- Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Ave. Box 626, Rochester, NY 14642, USA
Correspondence Address:
Mahlon Johnson
Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, 601 Elmwood Ave. Box 626, Rochester, NY 14642, USA
DOI:10.4103/2152-7806.117412
Copyright: © 2013 Johnson M This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Johnson M, Mazariegos J, Lewis PJ, Pomakova D. Crystal storing histiocytosis presenting as a temporal lobe mass lesion. Surg Neurol Int 31-Aug-2013;4:112
How to cite this URL: Johnson M, Mazariegos J, Lewis PJ, Pomakova D. Crystal storing histiocytosis presenting as a temporal lobe mass lesion. Surg Neurol Int 31-Aug-2013;4:112. Available from: http://sni.wpengine.com/surgicalint_articles/crystal-storing-histiocytosis-presenting-as-a-temporal-lobe-mass-lesion/
Abstract
Background:Crystal storing histiocytosis (CSH) is a disorder characterized by local or diffuse infiltration of histiocytes containing crystalline inclusions most commonly of immunoglobulin light chain. Involvement of the central nervous system is extremely rare. CSH may be misdiagnosed as an infection or tumor. In patients with involvement of other organs, it is frequently associated with lymphoplasmacytic diseases.
Case Description:A 20-year-old female was evaluated for 2 weeks of progressively worsening headaches. At presentation, she had no history of fevers but reported a sore throat without cough 3-4 days prior. Her past medical history was unremarkable. She denied intravenous drug use or sexually transmitted diseases but lived with an individual with a history of fungal meningitis. On examination she was afebrile, alert, and oriented with a blood pressure of 110/70 mmHg. She had no adenopathy or neurological deficits. Her white blood cell count was minimally elevated. Magnetic resonance imaging revealed a 3.5 × 1.3 × 1.9 cm contrast enhancing lesion of the left temporal lobe with a mild midline shift. Evaluation by multiple specialists suggested a differential diagnosis of an infectious or neoplastic process. Cultures for infectious agents were negative. The biopsy showed CSH. Postoperatively and at 1 month follow up, she was neurologically intact.
Conclusion:Radiographically and intraoperatively, CSH may mimic an infectious process or neoplasm. Its recognition is critical to facilitate appropriate therapy and prompt screening for an occult lymphoplasmacytic neoplasm, plasma cell dyscrasia or other underlying disease.
Keywords: Crystals, crystal storing histiocytosis, histiocytes, pseudotumor
INTRODUCTION
Crystal storing histiocytosis (CSH) is a disorder characterized by local or diffuse accumulation of histiocytes containing crystalline inclusions with a review and classification recently proposed by Dogan et al.[
CASE REPORT
A 20-year-old female was evaluated at an ambulatory care center for headaches that had become progressively worse over the prior 2 weeks. She had been treated for mycoplasma infection by a primary care physician. At presentation, the headaches were reportedly severe. The patient had no history of fevers but reported a sore throat 3-4 days prior without a cough or other discomfort. Her past medical history had no notable illnesses. Regarding her social history, she lived on a farm with a friend who had been treated for fungal meningitis in the past. No tobacco, alcohol, or intravenous drug use was admitted. She denied exposure or prior treatment for sexually transmitted diseases. She had no significant past medical history. On examination she was afebrile with a blood pressure of 110/70 mmHg. She was alert and oriented with no adenopathy. A complete blood count revealed a white blood cell (WBC) of 12.4 (4.2-9.1 thousand/microliter), 87% neutrophils with no bands. Serology was negative for mycoplasma and HIV-1. Her neurological exam was normal. Magnetic resonance imaging revealed a 3.5 × 1.3 × 1.9 cm contrast enhancing lesion of the left temporal lobe with a mild midline shift [
The mass was resected and aerobic, anaerobic, mycobacterial, and fungal cultures were taken. Anaerobic and aerobic cultures as well as gram, modified acid fast bacillus, and fungal stains were negative. Brain tissue polymerase chain reaction analysis at the state Department of Public Health laboratory public was negative for infectious agents. Due to initial uncertainty about the diagnosis and anticipation of the need for multiple studies in the laboratories, she was postoperatively treated with vancomycin IV for 10 days. Postoperative scans revealed gross total resection. On postoperative day 1 and 30 she was neurologically intact.
The sections revealed a fibrotic lesion with an extensive chronic inflammatory infiltrate of CD138-immunoreactive plasma cells, CD68-immunoreactive macrophages and monocytes and S100-immunoreactive histiocytes [
Figure 2
Brain lesion with crystal storing histiocytosis. (a) Numerous histiocytes contain rhomboid and needle-like kappa light chain restricted crystals are inconspicuous with standard stains (Hematoxylin and eosin, original magnification ×200). (b) Crystal deposition is apparent with periodic acid Schiff stain (PAS stain, original magnification ×200)
DISCUSSION
Recognition of CSH is important due to the association with occult but potentially treatable lymphoplasamcytic disorders. Nonetheless, in CSH involving the CNS, the limited number of cases suggests that many may not have systemic disease. Approximately 90% of CSH occurs in a setting of plasma cell dyscrasias, myeloma, or lymphomas. However, occasionally it develops in a setting of plasma cell granulomas, atypical infections, and other inflammatory processes such as rheumatoid arthritis, mastocytosis, Crohn's disease, and hypereosinophilic syndrome.[
Systemic CSH may present as either localized or multifocal/a multiorgan disease. Most reported and the present case of CSH presented as mass lesions in the cerebral hemispheres.[
The crystalline material in histiocytes is most commonly kappa light chain but, as in the present case, can be lambda light chain. The pathogenesis of this crystal formation remains speculative, attributed in some cases to over production or decreased clearance.[
Because an associated lymphoplasmacytic disorder may not be recognized and the crystals are hard to visualize in hematoxylin and eosin-stained sections, the diagnosis may be inapparent. The differential diagnosis includes a number of entities with a lymphoplasmacytic and histiocytic infiltrate. These include the aforementioned lymphoplasmacytic disorders, amyloidoma, Erdheim–Chester and Rosai–Dorfman disease, xanthogranulomas, rare drug reactions as from Clofazimine, and infections.[
References
1. Dogan S, Barnes L, Cruz-Vetrano WP. Crystal-storing histiocytosis: Report of a case, review of the literature (80 cases) and a proposed classification. Head Neck Pathol. 2012. 6: 111-20
2. Kaminsky IA, Wang AM, Olsen J, Schechter S, Wilson J, Olson R. Central nervous system crystal-storing histiocytosis: Neuroimaging, neuropathology and literature review. Am J Neuroradiol. 2011. 32: E26-8
3. Lebeau A, Zeindl-Eberhardt E, Muller EC, Muller-Hocker J, Jungblut PR, Emmerich B. Generalized crystal-storing histiocytosis associated with a monoclonal gammopathy: Molecular analysis of a disorder with rapid clinical course and review of literature. Blood. 2002. 100: 1817-27
4. Orr BA, Gallia GL, Rodriquez FJ. Case 2012-10. 53rd annual diagnostic slide session. Am Assoc Neuropathol. 2012. p.
5. Rodriguez FJ, Gamez JD, Vrana JA, Theis JD, Giannini C, Scheithauer BW. Immunoglobulin derived depositions in the nervous system: Novel mass spectrometry application for protein characterization in formalin-fixed tissue. Lab Invest. 2008. 88: 1024-37