Lymphoplasmacytic inflammatory pseudotumor of the liver

Orpha code: 555437OMIM code:

Definition

A subtype of inflammatory pseudotumor of the liver characterized by a benign, well-circumscribed tumor with diffuse lymphoplasmacytic infiltration with histological features of IgG4-related disease (numerous IgG4-positive plasma cells, prominent eosinophils, stromal fibrosis, fibroblastic proliferations and, frequently, obliterative phlebitis), and that is likely located around the hepatic hilum. Most often it is discovered as an incidental finding.

Disease data
Classification

Clinical subtype

Synonyms
IgG4-related inflammatory pseudotumor of the liver
Guz rzekomy limfoplazmatyczny zapalny wątroby związany z IgG4
ORPHA code
555437
OMIM code
-
ICD10 code
K75.8
ICD11 code
-

No additional description.

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