title: Paraneoplastic granulocyte colony-stimulating factor secretion in soft tissue sarcoma mimicking myeloproliferative neoplasia: a case report.
abstract: BACKGROUND
  While paraneoplastic leukocytosis is a common phenomenon in solid tumors, extreme elevations of white blood counts (WBC) in the range of more than 100,000/μl are uncommon in patients with non-hematologic malignancies. Leukocytosis with mature neutrophils due to a granulocyte colony-stimulating factor (G-CSF) producing tumor is only seen on rare occasions.
  
  
  METHODS
  Massive neutrophil leukocytosis of approximately 100,000/μl was diagnosed in a 57-year-old Caucasian woman with metastatic undifferentiated endometrial sarcoma. A bone marrow trephine biopsy revealed massively increased granulopoiesis, but no evidence of monoclonal myeloproliferative disease. After the primary tumor had been resected, white blood count (WBC) plummeted and went back to nearly normal levels within one week. With progressive metastatic disease, granulocyte colony-stimulating factor (G-CSF) plasma levels were found to be increased by 10-fold. White blood count (WBC) strictly correlated with tumor burden and response to chemotherapy. In the final stage of treatment resistent disease, white blood count (WBC) approximated 300,000/μl.
  
  
  CONCLUSIONS
  We report on a granulocyte colony-stimulating factor (G-CSF) secreting undifferentiated endometrial sarcoma, which was associated with extreme neutrophil counts. White blood count (WBC) were closely correlated with tumor burden and associated with an aggressive clinical course. We suggest that paraneoplastic neutrophilia represents a poor prognostic sign in soft tissue sarcoma. In patients with similar constellations, antitumor therapy must not be delayed.
authors.forename: Christiane
authors.initials: C
authors.lastname: Dorn
authors.forename: Stefanie
authors.initials: S
authors.lastname: Bugl
authors.forename: Elke
authors.initials: E
authors.lastname: Malenke
authors.forename: Martin R
authors.initials: MR
authors.lastname: Müller
authors.forename: Katja C
authors.initials: KC
authors.lastname: Weisel
authors.forename: Ulrich
authors.initials: U
authors.lastname: Vogel
authors.forename: Marius
authors.initials: M
authors.lastname: Horger
authors.forename: Lothar
authors.initials: L
authors.lastname: Kanz
authors.affiliation: Department of Medical Oncology, Hematology, Immunology, Rheumatology and Pulmology, Medical Center II, South West German Comprehensive Cancer Center, University Hospital of Tuebingen, Tuebingen, Germany. hans-georg.kopp@med.uni-tuebingen.de.
authors.forename: Hans-Georg
authors.initials: HG
authors.lastname: Kopp
mesh_terms: D001772:Blood Cell Count; D001853:Bone Marrow / Q000473:pathology / Q000601:surgery; D019046:Bone Marrow Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000378:metabolism*; D006801:Humans; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000097:blood / Q000150:complications* / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D013902:Radiography, Thoracic; D012509:Sarcoma / Q000150:complications* / Q000175:diagnosis* / Q000378:metabolism* / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D016179:Granulocyte Colony-Stimulating Factor