CHAPTER 53

I Primary Neoplasms
Geoffrey M. Graeber
David R. Jones
Peter C. Pairolero

DEFINITION
Chest wall tumors encompass a kaleidoscopic panorama Stelzer P, Gay WA Jr: Tumors of the chest wall. Surg Clin North Am
of bone and soft tissue pathologic conditions. Included 60:779, 1980.
are primary and metastatic neoplasms of both the bony
skeleton and soft tissues and the primary neoplasms that CLINICAL FEATURES
invade the thorax from adjacent structures such as the
breast, lung, pleura, and mediastinum. Nearly all of these The mean age of presentation for a patient with a benign
neoplasms have at one time or another been irradiated, tumor of the chest wall is approximately 15 years
and it is fairly common for these patients to present with younger than for those with primary malignancies. The
postradiation necrotic ulceration. The thoracic surgeon is average patient age for benign tumors is 26 years old; for
asked to evaluate all of these patients. Most are seen to malignant tumors, the average age is 40 years old (Pass,
establish a diagnosis, some to treat for cure, and a few 1989). The male to female ratio is approximately 2:1
to manage necrotic, foul-smelling, chest wall malignant (Gordon et al, 1991; Graeber et al, 1982; Sabanathan et
ulcers. Primary chest wall neoplasms previously consid- al, 1985) for most tumors, with the exception of the
ered unresectable because of their size or extension into desmoid tumors, which have a 1:2 male to female pre-
adjacent structures are now being resected, and the chest ponderance (Gordon et al, 1991; McKinnon et al, 1989).
wall is reconstructed with little morbidity. In many pa- Chest wall tumors generally present as slowly enlarging
tients, surgical extirpation is often the only remaining masses. Most are initially asymptomatic, but with contin-
modality of therapy. This may be compromised by an ued growth, pain invariably occurs. At first, the pain is
incorrect diagnosis or an inability to reconstruct large generalized, and the patient is frequently treated for a
chest wall defects (Pairolero and Arnold, 1985). neuritis or musculoskeletal complaint. The incidence of
a chest wall mass is 70%, and pain is seen in 25% to 50%
of patients (Gordon et al, 1991; King et al, 1986). These
HISTORICAL NOTE
Because primary chest wall neoplasms are uncommon,
relatively few series have previously been reported. More-
over, most reports have included only patients with bone PRIMARY CHEST WALL NEOPLASMS
tumors (Groff and Adkins, 1967; Pascuzzi et al, 1957;
Stelzer and Gay, 1980). When bone neoplasms are com- Malignant
bined with primary soft tissue tumors, however, the soft Myeloma
tissues become a major source of chest wall neoplasms Malignant fibrous histiocytoma
and account for nearly one half of these tumors treated Chondrosarcoma
surgically (Graeber et al, 1982; King et al, 1986; Pairolero Rhabdomyosarcoma
and Arnold, 1985). The incidence of malignancy in these Ewing's sarcoma
tumors is variable and has been reported to range from Liposarcoma
50% to 80%. The higher malignancy rates are found Neurofibrosarcoma
in those series that include soft tissue tumors. When Osteosarcoma
combined, malignant fibrous histiocytoma (fibrosar- Hemangiosarcoma
coma), chondrosarcoma, and rhabdomyosarcoma are the Leiomyosarcoma
most frequent primary malignant neoplasms that the tho- Lymphoma
racic surgeon is asked to manage. Cartilaginous tumors Benign
(osteochondroma and chondroma) and desmoid tumors Osteochondroma
are the most common primary benign tumors. Chondroma
Desmoid
• HISTORICAL READINGS Lipoma
Fibroma
Groff DB, Adkins PC: Chest wall tumors. Ann Thorac Surg 4:260, 1967.
Pascuzzi CA, Dahlia DC, Clagett OT: Primary tumors of the ribs and Neurilemmoma
sternum. Surg Gynecol Obstet 104:390, 1957.

