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 Isık 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¨ 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. *
Aslantaş 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 Münster 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.
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.