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Indeterminate cell tumor: a rare dendritic neoplasm.
Sherif A Rezk1, Dominic V Spagnolo, Russell K Brynes
1Department of Pathology and Laboratory Medicine, University of California Irvine (UCI), Irvine, CA, USA.
The American Journal of Surgical Pathology
|September 25, 2008
Summary
Indeterminate cell tumor (ICT) is a rare dendritic cell neoplasm. This study defines ICT, noting its association with B-cell lymphoma and distinguishing it from Langerhans cell tumors by the absence of Birbeck granules.
Area of Science:
- Dermatopathology
- Hematopathology
- Oncology
Background:
- Indeterminate cell tumor (ICT) is a rare neoplastic dendritic cell disorder.
- Its histogenesis and pathogenesis are poorly understood, with unclear relationships to Langerhans cells.
- ICTs mimic Langerhans cells immunophenotypically (CD1a+, S-100+) but lack Birbeck granules.
Purpose of the Study:
- To further define Indeterminate cell tumor (ICT).
- To investigate the relationship between indeterminate cells and Langerhans cells.
- To report clinical, morphologic, immunophenotypic, and ultrastructural features of 5 ICT cases.
Main Methods:
- Case series analysis of 5 ICT patients.
- Immunophenotypic analysis (CD1a, S-100, Langerin).
- Ultrastructural examination for Birbeck granules.
- Fluorescence in situ hybridization (FISH) for t(14;18) and PCR for immunoglobulin gene rearrangement.
Main Results:
- Four of 5 patients were female, and 4 were older than 68 years.
- Cutaneous lesions (3/5) and cervical lymph node involvement (2/5) were observed.
- All cases expressed CD1a and S-100, lacked Langerin and Birbeck granules.
- Two cases showed association with B-cell lymphoma (t(14;18) and kappa light chain rearrangement).
Conclusions:
- ICT is a rare neoplasm that can arise de novo or in association with B-cell lymphoma.
- The absence of Birbeck granules is a key diagnostic criterion for ICT.
- Langerin immunostaining can serve as a surrogate marker for the absence of Birbeck granules.
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