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Villonodular Synovitis

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Allergic reaction to a bovine dural substitute following spinal cord untethering

Case report

Andrew B. Foy, Caterina Giannini, and Corey Raffel

✓Bovine tissues are now routinely used for dural closure in cranial and spinal surgery. The authors report the case of an 18-year-old woman with a history of myelomeningocele who had symptoms of tethered cord syndrome and presented to a regional hospital. At that hospital she underwent a cord untethering procedure. The spinal dura was closed with Durepair, a dural substitute derived from fetal bovine skin. Her postoperative course was complicated by a cerebrospinal fluid leak that was surgically repaired. Following this, she developed erythroderma, intermittent fevers, eosinophilia, and marked elevation in serum immunoglobulin E. She was then transferred to the authors' institution. A skin antigen test to beef was administered, which revealed a positive reaction. A radioallergosorbent test to beef also yielded positive results. She was taken to the operating room for removal of the bovine graft due to concern for an allergic reaction to the graft. The graft material showed evidence of eosinophilic infiltration. Her clinical symptoms and laboratory values all improved after surgery. To the authors' knowledge this is the first reported case of an allergic reaction to bovine-based dural substitutes.

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Ganglioglioma in the cerebellopontine angle in a child

Case report and review of the literature

Brian D. Milligan, Caterina Giannini, and Michael J. Link

✓The authors report a case of a posterior fossa ganglioglioma centered in the cerebellopontine angle occurring in a child. As with cortically based gangliogliomas, the primary therapy is resection. When the tumor presents in the posterior fossa, often only partial resection can be accomplished without significant neurological deficit. The role of adjuvant chemotherapy and radiation therapy remains controversial, although these are usually reserved for high-grade lesions or progressive growth. The literature regarding the natural history, surgical outcomes, and indications for adjuvant therapy is reviewed. Although it occurs rarely, ganglioglioma should be included in the differential diagnosis of a posterior fossa mass in a child or young adult.

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Acute cerebellitis presenting as tumor

Report of two cases

Nelly Amador, Bernd W. Scheithauer, Caterina Giannini, and Corey Raffel

✓A pseudotumoral presentation of acute cerebellitis is rare. The authors report two cases of children with hemicerebel-lar involvement, neither case being postinfectious nor vaccination related. One patient underwent biopsy sampling revealing marked parenchymal and leptomeningeal inflammation, and in the other, near-complete resolution of neuro-imaging abnormalities was demonstrated. The literature is reviewed and therapy considerations are emphasized.

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Pigmented villonodular synovitis of the spine: a clinical, radiological, and morphological study of 12 cases

Caterina Giannini, Bernd W. Scheithauer, Doris E. Wenger, and Krishnan K. Unni

✓ Cases of pigmented villonodular synovitis (PVNS) that affect the axial skeleton are rare and thus information regarding its natural history, treatment, and prognosis remains limited. To characterize this lesion more fully, the authors reviewed their experience with 12 cases of PVNS of the spine (one of which had been previously reported), then reviewed the 11 cases that previously had been reported, and obtained additional follow-up data in six of them. On the basis of the cumulative data provided by these 22 cases, PVNS of the spine appears to occur over a wide range of ages (21–81 years) and, contrary to what was previously suggested, does not show definite gender predilection (12 women and 10 men). The lesion affects the posterior elements of the vertebrae at all levels and involves the facet joints in 89% of cases. Extension into the epidural spine is frequent (70%). Surgical resection appears to be the treatment of choice. Although PVNS of the spine tends to recur locally (18%), repeat surgical excision appears to be curative.

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Letter to the Editor: Fibrolipoma

Mark A. Mahan, Caterina Giannini, and Robert J. Spinner

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Iatrogenic cerebellar implantation of a vestibular schwannoma

