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| LETTER TO EDITOR |
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| Year : 2014 | Volume
: 62
| Issue : 6 | Page : 684-685 |
Primary central nervous system histiocytic sarcoma mimicking glioma
Jiwei Bai1, Guang Li2, Mi Shen3, Dali Sui1, Song Lin1
1 Department of Neurosurgery, Beijing Tiantan Hospital,Capital Medical University, Dongcheng, Beijing, China
2 Department of Pathology, Beijing Tiantan Hospital,Capital Medical University, Dongcheng, Beijing, China
3 Department of Radiology, Beijing Tiantan Hospital,Capital Medical University, Dongcheng, Beijing, China
| Date of Submission | 10-Apr-2014 |
| Date of Decision | 24-Aug-2014 |
| Date of Acceptance | 05-Dec-2014 |
| Date of Web Publication | 16-Jan-2015 |
Correspondence Address:
Jiwei Bai
Department of Neurosurgery, Beijing Tiantan Hospital,Capital Medical University, Dongcheng, Beijing
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.149409
How to cite this article:
Bai J, Li G, Shen M, Sui D, Lin S. Primary central nervous system histiocytic sarcoma mimicking glioma. Neurol India 2014;62:684-5 |
Sir,
Histiocytic sarcoma (HS) is a rare but aggressive hematopoietic tumor, and most patients die of progressive disease within two years. [1] These malignant neoplasms are characterized by the morphological and immunophenotypic features of mature tissue histiocytes. The most common sites for these tumors are lymph nodes and certain extranodal sites. [1] The involvement of the central nervous system (CNS) has rarely been reported. The median survival for CNS HS is even worse at approximately five months. [2] We present a case of primary CNS HS mimicking glioma with a relatively good outcome during the following period.
A 52-year-old man presented with weakness of left lower limb of 15 days. Neurologic examination revealed motor power of grade 4/5 in the left lower limb. Magnetic resonance imaging (MRI) [Figure 1] revealed an irregular intra-axial mass in the frontal lobe with significant peri-lesional edema. The solid part was iso-intense on both T1- and T2-weighted images. Cystic changes were present. On contrast administration, the lesion enhanced significantly. The solid portion showed slightly restricted diffusion on DWI (b = 1000). MR spectroscopy (MRS) showed increase in Cho/NAA and Cho/Cr, decrease in NAA/Cr and lactic acid. These appearances were consistent with the mass being a glioma. The patient underwent a craniotomy and total excision of the tumor under ultrasonic guidance. The postoperative screening for systemic malignancy, including a whole-body 18 F-FDG PET scan, was unremarkable. The patient underwent postoperative concomitant chemoradiotherapy. He received temozolomide concomitant with 1.8 Gy of radiotherapy five days a week. The radiotherapy was intensity-modulated radiation therapy for a total of 30 fractions. MRI performed six months after initial presentation showed no evidence of tumor recurrence. The patient is still in good condition when followed up by telephone interview after 16 months. | Figure 1: Gadolinium-enhanced T1-weighted sagittal (a) and T2-weighted coronal (b) MRI. A heterogeneous and irregular intra-axial mass was revealed in the right frontal lobe, and peripheral vasogenic edema was obvious. MR spectroscopy (c) showed increased Cho/NAA, increased Cho/Cr, and decreased NAA/Cr
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Microscopically, the tumor was composed of histiocytoid cells of variable size with abundant eosinophilic cytoplasm. These cells showed marked nuclear pleomorphism with prominent nucleoli and coarse chromatin [Figure 2]a. Mitotic figures were identified. A typical pathological feature of CNS HS is an associated prominent inflammatory component. [2],[3],[4] In the present case, the inflammatory background consisted primarily of lymphocytes. The presence of abundant inflammatory cells may skew the diagnosis of HS. [4] Immunohistochemically, the tumor cells were positive for CD68 and lysozyme [Figure 2]b and c. The cells were also focally positive for S-100 and vimentin but negative for EMA, CK, GFAP, LCA, myoglobin, synaptophysin, Melan A, HMB45, ALK, desmin, CD15, CD30, CD1a, CD21, CD45RO, CD38, CD138, CD99, CD56, MAP-2 and Olig2. It should be emphasized that no antibody is specific for histiocytic differentiation. The expression of CD68 and lysozyme must be positive in HS. CD163 is a new immunohistochemical marker of monocytes and histiocytes. Its expression is more specific than other monocytic and histiocytic markers. [2] Given that glioma was initially considered, IDH (1 and 2) and 1p/19q were investigated, and the results exhibited neither IDH mutations nor 1p/19q co-deletion. | Figure 2: Histological image of histiocytoid cells of variable size with abundant eosinophilic cytoplasm. These atypical cells showed marked nuclear pleomorphism with prominent nucleoli and coarse chromatin (2a, hematoxylin-eosin, ×400). The tumor cells were positive for CD68 (2b, ×200) and lysozyme (2c, ×200)
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The preferred initial therapy is surgery because most cases have a single lesion. [5] Complete surgical excision of a solitary CNS HS may contribute to a good outcome. [4] However, local recurrence is common, and extracranial relapse, including mediastinal and bony metastasis, occur occasionally. [3] Chemotherapy has been suggested for more aggressive or multifocal lesions, but the outcomes were frustrating. Our patient was treated with concomitant chemoradiotherapy after surgery and remained well during close clinical and radiological surveillance over a period of 16 months. In conclusion, preoperative diagnosis of CNS HS is challenging. CNS HS should be treated intensively with surgery, chemotherapy, and/or radiation therapy. We suggest that a concomitant chemoradiotherapy regimen for high-grade glioma should be considered postoperatively for primary CNS HS.
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| 1. | Takahashi E, Nakamura S. Histiocytic sarcoma: An updated literature review based on the 2008 WHO classification. J Clin Exp Hematop 2013;53:1-8.  |
| 2. | Laviv Y, Zagzag D, Fichman-Horn S, Michowitz S. Primary central nervous system histiocytic sarcoma. Brain Tumor Pathol 2013;30:192-5. |
| 3. | Cao M, Eshoa C, Schultz C, Black J, Zu Y, Chang C. Primary central nervous system histiocytic sarcoma with relapse to mediastinum: A case report and review of the literature. Arch Pathol Lab Med 2007;131:301-5. |
| 4. | Bell SL, Hanzely Z, Alakandy LM, Jackson R, Stewart W. Primary meningeal histiocytic sarcoma: A report of two unusual cases. Neuropathol Appl Neurobiol 2012;38:111-4. |
| 5. | Perez-Ruiz E, Delgado M, Sanz A, Gil AM, Dominguez AR. Primary leptomeningeal histiocytic sarcoma in a patient with a good outcome: A case report and review of the literature. J Med Case Rep 2013;7:127. |
[Figure 1], [Figure 2]
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