Last reviewed: 19 Sep 2021
Last updated: 24 Sep 2021

Summary

Definition

History and exam

Key diagnostic factors

  • mass
  • upper/lower gastrointestinal bleed
  • rash
  • purplish macular-papular lesions
  • dysfunctional uterine bleeding
  • increased abdominal girth
  • history 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
  • lymphoedema
  • history of exposure to chemical carcinogens

Diagnostic investigations

1st investigations to order

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

Investigations to consider

  • ultrasound of primary tumour
  • chest x-ray
  • PET scan
  • endoscopy
  • biopsy for histology
  • FBC
  • urea
  • creatinine
  • LFTs
  • echocardiogram or multi-gated acquisition (MUGA) scan
  • gene rearrangement testing

Treatment algorithm

Contributors

Authors

James S. Hu, MD, FACP

Associate 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 James S. Hu, Dr Swati Sikaria, 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 topic.

Disclosures

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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