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Primitive neuroectodermal tumor of the gallbladder.

Dong Eun Song¹, GaWon Choi, Sun-Young Jun

¹Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Archives of Pathology & Laboratory Medicine

|April 17, 2004

Summary

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This report details a rare case of primitive neuroectodermal tumor (PNET) in the gallbladder, a location previously undocumented for this cancer. The diagnosis was confirmed through characteristic cell morphology and immunohistochemical markers.

Area of Science:

Oncology
Pathology
Surgical Oncology

Background:

Primitive neuroectodermal tumors (PNETs) are rare malignancies typically arising in the soft tissues or paravertebral regions.
The gallbladder is an exceptionally uncommon site for PNET, with no prior documented cases.

Observation:

A 53-year-old woman presented with a PNET of the gallbladder.
Microscopic examination revealed monotonous small round cells forming Homer-Wright rosettes.
Immunohistochemistry showed strong membranous positivity for MIC2/CD99, neuron-specific enolase, and synaptophysin.

Findings:

The diagnostic criteria for PNET were met based on morphology and immunohistochemical profile.
Differential diagnoses, including lymphoma, rhabdomyosarcoma, and melanoma, were excluded by negative staining for leukocyte common antigen, desmin, and S100.

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The characteristic chromosomal translocations, t(11;22) and t(21;22), were absent, yet the diagnosis was established.

Implications:

This case expands the known anatomical distribution of PNETs.
It highlights the importance of immunohistochemistry in diagnosing PNETs, especially in rare locations and when genetic markers are absent.
Further research may be warranted to understand the specific oncogenesis of PNETs in the gallbladder.