Case description
A 3-year-old girl was admitted to our clinic with complaints of
persistent fever and rashes for the duration of a week in October 2013.
On her physical examination, pallor, petechias at lower extremities and
massive hepatosplenomegaly were observed. Routine laboratory
investigations, peripheral blood smear, bone marrow aspirate and
immunophenotyping were confirmed the diagnosis of B-cell precursor ALL.
The intensive chemotherapy was started according to ALL-IC BFM 2009
(Acute Lymphoblastic Leukemia-Intercontinental Berlin-Frankfurt-Münster
2009) and treatment was completed in October 2015. She was followed-up
in remission status for six years.
Seven years after the diagnosis of ALL, her family detected a mass on
her left chest wall. In physical examination, the mass was hard,
painless, immobile, and approximately the size of a lemon. It located on
the left of chest wall, near the back. Computed tomography (CT) scan was
reported a 6x9x12 cm large mass with heterogenous density including
partial necrosis (Figure 1). The mass destructed ninth, tenth, and
eleventh ribs and compressed the left lung and diaphragm. Except the
mass, there was not an abnormal activity in the positron emission
tomography (PET-CT) scan. An ultrasound-guided tru-cut needle biopsy was
performed and a histological study defined that the tumor was composed
of small, round, primitive cells that stain blue with conventional
staining and stained positive for CD99, periodic acid schiff (PAS), and
neuron spesific enolase (NSE). The t(11;22) translocation with theESWR/FL1 fusion transcript was detected in fluorescence in-situ
hybridization (FISH). The diagnosis of a primitive neuroectodermal tumor
of chest wall (Askin Tumor) was finalized with histologic and genetic
analysis. Also, hereditary risk factors (MSH6, BARD1, MRE11, ATM,
RAD51, PALB2, TP53, BRIP1, STK11, CHEK2, NBN, PTEN , and RAD51C )
were researched with next-generation sequencing and there was no
mutation identified.
A complete resection of the tumor was achieved without unfortunately
adequate measures from facial/soft tissue planes, and bony margins. The
multiagent chemotherapy was started according to the Children Oncology
Group interval compression arm of AEWS0031 regimen A2.