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2018 ; 10
(ä): 2036361318787626
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A nonrandom association of sarcoidosis in patients with gastrointestinal stromal
tumor and other sarcomas
#MMPMID30038741
Espejo AP
; Ramdial JL
; Wilky BA
; Kerr DA
; Trent JC
Rare Tumors
2018[]; 10
(ä): 2036361318787626
PMID30038741
show ga
In patients with sarcoma, concomitant malignancy is found in 1.2% -2.5% of cases.
Previous studies have demonstrated conflicting results in terms of positive or
negative effects on cancer prognosis with comorbid sarcoidosis. Additionally,
there are no data determining whether an association between sarcoidosis and
sarcomas exists. Finding an association between the two entities could prevent
inadvertent upstaging of a primary sarcoma based on pulmonary nodularity mistaken
for metastatic disease. Here, we will describe eight sarcoma patients with
concomitant occurrence of sarcoidosis identified since 2007. Eight patients with
diagnosis of both sarcoma and sarcoidosis were identified over the period of 2007
-2016 at a single sarcoma center. Clinical and historical data including
presentation, histology, treatment, and outcome was tabulated for analysis. The
standardized incidence ratio was calculated for the state of Florida and our
hospital catchment area. We compared the observed incidence to the expected
incidence if the two entities were to be unrelated. Sarcoma subtype was
gastrointestinal stromal tumor in five patients, the remaining three cases were
unclassified spindled and epithelioid cell sarcoma, uterine leiomyosarcoma, and
myxofibrosarcoma. Sarcoidosis was diagnosed before sarcoma in three patients,
after sarcoma in four patients, and at the same time of sarcoma diagnosis in one
patient. From our series, three patients have shown no progression of sarcoma,
two are alive with sarcoma, two died due to progression of sarcoma, and one was
lost to follow up. Statistical analyses showed a standardized incidence ratio of
305 (95% confidence interval: 131 -556) for the state of Florida and standardized
incidence ratio of 950 (95% CI: 407 -1727) for our catchment area. This case
series points to a statistically robust, nonrandom association between sarcoma
and sarcoidosis that has not been previously described. Presumed metastatic
sarcoma should be considered for biopsy particularly with demographic
characteristics or imaging features suggestive of sarcoidosis.