Primary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review

doi:10.1016/j.humpath.2012.11.002

Human Pathology

Volume 44, Issue 6, June 2013, Pages 1177-1183

https://doi.org/10.1016/j.humpath.2012.11.002 Get rights and content

Summary

Histiocytic sarcoma (HS) is a rare neoplasm that occurs most commonly in the intestinal tract, skin, soft tissue, and lymph node. The incidence of primary central nervous system (CNS) HS is even rarer, with a total of 6 cases reported in the literature. 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. The pathologic and immunophenotypic characteristics of this case, with a nearly obscuring heavy inflammatory infiltrate and expression of monocytic/histiocytic markers (CD163, CD68, CD4, fascin), are characteristic of CNS HS. A discussion of the differential diagnosis and review of relevant literature are presented.

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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Sarcomas are neoplasms arising from mesenchymal progenitor cells which differentiates them from other central nervous system (CNS) tumors, such as gliomas, which arise from cells that are ectodermal in origin. Primary tumors arising from these cell types are infrequently seen in the CNS. Many CNS sarcomas, when they do occur, most commonly arise in the pediatric population. Our understanding of their epidemiology is lacking given rarity. This chapter will detail sarcomas which arise within the CNS, as well as those which occur adjacent to the CNS, in bony tissue for example, and can unfavorably impact the CNS. These rare tumors are often managed by multidisciplinary teams which include neuro-oncologists, pediatric neuro-oncologists, oncologists, neurosurgeons, orthopedic surgeons, radiation oncologists, general surgeons, neuroradiologists, and pathologists.
Histiocytic sarcoma of the base of tongue treated with radical radiotherapy: A case report and review of the literature
2020, Clinical and Translational Radiation Oncology
Histiocytic sarcoma (HS) is an exceedingly rare and aggressive neoplasm of lymphoid and haematopoietic tissues and expresses histological and phenotypical characteristics of mature histiocytes. There have only been a few cases of documented HS in the head and neck region. Whilst patients with HS often have nodal or disseminated disease, patients can present with localised disease. There are currently no established treatment guidelines, and reported cases of localised disease have been managed with primary surgery and adjuvant radiotherapy and/or chemotherapy. Here we present, the case of a 49 year old man with a HS of the base of tongue treated with radical radiotherapy to a dose of 60 Gy in 30 fractions, achieving disease free survival of greater than 5 years with minimal toxicity. To our knowledge, this is the first reported case of HS treated with radical radiotherapy, and suggests that when the potential morbidity of surgery for localised disease is significant, radiotherapy may represent an alternative treatment.
Infection or Glioma? The False Dilemma of Primary Central Nervous System Histiocytic Sarcoma
2017, World Neurosurgery
Citation 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.
Primary central nervous system (CNS) histiocytic sarcoma is an extremely rare lymphoproliferative disorder that affects the CNS and behaves aggressively. Only 27 cases of primary CNS histiocytic sarcoma have been reported. The paucity of literature on this entity has made diagnosis and treatment difficult both for the surgeon and the pathologist.
In this case of primary CNS histiocytic sarcoma, a middle-aged woman presented from an outside institution with a supposed cerebellar abscess. Intraoperative frozen pathology was initially interpreted as high-grade glioma; however, final pathology demonstrated histiocytic sarcoma.
This report makes a significant contribution to the literature on this rare malignant disease by outlining a similar presentation among several cases and providing a thorough overview of existing criteria for diagnosis and management.
Radiation-Induced Sarcomas of the Central Nervous System: A Systematic Review
2017, World Neurosurgery
Citation 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%).
Radiation-induced sarcomas (RISs) of the central nervous system are an uncommon late risk of irradiation. We conducted a systematic review of individual patient data to characterize RISs.
We conducted a systemic search of the PubMed databases and compiled a comprehensive literature review. Student t tests were used to evaluate differences between variables. Kaplan-Meier analysis was used to estimate survival. Statistical significance was assessed using a log-rank test.
We analyzed 180 cases of RISs, including 59 osteosarcomas, 50 fibrosarcomas, and 16 malignant fibrous histiocytomas. The average age of onset for primary lesions was 28.8 ± 17.9 years, and the average dose of radiation delivered to the primary lesion was 51.4 ± 18.6 Gy. The latency period between radiotherapy and the onset of sarcomas was 12.4 ± 8.6 years. The median overall survival time for all sarcoma patients was 11 months, with a 5-year survival rate of 14.3%. The median survival in patients who received chemotherapy was 18 months, with a 2-year survival rate of 39.4%, whereas patients who did not receive chemotherapy had a median survival of 5 months and a 2-year survival rate of 13.7% (P < 0.0001).
The risk of secondary sarcomas in patients treated with cranial radiotherapy warrants longer follow-up periods beyond the standard time frame typically designated for determining the risk of primary tumor relapse. Moreover, chemotherapy should be considered a potential treatment option for RISs.
Primary histiocytic sarcoma of the central nervous system. A case report and review of the literature
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La afectación primaria del sistema nervioso central por un sarcoma histiocítico es extremadamente infrecuente, con apenas 17 casos publicados y un comportamiento clínico agresivo. Presentamos el caso de una mujer de 46 años cuyas imágenes de resonancia magnética mostraron masa tumoral intraparenquimatosa temporoparietal derecha con desplazamiento de la línea media y edema vasogénico asociado. El tumor fue resecado parcialmente, resultó ser un sarcoma histiocítico. La paciente falleció a los 3 meses de ser realizado el diagnóstico. La necropsia demostró un severo edema cerebral y recrecimiento del tumor sin afectación del resto de los órganos. El diagnóstico estuvo basado en las características microscópicas del tumor, complementado con un panel inmunohistoquímico amplio, que resulta vital para confirmar el origen histiocítico y excluir otras lesiones malignas.
Primary histiocytic sarcoma of the central nervous system is not only an extremely rare tumour, with only 17 published cases to date, but also a very aggressive one. We report the case of a 46 year old woman, who was seen on magnetic resonance to have a right intracerebral parietal temporal tumour with displacement of the midline and associated vasogenic oedema. The tumour was partially resected and seen to be a histiocytic sarcoma. The patient died 3 months after the diagnosis. Necropsy showed severe brain oedema and tumour regrowth but no involvement of other organs. Diagnosis was based on the microscopic characteristics of the tumour as well as an immunohistochemical panel, essential for the confirmation of its histiocytic origin and for the differential diagnosis with other malignant lesions.
A rare case of primary central nervous system histiocytic sarcoma harboring a novel ARHGAP45::BRAF fusion: a case report and literature review
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^☆: The authors declare no disclosures or conflicts of interest.

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Case studyPrimary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review☆

Summary

Introduction

Section snippets

Clinical history

Histopathology

Discussion

Mod Pathol

Clin Neurol Neurosurg

World Health Organization pathology and genetics of tumours of haematopoietic and lymphoid tissues

CD163: a specific marker of macrophages in paraffin-embedded tissue samples

Am J Clin Pathol

Expression of CD163 (hemoglobin scavenger receptor) in normal tissues, lymphomas, carcinomas, and sarcomas is largely restricted to the monocyte/macrophage lineage

Am J Surg Pathol

Monocyte/macrophage-specific marker CD163+ histiocytic sarcoma: case report with clinical, morphologic, immunohistochemical, and molecular genetic studies

Int J Hematol

Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases

Histopathology

Case study
Primary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review☆