Ciaran J. Powers, Catherine C. Pizzi, Thomas J. Cummings, and Allan H. Friedman
✓ The authors report on an unusual case of a primary intracranial myxoma in a 39-year-old woman. The patient presented with headache and generalized seizure. Magnetic resonance imaging revealed a large right frontal tumor resembling a parasagittal meningioma. A gross-total resection was performed, and histological review confirmed the lesion as a myxoma. Results of additional workup revealed the absence of a primary myxoma elsewhere. This case represents the third published report of a primary intracranial myxoma and the second report of a supratentorial myxoma.
Case report and review of the literature
Isaac O. Karikari, Lee A. Selznick, Thomas J. Cummings, and Timothy M. George
✓A 3-month-old boy presented to the pediatric neurosurgery service with central hypotonia. Magnetic resonance images of the brain revealed a homogeneously enhancing dumbbell-shaped mass located in the fourth ventricle and extending into the left cerebellopontine angle (CPA). A suboccipital craniotomy was performed and a gross-total resection of the mass was achieved. Pathological examination of the resected tissue confirmed the diagnosis of capillary hemangioma. To the authors’ knowledge, this is the first reported case of a capillary hemangioma occurring in this location. The authors provide a review of the current literature on intracranial capillary hemangiomas and conclude that, despite the rarity of these lesions, they should be considered in the differential diagnosis of lesions in the fourth ventricle and/or the CPA in children.
Matthew J. McGirt, Ketan R. Bulsara, Thomas J. Cummings, Kent C. New, Kenneth M. Little, Henry S. Friedman, and Allan H. Friedman
Object. The prognostic value of differentiating between recurrent malignant glioma and a lesion due to radiation effect by performing stereotactic biopsy has not been assessed. Thus, this study was undertaken to determine such value.
Methods. Between 1995 and 2001, 114 patients underwent magnetic resonance (MR) imaging—guided stereotactic biopsy to differentiate lesions caused by a recurrence of malignant astrocytoma and by radiation effect. All patients had previously undergone tumor resection (World Health Organization Grade III or IV) followed by radiotherapy. Disease diagnosis based on biopsy and patient characteristics were assessed as predictors of survival according to results of a multivariate Cox regression analysis. The diagnosis determined with the aid of biopsy was compared with that established during a subsequent resection in 26 patients.
Survival following stereotactic biopsy was markedly increased in patients suffering from radiation effect compared with those harboring recurrent malignant glioma (p < 0.0001). In patients with radiation effect on biopsy, an increasing patient age (p < 0.05), having had two compared with one prior resection (p < 0.05), and a decreasing time from radiotherapy to biopsy (p < 0.001) were factors associated with decreased survival. Nevertheless, in patients with biopsy-defined radiation effect at second progression or with an age younger than 50 years the survival rate remained higher than that in patients with recurrent tumor on biopsy (p < 0.01). A biopsy-based diagnosis of radiation effect obtained less than 5 months after radiotherapy was not associated with an increased rate of patient survival compared with a diagnosis of recurrent malignant glioma on biopsy (p = 0.286). Eighty-six percent of lesions initially determined to be due to radiation effect on biopsy fewer than 5 months after radiotherapy were characterized as recurrent glioma by a mean of 11 months later. In contrast, only 25% of lesions initially diagnosed as attributable to radiation effect on biopsy more than 5 months after radiotherapy were classified as recurrent glioma a mean of 12 months later (p < 0.05).
Conclusions. With the aid of stereotactic biopsy the authors demonstrated prognostic significance in differentiating recurrent malignant astrocytoma from a lesion due to radiation effect in patients presenting more than 5 months after having undergone radiotherapy. In patients who presented earlier than 5 months after radiotherapy, radiation effect on biopsy was not associated with an improved rate of survival compared with that in patients harboring recurrent malignant astrocytoma.
Peter M. Grossi, Michael J. Ellis, Thomas J. Cummings, Linda L. Gray, Takanori Fukushima, and John H. Sampson
✓Cholesterol granulomas (CGs) are benign lesions resulting from an inflammatory reaction to cholesterol and hemosiderin. These masses most often arise within the temporal bone or nasal sinuses; intracerebral CGs are extremely rare. In this report the authors present an unusual case of a CG arising within the lateral ventricle.
The patient presented with transient hemiparesis and numbness. Computed tomography and magnetic resonance imaging demonstrated a cystic partially enhancing midline mass within the right lateral ventricle, expanding the ventricle and displacing the septum pellucidum. The patient underwent an interhemispheric, transcallosal resection of the lesion. Microscopic examination revealed a granulomatous inflammatory lesion containing cholesterol clefts, macrophages, and hemosiderin. Embedded within the granulomatous response were foci of tiny cystlike structures lined by nonciliated flattened cuboidal epithelium, consistent with the diagnosis of CG.
