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Inflammatory myofibroblastic tumor with CNS involvement.

A Trojan¹, T Stallmach, S Kollias

¹Division of Oncology, Department of Internal Medicine, University Hospital Zurich, Switzerland.

|September 28, 2001

Summary

Inflammatory myofibroblastic tumors (IMT) are rare neoplasms. This case highlights an unusual IMT presentation with metastases and central nervous system dissemination, showing a notable response to chemotherapy.

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Area of Science:

Pediatric Oncology
Surgical Pathology

Background:

Inflammatory myofibroblastic tumors (IMT) are uncommon neoplasms primarily affecting the mesentery and retroperitoneum in pediatric and young adult populations.
Malignant transformation and distant metastases are rare occurrences in IMT.

Observation:

A 16-year-old patient presented with an IMT originating in the mesentery, complicated by mediastinal metastases.
Initial chemotherapy with ifosfamide, dactinomycine, and vincristine resulted in partial remission.

Findings:

The patient experienced a relapse with meningeal infiltration two months post-treatment.
This case exhibited unusual IMT characteristics, including early metastatic spread and central nervous system (CNS) dissemination.

Implications:

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This case underscores the aggressive potential of IMT, even with initial treatment response.
Further research into the biology and treatment of metastatic IMT, particularly CNS involvement, is warranted.