Last reviewed: 21 Aug 2021
Last updated: 06 Feb 2019

Summary

Definition

History and exam

Key diagnostic factors

  • mass
  • upper/lower GI bleed
  • rash
  • purplish macular-papular lesions
  • dysfunctional uterine bleeding
  • increased abdominal girth
  • hx of HIV infection
  • features of acute abdomen
  • neuropathic pain

Other diagnostic factors

  • weight loss
  • fatigue
  • anorexia
  • abdominal bloating, discomfort, pain
  • unilateral extremity swelling

Risk factors

  • genetically inherited syndromes
  • radiation
  • human herpesvirus-8 (HHV-8) infection
  • congenital disorders
  • lymphedema
  • hx of exposure to chemical carcinogens

Diagnostic investigations

1st investigations to order

  • CT scan of primary tumor
  • MRI of primary tumor
  • CT scan chest
  • HIV test

Investigations to consider

  • ultrasound of primary tumor
  • CXR
  • PET scan
  • endoscopy
  • biopsy for histology
  • CBC
  • BUN
  • creatinine
  • LFTs
  • echocardiogram or multigated acquisition (MUGA) scan
  • genetic testing

Treatment algorithm

Contributors

Authors

James S. Hu, MD, FACP

Assistant Professor of Clinical Medicine

USC Norris Comprehensive Cancer Center

Los Angeles

CA

Disclosures

JSH declares that he has no competing interests.

Swati Sikaria, MD

Fellow

USC Norris Comprehensive Cancer Center

Los Angeles

CA

Disclosures

SS declares that she has no competing interests.

Sant P. Chawla, MD, FRACP

Director

Sarcoma Oncology Center

Santa Monica

CA

Disclosures

SPC declares that he has no competing interests.

Acknowledgements

Dr Swati Sikaria, Dr James S. Hu, and Dr Sant P. Chawla would like to gratefully acknowledge Dr Jonathan C. Trent, Dr Saira Hassan, and Dr David Thomas, previous contributors to this monograph. JCT and SH each declare that they have no competing interests. DT has received research support from Pfizer, Amgen, and Novartis.

Peer reviewers

Alfio Ferlito, MD, DLO, DPath, FRCSEd

Director

Department of Surgical Sciences

Professor and Chairman

ENT Clinic

University of Udine

Udine

Italy

Disclosures

AF declares that he has no competing interests.

Adrienne Flanagan, MB BCh FRCPath PhD

Professor of Musculoskeletal Pathology

Institute of Orthopaedics and Musculoskeletal Science

University College London

Stanmore

UK

Disclosures

AF declares that she has no competing interests.

Robert Maki, MB, PhD

Medical Oncologist

Memorial Sloan-Kettering Cancer Center

New York

NY

Disclosures

RM declares that he has no competing interests.

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