Beruflich Dokumente
Kultur Dokumente
ABSTRACT
Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half
(53%) of these originate from the striated muscles and are called rhabdomyosarcomas (RMS) the remaining are nonrhabdomyosarcoma
soft tissue sarcomas (NRSTS). Almost two-thirds of RMS cases are diagnosed in children <6 years of age. They can arise at varied
locations like the head and neck region, genitourinary tract, extremities, trunk and retroperitoneum. Pathologically RMS is now classified
as superior, intermediate and poor outcome histologies. For stratification of treatment and also comparison of results the RMS are now
staged both by the clinical grouping and the TNM staging systems. The ultimate outcome depends on the site, extent of disease and
histology. Currently, approximately 70% of the patients survive for 5 years or more and are probably cured. This is credited to the use of
multi-modal, risk-adapted therapy, refinements in tumor grouping and better supportive care which has emerged out of cooperative
studies like Intergroup Rhabdomyosarcoma Study (IRS) and the International Society of Pediatric Oncology studies (SIOP). The treatment
involves chemotherapy, radiotherapy and organ/function preserving surgery. The gold standard chemotherapy is still vincristine, actinomycin
D and cyclophosphamide (VAC) regime with high doses of intensity bone marrow rescue with colony stimulating factors. The NRSTS
are rare and of heterogenous histologies and so it has been difficult to arrive at a treatment strategy for these. What is definitely
understood is that these are usually immature and poorly differentiated tumors that respond poorly to chemotherapy and so surgical
resection forms the mainstay of treatment with adjuvant radiotherapy and chemotherapy to prevent local recurrences. In all likelihood,
the molecular analysis of RMS will further refine current classification schemes and knowledge of genetic features of the tumors will
significantly improve the ability of investigators to identify patients at lower or higher risk of treatment failures, thus paving the way for
advances in risk-based therapy.
KEY WORDS: Children, nonrhabdomyosarcoma soft tissue sarcomas, rhabdomyosarcomas.
Superior prognosis
a) Botryoid
b) Spindle cell
II Intermediate prognosis
a) Embryonal
III Poor prognosis
a) Alveolar
b) Undifferentiated sarcomas
Percentage
59.4
18.5
0.5
0.5
13
3.6
Sites
Favorable
Unfavorable
Unfavorable
T
T1 or T2
T1 or T2
T1 or T2
Size
a or b
a
a
b
a or b
N
N0 or N1 or Nx
N0 or Nx
N1
N0 or N1 or Nx
N0 or N1
4
Either
T1 or T2
Definitions:
Site:
Favorable sites are orbit, head and neck (excluding parameningeal), or genitourinary (excluding bladder/prostate)
Unfavorable sites are bladder/prostate, parameningeal, extremities, trunk and all others
Tumor:
T1 = Tumor confined to anatomic site of origin
a) <5 cm in diameter
b) >5 cm in diameter
a) <5 cm in diameter
b) >5 cm in diameter
Regional nodes:
N0, Regional nodes not clinically involved
N1, Regional nodes clinically involved by tumor
Nx, Clinical status of regional nodes unknown (specially sites which preclude lymph node evaluation)
Metastasis:
M0, No distant metastases
M1, Metastases present
17
M
M0
M0
M0
M0
M1
19
Total
1062
375 (35)
109 (10)
111 (10)
155 (15)
167 (16)
110 (10)
202 (19)
208 (20)
Extremities
RMS involving the extremities comprise 19% of all RMS
[Table 4] and are characterized by a swelling in the
affected body part. They involve the lower extremity more
than the upper and distal limb involvement is more
common than proximal. Pain, tenderness and redness may
occur and almost 45% of these are of alveolar histology.[35]
Limb sparing, wide resection of the tumor is
recommended whenever feasible and without loss of
function since excision results in improved results. [36]
Amputation should be avoided. Since more than one
fourth of these patients have metastases to the regional
lymph nodes therefore IRS-IV recommends routine LN
sampling, even if clinically negative. Positive lymph nodes
warrant their inclusion in the RT portal. If surgical margins
are microscopically positive the re-excision should be done
prior to chemotherapy and RT. [37] Postoperatively all
patients get chemotherapy and radiotherapy. The survival
rate was 80% when lymph nodes were not involved as
compared to 46% when involved. [36] There is also an
increased rate of distant metastases when regional LN
was involved.[38]
Retroperitoneum
Retroperitoneal (RP) tumors, excluding the GU tract,
account for 11% of cases and can be either embryonal or
alveolar. Complete resection of these tumors is often
impossible for technical reasons and regional LN are often
involved and distant metastases present at the time of
diagnosis. Treatment includes chemotherapy and RT
according to the clinical grouping. Patients with RP-RMS
have the worst prognosis with a 5-year survival rate, of
nonmetastatic tumor, being only 50%.
Trunk
These sites include tumors of the chest wall, paraspinal
region and the abdominal wall in decreasing order of
frequency and constitute 10% of all cases of RMS. Most
of these are of alveolar histology and <30% are amenable
to complete resection at diagnosis.[32] Whenever excised
they have a tendency to recur locally. Treatment includes
chemotherapy and RT according to the clinical grouping.
NON-RMS SOFT
CHILDREN
TISSUE
SARCOMAS
IN
20
Rare
Angiosarcoma
Hemangiopericytoma
Clear cell sarcoma
Epitheloid cell sarcoma
Intra-abdominal desmoplastic
Malignant mesenchymoma
21
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22.
REFERENCES
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1.
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4.
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28.
29.
30.
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