This neoplasm is not reportable.

Name

POEMS syndrome

Not Reportable

This neoplasm is not reportable

Alternate Names

Crow-Fukase syndrome
Osterosclerotic myeloma
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin

Definition

POEMS syndrome is a paraneoplastic syndrome associated with a plasma cell neoplasm, usually characterized by fibrosis and osteroslcerotic changes in bone trabecula, and often with lymph node changes resembling the plasma cell variant of Castleman disease.

The POEMS acronym stands for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, but these components are not all required for diagnosis.

Abstractor Notes

The mandatory criteria for diagnosis of POEMS syndrome are a chronic progressive polyneuropathy and a monoclonal plasma cell proliferative disorder. There is usually an associated M protein of either IgG, IgA type, with lambda light chain restriction in all cases.

Two thirds of patients with lymphadenopathy have changes consistent with the plasma cell variant of Castleman disease. Osterosclerotic bone lesions are present in > 95% of cases. Organomegaly, primary hepatomegaly or splenomegaly, is present in at least half of patients. Skin changes occur in more than two thirds of cases.

Signs and Symptoms

Bone pain
Chronic progressive polyneuropathy
Clubbing
Endocrinopathy
Organomegaly
Radiographic bone abnormalities
Skin changes
Thrombocytosis
Weight loss

Epidemiology and Mortality

Most common causes of death are cardiorespiratory failure and infection

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature B-cell neoplasms
Pages: 256-257

National Cancer Institute
Section: General Information About Plasma Cell Neoplasms
Pages: https://www.cancer.gov/types/myeloma/hp/myeloma-treatment-pdq
Glossary