arises from medulla.
  • Periosteal chondroma : arises from periosteum.

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.

 

Osteochondroma

Osteochondroma 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.

Its associated with pain & localized tenderness.

Pathologic fracture can developed.

Chest x-rays : punched-out osteolytic lesion

Microscopic : consists of chronic granuloma.

Treatment

  • Solitary lesion : excision
  • Multiple lesions : radiotherapy

 

Osteoid Osteoma

Osteoid 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 Tumors

Tumors are a form of benign fibromatosis or low-grade fibrosarcoma.

They arise from deep fascia & connective tissue of muscles.

Theyre 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.

 

Lipoma

Lipoma 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

Primary tumor (T)

TX :   Minimum requirements to assess primary tumor cannot be met

T0 :   No demonstrable tumor

T1 :   Tumor 5 cm. or less in diameter

T2 :   Tumor more than 5 cm. in diameter

Tumor grade (G)

G1 : Well differentiated

G2 :  Moderately well differentiated

G3 :  Poorly differentiated

G4 :  Undifferentiated

Nodal involvement (N)

NX :  Minimum requirements to assess regional nodes cannot be met

N1 :   No lymph node metastasis

N2 :   Regional lymph node metastasis

Distant metastasis (M)

MX :  Minimum requirements to assess the presence of distant metastasis cannot be met

M0 :  No metastasis

M1 :  Distant metastasis present

 

Stage            Classification               5-yr Survival

I                  G1, T1-2, N0, M0                   >90%

II                 G2, T1-2, N0, M0                   70%

III                G3-4, T1-2, N0, M0               20-50%

IVA             Any G or T, N1, M1                <20%

IVB             Any G, T, or N, M1                 <5%

 

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 Sarcoma

Incidence : 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%.

 

Ewings Sarcoma

Ewings 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 Tumors

Hematologic 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

  • Readily available in operation room
  • Easy to adapt for any contour
  • Durable without erosion
  • Infection resistant
  • Permeable

 

Prosthetic materials ;

  • Prolene mesh
  • Gore-Tex
  • Methymethacrylate - impregnated mesh

 

Myocutaneous flaps

  • Latissemus dorsi muscle rotation flap
  • Pectoralis major muscle rotation flap
  • Rectus abdominis muscle rotation flap
  • Latissemus dorsi muscle rotation flap

 

Summarized By Thirayost Nimmanon

ػ ʶ ҹ

 

Ѻاش 15/06/2010

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