Tumor-to-tumor metastasis: esophageal carcinoma metastatic to an intracranial paraganglioma.

Tumor-to-tumor metastasis (TTM) is a relatively rare but well-documented phenomenon. The authors report a unique case of esophageal carcinoma metastatic to an intracranial paraganglioma. A sellar and suprasellar tumor was found using MR imaging in an 81-year-old man who presented with a 3-week history of progressive headache and blurred vision. A subtotal excision of the tumor was achieved. Histopathological examination of the tumor disclosed a neoplasm with two distinct components: one showing the classic Zellballen pattern of a paraganglioma, the other exhibiting malignant features leading to the diagnosis of a poorly differentiated carcinoma metastatic to a sellar/suprasellar paraganglioma. The primary esophageal carcinoma was not uncovered until 2 months later, after the patient presented with upper gastrointestinal bleeding. The patient died 4 months after initial presentation. This case expands the spectrum of TTM, and emphasizes the importance of TTM in the practice of pathology.

Bilateral agenesis of the hippocampal dentate gyrus in a neurologically normal adult.

BACKGROUND: Ontogenic development of granule cells in the hippocampal dentate gyrus is influenced by genes including WNT3, EMX2, NEUROD, and LEF1. Dentate granule cells continue to be generated from stem cell precursors postnatally and during adult life, and are implicated in normal and abnormal neurological function. Developmental privation of dentate granule cells is rare and essentially always occurs in the context of other neurodevelopmental abnormalities. We have found no previous reports of severe, selective agenesis of dentate granule cells in humans. METHODS: A gross and microscopic examination of the brain included appropriate histochemical and immunohistochemical preparations and examination of the hippocampal formation at multiple levels bilaterally. RESULTS: This neurologically normal 82-year-old man was found to have bilateral agenesis of the hippocampal dentate gyrus, no identifiable dentate granule cells, and moderate disorganization of the pyramidal cell layer of Ammon's horn. We found no neurodevelopmental abnormalities outside the hippocampus. CONCLUSIONS: The hippocampal architectural alterations in this patient are similar to those associated with a murine Lef1 mutation, but our human case does not have the other congenital deficits reported in the Lef1-null mouse. Bilateral agenesis of the hippocampal dentate gyrus, and apparent failure of regeneration of dentate granule cells from stem cells in adult life, may occur without overt clinical neurological deficits.

Primary angiitis of the spinal cord presenting as a conus mass: long-term remission.

BACKGROUND: Primary angiitis of the central nervous system (CNS) is a rare vasculitic disorder that typically involves the brain and, less frequently, the spinal cord without involvement of the blood vessels outside the CNS. CASE DESCRIPTION: We present a case of a 52-year-old woman who developed a conus syndrome linked to an enhancing mass of her lower thoracic spinal cord, lumbar cord, and conus. Spinal cord biopsy performed for diagnostic purposes in the setting of progressive neurological deficit confirmed angiitis of the spinal cord. Therapy with steroid and cyclophosphamide was associated with long-term (3 years) clinical and imaging remission of the lesion. CONCLUSIONS: The prognosis of primary CNS angiitis is dismal with most cases progressing to death. Long-term remission is unusual. Aggressive therapy with steroid and cytotoxic agents may improve survival.

Neuromyelitis optica with extensive active brain involvement: an autopsy study.

OBJECTIVE: To describe the clinical, molecular, and neuropathological findings of a patient with aquaporin 4-positive relapsing myelitis who developed extensive brain involvement followed by death. DESIGN: Case report. SETTING: Foothills Medical Center, Calgary, Alberta, Canada. PATIENT: A 51-year-old woman with neuromyelitis optica spectrum disorder. RESULTS: Neuropathological examination disclosed neuromyelitis optica lesions, even in areas that appeared normal radiologically and grossly. Immunostaining confirmed the massive disintegration of astrocytes in the acute and chronic lesions, indicating that astrocytes are targeted early in the disease process. Induction of the immune response was demonstrated by reverse-transcriptase polymerase chain reaction analysis of relevant immune response genes. CONCLUSIONS: This article supports and supplements current concepts of astrocyte disintegration in neuromyelitis optica and of immune mechanisms in its pathogenesis.

Neuroinflammation and demyelination in multiple sclerosis after allogeneic hematopoietic stem cell transplantation.

OBJECTIVE: To evaluate the effects of allogeneic hematopoietic stem cell transplantation (allo-HSCT) on the brains of persons with and without multiple sclerosis (MS) by means of postmortem histopathological examination. DESIGN: Postmortem histopathology, case studies, and case-control studies. Patients Four patients with MS who died at a median of 4.5 months (range, 3-9 months) after allo-HSCT for a concomitant hematologic malignant neoplasm; 5 patients without MS who died at a median of 10.0 months (1-29 months) after allo-HSCT; and 5 control subjects without MS who did not undergo allo-HSCT. SETTING: Referral centers. Intervention Allogeneic hematopoietic stem cell transplantation. MAIN OUTCOME MEASURES: Morphological features and immunohistochemical features, including the quantitative measures of chronic inflammatory cells. RESULTS: Demyelinating and inflammatory activities of MS persisted after allo-HSCT in all of the patients with MS. Active and chronic active MS lesions exhibited significantly higher numbers of CD3+ T cells and CD8+ cytotoxic T cells and significantly higher scores of CD68+ microglia/macrophages than did chronic inactive lesions or normal-appearing white matter. The normal-appearing brains of allo-HSCT recipients who did not have MS were found to have significantly higher numbers of CD3+ T cells and CD8+ cytotoxic T cells and higher scores of CD68+ microglia/macrophages compared with the controls; however, no demyelination was identified in these non-MS samples. CONCLUSION: Allo-HSCT fails to halt the demyelination and inflammation of MS.

Multifocal complex glioneuronal tumor in an elderly man: an autopsy study: case report.

OBJECTIVE: The clinicopathological spectra of a dysembryoplastic neuroepithelial tumor (DNT) and a rosette-forming glioneuronal tumor (RGNT) are expanding. We report here the autopsy findings of a case of complex glioneuronal tumor with combined histological features of both a DNT and an RGNT. CLINICAL PRESENTATION: A 79-year-old man presented with a 1-month history of confusion and gait difficulties. A magnetic resonance imaging scan revealed obstructive hydrocephalus attributed to a mass in the posterior third ventricle. INTERVENTION: A third ventriculostomy was performed. Postoperatively, the mass remained unchanged in size for more than 14 months. Thirty-eight months after his initial manifestations, he experienced minor head trauma and was then hospitalized. Despite placement of an external ventricular drain and other supportive treatment, he deteriorated and died. A full autopsy was performed, with emphasis on the brain. The mass lesion and a few independent microfoci situated primarily around the third ventricle showed histological features of pilocytic astrocytoma with recurrent hemorrhage. Far more numerous were microfoci with histological features of a DNT, including floating neurons, as well as typical RGNT-associated, synaptophysin-positive rosettes and perivascular pseudorosettes. CONCLUSION: The advanced age of the patient, the coexisting histological features of the DNT and RGNT, and the distinctive anatomic distribution of the lesions, being centered on the third ventricle, may lend insight into the histogenetic relationship of a DNT, an RGNT, and mixed glioneuronal tumors.