- Case report
- Open Access
- Open Peer Review
Primary leptomeningeal histiocytic sarcoma in a patient with a good outcome: a case report and review of the literature
© Pérez-Ruiz et al.; licensee BioMed Central Ltd. 2013
- Received: 3 January 2013
- Accepted: 11 April 2013
- Published: 13 May 2013
Histiocytic sarcoma is a rare neoplasm with few cases reported in the literature of which some were diagnosed in animals. This neoplasm arises from abnormal reticuloendothelial system cell proliferation of histiocytes and has an aggressive behavior especially if located in the central nervous system. We present the first case of a patient with histiocytic sarcoma that involved the meninges and had a good course after multidisciplinary treatment.
Our patient was a 41-year-old Caucasian woman with no previous history of disease who started with systemic symptoms such as headache and chills. Magnetic resonance imaging with gadolinium contrast of the brain suggested a mass 1.5×2cm in diameter in the temporal lobe with a non-uniform vasogenic edema. This lesion was implanted in the meninges and surgery was the first treatment. The histological findings revealed a histiocytic sarcoma. The patient received concomitant chemoradiotherapy after surgery with good tolerance and currently lives without disease.
Although histiocytic sarcomas in the brain present an unusual location and have a poorer prognosis, we have identified the first primary leptomeningeal histiocytic sarcoma with a disease-free survival greater than 3 years following multidisciplinary treatment with surgery and chemotherapy and radiotherapy.
- Histiocytic sarcoma
- Leptomeningeal sarcomas
- Multidisciplinary treatment
Histiocytic sarcoma (HS) is a rare neoplasm showing evidence of histiocytic differentiation. It often appears in the skin, lymph nodes, and intestinal tract but central nervous system (CNS) involvement is rare. The standard treatment for these sarcomas is surgery. The best option for treating these tumors is unclear and affected patients have a poor prognosis despite treatment.
HS also formerly known as “true” histiocytic lymphoma is a rare neoplasm showing evidence of histiocytic differentiation. It often appears in the skin, lymph nodes, and intestinal tract . Although CNS involvement is rare, there are a few cases reported in the literature [1–5] and other cases diagnosed in animals, such as cats, dogs, and camels [6–9]. The cases involving the CNS occurred by continuation of the disease except the case reported by Torres et al.  which appeared in the subarachnoid space.
HS has often been classified as being similar to diseases such as malignant histiocytosis, histiocytic lymphoma, and reticular sarcoma, based on morphological criteria . However, advances in molecular and biological markers have improved immunohistochemistry findings, allowing for differentiation between HS and B or T cells and non-Hodgkin lymphomas. These diseases have a prognosis and treatment different from sarcomas.
HS is composed of mononuclear tumor cells with large cytoplasmic vacuoles. Some cells present with phagocytosis phenomena. The diagnosis is based on immunohistochemistry markers of histiocytic cells such as positive CD68, lysozyme, α1-antitrypsin and CD163, which is regarded as a specific HS marker . However, HS presents negative markers of epithelial (epithelial membrane antigen or cytokeratin), melanocytic (CD34 or myeloperoxidase) and lymphoid (CD3 or CD20) neoplasms as in our case. Markers of Langerhans cell (CD1a), follicular dendritic cells, B cells, T cells, and myeloid cells are negative. Lack of expression of CD30 and ALK-1 excludes anaplastic large cell lymphoma, which is an important differential diagnosis. The focal staining for S-100 protein is not uncommonly seen in the true histiocytic lymphomas as reported in the literature .
Radiological findings have not been established due to the low number of cases. The HS image study reported in the literature addressing the brain is MRI , which describes the extent of the disease. The images point towards neoplastic disease, but there are no specific images suggesting HS. Some MRI studies in dogs are inconclusive because the image presented could be either a benign process (such as encephalitis) or a malignant process (such as meningioma) .
The standard treatment for these sarcomas is surgery. The best option for treating these tumors is unclear. Furthermore, when the tumor is located in the CNS the therapeutic options are reduced. Brain sarcomas have an aggressive course with an overall survival rate of around 4 to 5 months, regardless of treatment. There are no studies where the benefit of radiotherapy or chemotherapy for HS has been established.
In our case, the patient received concomitant chemoradiotherapy after surgery. We decided to administer temozolomide with the radiotherapy because the patient was young and she presented with a sarcoma affecting the CNS. It is also known that this type of chemotherapy crosses the blood–brain barrier and it has shown benefit over radiotherapy alone in other aggressive tumors . She had a good tolerance and currently lives without disease (disease-free interval of 42 months). The cases reported before were treated with different schedules of chemotherapy by the vein or with intrathecal treatment but none received concomitant radiotherapy. All the cases had an aggressive course except one case that relapsed after 3 years . In the literature, some cases of extranodal HS treated with surgery and some type of chemotherapy or radiotherapy had a good outcome. These reported patients lived varying months after treatment . Recently, a radiotherapy-induced HS has been treated with radiation alone and the patient remained free of recurrent disease after 1.5 years . Radiation alone could be beneficial but we do not know whether radiation-induced HS differ in their biological behavior.