1417
1418 CHAPTER 53 • Primary Neoplasms
chest wall masses may be large and have been present for
long periods. The size of these tumors may rarely prevent
the patient from dressing and thus cause the patient to
seek therapy. Pain is more common in malignant tumors
but cannot be used to exclude the diagnosis of benignity
because one third of patients with benign chest wall
neoplasms have associated pain. Less common symptoms
include weight loss, fever, lymphadenopathy and brachial
plexus neuropathy.
DIAGNOSIS
The evaluation of patients with suspected chest wall
tumors should include a careful history and physical and
laboratory examination followed by conventional plain
and tomographic chest radiography. Old chest radio-
graphs are important to determine the growth rate. Com-
puted tomographic scans (CT) should be obtained to
delineate soft tissue, pleural, mediastinal, and pulmonary
involvement. The role of magnetic resonance imaging
(MRI) is not yet fully known, but preliminary evaluation
indicates still further enhancement of tissue pathologic
findings, which may make it the diagnostic modality of
choice in the future. A bone survey should be done
if metastases are suspected. Pulmonary function testing
should also be obtained.
Most primary chest wall neoplasms should be diag-
nosed by excisional biopsy. The reasons for excisional
biopsy include (1) removal of the entire mass,"(2) ade-
quate tissue sampling to establish the tumor's histologic
type, and (3) earlier administration of adjuvant therapy
if necessary. Cavanaugh and others (1986) recommended
a limited incisional biopsy to establish the diagnosis and
allow appropriate management plans to be made that are
based on the histologic type. In this series, 73% of the
lesions were benign, and no further surgery was per-
formed. In most series, however, the rate of malignancy
is 50% to 80%, and all require en bloc resection (Graeber
et al, 1982; King et al, 1986; Pairolero and Arnold, 1985).
Incisional biopsies may confuse the histologic diagnosis
because certain tumors, particularly chondrosarcomas,
have areas that histologically appear benign and other
areas in which frank malignancy is present (Graeber et al,
1982). Clinical decisions based on the wrong pathologic
diagnosis may be catastrophic. If an incisional biopsy is
performed, it should be made in such a way that the
definitive excision will not be compromised. No flaps or
extensive dissection should be used to prevent tumor cell
seeding. Needle biopsy of a lesion in a patient with a
known prior malignancy may be helpful. Ayala and Zor-
nosa (1983) demonstrated a 79% accuracy rate in the
diagnosis of primary bone tumors with percutaneous
needle biopsy. Most thoracic surgeons still prefer exci-
sional biopsy whenever possible.
Laboratory analysis and diagnostic studies should in-
clude liver function tests, alkaline phosphatase levels,
and a CT or MRI of the chest. Many of these tumors
metastasize to the lungs or involve the lung. Involvement
of the underlying lung does not preclude resection, but
it is associated with a worse prognosis, particularly in a
patient with high-grade sarcomas (King et al, 1986; Perry
et al, 1990). Ultrasound of chest wall tumors helps to
localize the tumor's relationship to the pleura and lung
parenchyma (Saito et al, 1988). If the tumor is confined
to the chest wall, its movement during respiration is
synchronous with the chest wall movement and not with
the lung parenchyma.
BENIGN TUMORS
Benign chest wall tumors require diagnostic studies simi-
lar to those for malignant tumors. Radiographic studies
may suggest the diagnosis of benignity, but histologic
evidence is necessary. The more common benign chest
wall tumors are discussed earlier. Less common benign
tumors include lipomas, osteomyelitis, mesenchymomas,
fibroxanthomas, hemangioendotheliomas, and some neu-
ral tumors.
Chondroma
Chondroma is the most common benign tumor of the
chest wall (Graeber et al, 1982; Ryan et al, 1989; Sabana-
than et al, 1985). They usually arise in the ribs near the
costochondral junction anteriorly. These patients present
with a mass that may be painful. Radiographically, the
lesion has a lobulated radiodense appearance, which fre-
quently displaces the bony cortex but does not penetrate
it (Fig. 53-1). Calcification may be diffuse or focal with
a stippled pattern. Histologically, there is mature hyaline
cartilage with foci of myxoid degeneration and calcifica-
tion. These lesions may grow to enormous size if un-
treated, and the therapy of choice is wide local excision
with 2-cm margins.
Fibrous Dysplasia
Fibrous dysplasia occurs in young adults and presents as
a painless, asymptomatic mass. It can arise anywhere on
the chest wall but occurs frequently in the posterior
ribs (Boyd, 1986). There is an association with trauma.
Radiographs show a central, fusiform, expanded mass
with thinning of the cortex and absence of calcification
(Sabanathan et al, 1985). Cortical bone erosion is not
uncommon. Histologically, there is a characteristic fish-
hook configuration of the trabeculae and lack of transfor-
mation of the coarse bony fibers to lamellar bone. This
suggests that fibrous dysplasia represents a maturation
defect. Excision of this lesion is curative.
Osteochondromas
Osteochondroma is a rare chest wall tumor that occurs
in the first or second decade of life. The radiographic
appearance is typical. The lesion, which is usually located
in the metaphysis, grows in a direction opposite to that
of the adjacent joint (Fig. 53-2). Infrequently, it has a
focal radiolucent area surrounded by osteosclerotic tissue
(Sabanathan et al, 1985). Grossly, the tumor consists of
mature bone trabeculae covered by a cartilaginous cap.
Most lesions are greater than 4 cm in diameter but may
become larger if untreated. Solitary osteochondromas are
benign and rarely may degenerate into malignancy. Multi-
 CHAPTER 53 • Primary Neoplasms 1419

FIGURE 53-1 • A computed

tomography image in a 42-year-old
man of a lobulated chondroma
arising near the costochondral
junction in the left fourth rib. The
therapy was resection with adequate
margins of excision.

pie osteochondromas have a higher incidence of malig-
nancy (Boyd, 1986). The therapy is wide local excision.
Eosinophilic Granuloma
Eosinophilic granuloma is a disease of the lymphoreticu-
lar system and not a true bone tumor. It may be solitary
or multifocal and is a unifying feature of the conditions
designated as histiocytosis X. Microscopically, there is an
abundance of Langerhans cells, giant cells, eosinophils,
and neutrophils. The peak incidence is between 5 and 15
years. It occurs in either the metaphysis or diaphysis of
the bone and has no malignant potential. These lesions
show osteolytic activity with adjacent osteosclerosis by
radiography. They are frequently confused with Ewing's
sarcoma or osteomyelitis. The therapy consists of either
resection or radiotherapy.
Desmoid Tumor
Desmoid tumors' occur most commonly in the third to
fourth decades of life and have a 2:1 female to male
predominance. The desmoid tumor is frequently difficult
to differentiate from the low-grade fibrosarcoma. Histo-
logically, the desmoid tumor contains sheets of fibroblasts
with well-differentiated abundant collagen, which lacks
encapsulation. The fibrosarcoma is usually well encapsu-
lated with a herringbone pattern and distinct mitoses
(McKinnon et al, 1989). Although one third of patients
with Gardner syndrome have desmoid tumors, only 2%
of patients with a desmoid tumor have Gardner syndrome
(Hayery and Scheinin, 1988). Desmoids have also been
reported to occur after trauma and to be associated with
estrogen-induced growth (Hayery and Scheinin, 1988;
McKinnon et al, 1989).
The clinical presentation is usually one of a dull,
aching mass, which may be fixed to the underlying tis-
sues but not to the skin (Graeber et al, 1985). The growth
of the mass is slow, and it does not metastasize. There are
no characteristic radiographic findings, and the diagnosis
should be made by excisional biopsy.
The therapy is wide local excision with margins of at
least 4 cm. Because desmoids may spread along fascial

