- Received November 30, 2022
- Accepted February 03, 2023
- Publication March 16, 2023
- Visibility 3 Views
- Downloads 0 Downloads
- DOI 10.18231/j.ijpo.2023.014
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CrossMark
- Citation
An unusual case of mediastinal mass with pleural and pericardial effusion
- Author Details:
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Shirley Maria Dsouza *
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Purnima S Rao
Shirley Maria Dsouza *
Purnima S Rao
Introduction
Myeloid sarcoma is a tumor mass consisting of myeloid blasts, with or without maturation, occurring in an anatomical site other than bone marrow.[1]
Myeloid Sarcoma (MS) is only called so if there is a mass formation with complete effacement of tissue architecture.
More than 2000 cases have been reported so far, with about 21% cases occurring in the mediastinum.[2]
Clinical features depend on the location, with symptoms due to tumor mass effect or local organ dysfunction. It may sometimes precede/ coincide with AML or constitute blastic transformation of MDS/ MPN.[1]
Case Report
A 17-year-old female came with the complaints of swelling in the neck since 1 month, breathlessness for the past 5 days.
Pet-CT
Metabolically active disease in the large soft tissue mass occupying the entire mediastinum, encasing the mediastinal vessels, trachea and esophagus.
Metabolically active disease in the large ill-defined soft tissue mass in bilateral supraclavicular region.
Mild pleural effusion and pericardial effusion.
Metabolic activity in the cardiophrenic nodes.
Diffuse hypermetabolism in the marrow of all bones of axial and proximal appendicular skeleton.
Pericardiocentesis
Appearance: Turbid
Color: Red
Coagulum: Present
Cell count: 74,000 cells/cumm
Cell type: Mononuclear atypical cells: 90%
Neutrophils: 10%
Protein: 6.8 g/dL
Glucose: 10 mg/dL
Myelopoiesis: Increased in number, shows predominant population of blasts (86%), having high N:C ratio, fine chromatin, 1-2 nucleoli, scanty cytoplasm. Few blasts are seen to have nuclear convolutions.
Impression: Acute Leukemia, immunophenotyping indicated for further subtyping.
Flowcytometry
Low side scatter in CD45 and CD13
Moderate expression of CD34, CD33, CD117, HLA-DR, CD7. Rest of the markers were negative.
CT-guided biopsy of the mediastinal mass
Discussion
Myeloid sarcoma is a rare neoplasm with a greater chance of it being misdiagnosed as malignant lymphoproliferative disorders including Hodgkin’s lymphoma, MALT lymphoma, DLBCL, Ewing’s sarcoma, thymoma, round blue cell tumors or poorly differentiated carcinoma.[3] The misdiagnosis was commonly due to inadequate immunophenotyping of the MS lesion and was not corrected until a diagnosis of acute leukemia was later established by bone marrow biopsy or peripheral blood examination.[4]
In a study by J E Takasugi et.al, mediastinum was the commonest intrathoracic site for MS and 6 out of 9 cases presented with pleural effusion.[5]
Very few cases of MS with pleural and pericardial effusion have been reported.
Conclusion
Recognition of MS with or without AML is necessary for prognosis.
Correlating radiological, hematological, bone marrow and flowcytometry features with histomorphology and immunohistochemistry of the tumor is essential.
A rare possibility of MS should be kept in mind for mediastinal masses for timely diagnosis and treatment.
Source of Funding
None.
Conflict of Interest
None.
References
- . WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. 2017. [Google Scholar]
- . Central Nervous System Tumours: WHO Classification of Thoracic Tumors. 2021. [Google Scholar]
- PA Hagen, C Singh, M Hart, AH Blaes. Differential Diagnosis of Isolated Myeloid Sarcoma: A Case Report and Review of the Literature. Hematol Rep 2009. [Google Scholar]
- M Magdy, NA Karim, I Eldessouki, I Gaber, O Rahouma, M Ghareeb. Myeloid Sarcoma. Oncol Res Treat 2019. [Google Scholar]
- JE Takasugi, JD Godwin, SI Marglin, SH Petersdorf. Intrathoracic granulocytic sarcomas. J Thorac Imaging 1996. [Google Scholar]