Primary histiocytic sarcoma of the brain mimicking cerebral abscess

Case report

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Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation.

This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.

Abbreviation used in this paper:HS = histiocytic sarcoma.

Article Information

Address correspondence to: Peter Nakaji, M.D., Neuroscience Publications, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, 350 W. Thomas Rd., Phoenix, AZ 85013. email: neuropub@dignityhealth.org.

Please include this information when citing this paper: published online July 26, 2013; DOI: 10.3171/2013.6.PEDS12533.

© AANS, except where prohibited by US copyright law.

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Figures

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    Left: Axial T1-weighted Gd-enhanced MR image of the brain at initial presentation showing a left parietal multiloculated mass with local vasogenic edema. Right: The loculations are diffusion restricted and intraoperatively were found to be full of a purulent fluid.

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    Intraoperative photograph demonstrating the purulent fluid. Used with permission from Barrow Neurological Institute.

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    Left: The abscess-like areas were filled with purulent necrotic debris. Right: Atypical large, often bizarre “discohesive” cells with folded nuclei and abundant granular cytoplasm. Scattered neutrophils and small mononuclear inflammatory cells are present in the background. H & E, original magnification × 400.

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    Eighteen days after initial presentation, the mass has enlarged substantially despite internal drainage and an aggressive course of antibiotics.

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    The mass is even larger, despite another drainage and debulking procedure and additional antibiotics.

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    Left: The atypical cells express CD-68 consistent with a histiocytic origin. Note that CD68 positivity is not sufficient to prove the diagnosis. The tumors must also be CD45 positive and negative for other lymphocyte specific markers, myeloid markers, and markers of different histiocytic lineages such as Langerhans cells. Right: The atypical cells also express CD45, a pan-lymphocytic and monocyte lineage marker. DAB with hematoxylin, original magnification × 200.

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    A: Four months after initial presentation, the mass has grown substantially despite a course of radiation therapy. B: Metastatic deposits are now seen in both hemispheres and the posterior fossa. C: The loculations continue to be strongly diffusion restricted.

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