Purpose: Microsatellite instability (MSI) is found in 10%

Intranodal palisaded myofibroblastoma (IPM) is a rare primary nonlymphoid tumor of the lymph node, which can easily be mistaken for other spindle cell tumors. Intranodal palisaded myofibroblastoma is thought to arise from intranodal myofibroblasts, a finding that is supported by its immunophenotype, positive immunostaining for actin and vimentin, and negative immunostaining for desmin. Characterized by a benign clinical course, IPM is treated by simple surgical excision. We describe a 49-year-woman, who had cadaveric renal transplantation in 1992 and recurrent IPM 4½ years after its original excision. To our knowledge, this case represents only the second known case of recurrent IPM. The histologic feature of metaplastic bone formation in this case has not been previously described in IPM.

A 44-year-old woman presented with a several month history of fatigue, night sweats, increasing abdominal girth, bilateral leg swelling, and a 25-pound weight loss. Her past medical history was significant for intravenous drug use, a habit she claimed to have discontinued 5 years earlier, and chronic, asymptomatic hepatitis C infection.On physical examination, her vital signs were normal including a temperature of 36.7°C. Generalized, nontender lymphadenopathy was found throughout the cervical, axillary, and inguinal regions bilaterally, the largest nodes measuring approximately 2 × 4 cm. The liver and spleen were noted to be slightly enlarged and nontender, and there was 2+ ankle edema present in the lower extremities.

X-linked lymphoproliferative disease (XLP) is a rare familial disorder resulting in selective immunodeficiency to the Epstein-Barr virus (EBV), characterized by uncontrolled proliferation of EBV-infected lymphocytes. Phenotypes of this disease are variable and include fulminant infectious mononucleosis, hypogammaglobulinemia, and malignant lymphoma. In this article, we describe a case of a previously healthy 4-year-old boy with serologic evidence of acute EBV infection who died of fulminant hepatic failure. Histopathological examination of tissue obtained postmortem showed hemophagocytosis and prominent polymorphous infiltrates associated with necrosis in the liver, spleen, and lymph nodes. Semiquantitative polymerase chain reaction (PCR) utilizing primers complementary to the EBV gene LMP2a performed on samples of liver tissue demonstrated approximately 0.6 copies of the EBV gene per cell. Immunohistochemistry demonstrated light chain restriction and PCR studies of the immunoglobulin V-D-J region revealed two strong bands, consistent with a clonal B cell proliferation. Extended family history revealed that the boy's family was followed by the XLP Registry, which was established in 1978 to follow kindreds with XLP. The genetic abnormality associated with XLP has been localized to the Xq25, allowing RFLP analysis to identify female carriers and affected boys.

No abstract