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\begin{document}

\title{Myeloid Sarcoma presentetion as brain tumor in pediatric acute myeloid
lekeumia}



\author[1]{Huseyin Solgun}%
\author[1]{Sibel Tekgunduz}%
\author[1]{Cengiz Bayram}%
\affil[1]{Saglik Bilimleri Universitesi}%


\vspace{-1em}



  
  \date{May 24, 2024}


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\selectlanguage{english}
\begin{abstract}
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%
\end{abstract}%



\sloppy


\textbf{Myeloid Sarcoma presentetion as brain tumor in pediatric acute
myeloid lekeumia}

\textbf{Authors:} Huseyin Avni Solgun\textsuperscript{1}, Cengiz
Bayram\textsuperscript{2} , Sibel Tekg\selectlanguage{ngerman}ündüz\textsuperscript{3}

\textbf{Short title: Myeloid Sarcoma presentetion as brain tumor}

SOLGUN Huseyin A., M.D., Department of Pediatric Hematology and
Oncology, Health Sciences University, Basaksehir Cam and Sakura Training
and Research Hospital /Istanbul

TEKGÜNDÜZ Sibel, M.D., Department of Pediatric Hematology and Oncology,
Health Sciences University, Basaksehir Cam and Sakura Training and
Research Hospital /Istanbul

BAYRAM Cengiz., Assoc. Prof. M.D., Department of Pediatric Hematology
and Oncology, Health Sciences University, Basaksehir Cam and Sakura
Training and Research Hospital /Istanbul

\emph{Key clinical massege}

In our case the tumor localization was pariatotemporal as it is not
usuall presentation in Myeloid sarcoma (MS) of childhood. By this
knowledge; we presented this case report to refresh the data in the
literatüre and to emphasize the atypicall clinical prensentation, rare
incidence and management protocols of granulocytic sarcoma.

\textbf{Absract}

\textbf{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
.

\textbf{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. (\textbf{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
in\textbf{table 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 \textbf{figure 1a-b and 2a-b} respectively.

\textbf{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.

\textbf{Keywords:} Childhood, Acute myeloid lekeumia, Myeloid Sarcoma,
Brain tumors

\textbf{Introduction}

Granulocytic sarcoma (GS) is a rare extramedullary tumor of immature
myeloid cells and is frequently associated with acute or chronic
leukemias {[}1{]}. GS is seen in 3\% to 5\% of patients with
acutemyeloid leukemia (AML).When GS is associated with AML, the
prognosis is very poor {[}2{]}. Certainmorphological subtypes of AML
(M2,M4, M5) are more frequently associated with extramedullary myeloid
tumors {[}3, 4{]}. Although most cases of GS arise synchronously with
leukemias, GS may rarely be seen prior to leukemic development, and most
of these cases are falsely diagnosed as lymphoma, neuroblastoma, or
rhabdomyosarcoma, among others. In the pediatric age group, GS is
generally localized in the orbital area, but it may also be found in the
skin, bone, lymph node, and soft tissues {[}5{]}. Orbital GS, primarily
a childhood disease and rarely seen in adults, is mostly observed in
children under 10 years of age and is responsible for 2\% to 6\% of
childhood orbital tumors {[}6--7{]}.

In our case the tumor localization was pariatotemporal as it is not
usuall presentation. By this knowledge; we presented this case report to
refresh the data of granulocytic sarcoma in the literatüre and to
emphasize the atypicall clinical prensentation and rare incidence.

\textbf{Case Presentation}

We present a 2-year-old male patient who was referred to the pediatric
hematology oncology clinic with left pariatotemporal mass. The
laborautary test results were for white blood cell (WBC) 71320 10'9/L,
hemaglobuline 8,3 g/dl and platalet 81000 10'9/L respectevily. 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. (\textbf{figure 1a)} A rapid
peripheral blood smear (PBS) planned to identify the leukocytosis and
cytopenia.The observation of PBS reported \%5-6 myeloid blast formation.
The flow cytometric evaulation of peripheral blood showed \%10
myelobalasts. 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 confirmation of diagnosis AML, \%20 and
higher blast percentage was needed. In this period to decrease the WBC
count to safe values cytosine arabinoside treatment of 100mg/m2 dosage
for 24 hours was iniated. 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
myeloblasts. 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 markable
involution of tumoral mass from first day to first month serebral CT and
physical view are in \textbf{figure 1a-b and figure 2a-b} respectively.

