Zapalny guz miofibroblastyczny

Kod Orpha: 178342Kod OMIM:

Definicja

Inflammatory myofibroblastic tumor is a rare neoplastic lesion of the submucosal stroma, which can develop in any organ, often occurring in the lung, mesentery, omentum and the retroperitoneal region. It is histologically heterogenous, composed of spindle-shaped cells, myofibroblasts and inflammatory cells. It is usually benign, however local invasion, recurrence, malignant transformation with vascular invasion and metastases may occur. The presentation is nonspecific and depends on the organ involved. Some patients may present with paraneoplastic syndrome (fever, malaise, weight loss, anemia, thrombocytosis) or symptoms related to compression of adjacent organs, such as bowel obstruction.

Dane
Klasyfikacja

Choroba

Kod ORPHA
178342
Kod OMIM
-
Kod ICD10
D48.7
Kod ICD11
2E92.1

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