FIGURE 53-2 • A representative computed

tomography scan of a solitary osteochondroma of
the posterior left scapula with displacement of the
third rib anteriorly. This produced dull posterior
chest wall pain in the patient.
1420 CHAPTER 53 • Primary Neoplasms
planes well beyond the primary, the wider resection mar-
gins are recommended. The recurrence rates for desmoid
tumors after excision range from 4% to as high as 50%
(McKinnon et al, 1989; Posner et al, 1989). The recur-
rence rates were directly related to resection margin status
in a study by McKinnon and coworkers (1989), and 45%
of patients with positive resection margins had recur-
rences. Only 4% with negative resection margins had
relapses. In patients with recurrence or gross residual
disease, radiotherapy is effective for local control (Leibel
et al, 1983; Sherman et al, 1990). The recommended
radiation doses of 50 to 60 Gy at 1:8 Gy/fraction prevent
the dose-related complications of radiotherapy (Sherman
et al, 1990). Chemotherapy plays no role in the therapy
of desmoid tumors. Because of the hormonal influence
on the desmoid's growth, tamoxifen has been reported to
decrease both the size and symptoms of these tumors
(Kinzbrunner et al, 1983).
The actual survival rates after wide local excision are
90% at 10 years, with a cause-specific survival rate of
100% (Graeber, 1989). Local recurrence remains the most
difficult challenge for this locally aggressive, benign tu-
mor.
MALIGNANT TUMORS
Malignant primary chest wall tumors can be cured if
certain surgical principles are followed. These tumors
may require extensive chest wall resection, but with the
aid of muscle flaps, chest wall reconstruction is success-
ful. Adjuvant therapy has become increasingly important
in the management of these tumors. The most common
primary malignant chest wall tumors were shown pre-
viously. Less common malignant tumors include neu-
rofibrosarcomas, malignant hemangioendotheliomas, and
leiomyosarcomas. The survival rates after therapy for
these tumors vary, but all histologic subtypes have some
long-term survivors (Fig. 53-3).
Chondrosarcoma
Chondrosarcoma is the most common primary chest wall
malignant tumor. It accounts for 50% of the malignant
neoplasms and 25% of all primary chest wall tumors
(Sabanathan et al, 1985). Eighty percent of these tumors
arise in the ribs, and 20% arise in the sternum (McAfee
et al, 1985).
Most of these tumors are solitary and have been pres-
ent an average of 18 months prior to presentation
(McAfee et al, 1985).
The conventional radiographic findings of a chondro-
sarcoma include a lobulated mass that arises in the med-
ullary portion of the rib or sternum, often with cortical
bone destruction. Calcification of the tumor is missed in
45% of chest radiographs but detected on chest CT scan.
A stippled calcification pattern is most common, but
rings and arcs of calcification may be present (Aoki et
al, 1989).
The diagnosis of these tumors should be made by an
excisional biopsy. The incisional biopsy has no place in
the diagnosis of these lesions because the histologic find-
ings vary from a poorly differentiated cellular appearance
to an extremely well-differentiated lesion that is indistin-
guishable from a benign chondroma (Fig. 53-4) (McAfee
et al, 1985; Sabanathan et al, 1985). The incidence of
chondrosarcomatous change in a solitary osteochon-
droma is reportedly 1% to 2% (Lichtenstin, 1977). The
natural history of these tumors is one of slow growth,
with frequent local recurrence and late metastasis. Chon-
drosarcomas have been related to previous chest wall
trauma in 12.5% of patients (McAfee et al, 1985).
The therapy of choice is wide local excision, including
several partial ribs above and below the lesion, with
surgical margins of at least 4 cm. If the lesion originates
in the sternum, a sternotomy with a corresponding resec-
tion of the costal arches bilaterally should be performed
(Arnold and Pairolero, 1978). Chest wall reconstruction
is frequently necessary.
Chondrosarcomas are extremely radioresistant and
chemoresistant. The prognostic factors include the tu-
mor's grade, diameter, and location. Tumors less than 6
cm and sternal tumors have a better patient prognosis.
The 10-year survival rates are 96% with wide local exci-
sion, 65% with local excision, and 14% with palliative
excision (McAfee et al, 1985). The local recurrence rate is
higher with local excision (50%) than wide local excision
FIGURE 53-3 • Survival rates in patients
with malignant neoplasms of the chest wall.
The total number of patients used to
generate this graph was 44 rather than 47
because 2 patients with fibrosarcomas were
lost to follow-up and an 84-year-old man
with a massive chondrosarcoma of the chest
wall was not considered a candidate for
resection.

