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Malignant granular cell tumor of the median nerve: a case report with a literature review of 157 cases

  • Shinji Tsukamoto
  • Maiko Takeda
  • Kanya Honoki
  • Shohei Omokawa
  • Yasuhito Tanaka
Case Report
  • 52 Downloads

Abstract

Malignant granular cell tumors are an extremely rare, high-grade sarcoma with a schwannian phenotype and are composed of malignant granular cells with cytoplasmic lysosomal inclusion. To date, 157 cases of malignant granular cell tumors have been reported. We report the first case of a malignant granular cell tumor arising from the digital nerve to the median nerve in the palm, and we review the 157 previously reported cases and summarize the clinical profile, treatment, and outcome of this tumor. The median age, tumor size, and follow-up periods were 51 years, 6 cm, and 24 months respectively. With respect to the oncological result, 53 patients (33.8%) had no evidence for disease, 31 (19.7%) were alive with the disease, and 51 (32.5%) died because of the disease. Our case report indicates that rare malignant tumors can arise from the digital nerve to the median nerve in the palm, an anatomical site that is usually affected by benign lesions. Exhaustive discussions between surgeons and pathologists are necessary for the treatment of this rare malignant tumor.

Keywords

Malignant granular cell tumor Granular cell tumor Median nerve Nerve sheath tumor 

Notes

Acknowledgements

The authors would like to thank Dr Masanori Hisaoka (Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, Japan) for consultation regarding the pathological diagnosis of this case.

Author statement

All authors had access to the data and a role in writing the manuscript.

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© ISS 2018

Authors and Affiliations

  • Shinji Tsukamoto
    • 1
  • Maiko Takeda
    • 2
  • Kanya Honoki
    • 1
  • Shohei Omokawa
    • 1
  • Yasuhito Tanaka
    • 1
  1. 1.Department of Orthopaedic SurgeryNara Medical UniversityKashihara-cityJapan
  2. 2.Department of Diagnostic PathologyNara Medical UniversityKashiharaJapan

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