Diagnostically Challenging Spindle Cell Neoplasms of the Retroperitoneum

Surg Pathol Clin. 2015 Sep;8(3):353-74. doi: 10.1016/j.path.2015.05.007.


The diagnostic spectrum of spindle cell neoplasms arising in the retroperitoneum is wide and, in the presence of commonly shared morphologic features, it may be challenging to establish a correct diagnosis in certain cases. Beyond seemingly undifferentiated spindle cell morphology, most neoplasms may reveal distinctive adipocytic, smooth muscle or myofibroblastic or nerve sheath differentiation and show additional diagnostic clues or characteristic molecular abnormalities. Obtaining sufficient and representative biopsy material, a thorough work-up, and extensive sampling of gross specimens followed by a combined histopathologic, immunohistochemical, and, if necessary, molecular work-up of these cases is advisable so as not to miss important diagnostic and/or prognostic indicators.

Keywords: Liposarcoma; Nerve sheath tumor; Retroperitoneum; Smooth muscle tumor; Spindle cell.

Publication types

  • Review

MeSH terms

  • Diagnosis, Differential
  • Humans
  • Leiomyoma / diagnosis
  • Leiomyoma / pathology
  • Leiomyosarcoma / diagnosis
  • Leiomyosarcoma / pathology
  • Liposarcoma / diagnosis
  • Liposarcoma / pathology
  • Neoplasms, Muscle Tissue / diagnosis
  • Neoplasms, Muscle Tissue / pathology
  • Nerve Sheath Neoplasms / diagnosis
  • Nerve Sheath Neoplasms / pathology
  • Neurilemmoma / diagnosis
  • Neurilemmoma / pathology
  • Prognosis
  • Retroperitoneal Neoplasms / diagnosis*
  • Retroperitoneal Neoplasms / pathology
  • Sarcoma / diagnosis*
  • Sarcoma / pathology
  • Soft Tissue Neoplasms / diagnosis*
  • Soft Tissue Neoplasms / pathology