FIGURE 53-4 • This photomicrograph presents a characteristic
field from a histologic section of a chondrosarcoma. The
histologic characteristics of this tumor include a cartilaginous
neoplasm, which has anaplastic cells with one or more bizarre,
hyperchromatic nuclei. This malignant neoplasm is also known
to have frequent variations in the grade of tumor cells present
throughout the presenting mass (H & E, x 200).
(17%). The possibility of late local recurrences of chon-
drosarcomas necessitates long-term follow-up of these
patients (Graeber et al, 1982).
Ewing's Sarcoma and Askin's Tumor,
Ewing's sarcoma is a small, round tumor with characteris-
tics of a primitive neuroectodermal tumor (PNET) and a
neural histogenesis, as indicated by experimental studies
(Cavazanna et al, 1987). It is the most common primary
chest wall malignancy in children and occurs in 8% to
22% of malignant chest wall lesions in adults (Graeber
et al, 1982; King et al, 1986; Sabanathan et al, 1985;
Shamberger et al, 1989). The differential diagnosis of
small, round cell malignant tumors includes neuroblasto-
mas, embryonal rhabdomyosarcomas, and lymphomas in
addition to Ewing's sarcomas (Stefanco et al, 1988). A
highly malignant alternative to Ewing's sarcoma is the
PNET, which was first described by Askin and colleagues
(1979). PNET is considered similar to Ewing's sarcoma
because of a common neuroectodermal differentiation
and a frequently seen translocation between the long
arms of chromosomes 11 and 22 [t(ll:22)(q24:ql2)]
(Turc-Cavel et al, 1983; Whang-Peng et al, 1984). PNET
and Ewing's sarcoma are grouped together because the
diagnosis and therapy of each are similar.
Most patients are between 5 and 30 years old and
present with progressive chest wall pain with or without
the presence of a mass. Some patients have a modest
leukocytosis and elevated erythrocyte sedimentation rate.
The typical radiographic picture is the characteristic on-
ion-peel appearance, which is produced by multiple lay-
ers of periosteal new bone formation (Sabanathan et al,
1985). Bony destruction, sclerosis of the widened cortex,
and a widened medulla are also common radiographic
findings. The tumor may involve several ribs but usually
is confined to one rib. The diagnosis may be made with
percutaneous needle biopsy (Ayala and Zornosa, 1983),
but as with other primary chest wall malignancies, an
CHAPTER 53 • Primary Neoplasms 1421
excisional biopsy is best (Fig. 53-5). The preoperative
workup should include standard chest imaging and bone
marrow aspiration.
These patients are best treated through a multimo-
dality approach. The entire marrow cavity of the rib is
considered to be at risk for malignancy; therefore, the
entire involved rib is removed along with a partial rib
resection above and below the lesion (Shamberger et al,
1989). Postoperative external beam irradiation to the
tumor bed provides excellent local control. If complete
surgical resection and irradiation are performed, local
control rates of 93% have been reported (Thomas et
al, 1983).
Chemotherapy is used to control distant disease and
has been shown to decrease the incidence of distant
metastases and improve survival rates (Hayes et al, 1983;
Thomas et al, 1983). Doxorubicin, dactinomycin, cyclo-
phosphamide, and vincristine are the four drugs used in
combination most frequently. Failure to include doxoru-
bicin in this combination has detrimental results (Perez
et al, 1981). Preoperative chemotherapy has been re-
ported to facilitate subsequent local therapy, but its use
is not well established (Brown et al, 1987; Shamberger et
al, 1989). The survival rate was improved with multi-
modality therapy to 52% at 5 years in one study (Hayery
and Scheinin, 1988). Patients with distant metastasis
rarely survive 5 years.
The complications of extensive chest wall resections
in children with Ewing's sarcoma include scoliosis and
restrictive pulmonary disease (Grosfeld et al, 1988; Ma-
langoni et al, 1980). Harrington rod fusion may be neces-
sary for severe scoliosis. The restrictive pulmonary func-
tion usually does not result in any long-term respiratory
difficulties.
Osteosarcoma
Osteosarcomas occur between the ages of 10 and 25 years
and again after age 40 years in association with several
other disease processes. They frequently present as a
FIGURE 53-5 • This photomicrograph shows a characteristic
sample taken from a Ewing sarcoma. The histologic
characteristics of this tumor include closely packed, small,
round cells, which are infiltrating muscle fibers (H & E, x 200).
1422 CHAPTER 53 • Primary Neoplasms
painful mass with a duration of symptoms prior to pre-
sentation that lasts from weeks to months. Most osteosar-
