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2014 ; 15
(11
): 1439-43
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Grade 4 unclassified renal cell carcinoma with sarcomatoid component expressing
S-100 protein A case report with peculiar diagnostic and therapeutic
implications
#MMPMID25482943
Miolo G
; Ash A
; Buonadonna A
; Lo Re G
; Torrisi E
; Cervo S
; Santeufemia DA
; Tuzi A
; Canzonieri V
Cancer Biol Ther
2014[]; 15
(11
): 1439-43
PMID25482943
show ga
Grade 4 unclassified renal cell carcinoma, with a sarcomatoid component (URCCSC)
is a rare high grade tumor presumptively derived from all histological subtypes
of renal cell carcinoma (RCC). Even though rare, URCCSC generates a great deal of
interest, as it is a particularly aggressive variant of RCC, that is poorly
responsive to chemo-immunotherapy. Whether it originates from a separate
sarcomatoid cell clone within the tumor or from true cell dedifferentiation from
RCC has yet to be established. The diagnosis of URCCSC is usually based on
morphological and immunohistochemical characteristics of the neoplastic cells
which show transitional epithelial/mesenchymal features. In fact, the frequent
loss of epithelial markers and gain of mesenchymal phenotypes, can result in
difficulties in interpreting diagnostic data. Consequently assigning the optimal
therapeutic treatments can be hindered due to this biological "complexity." Here
we present the clinicopathological records of a 51 year-old patient who underwent
an excision of a periureteral retroperitoneal mass, and whose first pathological
diagnosis was malignant peripheral nerve sheath tumor (MPNST). Eleven months
after surgery, a CT-scan revealed a local recurrence of the disease. Later on the
patient was admitted to our hospital and a systemic, sarcoma-oriented, treatment
was initiated. A partial remission was observed but only with a dacarbazine based
regimen administered as a third line therapy, after which a second surgery took
place. The removed tumor was diagnosed as URCCSC based on the peculiar
morphologic and immunohistochemical characteristics of the cells. Pathological
assessment of the first intervention was re-evaluated, resulting in a diagnosis
of URCCSC. This case-report therefore highlights the implications that an
erroneous pathologic diagnosis can have for the clinical management of this
disease. Furthermore, the unexpected response to a dacarbazine based regimen,
indicates that this drug should be included among the therapeutic options
available against this type of renal carcinoma.
|Antineoplastic Combined Chemotherapy Protocols/therapeutic use
[MESH]