Absract
Background: Myeloid sarcoma (MS), also known as granulocytic
sarcoma or chloroma, is a rare extramedullary tumor consisting of
myeloblasts or immature myeloid cells that disrupt the involved tissue
and typically occurs concurrently with acute myeloid leukemia (AML). It
can also occur in association with accelerated-phase chronic myeloid
leukemia or myelodysplastic syndrome; as an extramedullary relapse of
AML, including in the post-bone marrow transplant setting; and
occasionally as the first presenting manifestation, even before bone
marrow involvement. Bone, periosteum, skin, orbit, lymph nodes, the
gastrointestinal tract, and the central nervous system are the most
commonly involved sites in patients presenting with MS; however, skin
and orbital localizations are the most often reported sites in children
.
Case: We present a 2-year-old male patient who was referred to
the pediatric hematology oncology clinic with left pariatotemporal mass.
In computed tomography (CT) of brain; a mass lesion of approximately 8x9
cm in size extending from the left parietotemporal lobe to the inferior,
extending towards the skin / subcutaneous, causing lytic changes in the
left posterior temporal bone was observed. (figure 1a) . A
neurosurgery consultation was planned. The brain antieudema protective
treatment with prednisilone 40 mgr/kg first 3 days and subsuquent to 30
mgr/kg 4 days was initiated. Soft tissue tumor and the bone marrow
metastasis or AML and granulocytic sarcoma were the initiall diagnositc
features. Flowcytometric peripheral blood test was reported %8
myeloblast. In this period to decrease the WBC count: 76000 10’9/L to
safe values cytosine arabinoside treatment of 100mg/m2 dosage for 24
hours was iniated. The control values in treatment hours and days are intable 1 . In the second day of follow ups, WBC count was 42000
10’9/L and bone marrow aspiration (BMA) and cerebrospinal fluid was
planned. The flow cytometric evaulation of BMA showed %10 myelobalasts.
The genetic panel for AML was sent form bone marrow aspirate. In the 3th
day of treatment; the genetic tests reported t(8,21) positivity in this
paitent. The AML diagnosis was comfirmed and AML BFM 2019 protocol was
initiated to the patient. The involution of tumoral mass from first day
and first month of CT and physical view are in figure 1a-b and
2a-b respectively.
Conclusion: The most common presentation sites in children with
MS is skin and orbital localizations. Dexamethosene and conventianol
chemathearphy is first steps in treatment. Our case presented exterior
pariatotemporal mass extending to interior left pariatel lobe with
extraordinary feature. This presentation may need differantial diagnosis
even with brain tumors.
Keywords: Childhood, Acute myeloid lekeumia, Myeloid Sarcoma,
Brain tumors