2012 EDUCATIONAL BOOK - American Society of Clinical Oncology

Surgical Options for Recurrent
Uterine Sarcomas
By Sharmilee B. Korets, MD, and John P. Curtin, MD
Overview: Leiomyosarcoma, the most frequent pure uterine
sarcoma, is an aggressive tumor with a tendency toward early
relapse. Survival for patients with recurrent disease is poor.
In contrast, endometrial stromal sarcoma, the second most
common uterine sarcoma, is a more indolent malignancy with
a tendency toward recurrence after a long latency period. The
relative infrequency of both diseases makes the study and
standardization of treatment for recurrent disease challenging.
Treatment of recurrence with cytotoxic chemotherapy,
radiation therapy, or hormone therapy produces modest to
poor response rates. Surgical resection is one treatment
modality offering the potential for cure and perhaps a more
durable response than is seen with medical management.
Although initial studies focused on pulmonary metastasec-
Case History: An otherwise healthy, middle-age woman was
diagnosed with leiomyosarcoma at the time of a myomectomy
for symptomatic fibroids in 2000. Six weeks later, she underwent
a total abdominal hysterectomy with bilateral salpingooophorectomy
for leiomyosarcoma and was diagnosed with
stage IB disease. Surgery was followed by three cycles of
adjuvant chemotherapy. She had an initial disease-free interval
of 7.5 years. However, in fall 2008, she was diagnosed
with a large left upper lobe thoracic metastasis and a
synchronous left acetabular lesion. She underwent a videoassisted
thoracoscopic left upper lobectomy and mediastinal
lymph node dissection for a 5 cm mass. All pulmonary
disease was completely resected and lymph nodes were negative
for disease. Three months later, she had intermittent
abdominal discomfort and vague bowel symptoms and was
noted to have a jejunal mass that was suspected to be
recurrent disease. The mass was completely removed by
small bowel resection with reanastomosis. At the time of
surgery, there was no evidence of residual intra-abdominal
disease. She had resection of the acetabular lesion in spring
2009. At that time, she was thought to be free of disease.
However within 1 month, she a calcaneal metastasis was
found, and after a course of radiation therapy, systemic
chemotherapy was initiated. She was treated with multiple
chemotherapy regimens over the course of the next 16 months.
She then chose to pursue palliative treatment and died
approximately 10 years after the initial diagnosis and 30
months after the initial surgical resection for recurrence.
This case illustrates several key points in our review of
surgical management of recurrent uterine sarcoma and provides
illustrative radiographic images of resectable metastases
in uterine sarcoma (Fig. 1).
MALIGNANT MESENCHYMAL tumors of the uterus
are rare, accounting for less than 3% of all uterine
malignancies. The annual incidence of uterine sarcoma
approaches two per 100,000 women. 1 Recent changes in
terminology and classification now exclude carcinosarcomas
from this group, as the biology and clinical behavior of those
tumors point toward an epithelial origin. This review focuses
on surgical management of recurrence in the most
common pure uterine sarcomas, listed in order of incidence:
362
tomy in recurrent soft tissue sarcoma, an increasingly large
body of data specifically evaluating outcomes after both
thoracic and extrathoracic metastasectomy in patients with
recurrent uterine sarcoma is now available. Though no prospective
trials have been conducted, retrospective comparisons
of chemotherapy or radiation therapy with surgery for
recurrent uterine sarcoma suggest improvement in diseasespecific
survival for the surgery group. Clearly defined factors
are associated with better prognosis after surgical resection
of recurrence, including a prolonged disease-free interval
and complete resection of disease. In properly selected
women, surgery and even repeated metastasectomy for
recurrent disease may improve survival and should be considered.
leiomyosarcoma, which accounts for the majority of uterine
sarcomas, followed by endometrial stromal sarcoma (ESS,
previously called low-grade endometrial stromal sarcoma),
undifferentiated endometrial sarcoma (previously called
high-grade endometrial stromal sarcoma), and adenosarcoma.
Even early-stage uterine sarcomas demonstrate aggressive
clinical behavior and confer a poor prognosis. Leiomyosarcomas
have a propensity toward hematogenous spread
and early recurrence. Five-year survival rates range from
30% to 48%, and relapse rates approach 60%, with 42% of
relapsed disease occurring outside the pelvis. 2 Most extrapelvic
relapses occur in the lung. As with other soft tissue
sarcomas, leiomyosarcomas are relatively chemoresistant, 3
making treatment of recurrent disease challenging. In contrast,
ESS is more indolent, with a tendency toward local or
distant relapse after a long latency period. Five-year survival
rates are between 80% and 100%, 1 the median time to
recurrence in women with stage I disease is 65 months, and
the rate of relapse ranges from 36% to 56%. 4 Recurrences
are primarily pelvic, intra-abdominal, or pulmonary;
however, intravascular, cardiac, and central nervous
system metastases have also been reported. Because of
their slow growth, repeated resection may be warranted,
and even secondary and tertiary cytoreductions likely improve
prognosis. 4 Adenosarcomas are similarly indolent,
with excellent survival when disease is at an early stage at
the time of diagnosis and there is a long latency period
before relapse.
The relative rarity of uterine sarcomas makes clinical
investigation difficult and prospective randomized trials
nearly impossible, especially for evaluating the management
of recurrent disease. Most studies are retrospective,
and various primary disease sites and histologic subtypes
From the Division of Gynecologic Oncology, New York University School of Medicine, New
York, NY.
Authors’ disclosures of potential conflicts of interest are found at the end of this article.
Address reprint requests to John P. Curtin, MD, NYU Clinical Cancer Center, 160 E. 34th
St., 4th Floor, New York, NY 10016; email: john.curtin@med.nyu.edu.
© 2012 by American Society of Clinical Oncology.
1092-9118/10/1-10