Synchronous subcutaneous granular cell tumours, a rare presentation

Ann R Coll Surg Engl. 2018 Apr;100(4):e85-e87. doi: 10.1308/rcsann.2018.0016. Epub 2018 Feb 27.

Abstract

We describe a unique presentation of a rare disease presentation of a granular cell tumour. A 36-year-old woman presents with a large symptomatic left flank mass that had been slowly increasing in size. Multiple synchronous subcutaneous masses were found at presentation on the left breast, right auricle and right cheek. After diagnosis of granular cell tumour by core needle biopsy, the masses were excised with histopathological and immunohistochemical analysis of both specimens confirming the presence of non-malignant granular cell tumours. Granular cell tumours are rare Schwann cell derived tumours that are typically asymptomatic and benign. These tumours are most often located in the head and neck, with multifocal disease present in approximately 5-16% of patients. Final pathology is necessary for diagnosis and frozen section is rarely helpful. Malignancy is present in approximately 2% of cases and can be diagnosed by the presence of a high mitotic rate, large nucleoli, necrosis, spindling and pleomorphism are other suspicious features. Granular cell tumours do not generally require adjuvant treatment. The mainstay of therapy is surgical resection with surveillance.

Keywords: Granular cell tumours; Soft tissue neoplasm; Surgical oncology.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy, Large-Core Needle
  • Breast / pathology
  • Breast / surgery
  • Cheek / pathology
  • Cheek / surgery
  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit / genetics
  • Ear Auricle / pathology
  • Ear Auricle / surgery
  • Female
  • Granular Cell Tumor / genetics
  • Granular Cell Tumor / pathology
  • Granular Cell Tumor / surgery*
  • Granulosa Cell Tumor / pathology
  • Granulosa Cell Tumor / surgery*
  • High-Throughput Nucleotide Sequencing
  • Humans
  • Immunohistochemistry
  • Neoplasms, Multiple Primary / genetics
  • Neoplasms, Multiple Primary / pathology
  • Neoplasms, Multiple Primary / surgery*
  • Proto-Oncogene Proteins c-kit / genetics
  • Schwann Cells / pathology
  • Soft Tissue Neoplasms / genetics
  • Soft Tissue Neoplasms / pathology
  • Soft Tissue Neoplasms / surgery*
  • Subcutaneous Tissue / pathology
  • Subcutaneous Tissue / surgery
  • Tuberous Sclerosis Complex 2 Protein
  • Tumor Suppressor Proteins / genetics

Substances

  • Cyclic AMP-Dependent Protein Kinase RIalpha Subunit
  • PRKAR1A protein, human
  • Tuberous Sclerosis Complex 2 Protein
  • Tumor Suppressor Proteins
  • Proto-Oncogene Proteins c-kit

Supplementary concepts

  • Abrikosov's tumor