comas arise de novo and are located in the metaphysial
portion of the long bones, such as the femur, the tibia,
and the humerus. They do, however, account for a small
but significant number of rib-based malignancies (Fig.
53-6) (Graeber et al, 1982). There is an association
between the development of osteosarcomas and previous
irradiation, Paget's disease, and chemotherapy (Huvos,
1986; Souba et al, 1986; Tucker et al, 1987). The latency
period for the development of osteosarcoma after irradia-
tion is approximately 10 years (Huvos et al, 1985).
The preoperative evaluation of a patient considered to
have an osteosarcoma should include an excisional bi-
opsy to confirm the diagnosis. An elevated serum alkaline
phosphatase level may be present by laboratory analysis,
but this is nonspecific. One study showed that tumors
associated with a serum elevation of this enzyme had
increased metastatic rates (Raymond et al, 1987). Radio-
graphically, the classic "sunburst" pattern of new perios-
teal bone formation is frequently seen (Boyd, 1986; Pass,
1989). Triangular elevation of the periosteum secondary
to reactive new bone formation may be seen radiographi-
cally and is known as Codman's triangle sign. Histologi-
cally, we see eosinophilic staining and a glassy appearance
with irregular contours of the osteoid. Interspersed with
the osteoblastic cells are foci of fibroblastic and chon-
droblastic cells, which help divide osteosarcomas into
those three subtypes (Fig. 53-7) (Rosai, 1989).'
The therapy for osteosarcoma of the chest is preopera-
tive chemotherapy, which usually consists of a combina-
tion of doxorubicin, high-dose methotrexate, and cis-
platin (Winkler et al, 1984). This is done to shrink the
tumor prior to resection and to evaluate the tumor's
response to chemotherapy. Tumors with a significant
amount of tumor necrosis postchemotherapy are associ-
ated with better patient survival rates (Raymond et al,
1987). Preoperative intra-arterial chemotherapy with cis-
platin has produced significant disease-free survival rates
in patients who have a complete or partial response
(laffe et al, 1989). Radiotherapy is usually ineffective for
osteosarcomas.
The prognostic factors include the response to preop-
erative chemotherapy, an association with Paget's disease
(worse prognosis), and unifocal osteosarcoma. The addi-
FIGURE 53-6 • Osteosarcoma. A computed tomography scan
of a right third rib-based osteosarcoma with destruction of the
bone is shown here. The diagnosis was confirmed by an
excisional biopsy.
FIGURE 53-7 • This photomicrograph shows a characteristic
section taken from an osteosarcoma. The histologic
characteristics include anaplastic osteoblasts in an osteoid
matrix with atypical calcification (H & E, x 200).
tion of multidrug chemotherapy to the therapy of osteo-
sarcoma has increased 5-year disease-free survival rates
to greater than 50% (Lane et al, 1986).
Plasmacytoma
Solitary plasmacytomas that arise in bone account for
10% to 30% of primary chest wall malignancies (Graeber
et al, 1982; Pass, 1989). They are more common in male
patients and usually occur later in life, with a mean age
of 60 years (Graeber et al, 1982). The most common
chest wall location is the ribs, followed by the clavicle
and sternum. Soft tissue invasion from bone lesions may
occur. The radiographic appearance of the plasmacytoma
demonstrates an osteolytic process with several paracostal
opacities frequently present (Galluccio et al, 1989).
Confirmation that the plasmacytoma is localized to
the chest wall requires several studies. The patient should
undergo a bone marrow aspiration, skeletal radiographs,
and immunoelectrophoretic examination of the serum
and urine. A patient with a solitary plasmacytoma usually
has a normal calcium level and is not anemic. Evidence
of monoclonality of one of the immunoglobulins with
normal levels of the other circulating immunoglobulins
strongly suggests that the plasmacytoma is solitary. Serum
[^-microglobulin levels are usually normal in the solitary
plasmacytoma. Most bone lesions show a predominance
of immunoglobulin reactivity; upper respiratory tract le-
sions are predominantly immunoglobulin (Rosai, 1989).
The diagnosis of a solitary plasmacytoma should be made
only if all studies for disseminated disease have negative
findings.
Microscopically, plasmacytomas are composed of
sheets of plasma cells and are often hypervascular. The
nucleoli are prominent and have a characteristic pinwheel
appearance. Amyloid may be present in 25% of the le-
sions (Fig. 53-8) (Meis et al, 1987).
The role of surgery is to establish the diagnosis by
excisional biopsy. High-dose radiotherapy (5000 to 6000
cGy) has been shown to be successful for the local con-

