Z. Q. Zhang et al. / Case Reports in Clinical Medicine 2 (2013) 366-369
368
Glycogen granules or nerve secretory granules can be
seen under an electron microscope [9,10].
From the molecular pathological view, PNET/EWS
have the same t(11; 22) (q24; q12), or less t(21; 22) (q22;
q12) or t(7; 22) (p22; q12). All these chromosomal rear-
rangements fuse the EWS gene on chromosome 22 to
FLI21 ERG or ETV1 gene members of the ETS tran-
scription factor family, resulting in EWS/FLI21, EWS/
ERG or EWS/ETV1 fusion genes, leading to tumori-
genesis. 95% of the tumors can be detected by RT-PCR
and FISH with high sensitivity and specificity [11].
Identification and diagnosis of this type of tumor pri-
marily relies on comparisons with other intra-abdominal
and retroperitoneal soft tissue tumors, including the loca-
tion, age, tumor morphological alterations, immunohis-
tochemical illustration, and genetic traits.
3.5. Treatment and Prognosis
PNET/EWS is a systemic disease and its therapy re-
quires comprehensive treatment, including surgery, ra-
diotherapy, chemotherapy and biological therapy. There
is no uniform regimen for intraperitoneal or retroperito-
neal PNET/EWS. Rud et al. [12] and Covelli et al. [13]
suggested that the surgery may be more important for
EWS outside bone than EWS in the bone and total resec-
tion yields better prognosis. Based on the literature,
complete surgical removal may be the most effective
means for cure; even for patients with local recurrence, if
possible, surgical treatment should be implemented. In
order to ensure complete resection, removal of some ad-
jacent organs, the most commonly kidney, adrenal, colon,
pancreas, and spleen, is often necessary. With the devel-
opment of vascular surgery, resection of involved vessels
and implantation of artificial blood vessels may be coap-
plied, though the long-term efficacy and benefits await
further studies to confirm.
As far as radiotherapy and chemotherapy, there are
different views. Kinsella et al. [14] believed that high
doses of local radiotherapy and VAC procedures are
adequate for EWS outside bone and total surgical resec-
tion may be unnecessary. Local adjuvant radiotherapy
after complete resection cannot significantly reduce the
local recurrence because of high doses and side effects
that require stringent protective measures [12,15]. Ven-
kitaraman et al. [16] proposed that intensive chemother-
apy is necessary after complete resection of the tumor or
radiotherapy with doses >50 Gy, for patients with PNET/
EWS sarcoma outside bone in order to obtain good effect.
Of 14 PNET/EWS patients without metastases who un-
derwent combinational chemotherapy (5 with VAC pro-
cedures, 5 with VACA procedures, four with VAC/IE
procedures), 8 were cured, 2 partially cured, and 4 had
progressive deterioration [16].
Venkitaraman et al. [16] believed that high hemoglo-
bin, low lactate dehydrogenase, VAC/IE chemotherapy,
and complete response to chemotherapy are the factors
for raising tumor-free survival rate and overall survival
rate. Martin et al. [15] considered the initial perform-
ance of tumor (primary or recurrent) is an important pro-
gnostic factor, whereas tumor location, size, and age
make no statistical significance [15,16]. PNET/EWS pa-
tients without metastatic who received combination ther-
apy had 5-year survival rate of 60% and overall survival
rate of 30% [16]. Aggressive PNET/EWS causes distant
metastases at early stages, frequent local recurrence and
regional lymph nodes, lungs, liver, bone and bone mar-
row metastasis, and has poor prognosis with the median
survival time of about two years; the leading cause of
death is metastasis to the lung, bone, liver and other or-
gans.
4. CONCLUSION
The abdominal cavity and retroperitoneal PNET/EWS
are relatively rare, but they grow rapidly in larger size,
oppress surrounding organs and large vessels, and make
surgical resection difficult. It is often in high degree of
malignancy and already has widespread subclinical me-
tastasis at the time of going to a clinic. Moreover, it can
quickly transfer to everywhere throughout the body ren-
dering poor prognosis. Clinicians should be highly alert
to this condition. Imaging is the primary means of early
detection and early diagnosis. Complete surgical resec-
tion may be the most effective way of cure. Whenever
possible, surgery should be implemented even in patients
with local recurrence. In order to ensure complete re-
section, removal of any neighboring organs, most com-
monly kidney, adrenal, colon, pancreas, spleen, and so on,
may be required. With the development of vascular sur-
gery, resection of involved vessels and implantation of
artificial blood vessels may be conducted. The efficacy
of radiotherapy is still unclear. Future studies are war-
ranted to develop surely effective surgical treatment op-
tions and preoperative/postoperative adjuvant therapies.
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