2012 EDUCATIONAL BOOK - American Society of Clinical Oncology

SURGICAL OPTIONS FOR UTERINE SARCOMAS
have been combined in order to attain sufficient power.
Thus, optimal management strategies for recurrent disease
and definition of favorable clinicopathologic characteristics
for secondary cytoreduction or metastasectomy are not well
established. The purpose of this chapter is to better characterize
candidates for resection of recurrent disease and to
discuss factors associated with better prognosis.
Treatment Options for Recurrent Disease
Survival for patients with recurrent disease is poor. Systemic
therapy is often recommended; in ESS, hormone
therapy is frequently used, whereas in leiomyosarcoma,
cytotoxic chemotherapy may be the treatment of choice. In
uterine leiomyosarcoma, doxorubicin is the most active
single agent, with a response rate of 25%. 5 Combination
therapies containing doxorubicin and ifosfamide and gemcitabine
and docetaxel also have demonstrated activity in
metastatic or recurrent disease. 6,7
External-beam radiation therapy has been used in the
adjuvant setting for uterine sarcoma, and most studies have
demonstrated an improved local control rate without a
substantial effect on overall survival. Radiation therapy
may have a role in the palliative treatment of distant
metastases. 1 However, of all possible treatments for recurrent
disease, including cytotoxic chemotherapy, hormone
therapy, radiation therapy, or surgery, only surgery is
associated with a high cure rate and prolonged survival. 8
Surgical Resection of Metastatic Disease
In a single-institution retrospective cohort study of 33
patients with recurrent or metastatic uterine leiomyosarcoma,
survival was improved for patients who had surgical
resection of metastases compared with patients who
did not have surgery, with a median overall survival 45
compared with 31 months, respectively. 9 In a larger study
(128 patients), survival after secondary cytoreduction for
recurrent uterine leiomyosarcoma was compared with survival
after chemotherapy and/or radiation therapy for recur-
KEY POINTS
● Recurrent uterine sarcoma is an aggressive disease in
which nonsurgical treatment modalities offer limited
benefit.
● Due to the relative rarity of uterine sarcoma, few
prospective data on optimal management of recurrent
disease are available.
● Retrospective data demonstrate a survival benefit for
well-selected women in whom surgery is performed
for the management of recurrent disease.
● Although initial data suggested that surgery for metastastic
disease should be limited to pulmonary
metastasectomy only, additional studies have shown
that extrathoracic metastasectomy offers a similar
survival advantage.
● Prolonged disease-free interval and complete resection
of tumor correlate with improved outcome for
patients undergoing surgery for metastatic uterine
sarcoma.
rent disease. No distinction was made between abdominal,
pelvic, or thoracic procedures for resection. Disease-specific
survival as well as overall survival were substantially improved
for the surgery group compared with the medical
management group, with a mean overall survival of 2.0
compared with 1.1 years from the time of recurrence. 10
Due to the rarity of uterine sarcoma, much of the data
regarding surgical management of recurrence come from
studies in which heterogeneous histologic subtypes and
primary disease sites are pooled. Because the lung is a
preferred site of recurrence for soft tissue sarcoma, a large
body of literature focuses on indications for pulmonary metastasectomy
and factors affecting survival after resection.
Pulmonary Metastasectomy
Indications for pulmonary metastasectomy in recurrent
uterine sarcoma can be extrapolated from studies of the
procedure for patients with all types of soft tissue sarcomas.
In the surgical and thoracic oncology literature, indications
for pulmonary metastasectomy include medical suitability
for surgery, sufficient pulmonary reserve to tolerate loss of
lung capacity, control of disease at the primary site, no
evidence of extrapulmonary disease, and no better therapy
available. 11 Pulmonary metastasectomy with curative intent
is widely accepted for well-selected patients with sarcomatous
lung metastases. 3,12
Thoracic procedures for metastasectomy range from thoracoscopic
wedge resection to open pneumonectomy or bilobectomy
via median sternotomy. Wedge resection is
performed in most women undergoing surgery for sarcomatous
lung metastases. 11,13,14 In a series from Brigham and
Women’s hospital, approximately 75% of patients who had
pulmonary metastasectomy for recurrent leiomyosarcoma
were treated with wedge resection. In that study, it was also
demonstrated that disease-specific and overall survival were
similar for patients who underwent video-assisted thoracic
surgery (VATS) compared with thoracotomy or sternotomy.
Because recurrent lung metastases will develop in many
women, procedures that preserve the ability to tolerate
repeated resections, such as VATS and wedge resection, are
preferred. 13
In multiple series, 34 to 48% of women who underwent
initial pulmonary metastasectomy developed recurrent lung
metastasis and had repeat resection. 13-15 Sixteen to thirtyseven
percent of those patients had a tertiary resection. 14,15
Repeated metastasectomies correlate with improved survival,
likely because tumor biology is more favorable in
patients who survive long enough for more than one metastasectomy.
16
Extrathoracic Metastasectomy
More recently, outcomes after extrathoracic metastasectomy
have been evaluated in studies of recurrent sarcoma.
13,16,17 For patients undergoing resection of recurrent
uterine leiomyosarcoma, survival associated with thoracic
metastasectomy is similar to that associated with nonthoracic
procedures for recurrent disease, with a median
disease-specific survival of nearly 4 years from the time of
first metastasectomy. 17 A long-term survival benefit may be
conferred by metastasectomy for patients with extrapulmonary
disease in the case of complete resection. These data
suggest that the traditional criteria for pulmonary metasta-
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