FIGURE 53-8 • This is a representative photomicrograph
taken from a plasmacytoma. The characteristic features of this
neoplasm include a large field of well-differentiated plasma
cells that have eccentric nuclei that are surrounded by an
adjacent "halo" (H & E, x 200).
trol of solitary plasmacytomas (Mill and Griffith, 1980).
If the lesion is refractory to radiotherapy, then a more
extensive surgical excision can be done. Systematic che-
motherapy should only be given for evidence of disease
progression (Pass, 1989). Local recurrence is uncommon
for plasmacytomas. Spontaneous regression, of a chest
wall plasmacytoma has been reported, but this is rare
(Arunabh et al, 1988). After they are treated for a solitary
plasmacytoma, in approximately 35% to 55% of patients,
multiple myeloma develops, often 10 to 12 years after
the initial diagnosis (Meis et al, 1987). The presence of
nuclear immaturity with prominent nucleoli may have a
positive predictive value for the development of multiple
myeloma. The presence of a monoclonal protein in the
serum or urine has no predictive value for the develop-
ment of multiple myeloma (Rosai, 1989).
The 10-year survival rate for all bony locations of
solitary plasmacytoma is 68% (Bataille and Sany 1981).
A 25% to 37% 5-year survival rate after therapy for
primary chest wall plasmacytomas is expected (Gordon
et al, 1991; Graeber et al, 1982). Close follow-up of these
patients with frequent urine and serum electrophoretic
studies is necessary because the development of multiple
myeloma is fairly common.
Soft Tissue Sarcoma
Primary soft tissue sarcomas of the chest wall are uncom-
mon, and few centers have treated extensive series of
these tumors. The more common tumors include fibro-
sarcomas, liposarcomas, malignant fibrous histiocytomas,
rhabdomyosarcomas, dermatofibrosarcomas protuberans,
and angiosarcomas (Gordon et al, 1991; Graeber et al,
1987). These tumors compromise nearly 50% of all pri-
mary chest wall sarcomas (Ryan et al, 1989; Souba et al,
1986). The factors that may predispose the patient to the
development of soft tissue sarcomas include a history of
previous irradiation and syndromes such as von Reck-
linghausen's disease (neurofibromatosis), Gardner's syn-
CHAPTER 53 • Primary Neoplasms 1423
drome, and Werner's syndrome (Lynch et al, 1973;
Seyer, 1988).
Fibrosarcomas are large, painful masses that occur in
all age groups and often involve adjacent structures (Fig.
53-9) (Boyd, 1986). The radiographic findings show a
large irregular mass with frequent destruction of the
bone. The therapy includes wide local excision, with
tumor-free margins for low-grade sarcomas and the addi-
tion of chemotherapy for high-grade lesions (Gordon et
al, 1991). The difficulty in treating this neoplasm is
related to the significant incidence of local recurrence
and a propensity for the tumor to metastasize to the
lungs (Graeber et al, 1982). The 5-year survival rate is
53% to 86% after surgery, with or without adjuvant ther-
apy (Graeber et al, 1982).
Rhabdomyosarcoma is a rare primary chest wall tumor.
It accounts for 4% to 26% of primary malignant tumors.
These tumors arise from undifferentiated mesoderm and
are usually diagnosed after an incisional biopsy. Micro-
scopically, the tumor cells are small and spindle shaped
(Fig. 53-10). There are highly cellular regions that sur-
round blood vessels and alternate with abundant parvicel-
lular regions of muscle intercellular material. Immunocy-
tochemical analysis of the tissue has been very useful in
the diagnosis of rhabdomyosarcoma. The markers used
include myoglobin, desmin, myosin, actin, and antiskele-
tal muscle antibody from myasthenic patients. The ther-
apy is wide surgical excision and multidrug chemother-
apy. Radiotherapy is usually not effective in this tumor.
Malignant fibrous histiocytoma (MFH) is an uncom-
mon primary tumor to the chest wall. These tumors arise
from tissue histiocytes and have the potential to produce
collagen (Fig. 53-11) (Ozzello et al, 1963). CT scanning
aids in the operative planning and in the evaluation of
metastatic disease. The therapy is by wide local excision
for primary and locally recurrent tumors (Venn et al,
1986). MFH frequently has local recurrence and distant
metastasis. However, MFH is generally resistant to che-
motherapy. Adjuvant brachytherapy for soft tissue sarco-
FIGURE 53-9 • This photomicrograph shows a representative
sample of a fibrosarcoma. The histologic characteristics of this
tumor show malignant spindle cells, which are arranged in a
"herring bone" fashion. This alignment is characteristic of the
tumor (H & E, X 200).
1424 CHAPTER 53 • Primary Neoplasms
FIGURE 53-10 • This photomicrograph depicts some of the
histologic characteristics associated with a rhabdomyosarcoma.
Included in the field are large, elongated tumor cells that have
abundant eosinophilic cytoplasm, racket cells, and more
primitive rhabdomyoblasts (H & E, x 200).
mas has been shown to be beneficial if the patients are
undergoing resection for locoregional recurrence in a
previously irradiated site (Wallner et al, 1991). Postoper-
ative external beam radiation may be effective, particu-
larly if the resection margins are inadequate (Venn et al,
1986; Wallner et al, 1991).
Liposarcoma accounts for 15% of primary chest wall
soft tissue sarcomas (Gordon et al, 1991). Most (70%)
are low grade, and en bloc resection is the therapy of
choice. Local recurrence was found in 33% of patients in
the study by Greager and colleagues (1987), and was
treated by wide local excision alone. The presence of
local recurrence has no significant effect on the overall
survival rate (Gordon et al, 1991). Radiotherapy may be
effective in the control of local recurrence, but its role is
FIGURE 53-11 • This photomicrograph shows a
representative field from a malignant fibrous histiocytoma.
The characteristics of this neoplasm include a pleomorphic
tumor, which has many large, bizarre-shaped cells in a fibrous
stroma (H & E, x 200).
unclear. The 5-year survival rate is 83% (Graeber et
al, 1982).
The prognostic indicators for primary tissue sarcomas
include the tumor grade, presence of distant metastases,
and positive surgical resection margins (Gordon et al,
1991; Graeber et al, 1982; Perry et al, 1990). Tumors that
are low grade are associated with a 90% 5-year survival
rate; high-grade sarcomas have a 49% 5-year survival rate
(Souba et al, 1986). Positive resection margins negatively
affect both the disease-free survival and overall survival
rates in high-grade sarcomas, which emphasizes the im-
portance of negative margins (Perry et al, 1990). Radio-
therapy and chemotherapy have no prognostic value for
high-grade sarcomas in the adult patient. Because sarco-
mas tend to metastasize to the lungs, CT scans of the
chest should be performed. However, up to 50% of lung
parenchyma nodules discovered at surgery are not seen
on preoperative CT scans (J a bl° n s et al, 1989). The
presence of these synchronous pulmonary metastases is
associated with a worse prognosis (Perry et al, 1990).
SURGERY
Chest Wall Resection
Wide resection of primary malignant chest wall neoplasm
is essential to successful management. However, the ex-
tent of resection should not be compromised because of
an inability to close a large chest wall defect (Arnold
and Pairolero, 1979, 1984a; Pairolero and Arnold, 1985,
1986a, b). Opinions differ as to what constitutes wide
resection. In a recent report from the Mayo Clinic (King
et al, 1986), in which the effect of the extent of resection
on the long-term survival of patients with primary malig-
nant chest wall tumors was analyzed, 56% of patients
with a 4-cm or greater margin of resection remained free
from recurrent cancer at 5 years compared with only 29%
for patients with a 2-cm margin (Fig. 53-12). For many
surgeons, a resection margin of 2 cm would be considered
adequate. Although this margin may be adequate for
chest wall metastases, benign tumors and certain low-
grade malignant primary neoplasms, such as chondrosar-
coma, a 2-cm resection margin is inadequate for more
malignant neoplasms, such as osteogenic sarcoma and
malignant fibrous histiocytoma, which have the potential
to spread within the marrow cavity or along tissue planes,
such as the periosteum or parietal pleura. Consequently,
all primary malignant neoplasms initially diagnosed by
excisional biopsy should undergo further resection to
include at least a 4-cm margin of normal tissue on all
sides. High-grade malignancies should also have the en-
tire involved bone resected. For neoplasms of the rib
cage, this would include removal of the involved ribs,
the corresponding anterior costal arches if the tumor is
located anteriorly, and several partial ribs above and be-
low the neoplasm. For tumor of the sternum and manu-
brium, resection of the entire involved bone and corres-
ponding costal arches bilaterally is indicated. Any
attached structures, such as the lung, thymus, pericar-
dium, or chest wall muscles, should also be excised.

