Gary S. Pearl

Middle ear adenoma.

Middle ear adenoma is a benign tumor of the middle ear that can have exocrine (mucinous) and/or neuroendocrine differentiation. Early authors described a separate tumor with predominantly neuroendocrine differentiation as a middle ear carcinoid tumor, but these are now known to be the same tumor. We review the literature of this tumor, including the clinical presentation, gross pathology, histopathology, immunohistochemistry, differential diagnosis, and prognosis.

Sudden death in a 27-year-old man with Chiari I malformation.

We present a case of a witnessed sudden death of a 27-year-old adult man with no antecedent trauma who subsequently was found to have a previously undiagnosed Chiari I malformation. Cases of sudden unprovoked respiratory collapse in children and adults with Chiari I malformation have been well documented, leading to death in some children. There have also been rare examples of sudden death in adults with Chiari I malformation; however, these decedents experienced recent trauma. This is a unique example of a witnessed sudden death of an adult with previously undiagnosed Chiari I malformation in the absence of trauma.

Intussusception due to Yersinia enterocolitica enterocolitis in a patient with beta-thalassemia.

Patients who are homozygous for thalassemia major are at risk for Yersinia enterocolitica infections. We present a case of a 4-year-old child with intussusception of the terminal ileum whose past medical history was significant for beta-thalassemia. His monthly blood transfusions for this condition may have put him at risk for Y enterocolitica enterocolitis. The pathogenesis of this disease relates to the role of iron as an essential growth factor for Yersinia, and this patients transfusions left him in an iron-overloaded state, despite treatment with Desferal. Our patients unusual presentation of intussusception was secondary to the mass effect caused by lymphoid hyperplasia, specifically hypertrophied Peyers patches in the ileum caused by Y enterocolitica infection. To our knowledge, this is the first such case of intussusception caused by Yersinia to be reported.

Review of Pineal Anlage Tumor With Divergent Histology

Pineal anlage tumor is an extremely rare tumor that is not listed in the 2000 World Health Organization Classification of nervous system tumors. It has been defined as a primary pineal tumor with both neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. We review the literature on this tumor, including the clinical presentation, gross pathology, histopathology, immunohistochemistry, differential diagnosis, and prognosis.