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Case Reports
. 2012:3:96.
doi: 10.4103/2152-7806.100183. Epub 2012 Aug 27.

A rare case of primary extranodal, extradural histiocytic sarcoma of the thoracic spine and review of literature

Affiliations
Case Reports

A rare case of primary extranodal, extradural histiocytic sarcoma of the thoracic spine and review of literature

Ashis Patnaik et al. Surg Neurol Int. 2012.

Abstract

Background: Histiocytic sarcoma (HS) is a rare aggressive malignant neoplasm of hematopoietic cell origin showing features of fever, weight loss, adenopathy, hepatosplenomegaly, and pancytopenia. Primary histiocytic sarcoma of spine without involvement of lymph nodes, bone marrow or systemic features is very rare. Due to highly malignant, aggressive behavior and frequent local and distant metastases, prognosis of histocytic sarcoma is poor.

Case description: We report a case of primary extranodal variety of histiocytic sarcoma without involvement of bone marrow at extradural location of thoracic spine. Immunohistochemistry panel ruled out other more common lesions in this location. We could not find such presentation of extradural histiocytic sarcoma at the thoracic region in the literature.

Conclusion: Primary involvement of extradural space by histiocytic sarcoma could arise from the proliferation of multipotent hematopoietic stem cells which are left in this space embroyologically.

Keywords: Extranodal; histiocytic sarcoma; primary; spine.

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Figures

Figure 1
Figure 1
(a) T1-weighted contrast sagittal image showing the tumor with irregular margin and intense enhancement on contrast. Bodies of T8 and T9 vertebrae show infiltration. (b) T1-weighted contrast axial image showing the enhancing tumor surrounding and compressing the tumor from all side like a cylinder. (c) Coronal image showing the infiltrative nature of the tumor
Figure 2
Figure 2
Tumor specimen showing the pinkish white color, soft, friable nature of the tumor
Figure 3
Figure 3
(a) Routine staining show pleomorphic, large cells with abundant cytoplasm (arrowhead) with large numbers of lymphocytic infiltrate (long arrow). (b) Numerous mitotic figures (arrow). (c) Tumor giant cells (arrow). (d) Emperipolesis: Phagocytosis of red cells, leukocytes and tumor cells by the histiocyte
Figure 4
Figure 4
Immunohistochemistry with S-100 positivity
Figure 5
Figure 5
CD68 immunopositivity
Figure 6
Figure 6
Lysozyme immunopositivity

References

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