FIGURE 53-12 • Percentage of patients with
malignant chest wall tumors free from recurrent
tumors by extent of resection margin. Zero time on
the abscissa represents the day of the chest wall
resection. (From King RM, Pairolero PC, Trastek VH
et al: Primary wall tumors: Factors affecting
survival. Ann Thorac Surg 41:597, 1986.)
Chest Wall Reconstruction
The ability to close large chest wall defects is of prime
importance in the surgical therapy of chest wall neo-
plasms. The critical questions of whether the recon-
structed thorax will support respiration and protect the
underlying organs must be answered when we consider
that both the extent of resection and dependable recon-
struction are the mandatory ingredients for successful
therapy. These two important items are accomplished
most safely by the joint efforts of a thoracic and a plastic
surgeon (Arnold and Pairolero, 1984a). .
Reconstruction of chest wall defects involves a consid-
eration of many factors. The location and size of the
defect are of the utmost importance, but the medical
history and local conditions of the wound may drastically
alter a reconstructive choice. Primary closure remains the
best option available if possible. If full-thickness recon-
struction is required, which is usually the situation in
most primary neoplasms that have not been previously
treated, consideration must be given to both the struc-
tural stability of the thorax and the soft tissue coverage.
CONSIDERATION FOR RECONSTRUCTION
OF CHEST WALL DEFECTS
Location
Size
Depth
Partial thickness
Full thickness
Duration
Condition of local tissue
Irradiation
Infection
Residual tumor
Scarring
General condition of patient
Chemotherapy
Corticosteroid
Chronic infection
Lifestyle and type of work
Prognosis
CHAPTER 53 • Primary Neoplasms 1425
Skeletal Reconstruction
Reconstruction of the bony thorax is controversial. Dif-
ferences of opinion exist both as to which patients should
undergo reconstruction and what type of reconstruction
should be done. The decision not to reconstruct the
skeleton depends on the size and location of the defect
and whether the wound is infected. In general, infected
wounds should not be reconstructed simultaneously. Sim-
ilarly, defects less than 5 cm in greatest diameter any-
where on the thorax are usually not reconstructed. Like-
wise, high posterior defects less than 10 cm do not
require reconstruction because the overlying scapula pro-
vides support. However, if the defect is located near the
tip of the scapula, the defect, even if 5 cm or less in size,
should be closed to avoid impingement of the tip of
the scapula into the chest with movement of the arm.
Alternatively, the lower half of the scapula could be re-
sected. Finally, all larger defects located anywhere on the
chest should be reconstructed, and either autogenous
tissue or prosthetic material may be used.
Stabilization of the bony thorax is best accomplished
with prosthetic material, such as Prolene mesh (Ethicon,
Somerville, NJ) or 2-mm-thick Gore-Tex (polytetrafluro-
ethylene) soft tissue patch. When either of these materials
is placed under tension, the rigidity of the prosthesis is
improved in all directions. Currently, the Gore-Tex soft
tissue patch is superior because this material has the
added advantage of preventing movement of fluid and air
across the reconstructed chest wall. Marlex mesh (Daval,
Providence, RI) is used less frequently because when it
is placed under tension, this material is rigid in one
direction only. Reconstruction with rigid material, such as
methylmethacrylate-impregnated meshes is not necessary.
All large, full-thickness skeletal defects that result from
the resection of a neoplasm in both the sternum and
lateral chest wall should be reconstructed if the wound
is not contaminated. If the wound is contaminated from
previous radiation necrosis or nectrotic neoplasm, recon-
struction with prosthetic material is not advised because
the prosthesis may subsequently become infected, which
would result in obligatory removal. In this situation,
reconstruction with a musculocutaneous flap alone is
preferred. Similarly, resection of full-thickness bony tho-
rax in a patient who has been previously irradiated may
1426 CHAPTER 53 • Primary Neoplasms
not require skeletal reconstruction because the lung is
frequently adherent to the underlying parietal pleura and
pneumothorax may not occur with chest wall resection.
AUTOGENOUS TISSUE AVAILABLE
FOR CHEST WALL RECONSTRUCTION
Muscle
Latissimus dorsi
Pectoralis major
Rectus abdominis
Serratus anterior
External oblique
Trapezius
Omentum
Soft Tissue Reconstruction
Both muscle and omentum can be used to reconstruct
soft tissue chest wall defects. Muscle can be transposed
as muscle alone or as a musculocutaneous flap and is the
tissue of choice for closure of most full-thickness soft
tissue defects. All major chest wall muscles can be mobi-
lized on a single axis of rotation and transposed to an-
other location of the chest wall (McGraw and Arnold,
1986). If muscle is not available because of previous
radiation damage or an operation, free muscle flaps from
another location can be reimplanted with the expectation
of dependable long-term coverage. The omentum should
be reserved for partial-thickness reconstruction or as a
back-up procedure when muscle is either not available
or has failed in a previous full-thickness repair.
Latissimus Dorsi
The latissimus dorsi is the largest flat muscle in the
thorax. Its dominant thoracodorsal neurovascular leash
has an arc of rotation that allows coverage of the lateral
and central back and the anterolateral and central front
of the thorax (Bostwick et al, 1979; Campbell, 1950).
Its dependable, musculocutaneous vascular connections
permit it to be used also as a reliable musculocutaneous
flap. This muscle flap can cover huge chest wall defects
because virtually one half of the back can be elevated on
the blood supply of a single latissimus dorsi in the unin-
jured, nonirradiated patient. The donor site posteriorly
may require skin grafting when large musculocutaneous
flaps are elevated, but this represents a minor disadvan-
tage when we consider that large, robust flaps can be
transposed to either the anterior or the posterior chest
for full-thickness reconstruction. If the dominant blood
supply has been compromised by previous trauma or
surgery, the muscle can still be transposed dependably
on the branch of the adjacent serratus anterior (Fisher et
al, 1983).
Pectoralis Major
The pectoralis major is the second largest flat muscle on
the chest wall and in many respects is the mirror image
of the latissimus dorsi. Its dominant thoracoacromial
neurovascular leash, which enters posteriorly about mid-
clavicle, allows both elevation and rotation centrally of
the muscle as either a muscle or a musculocutaneous
flap (Arnold and Pairolero, 1979). The pectoralis major
flap is as reliable as the latissimus dorsi flap. It is of
major benefit in the reconstruction of anterior chest wall
defects, such as those that result from sternal tumor
excisions (Arnold and Pairolero, 1978; Pairolero and Ar-
nold, 1984, 1986). Generally, only the muscle without
the overlying soft tissue and skin is transposed, which
thus avoids the distortion created by a centralization of
the breast. Reconstruction in this manner is more sym-
metric and more aesthetically acceptable. If sternal skin
must be excised, the symmetry of the breast can still be
maintained because the transposed muscle readily accepts
and supports a skin graft. If necessary, the muscle may
also be transposed on its secondary blood supply through
the perforators from the internal mammary vessels.
Rectus Abdominis
Use of the rectus abdominis for chest wall reconstruction
is based on the internal mammary neurovascular leash.
The inferior epigastric vessels must be divided to allow
rotation to the chest wall. This muscle can be mobilized
and moved either as a muscle or as a musculocutaneous
flap, with the skin component oriented either horizon-
tally, vertically, or both. The vertical skin flap, however,
is more reliable because it is oriented along the long axis
of the muscle and thus maintains more musculocuta-
neous perforators. The donor site is usually closed pri-
marily.
The rectus abdominis is most useful in the reconstruc-
tion of lower sternal wounds. Either muscle can be used
because their arc of rotation is identical. Care must be
taken to choose the muscle that has patent and uninjured
internal mammary vessels. Angiographic demonstration
of vessel patency may be helpful to determine which
musculocutaneous unit would be the most reliable, par-
ticularly in previously irradiated patients or in patients
who had prior coronary artery bypass surgery.
Serratus Anterior
The serratus anterior is a smaller, flat muscle that is
located along the midaxillary chest wall. Its blood supply
comes from the serratus branch of the thoracodorsal
vessels and from the long thoracic artery and vein. Al-
though this muscle can be used alone, it is more com-
monly utilized in chest wall reconstruction as an ad-
junctive muscle in tandem with either the pectoralis
major or the latissimus dorsi to close larger defects. The
muscle also augments the skin-carrying ability of either
adjacent muscle (Arnold and Pairolero, 1984b). This
muscle is particularly useful as an intrathoracic muscle
flap (Arnold and Pairolero, 1984b; Pairolero et al, 1983).
External Oblique
The external oblique muscle may also be transposed as
either a muscle or a musculocutaneous flap, and it is
most useful in closing defects of the upper abdomen and
lower thorax. It reaches the inframammary fold without