Case report

Todd A. Patrick, Caterina Giannini, Michael J. Ebersold, and Michael J. Link

✓ Metastatic seeding or iatrogenic implantation of numerous types of primary central nervous system tumors, typically along cerebrospinal fluid pathways, is a frequently described albeit rare phenomenon and has never been reported in association with vestibular schwannoma (VS). The authors present a case of inadvertent surgical implantation of VS into the cerebellar hemisphere during resection of a recurrent VS in the cerebellopontine angle and internal auditory canal. A 42-year-old man presented with a 2.5-cm right VS that was removed without complication via a retrosigmoid approach. Routine imaging performed 5 years later revealed a 1.5-cm recurrence of the VS that was subsequently removed by reopening the retrosigmoid craniotomy. Five years later—10 years after initial presentation—follow-up imaging revealed a 1-cm recurrence of the VS and a separate 2.2-cm tumor in the inferior cerebellar parenchyma with surrounding edema. Both tumors were removed without complication by reopening the previous retrosigmoid craniotomy. Histological evaluation of these tumors revealed features typical of VS and similar to those of the tissue obtained from the two prior resections. Given the similarities among these tumors in pathological appearance and mitotic index, the presence of the intraparenchymal cerebellar schwannoma was probably due to intraoperative iatrogenic implantation.

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Brain carcinoid metastases: outcomes and prognostic factors

Clinical article

Grant W. Mallory, Shanna Fang, Caterina Giannini, Jamie J. Van Gompel, and Ian F. Parney

Object

Carcinoid tumors are rare and have generally been regarded as indolent neoplasms. Systemic disease is often incurable; however, patients may live years with this disease. Furthermore, metastatic brain lesions are extremely uncommon. As such, few series have examined outcomes and prognostic factors in those with brain involvement.

Methods

The authors performed a retrospective review of patients who underwent primary treatment at Mayo Clinic in Rochester, Minnesota, for metastatic carcinoid tumors to the brain between 1986 and 2011. Progression-free survival (PFS) and overall survival (OS) were analyzed using Kaplan-Meier statistics. Cox proportional hazards were used to determine predictors of survival.

Results

Fifteen patients underwent primary treatment for metastatic carcinoid tumors to the brain between 1986 and 2011. Their mean age was 58 ± 12 years. Eighty percent (n = 12) of patients underwent surgery, whereas 2 received stereotactic radiosurgery and 1 had whole-brain radiation therapy (WBRT) as the primary treatment. The median follow-up duration was 19 months (maximum 124 months). Systemic disease progression occurred in 73% and was the leading cause of death in known cases, while intracranial disease recurred in 40%. The median PFS and OS were 21 and 19 months, respectively. The use of adjuvant WBRT correlated with improved PFS (HR 0.15, CI 0.0074–0.95, p = 0.044). Those who underwent surgery as primary modalities trended toward longer progression-free intervals (p = 0.095), although this did not reach significance.

Conclusions

Metastatic carcinoid disease to the brain appears to have a worse prognosis than that of other extracranial metastases. Although there was a trend toward a survival advantage in patients who underwent surgery and WBRT, further study is needed to establish definitive treatment recommendations.

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Long-term outcomes for low-grade intracranial ganglioglioma: 30-year experience from the Mayo Clinic

Clinical article

Julia J. Compton, Nadia N. Issa Laack, Laurence J. Eckel, David A. Schomas, Caterina Giannini, and Fredric B. Meyer

Object

Gangliogliomas comprise less than 1% of all brain tumors and occur most often in children. Therefore, there are a limited number of patients and data involving the use or role of adjuvant therapy after subtotal resections (STRs) of gangliogliomas. The objective of this study was to examine and review the Mayo Clinic experience of 88 patients with gangliogliomas, their follow-up, risk of recurrence, and the role of radiation therapy after STR or only biopsy.

Methods

Eighty-eight patients with gangliogliomas diagnosed between 1970 and 2007 were reviewed. Data on clinical outcomes and therapy received were analyzed. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival.

Results

The median age at diagnosis was 19 years. The median potential follow-up as of June 2008 was 142 months (range 9–416 months). Fifteen-year overall survival was 94%, median PFS was 5.6 years, with a 10-year PFS rate of 37%. Progression-free survival was dramatically affected by extent of initial resection (p < 0.0001).

Conclusions

This single-institution retrospective series of patients with gangliogliomas is unique given its large cohort size with a long follow-up duration, and confirms the excellent long-term survival rate in this group. The study also shows the importance of resection extent on likelihood of recurrence. Patients with gangliogliomas who undergo STR or biopsy alone have poor PFS. Radiation therapy may delay time to progression in patients with unresectable disease.

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Editorial

Biopsies and neuropathology

Caterina Giannini and Fredric B. Meyer