This neoplasm is not reportable.

Name

Paraneoplastic syndromes

Reportability

This neoplasm is not reportable

Abstractor Notes

Plasma cell neoplasms with associated paraneoplastic syndrome are part of the Plasma cell neoplasms and other diseases with paraproteins lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B17)

This is a group of rare paraneoplastic syndromes associated with plasma cell neoplasms. These syndromes are not reportable; however, these may occur in the setting of a reportable neoplasm. If that happens, code to the reportable neoplasm.

AESOP syndrome (adenopathy and extensive skin patch overlying plasmacytoma). Rare occurrence in the presentation of solitary plasmacytoma.

POEMS syndrome (see POEMS entry)

TEMPI syndrome (see TEMPI entry)

Definition

This is a group of rare paraneoplastic syndromes associated with plasma cell neoplasms: POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes), TEMPI syndrome (telangiectasias, elevated erythropoietin and erythrocytosis, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting), and AESOP syndrome (adenopathy and extensive skin patch overlying plasmacytoma). (WHO 5th edition)

Signs and Symptoms

Arthralgias
Bone pain
Chronic progressive polyneuropathy
Clubbing
Edema
Endocrinopathy
Fatigue
Organomegaly
Papilledema
Radiographic bone abnormalities
Serous cavity effusions
Skin changes
Thrombocytosis
Weight loss

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Plasma cell neoplasms and other diseases with paraproteins
Pages: Part B: 631-633

PDQ® Adult Treatment Editorial Board. PDQ Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment. Bethesda, MD: National Cancer Institute. Updated <03/28/2025>. Available at: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq. Accessed <03/30/2025>. [PMID: 26389362]
Section: Plasma Cell Neoplasms (Including Multiple Myeloma) Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary