Rare intraparenchymal choroid plexus carcinoma resembling atypical teratoid/rhabdoid tumor diagnosed by immunostaining for INI1 protein.

The authors present the case of a rare extraventricular, intraparenchymal choroid plexus carcinoma (CPC). This 6-year-old girl presented to the emergency department with a 1-week history of headaches, nausea, and vomiting. Imaging studies revealed an intraaxial cystic and solid mass located in the right frontal lobe with central nodular enhancement and minimally enhancing cyst walls. Gross-total resection was accomplished via craniotomy without complications. The initial pathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT); however, immunostaining for INI1 protein (using the BAF47/SNF5 antibody) showed retention of nuclear staining in the tumor cells, resulting in a change in the diagnosis to CPC. There was no evidence of recurrence at the last follow-up 2.5 years after treatment, which supports the diagnosis of CPC over AT/RT. This case emphasizes the importance of immunostaining for INI1 protein for distinguishing CPC from AT/RT in cases with atypical or indeterminate features.

Ultrasonographic Tinel sign.

High-resolution ultrasound has become increasingly useful in the evaluation of peripheral nerves. As we have gained experience, clinically helpful ultrasonographic signs have been detected. One of these is the ultrasonographic Tinel sign, in which paresthesias are produced when the transducer compresses a nerve lesion. This sign has not been previously described. We present a case in which the ultrasonographic Tinel sign helped accurately guide a nerve biopsy in a woman with mononeuritis multiplex.

Primary neuroendocrine tumor of the orbit progressing to neoplastic meningitis.

A 60-year-old man presented with multiple cranial neuropathies and an identifiable left orbital lesion along the course of the supraorbital nerve. The pathologic features of the excised orbital lesion were consistent with a poorly differentiated primary neuroendocrine carcinoma. Four years after his diagnosis, the patient succumbed to neoplastic meningitis. No other primary tumor site was identified or clinically apparent during his illness.

Current controversies in the radiotherapeutic management of adult low-grade glioma.

Over the last decade, the results of four prospective clinical trials of supratentorial low-grade glioma (LGG) in adults have been published. The data from the nearly 1,000 patients treated on these studies are summarized in this presentation, addressing the following three current controversies in the radiotherapeutic management of these patients: (1) optimum timing of radiation therapy (RT); (2) optimum RT dose; and (3) addition of chemotherapy to RT. The 5-year overall survival (OS) and progression-free survival (PFS) rates in these four studies ranged from 58% to 72% and from 37% to 55%, respectively. Significant prognostic factors included extent of surgical resection, histology, tumor size, and age. The European Organization for Research and Treatment of Cancer (EORTC) study 22845 randomized 311 adults to postoperative observation or RT. There was no difference in the 5-year OS rate between the two arms, but the irradiated patients had a significantly improved 5-year PFS rate. EORTC study 22844 randomized 379 adults to low- versus high-dose RT. Similarly, an intergroup study conducted by the North Central Cancer Treatment Group (NCCTG), Radiation Therapy Oncology Group (RTOG), and Eastern Cooperative Group (ECOG) randomized 211 adults to low- versus high-dose RT. There was no difference in the 5-year OS or PFS rates between the two dose groups in either study. A Southwest Oncology Group (SWOG) study randomized 60 adults with incompletely resected LGG to RT alone or RT plus lomustine (CCNU) chemotherapy. There was no difference in outcome between the two treatment arms. Further prospective clinical trials are needed to define the optimal management strategy for adults with supratentorial LGG. The schemata from recently completed and ongoing LGG studies will be presented.

Soft tissue amyloidoma of the extremities: a case report and review of the literature.

The deposition of amyloid as a distinct, clinically apparent mass is uncommon, particularly in soft tissues. Among reported sites of soft tissue amyloidomas, the extremities are quite rare. Amyloid tumors can mimic malignant neoplasms both clinically and radiologically. We report a case of AA amyloidoma presenting in the deltoid region with radiological features suggesting sarcoma. Cytomorphology from fine-needle aspiration material, tissue histology, and appearance by magnetic resonance imaging are described. The literature on soft tissue amyloidoma is reviewed.