\textbf{Discussion}

MS may occur at any site of the body, and therefore
clinicalmanifestations of MS exhibit diversity depending on the
specificlocation and size, which leads to significant diagnostic
challenges,in particular in patients without initial bone marrow
involvement. Malignant lymphoproliferative disorders, Ewing's sarcoma,
thymoma, melanoma, round blue cell tumors,or poorly differentiated
carcinoma has been reported at a

rate of 25\%-47\% in patients subsequently diagnosed with MS. {[}8,9{]}.

Diagnostic tools for the correct diagnosis of MS are also important in
this context and should include MRI and/or computed tomography scan for
evaluation of the size and location of the tumor and for distinguishing
the tumor from other lesions, morphological and flow cytometric analysis
of bone marrow and peripheral blood, or biopsy of the tumor and
immunohistochemical staining in patients without bone marrow involvement
{[}4{]}.

Treatment of MS includes AML-based protocols and, as in our case,
surgery and/or radiotherapy may be indicated for symptomatic lesions or
tumors causing local organ dysfunction {[}10{]}. Considering the most
common presentation sites in children with MS, which are skin and
orbital localizations, the current patient is presented to highlight a
rarely encountered presenting feature of MS.

Pamir Is\selectlanguage{polish}ı\selectlanguage{english}k and et al. Reported; 2 extramedullary orbital granulocytic
sarcoma (GS) cases without bone marrow involvement in view of their
rarity and also to reevaluate the treatment approach in this disease.
Seven days of high-dose methyl prednisolone (HDMP) treatment (3 days 30
mg/kg/day and 4 days 20 mg/kg/day)was administered initially, and
subsequently AcuteMyeloid Leukaemia--Berlin Frankfurt M\selectlanguage{ngerman}¨ unster
(AML-BFM) 2004 treatment protocol was continued for 2 cases. Eye
findings of the cases resolved considerably with HDMP treatment. In our
case after chematheraphy initiated there was markable involution in
pariatofrantal mass in first week as Pamir study decleraed. *

Aslanta\selectlanguage{polish}ş \selectlanguage{english}and et al reported a 4-year-old boy admitted with the complaint
of hemiparesis and a subsequent thoracolumbar mass was detected by
magnetic resonance imaging (MRI) Bone marrow aspiration showed 30\%
blasts compatible with AML. The pathology of the mass revealed MS.
{[}11{]}. After administration of radiotherapy, given at a dose of 18 Gy
in 10 daily fractions in 2 weeks, and dexamethasone therapy, the patient
achieved neurological improvement. He was treated with the AML-Berlin
Frankfurt M\selectlanguage{ngerman}ünster 2012 protocol and achieved both remission and mass
reduction following AML induction chemotherapy. The patient is still in
remission without any residual tumor on follow-up MRI. In our case we
iniated dexamethasone treatment reveresly radiothearphy was not
preferred.

\textbf{In conclusion;} AML-based protocols are stil remians the main
course reatment of MS and in indivudal cases, surgery and/or
radiotherapy may be indicated for symptomatic lesions or tumoral mass
causing local organ dysfunction. 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.

\textbf{References}

\textbf{1-} Inoue D, Nagai Y, Kimura T. Refractory de novo myeloid
sarcoma: a case report and therapeutic strategy based on bone marrow
minimal residual disease. Int J Hematol. 2009;90:120--123.

\textbf{2-} Maka E, Lukats O, T ´ oth J. Orbital tumour as initial
manifestation of acute myeloid leukemia: gran- ´ ulocytic sarcoma: case
report. Pathol Oncol Res. 2008;14:209--211.

