Case studyPrimary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review☆
Introduction
Histiocytic sarcoma (HS) is a rare neoplasm that occurs most commonly in the intestinal tract, skin, soft tissue, and lymph node [1]. The incidence of primary central nervous system (CNS) HS is even rarer, with a total of 6 cases reported in the literature [[2], [5]]. An etiologic link has not been identified for CNS HS, and the current case of primary CNS HS is unique in that an etiologic link to prior radiation therapy is identified, associated with complex cytogenetic abnormalities in the tumor. Although radiation-associated sarcomas can present as any number of different pathologic entities, this is the first reported case of a radiation-associated CNS HS.
Section snippets
Clinical history
A 50-year-old male Asian with a history of recurrent pineal cavernous hemangioma, status post multiple resections and radiation, presented in 2011 with new growth of a right parieto occipital mass.
7In 2003, the patient presented with double vision and tinnitus, followed by worsening headaches. Magnetic resonance imaging (MRI) revealed a cystic-appearing focus in the pineal region with thin marginal enhancement; the imaging features were considered to be compatible with a benign pineal cyst. The
Histopathology
Histologic examination demonstrated a vaguely spindled, highly cellular, diffuse proliferation, consisting of a polymorphous infiltrate (Fig. 2A). The predominant population consisted of large cells with enlarged, round to ovoid nuclei, irregular nuclear contours occasionally with nuclear folds or grooves, distinct nucleoli, and epithelioid to spindled cell shape (Fig. 2B). There were scattered giant, highly atypical, epithelioid cells with eccentrically-placed polylobated nuclei arranged in a
Discussion
According to the World Health Organization, HS is defined as a malignant neoplasm of mature tissue histiocytes [1]. The typical morphology consists of a diffuse noncohesive proliferation of large (>20 μm), usually pleomorphic cells, round to oval in shape, although focal spindling/sarcomatoid areas may be seen. The cytoplasm is usually abundant and eosinophilic, often with some fine vacuoles. The nuclei are generally large, round to oval to convoluted/irregularly folded, including grooves and
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2017, World NeurosurgeryCitation Excerpt :These conditions were later referred to as “malignant histiocytosis.” With new developments in immunohistochemical staining at the end of the 20th century, it was discovered that histiocytic neoplasms constituted a completely different entity from T and B cell lineages.2,3,5,6 The term “histiocytic sarcoma” was proposed to describe neoplasms consisting of cells from monocytic or phagocytic lineage without T or B cell involvement.
Radiation-Induced Sarcomas of the Central Nervous System: A Systematic Review
2017, World NeurosurgeryCitation Excerpt :The selection steps and the underlying reasons for exclusion are summarized in Figure 1. During our review of the literature, we identified 180 (86 men, 78 women, and 16 sex unknown) cases of radiation-induced central nervous system sarcomas that occurred between 1956 and 2015 (Table 1 and Supplementary Table S1).3,4,8-114 The primary tumors were characterized as hematologic malignancies in 15 patients (8.3%), gliomas in 48 patients (26.6%), pituitary adenomas in 49 patients (27.2%), medulloblastomas in 8 patients (4.4%), craniopharyngiomas in 5 patients (2.7%), germ cell tumors in 4 patients (2.2%), meningiomas or neurinomas in 8 patients (4.4%), metastatic brain tumors in 2 patients (1.1%), other cancers in 23 patients (12.7%; subsequently discussed), head and neck sarcomas in 6 patients (3.3%), tinea capitis or scalp lesions in 4 patients (2.2%), and other types in 8 patients (4.4%).
Primary histiocytic sarcoma of the central nervous system. A case report and review of the literature
2015, Revista Espanola de Patologia
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The authors declare no disclosures or conflicts of interest.