and 8 have no evidence of disease (NED), with a median
follow-up since initial diagnosis of 37 months (range 4-290).
There have been no local recurrences among the IRS group I
or group II patients but 4 developed distant metastases.
Of the patients with recognized metastatic disease at
diagnosis (IRS group IV), 5 of 7 initially underwent
incisional or core biopsy, but 2 had excisional biopsy. In
this group of 7 patients, 2 are dead of disease (DOD), 4 are
alive with disease (AWD), and one has NED after multiple
metastasectomies, with a median follow-up since diagnosis
for these 7 patients of 21 months (range 6-239).
2.4. Surgery for metastases
Thirty-one operations for metastases were carried out,
distributed among almost half (9/20 or 45%) of the patients.
The median number of metastasectomies performed for this
group was 3, with a range of 1 to 8. Operations included
thoracoscopy (n = 2), thoracotomy (n = 17), laparotomy
(n = 5), craniotomy (n = 6), and curettage of the acetabulum
(n = 1). Four patients (#1, #7, #10, and #18) had
metastasectomies 10 years or later after their initial diagnosis.
Of the 9 patients who had metastasectomies, 2 are DOD, 4 are
alive with disease, and 3 have NED, with a median follow-up
since diagnosis of 135 months (range 21-354).
2.5. Use of chemotherapy and biologic therapy
Chemotherapy and/or biologic therapy was given to 11
(55%) of 20 patients. Of these 11, 6 were treated owing to the
presence of IRS group IV disease at diagnosis, and 4 were
given chemotherapy after the onset of metastases later in their
course. However, 1 patient (#5), who was IRS group I,
received multiagent chemotherapy after a wide local excision
despite there being no residual local or distant disease.
Of the 11 receiving chemotherapy, 3 are DOD, 5 are alive
with disease, and 3 have NED, with a median follow-up from
diagnosis of 135 months (range 6-354). As there is no
standard regimen of chemotherapy for alveolar soft part
sarcoma, a vast assortment of chemotherapeutic and biologic
agents were administered to patients in this series, including
antimetabolites, alkylating agents, mitotic inhibitors, anthra-
cyclines, antimicrotubule agents, antineoplastics, antifolates,
COX-2 inhibitors, interleukin-2, interferon-a, and TNP-470.
No complete or partial responses were seen with any agent.
2.6. Use of radiation therapy
External beam radiation (XRT) was used on the primary
tumor site in 5 (25%) of 20 patients and to metastatic sites in
4 (20%) of 20 patients. Of the 5 receiving primary site XRT,
3 have died of disease, 1 is alive with metastatic disease,
and 1 has NED. One patient, who received whole-brain
radiation, had XRT without resection. External beam
radiation was combined with surgical metastasectomy in
2 patients (1 now NED, 1 DOD) and with metastasectomy
plus brachytherapy in another patient (now AWD 354 months
after diagnosis). Brachytherapy, without XRT, was used after
resection in 1 patient now NED 148 months after diagnosis.
Although the results particularly with brachytherapy are
intriguing, given the small numbers, no conclusion can be
drawn regarding the role of XRT, brachytherapy, or chemo-
therapy for alveolar soft part sarcoma.
Fig. 2
A, Kaplan-Meier curve showing overall follow-up for
patients with alveolar soft part sarcoma. B, Kaplan-Meier curve
showing progression-free survival in patients with alveolar soft part
sarcoma. The relatively long overall follow-up compared with the
short time to progression indicates that patients are living long
despite the progression of disease. C, Kaplan-Meier curve showing
progression-free survival for those with primary tumors of 5 cm or
smaller (upper line) vs those larger than 5 cm (lower line) ( P = .03).
Clinical presentation, treatment, and outcome of alveolar soft part sarcoma
191