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.