Adult orbital langerhans cell histiocytosis with frontal bone involvement.

A 28-year-old woman presented with a 2-week history of right upper eyelid swelling and intermittent frontal headaches. CT demonstrated an ill-defined superior right orbital mass with adjacent right frontal bone erosion and undeveloped frontal sinuses. The orbital biopsy revealed tissue strongly positive for CD1a and S100, diagnostic of Langerhans cell histiocytosis. The systemic workup was negative for multifocal lesions and for diabetes insipidus. In addition to subtotal resection, the patient was treated with a 6-month course of oral prednisone and intravenous vinblastine.

Isolated lymphoma of the anterior visual pathway diagnosed by optic nerve biopsy.

A 72-year-old previously healthy man developed rapidly progressive visual loss, and brain imaging showed features suggestive of a malignant glioma of the anterior visual pathway. Biopsy of one optic nerve yielded a diagnosis of lymphoma. There was no evidence of an extracranial non-Hodgkin lymphoma, so the conclusion was that this represented a primary central nervous system lymphoma (PCNSL). PCNSL isolated to the optic chiasm has been described only once in an immunocompetent patient. Our patient is unusual in that the lymphoma involved the optic nerve, chiasm, and tract in an immunocompetent patient.

Neuro-Behçet disease presenting with acute psychosis in an adolescent.

Behçet disease is a systemic vasculitis of unknown etiology that can affect the neurologic system. Neuro-Behçet disease is not well defined in children and adolescents, and the diagnosis is difficult to make in this population as they often present with insufficient symptoms to meet diagnostic criteria. Psychiatric symptoms as the initial manifestation of neuro-Behçet disease has rarely been reported. We describe a 17-year-old boy who presented with acute psychosis and was subsequently diagnosed with neuro-Behçet disease. A rare combination of both cerebral venous thrombosis and parenchymal central nervous system involvement was identified by neuroimaging. Although treatment guidelines for neuro-Behçet disease are limited, the patient made demonstrative clinical and radiographic improvement with a combination of corticosteroids, anticoagulation, and immunosuppressants, including a tumor necrosis factor-α (TNFα) blocking agent.

Orbital compression syndrome in sickle cell disease.

PURPOSE: To present 3 cases of orbital compression syndrome caused by infarction of the greater wing of the sphenoid in patients with sickle cell disease. METHODS: Case report and review of the literature. RESULTS: Three patients with sickle cell disease (2 males aged 22 and 16 years, and a 10-year-old girl) who presented with proptosis, limited ocular motility, and chemosis were found to have an infarction of the marrow space of the greater wing of the sphenoid that produced an orbital subperiosteal hemorrhage and exudate demonstrated on MRI. Two patients suffered compressive optic neuropathy; both patients recovered normal optic nerve function. Orbital edema resolved within 48 hours of receiving 1 g methylprednosolone daily. The third patient had normal optic nerve function and his orbital edema improved with methylprednisolone 250 mg/day and intravenous Kefzol over 3 days. In the literature, there are 27 similar cases; 5 were treated surgically and the remainder were managed medically. CONCLUSIONS: Patients with sickle cell disease are at risk for orbital compression syndrome secondary to orbital bone infarction, in the setting of vaso-occlusive crises. This diagnosis should be considered when a patient with sickle cell disease presents with headache, proptosis, decreased motility, and/or optic nerve compromise.

Osteosarcoma of the lumbosacral spine invading the central venous pathways, right-sided cardiac chambers, and pulmonary artery.

We report an unusual case of lumbosacral osteogenic sarcoma with cauda equina syndrome and invasion into the central venous and cardiac system. A 41-year-old Hispanic man presented to the emergency department complaining of severe low back pain, cauda equina syndrome, bilateral lower extremity edema, and an extra heart sound on physical examination. CT of the lumbosacral spine done in the emergency department demonstrated a sclerotic lesion in the sacrum with cortical destruction, extension into the spinal canal and a bulky soft tissue mass containing calcifications. Supplemental MRI demonstrated marrow replacement of L4, L5, and the sacrum, soft tissue extension of the tumor, and invasion iliac veins extending into the IVC; however, the full extent of the intravascular tumor was not seen on this examination. Surgical laminectomy and biopsy of the spinal tumor provided the diagnosis of osteogenic sarcoma. A transthoracic echocardiogram was performed while the patient was recovering due to nonsustained ventricular tachycardia, which showed an echogenic mass within the right atrium and ventricle. CT pulmonary angiogram confirmed the echocardiogram showing a tumor extending through the pulmonary valve into the main pulmonary artery. The patient underwent en bloc resection of the tumor from the venous and cardiac systems. Histologic examination of the tumor confirmed osteogenic sarcoma. While vertebral osteogenic sarcoma is uncommon, invasion of the spinal canal is common in these tumors. However, tumor extending into the central venous and cardiac system is rare. The previously reported cases of central venous and cardiac involvement have been related to distant metastases or primary cardiac osteosarcomas. There is only one other reported case of direct extension into the venous system by an iliac bone osteosarcoma in an adolescent; however, the tumor did not extend into the pulmonary circulation.