The following is an excerpt from The Moss Report on a rare type of
cancer called "epithelioid sarcoma." This is presented as a sample
of the kind of reports we prepare, even on very rare tumor types. It is
not to be taken as a guide to treatment of this or other kinds of cancer.
There are Moss Reports on virtually every type of cancer in existence,
including those which have metastasized to various sites. These reports
are all researched, written and kept up-to-date by Ralph W. Moss, Ph.D.
himself. Further information on these reports is available by calling coordinator
Anne Beattie at 718-636-4433.
You have asked for a report on epithelioid sarcoma. I will discuss
this tumor, then go on to a discussion of soft tissue sarcomas in general,
and finally talk about alternative and complementary treatments.
As stated, this is a very rare tumor, a histological subtype of sarcoma,
which occurs mainly in young adults (20 to 40 years of age) or even adolescents.
The diagnosis is often delayed because of the unusual nature of the tumor.
(Many doctors have never seen a case.) Sometimes under the microscope it
looks like a non-malignant lymphedema (swelling) (Skeletal Radiol 1997;26:606).
HISTORY: It is sometimes said that this tumor was first described
in 1970 (Hautarzt 1990;41:562). However, surgeons at M.D. Anderson cancer
center in Houston have been treating it since at least 1961 (Arch Surg 1991;126:1485).
In fact, in 1874 Prof. Zahn described a "sarcoma epithelioides,"
which from the description sounds very similar to the present disease.
METASTASES: Unlike most sarcomas, lymph node metastases are common
in epithelioid sarcoma. The lung is the most common site of distant metastases.
Bone and brain metastases have also been recorded. In a 1978 survey, 5 out
of 22 patients had vascular invasion from this tumor.
RARITY: To demonstrate how rare this tumor is, the world's largest
private cancer center, Memorial Sloan-Kettering (N.Y.), which has an international
reputation as a center for the treatment of rare malignancies, saw a total
of 16 such patients between 1982 to 1995, little more than one per year.
Eleven of these (69 percent) were male; five (31 percent) female. The average
age at diagnosis was 33 years. The length of time that symptoms were noted
before a diagnosis was finally made was 18 months. Similarly, in 11 years,
doctors at the St. Jude Children's Research Hospital, in Memphis (which
also specializes in the treatment of rare cancers) treated a total of 8
such cases.
INFORMATION: It is difficult to obtain detailed information on
this tumor. The National Cancer Institute's statement for physicians simply
alludes to this tumor's propensity to metastasize. The main orthodox textbook
on cancer (DeVita, et al.) contains just one small paragraph on it. Other
textbooks omit all mention of it.
Yet epithelioid sarcoma is certainly worthy of separate study, since
it presents some features that set it apart from the typical soft tissue
sarcoma.
APPEARANCE: An epithelioid sarcoma is usually first seen as a
small, firm nodule in the subcutaneous tissue (below the skin). Some doctors
say that these tumors are most common in the extremities, especially the
fingers, hands, and wrist. The tumor characteristically has multiple recurrences,
usually appearing along either a pathway of tendons or of fascial planes.
At Memorial Sloan-Kettering they found that tumors were first seen in the
trunk in 44 percent of cases, in the lower extremities in 31 percent, and
in the upper extremities in 25 percent.
At that center, the median follow-up time was 45 months. (Frequent follow-up
is definitely called for.) Sixty-nine percent of patients experienced at
least one local recurrence of the tumor. There were metastases to regional
lymph nodes during the course of the disease in 44 percent and to the lungs
in an equal percentage of cases.
SURVIVAL: The median survival with this disease was 88.8 months.
The five-year survival rate (conventionally called a "cure") was
66 percent. The main danger to patients was and is presented by the appearance
of lung metastases.
TREATMENT: In 1978, Memorial Sloan-Kettering doctors concluded
that "Our data suggest that cure may best be achieved by amputation
or by an exceptionally wide en bloc excision as early as possible, depending
upon the location and extent of the original tumor" (Cancer 1978;41:1472).
A 1985 paper concluded "Radical excision or amputation still appears
to be the initial treatment of choice, though adjunctive high-dose radiotherapy
to the excision site may prove to be of additional value" (Am J Surg
Pathol 1985;9:241).