In conclusion, HS is a rare neoplasm with a diagnosis based on immunohistochemistry and biological findings. Although HS in the brain present an unusual location and have a poorer prognosis, we have identified the first primary leptomeningeal HS with a disease-free survival greater than 3 years following treatment with surgery and chemotherapy and radiotherapy.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Cheuk W, Walford N, Lou J, Lee AK, Fung CF, Au KH, Mak LS, Chan JK:Primary histiocytic lymphoma of the central nervous system. Am J Surg Pathol. 2001, 25: 1372-1379. 10.1097/00000478-200111000-00004.View ArticlePubMedGoogle Scholar
- Cao M, Eshoa C, Schultz C, Black J, Zu Y, Chang CC:Primary central nervous system histiocytic sarcoma with relapse to mediastinum. Arch Pathol Lab Med. 2007, 131: 301-305.PubMedGoogle Scholar
- Sun W, Nordberg M, Fowler M:Histiocytic sarcoma involving the central nervous system. Am J Surg Pathol. 2003, 27: 258-265. 10.1097/00000478-200302000-00017.View ArticlePubMedGoogle Scholar
- Colovic N, Jurisic V, Colovic M:Malignant histiocytosis with central nervous system involvement and hepatic mucinous cystadenoma in a single patient with review of the literature. J BUON. 2007, 12: 539-542.PubMedGoogle Scholar
- Torres CF, Korones D, Powers J, Vadasz AG:Primary leptomeningeal histiocytic lymphoma in a young child. Med and Pediat Oncology. 1996, 27: 547-550. 10.1002/(SICI)1096-911X(199612)27:6<547::AID-MPO7>3.0.CO;2-L.View ArticleGoogle Scholar
- Molenaar FM, Breed EJ, Flach I, McCandlish IA, Pocknell AM, Strike T, Routh A, Taema M, Summers BA:Brain tumours in two Bactrian camels: a histiocytic sarcoma and a meningioma. Vet Rec. 2009, 164: 684-688. 10.1136/vr.164.22.684.View ArticlePubMedGoogle Scholar
- Tamura S, Tamura Y, Nakamoto Y, Ozawa T, Uchida K:MR imaging of histiocytic sarcoma of the canine brain. Vet Radiol Ultrasound. 2009, 50: 178-181. 10.1111/j.1740-8261.2009.01512.x.View ArticlePubMedGoogle Scholar
- Uchida K, Morozumi M, Yamaguchi R, And TS:Diffuse leptomeningeal malignant histiocytosis in the brain and spinal cord of a Tibetan Terrier. Vet Pathol. 2001, 38: 219-222. 10.1354/vp.38-2-219.View ArticlePubMedGoogle Scholar
- Ide T, Uchida K, Tamura S, Nakayama H:Histiocytic sarcoma in the brain of a cat. J Vet Med Sci. 2010, 72: 99-102. 10.1292/jvms.09-0312.View ArticlePubMedGoogle Scholar
- Hirose S, Shimada S, Takebe Y, Tokuriki Y, Kawano H, Kubota T:Recurrent malignant histiocytosis with cerebrospinal involvement. Neurol Med Chir. 1994, 34: 233-236. 10.2176/nmc.34.233.View ArticleGoogle Scholar
- Vos JA, Abbdondanzo SL, Barekman C, Andriko JW, Miettinen M, Aguilera NS:Histiocytic sarcoma: a study of five cases including the histiocyte marker CD163. Mod Pathol. 2005, 18: 693-794. 10.1038/modpathol.3800346.View ArticlePubMedGoogle Scholar
- Pileri SA, Grogan TM, Harris NL, Banks P, Campo E, Chan JK, Favera RD, Delsol G, De Wolf-Peeters C, Falini B, Gascoyne RD, Gaulard P, Gatter KC, Isaacson PG, Jaffe ES, Kluin P, Knowles DM, Mason DY, Mori S, Müller-Hermelink HK, Piris MA, Ralfkiaer E, Stein H, Su IJ, Warnke RA, Weiss LM:Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology. 2002, 41: 1-29.View ArticlePubMedGoogle Scholar
- Stupp R, Mason WP, Van den Bent M, Weller M, Fisher B, Taphorn MJ, Belanger K, Brandes AA, Marosi C, Bogdahn U, Curschmann J, Janzer RC, Ludwin SK, Gorlia T, Allgeier A, Lacombe D, Cairncross JG, Eisenhauer E, Mirimanoff RO:Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med. 2005, 352: 987-996. 10.1056/NEJMoa043330.View ArticlePubMedGoogle Scholar
- Hornick JL, Jaffe ES, Fletcher C:Extranodal histiocytic sarcoma. Clinicopathological analysis of 14 cases of a rare epithelioid malignancy. Am J Surg Pathol. 2004, 28: 1133-10.1097/01.pas.0000131541.95394.23.View ArticlePubMedGoogle Scholar
- Wu W, Sayit T, Vinters HV, Pope W, Mirsadraei L, Said J:Primary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review. Hum Pathol. 2013, 26: Epub ahead of print,Google Scholar
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