Atypical teratoid/rhabdoid tumor of the spine in an adult: case report and review of the literature.

Atypical teratoid/rhabdoid tumors (AT/RTs) are rare, malignant brain tumors which occur almost exclusively in infants and young children. There have been only 17 cases of AT/RT in adults reported in the medical literature and the rarity of this tumor makes the diagnosis in adults difficult. We describe a case of an AT/RT of the spinal cord in an adult. A 43-year old woman presented with neck and left upper extremity pain. An MRI demonstrated a mass lesion in the dorsal spinal cord extending from C4 to C6. The patient underwent a C3 through C7 laminectomy. In consultation with senior pathologists at other institutions, the lesion was initially diagnosed as a rhabdoid meningioma. Molecular genetic studies revealed monosomy 22 and loss of expression of the INI1 gene in 22q11.2. Subsequently, immunohistochemical studies revealed the absence of INI1 gene expression in the malignant cells, supporting the diagnosis of AT/RT. The patient underwent three additional surgical procedures for recurrent disease throughout the neuraxis secondary to leptomeningeal spread of the tumor. Despite aggressive surgical resection, adjuvant chemotherapy and radiation therapy, the patient succumbed to the disease two and a half years after her initial presentation. An unrestricted autopsy was performed. To our knowledge, this is the first case of a spinal atypical teratoid/rhabdoid tumor in an adult fully documented with molecular, immunohistochemical, cytogenetic and autopsy findings.

Carcinomatous meningitis arising from primary nasopharyngeal carcinoma.

Carcinomatous meningitis, also known as leptomeningeal metastasis and meningeal carcinomatosis, is the invasion of neoplastic cells into the leptomeninges. Head and neck cancers, especially nasopharyngeal carcinoma, give rise to carcinomatous meningitis very infrequently. In this case report, we present a rare case of carcinomatous meningitis with nasopharyngeal carcinoma as the primary source. In 1987, a 45-year-old white female presented with a few year history of chronic bilateral serous otitis media. She also complained of intermittent diplopia, right facial pain, right-sided headache, and, finally, right facial palsy. The patient was subsequently diagnosed with nasopharyngeal carcinoma by biopsy and treated with radiation as well as chemotherapy. Her neurological symptoms improved, and she did fairly well for several years. However, various neurologic symptoms started to recur, including right facial weakness, right facial numbness in the distribution of all 3 divisions of cranial nerve (CN) V, loss of taste as well as smell, and diplopia. In 1993, magnetic resonance imaging scan of the head revealed recurrence of nasopharyngeal carcinoma with involvement of the ethmoid sinuses as well as extension of the tumor into the frontotemporal leptomeninges. Over the course of the next 3 years, the patient experienced a very gradual decline with involvement of almost all of the CNs (CN I, II, III, V, VI, VII, VIII, IX, X, XII). This case report of carcinomatous meningitis from primary nasopharyngeal carcinoma is one of the few reported in the literature. Although very rare, nasopharyngeal carcinoma can give rise to carcinomatous meningitis, probably by direct invasion of malignant cells. We also review the literature with respect to the diagnosis and treatment of carcinomatosis meningitis.

A Phase II trial of paclitaxel and topotecan with filgrastim in patients with recurrent or refractory glioblastoma multiforme or anaplastic astrocytoma.

PURPOSE: Therapy for high-grade gliomas remains unsatisfactory. Paclitaxel and topotecan have separately demonstrated activity against gliomas. We conducted a Phase II trial of these agents in combination with filgrastim (G-CSF) in patients with recurrent or refractory glioblastoma multiforme or anaplastic astrocytoma. PATIENTS AND METHODS: Adult patients with radiographic evidence of recurrent or progressive tumor following primary therapy were eligible for study. Patients received paclitaxel 175 mg/m2 IV over 3 h on day 1 and topotecan 1.0 mg/m2 IV over 30 min on days 1-5. Filgrastim 5 microg/kg was given days 6-14 for neutrophil support. Treatment cycles were repeated every 21 days. RESULTS: Twenty patients were enrolled on study, and seventeen were considered evaluable for response. Two patients (12/%) exhibited partial remission and seven patients (41/%) exhibited stable disease in response to therapy. Hematologic toxicity was common with 25 /% of patients experiencing grade III or IV leukopenia despite G-CSF support. Two patients died of infectious complications on protocol, prompting suspension of further accrual. CONCLUSION: Paclitaxel and topotecan with G-CSF support exhibits modest activity in adults with recurrent or refractory glioblastoma and anaplastic astrocytoma. The significant hematotoxicity encountered, however, cannot justify further investigation of this combination in patients with high grade brain tumors.