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

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.

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

“…Less often, primary lung disease resembling pulmonary LCH [196], and primary parotid gland involvement have been described [6]. The disease may spread to other organs, i. e. lungs, liver, spleen [20], bile ducts [141], and CNS; in the latter, both intracerebral lesions [46,80,164] and lesions in the spinal cord have been described [161]. Also, leukemia-like spread of cells in the blood may occur [36].…”

“…Also, leukemia-like spread of cells in the blood may occur [36]. Unspecific symptoms such as fever, weight loss, hepatosplenomegaly and pancytopenia are observed as well [2,7,96,161,194]. Typically, the disease has an aggressive course, with poor prognosis [80,182,194].…”

A rare case of paediatric histiocytic sarcoma of the maxilla and mandible

“…6,10 Treatment protocols have evolved over time and recent studies suggest that a combination of radiotherapy and chemotherapy can be useful. 11 In our case, the patient was treated with ADE chemotherapy regime, which is often used for patients with acute myeloid leukaemia. After three cycles of treatment over a 3-month period, there was significant interval improvement, suggesting a good response to treatment.…”

A case of CD68 negative histiocytic sarcoma of axilla masquerading as metastatic breast cancer

“…HS is a hematopoietic neoplasm with aggressive behavior and poor outcome. It can present as localized disease confined to the skin, lymph nodes and intestinal tract, or as disseminated disease [ 6 ]. The median age of presentation is 46 years with slight male preponderance.…”