Skip to main content
SpringerLink
Log in

A Case of Generalized Crystal Storage Histiocytosis Presenting as Soft Tissue Sarcoma: Diagnostic Dilemma

  • Medicine
  • Published:
SN Comprehensive Clinical Medicine Aims and scope Submit manuscript

Abstract

Crystal storage histiocytosis (CSH) is a rare clinical entity. It comprises of benign histiocytes showing accumulation of refractile generally immunoglobulin crystals in the cytoplasm. Accumulation of non-immunoglobulin substances is also described, common being clofazimine, charcot-leyden, and cystine. Here, we present a case of generalized CSH presenting as a chest wall mass in a 62-year-old gentleman. X-ray showed a chest wall mass with destruction of the right sixth rib. Histopathological examination revealed plasmacytoma with the presence of histiocytes showing abundant pink cytoplasm. Bone marrow examination revealed the presence of plasma cells with dense azurophilic granules. The patient was started on chemotherapy for multiple myeloma. It is clinically important to identify these cases as part of published literature since it annotates poor prognosis to the generalised form of CSH compared to localized one. The unusual location, presence of morphologically distinct plasma cells in marrow, made the diagnosis challenging. The case also highlights the role played by immunohistochemistry to arrive at the right diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

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. https://doi.org/10.1007/s12105-011-0326-3.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Fang H, Chiu A, Reichard KK. Crystal-storing histiocytosis in bone marrow. A clinicopathologic study of eight cases and review of the literature. Am J Clin Pathol. 2018;149:148–63n. https://doi.org/10.1093/AJCP/AQX150.

    Article  PubMed  Google Scholar 

  3. Kuyama J, Kosugi S, Take H, Matsuyama T, Kanayama Y. Non-secretory multiple myeloma with azurophilic granules and vacuoles: an immunological and ultrastructural study. Intern Med. 2004;43:590–4.

    Article  Google Scholar 

  4. Lebeau A, Zeindl-Eberhart E, Müller EC, Müller-Höcker J, Jungblut PR, Emmerich B, et al. Generalized crystal-storing histiocytosis associated with monoclonal gammopathy: molecular analysis of a disorder with rapid clinical course and review of the literature. Blood. 2002;100:1817–27.

    Article  CAS  Google Scholar 

  5. Kanagal-Shamanna R, Xu-Monette ZY, Miranda RN, Dogan A et al. Crystal-storing histiocytosis: a clinicopathological study of 13 cases. Histopathology. 2016; 68:482–491.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Laboratory Medicine, Basvatarakam Indo American Cancer hospital and Research Institute, Rd No 14 Banjara Hills, Hyderabad, 500 034, India

    Manasi C. Mundada, Faiq Ahmed & Sudha Murthy

  2. Department of Medical Oncology, Basavatarakam Indo American cancer hospital and Research Institute.Road No 10, Banjarahills, Hyderabad, 500034, India

    Senthil Rajappa

Authors
  1. Manasi C. Mundada
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Faiq Ahmed
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sudha Murthy
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Senthil Rajappa
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Manasi C. Mundada.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Ethical Approval

Ethical approval was obtained.

Informed Consent

Informed consent was obtained.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Medicine

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mundada, M.C., Ahmed, F., Murthy, S. et al. A Case of Generalized Crystal Storage Histiocytosis Presenting as Soft Tissue Sarcoma: Diagnostic Dilemma. SN Compr. Clin. Med. 2, 228–231 (2020). https://doi.org/10.1007/s42399-019-00201-2

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s42399-019-00201-2

Keywords

Search

Navigation