However, as we study the world literature we note that treatment strategies
have been changing. One extremely important paper from M.D. Anderson concluded
that in the case of patients with epithelioid sarcomas of the extremity,
"Primary amputation offered no apparent overall survival benefit to
patients presenting with regional metastasis. The favorable outcome after
local resections for localized disease indicates that wide local excision
with margins that test negative on pathologic examination is preferable
to radical amputation in these patients" (Whitworth PW, et al.,
Extremity epithelioid sarcoma. Amputation vs local resection. Arch Surg
1991;126:1485). Certainly, it is worth discussing these issues with one's
surgeons and seeking a second opinion if you think that is at all warranted.
CHEMOTHERAPY: At the Mayo Clinic, Rochester, MN , E. Galanis and
colleagues reported on the use of chemotherapy to treat epithelioid sarcoma.
They state that eventually up to 50 percent of patients have systemic progression
that is not amenable to surgery. They reviewed the data on nine patients
who were treated with chemotherapy for this disease (5 males, 4 females).
The median age was 30 years, with a range of 16 to 77. Eight of the nine
had diffusely progressive disease after surgery, while one presented with
a widely metastasized disease. All had lung metastases and four had other
metastases as well.
All nine patients were given various doxorubicin (i.e. Adriamycin) based
combinations, either as first or as second or third-line treatments. Four
received a combination of ifosfamide + etoposide, three as first-line and
one as second-line treatments.
All four who received ifosfamide + etoposide progressed. However, results
appeared to be better in the Adriamycin group. There, the median duration
of regression (either complete or partial responses) was 28 months. Two
patients had excellent long-term survival, one alive at 3.5 years and the
other alive at over 19 years. Thus, the authors conclude that durable complete
responses are "feasible," although they caution that epithelioid
sarcomas can have "an indolent course" as is indicated by the
median survival in this series (ASCO Abstract #1817, 1997)
NCI LACKS INFORMATION: We have already alluded to the difficulty
of getting information on this tumor. The National Cancer Institute (NCI)
statement on soft tissue sarcomas contains no information specifically
about epithelioid sarcomas. However, about stage IVA soft tissue sarcomas
in general it states, "local control of the primary tumor probably
is best obtained by resection with negative margins, lymphadenectomy, and
postoperative external-beam radiation therapy." NCI cites an article
by RF Eilber, et al. entitled "Advances in the treatment of sarcomas
of the extremity: current status of limb salvage" (Cancer 1984;54:2695).
This cited article states the following:
"Nonamputative limb salvage is possible for many patients
with malignant skeletal or soft tissue sarcomas. Significant advances in
pathology, radiology, chemotherapy, radiation therapy, and surgical and
biomechanical techniques have contributed to a better understanding of
these diseases, and have provided the necessary techniques to achieve
local tumor control without amputation.
"Integration of diagnostic and treatment modalities has reduced
local recurrence and improved overall patient survival. Preoperative (neoadjuvant)
therapy permits assessment of the treatment effect and appears to significantly
improve selection of effective postoperative adjuvant therapy. As overall
patient survival has improved, efforts to increase local tumor control
and to evaluate long-term functional stability of the salvaged extremity
are now possible."
SECOND OPINIONS: Thus, if you are facing a treatment decision,
our advice would be to go for the most conservative option consistent
with good medical practice. In particular, if you are being told that
amputation is your only treatment option for an epithelioid sarcoma, you
should bring this report or the above-mentioned Whitworth and Eilber articles
to the attention of your doctor.
If you have any doubts at all about the advisability of a treatment,
you should seek out a second opinion with a doctor who believes in conservative
treatment options. [Some suggestions are given.]
EXPERTS: Rare tumors are best treated at large comprehensive cancer
centers, whose doctors are likely to have accumulated significant experience
in their management, especially using a multi-disciplinary approach. We
have mentioned Memorial Sloan-Kettering, M.D. Anderson and St. Jude's. Some
other such centers include....
(There follows information on international
experts, on soft tissue sarcomas in general, and then on alternative
and complementary treatments for such tumors.)