tension but does not readily extend higher (Hodgkinson
and Arnold, 1980). The primary blood supply is from
the lower thoracic intercostal vessels. The advantage of
this muscle is that lower chest wall defects can be closed
without a distortion of the breast.
Trapezius
The trapezius muscle is useful to close defects at the base
of the neck or the thoracic outlet, but it is not a consis-
tently useful muscle as far as the remainder of chest wall
reconstruction is concerned. Its primary blood supply is
the dorsal scapular vessels.
Omentum
Omental transposition has been useful in the reconstruc-
tion of the partial-thickness chest wall defects that may
occur with certain soft tissue neoplasms or radiation
necrosis (Arnold and Pairolero, 1986; Jurkiewicz and
Arnold, 1977). In the latter situation, the skin and soft
tissue are debrided down to what remains of the thoracic
skeleton, which may be either bone or cartilage but
frequently is only irradiated ischemic scar. The trans-
posed omentum, with its excellent blood supply from the
gastroepiploic vessels, adheres to the irradiated wound
and readily accepts and supports an overlying skin graft.
Because the omentum has no structural stability on its
own, it is not useful in full-thickness defects because
additional support with fascia lata, bone, OT prosthetic
material would be necessary.
Omental transposition is exceedingly helpful in situa-
tions in which planned muscle flaps have been used but
have failed because of partial necrosis. Generally, this
results in only a soft tissue defect, and a pleural seal with
respiratory stability is not required, which thus allows a
most threatening situation to be salvaged.
Late Results
During the past 10 years, more than 60 chest wall resec-
tions for primary neoplasms were performed at the Mayo
Clinic by one team of surgeons (unpublished data).
Nearly two thirds of these neoplasms were malignant.
Malignant fibrous histiocytoma and chondrosarcoma
were the most common malignant neoplasms, and des-
moid tumor was the most common benign tumor. The
patients' ages ranged from 12 to 80 years (median, 43.5
years). An average of 3.9 ribs were resected. Total or
partial sternectomies were performed in 13 patients. Skel-
TABLE 5 3 - 1 • Estimates of Number of New Cases of
Primary Malignant Chest Wall Tumors
in the United States in 1993
All Sites Chest
Tumor (No.) Wall (No.)
Soft tissue sarcoma 6,000 360
Chondrosarcoma 400 60
Ewing's sarcoma 300 45
Solitary plasmacytoma 125 25
Osteosarcoma 600 18
Total 7,425 508
CHAPTER 53 • Primary Neoplasms 1427
etal defects were closed with prosthetic material in 2
patients and with autogenous ribs in 5. Fifty-four patients
underwent 68 muscle transpositions; these included 24
pectoralis major, 23 latissimus dorsi, 6 serratus anterior,
3 external oblique, 2 rectus abdominis, 2 trapezius, and
8 other. The omentum was transposed in eight patients.
The median hospitalization was 9 days. There were no
30-day operative deaths. The patients were generally ex-
tubated during the evening of the operation or on the
following morning. Two patients required tracheostomy.
Most other patients had only minor changes in pulmo-
nary function (Meadows et al, 1985).
The long-term survival of patients with primary chest
wall malignant neoplasms is dependent on the cell type
and the extent of chest wall resection. In the Mayo Clinic
series, the overall 5-year survival rate was 57% (King et
al, 1986). Wide resection for chondrosarcoma resulted in
a 5-year survival rate of 96% (McAfee et al, 1985) com-
pared with only 70% for patients who had local excision
(Fig. 53-13). The 5-year overall survival rate for patients
with either chondrosarcoma or rhabdomyosarcoma was
70% (King et al, 1986), in contrast to a rate of only 38%
for patients with malignant fibrous histiocytomas (Fig.
53-14). Recurrent neoplasm, however, was an ominous
sign; only 17% of patients in whom recurrence developed
survived 5 years.
SUMMARY
The key to successful therapy of primary chest wall
neoplasms remains early diagnosis and aggressive surgical
resection. This procedure can generally be performed in
one operation, with minimal respiratory insufficiency and
with low operation mortality rates. When combined with
current methods of reconstruction, potential cure is likely
for most patients with primary chest wall neoplasms.
COMMENTS AND CONTROVERSIES
As documented by Drs. Graeber, Jones, and Pairolero,
primary malignant tumors of the chest wall are relatively
uncommon. Because almost all primary malignant chest
wall neoplasms can be classified as either soft tissue
sarcomas or malignant neoplasms of bone or cartilage,
estimates can be made of the number of tumors expected
to be diagnosed in the United States in 2000. Approxi-
mately 500 new cases of primary malignant chest wall
tumors will be diagnosed yearly in the United States.
Because it is estimated that there will be 1,170,000 new
cases of cancer diagnosed in the United States yearly,
primary malignant tumors of the chest wall comprised
only 0.04% of all new cancers. Because primary malig-
nant tumors of the chest wall are relatively uncommon,
data to support therapy options are sparse, but nicely
outlined in this chapter.
There is only one area of disagreement, and that is the
classification by the authors that chest wall desmoid tu-
mors are benign. Many pathologists currently accept the
desmoid tumor as a low-grade fibrosarcoma and not a
benign disease (Brodsky et al, 1992; Posner et al, 1989).
M. E. B.
1428 CHAPTER 53 • Primary Neoplasms

FIGURE 53-13 • Survival of patients with chest

wall chondrosarcomas by extent of operation. Zero
time on the abscissa represents the day of chest
wall resection. (From McAfee MK, Pairolero PC,
Bergstrahl EJ et al: Chondrosarcoma of the chest
wall: Factors affecting survival. Ann Thorac Surg
40:535, 1985.)

FIGURE 53-14 • Survival for patients with

chondrosarcomas and rhabdomyosarcomas
compared with those with malignant
fibrous histiocytomas. Zero time on the
abscissa represents the day of chest wall
resection. (From King RM, Pairolero PC,
Trastek VH et al: Primary wall tumors:
Factors affecting survival. Ann Thorac Surg
41:597, 1986.)

• KEY REFERENCES
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These authors present the Armed Forces Institute of Pathology's
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Included are both soft tissue and bone neoplasms. The roles of
chemotherapy and radiotherapy for each type of malignant neo-
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King RM, Pairolero PC, Trastek VF et al: Primary chest wall tumors:
Factors affecting survival. Ann Thorac Surg 41:597, 1986.
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McAfee MK, Pairolero PC, Bergstralh EJ et al: Chondrosarcoma of the
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Pairolero PC, Arnold PG: Chest wall tumors: Experience with 100
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