\textbf{3-} Porto L, Kieslich M, Schwabe D. Granulocytic sarcoma in
children. Neuroradiology. 2004;46:374--377.

\textbf{4-} Slavcheva V, Lukanov T, Tzvetkov N. Two cases of
extramedullary myeloid tumor in patients with continuous remission of
acute myeloblastic leukemia. J Buon. 2008;13:589--592. \textbf{5-} Lee
SG, Park TS, Cheong JW. Preceding orbital granulocytic sarcoma in an
adult patient with acute myelogenous leukemia with t(8;21): a case study
and review of the literature. Cancer Genet Cytogenet. 2008;185:51--54.

\textbf{6-} Hmidi K, Zaouali S, Messaoud R. Bilateral orbital myeloid
sarcoma as initial manifestation of acute myeloid leukemia. Int
Ophthalmol. 2007;27:373--377.

\textbf{7-} Chung EM, Murphey MD, Specht CS. From the archives of the
AFIP. Pediatric orbit tumors and tumorlike lesions: osseous lesions of
the orbit. Radiographics. 2008;28:1193--1214.

8. Bakst RL, Tallman MS, Douer D, Yahalom J. How I treat extramedullary
acute myeloid leukemia. Blood 2011;118:3785-3793.

9. Redner A, Kessel R. Acute myeloid leukemia. In: Lanzkowsky P, Lipton
JM, Fish JD (eds). Lanzkowsky's Manual of Pediatric Hematology and
Oncology. 6th ed. Amsterdam, Elsevier, 2016.

10. Samborska M, Derwich K, Skalska-Sadowska J, Kurzawa P, Wachowiak J.
Myeloid sarcoma in children - diagnostic and therapeutic difficulties.
Contemp Oncol (Pozn) 2016;20:444-448. 5. Almond LM, Charalampakis M,
Ford SJ, Gourevitch D, Desai A. Myeloid sarcoma: presentation,
diagnosis, and treatment. Clin Lymphoma Myeloma Leuk 2017;17:263-267

11. Esra Arslanta\selectlanguage{polish}ş , \selectlanguage{english}Cengiz Bayram , I\selectlanguage{polish}şı\selectlanguage{english}k Odaman and et al. A Rare Cause
of Paraplegia: Myeloid Sarcoma. Turk J Hematol 2018;35:200-216

\textbf{Figure legends}

\textbf{Figure 1a:} The first day of serebral CT imagining showed a mass
lesion of approximately 8,4x6,6 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
is observed. Perifocal edema is noticeable. The mass lesion extending
towards the anterior of the left cerebellar hemisphere in the posterior
fossa caused the left cerebellar hemisphere to be pushed laterally.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image1/image1}

\end{center}
\end{figure}

\textbf{Figure 1b:} In the 28th day of treatment serebral CT imagining
showed a total recovery in the mass lesion and lateral ventricle, 3rd
ventricle was observed dilarerally, there was a hypodenosis appearance
in the left temporal lobe and it was considered in favor of
encephalomalacia-glyphosis.The left lateral ventricle temporal horn was
dilated compared to the symmetrical needle.Midline structures of the
cerebral parenchyma were observed in the normal position.

\textbf{Hosted file}

\verb`image2.tmp`  available at \url{https://authorea.com/users/373217/articles/710332-myeloid-sarcoma-presentetion-as-brain-tumor-in-pediatric-acute-myeloid-lekeumia}

\textbf{Figure 2a:} First day physical view of mass in parietotemporal
skull.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image3/image3}

\end{center}
\end{figure}

\textbf{Figure 2b:} The markable involution of physical mass view in
28th day of treatment in parietotemporal skull.\selectlanguage{english}
\begin{figure}[H]
\begin{center}
\includegraphics[width=0.70\columnwidth]{figures/image4/image4}

\end{center}
\end{figure}

\selectlanguage{english}
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