To the authors' knowledge this is the first reported case of CG presenting as an intraventricular mass. The origin of this lesion is unclear, but it may relate to prior traumatic brain injury. The authors describe the presentation, imaging findings, histopathological characteristics, and surgical treatment of this rare lesion and related pathological entities.
Lefko T. Charalambous, Anirudh Penumaka, Jordan M. Komisarow, Amanda C. Hemmerich, Thomas J. Cummings, Patrick J. Codd, and Allan H. Friedman
Intracranial intravascular papillary endothelial hyperplasia (IPEH), also referred to as Masson’s tumor, is a condition that rarely occurs in the nervous system. IPEH most frequently occurs extracranially in the skin of the face, skull, neck, and trunk and can easily be mistaken clinically, radiologically, and histologically for angiosarcoma, organizing hematoma, or other vascular malformations. IPEH accounts for roughly 2% of all vascular tumors and is extremely rare intracranially, with only 23 reported cases compared with more than 300 cases of IPEH occurring in the skin and subcutaneous tissue. To date, it has never been reported to occur in the pineal region. The authors report the case of a patient with an IPEH in the pineal region who underwent complex resection and experienced reversal of neurological symptoms.
Stephen R. Parker, Peggy Harris, Thomas J. Cummings, Timothy George, Herbert Fuchs, and Gerald Grant
Posterior fossa decompression with duraplasty for Chiari malformation Type I (CM-I) is a common pediatric neurosurgery procedure. Published series report a complication rate ranging from 3% to 40% for this procedure. Historically, many dural substitutes have been used, including bovine grafts, human cadaveric pericardium, synthetic dura, and autologous pericranium. The authors hypothesized that a recently observed increase in complications was dependent on the graft used.
Between January 2004 and January 2008, 114 consecutive patients ≤ 18 years old underwent primary CM-I decompression using duraplasty. Records were retrospectively reviewed for short- and intermediate-term complications and operative technique, focusing on the choice of duraplasty graft with or without application of a tissue sealant.
The average age of the patients was 8.6 years. The dural graft used was variable: 15 were treated with cadaveric pericardium, 12 with Durepair, and 87 with EnDura. Tisseel was used in 75 patients, DuraSeal in 12, and no tissue sealant was used in 27 patients. The overall complication rate was 21.1%. The most common complications included aseptic meningitis, symptomatic pseudomeningocele, or a CSF leak requiring reoperation. The overall complication rates were as follows: cadaveric pericardium 26.7%, Durepair 41.7%, and EnDura 17.2%; reoperation rates were 13%, 25%, and 8.1%, respectively. Prior to adopting a different graft product, the overall complication rate was 18.1%; following the change the rate increased to 35%. Complication rates for tissue sealants were 14.8% for no sealant, 18.7% for Tisseel, and 50% for DuraSeal. Nine patients were treated with the combination of Durepair and DuraSeal and this subgroup had a 56% complication rate.
Complication rates after CM-I decompression may be dependent on the dural graft with or without the addition of tissue sealant. The complication rate at the authors' institution approximately doubled following the adoption of a different graft product. Tissue sealants used in combination with a dural substitute to augment a duraplasty may increase the risk of aseptic meningitis and/or CSF leak. The mechanism of the apparent increased inflammation with this combination remains under investigation.
Austin K. Mattox, Ankit I. Mehta, Peter M. Grossi, Thomas J. Cummings, and D. Cory Adamson
Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, malignant cutaneous tumor that sparingly presents on the scalp. Dermatofibrosarcomas often result from the formation of a fusion oncogene on translocated or supernumerary ring chromosomes 17 and 22, causing the overexpression of PDGFRβ driven by the COL1A1 promoter. Because of uncertainty surrounding appropriate treatment of aggressive scalp DFSP, the authors performed an extensive review of the available data from a MEDLINE (Ovid) search to describe the clinical presentation and treatment options for this rare tumor. Their search identified 39 different cases, including the illustrative case presented in this study.
Adjuvant therapy for this malignant lesion is not universally established in the literature. In the present case, the authors successfully treated a locally invasive scalp DFSP with presurgical therapy that specifically inhibited the PDGFβ receptor. Imatinib significantly shrank the DFSP tumor mass, reduced hypervascularity, reduced metabolic activity on PET scanning, and permitted a safe gross-total resection. Although wide excision and Mohs micrographic surgery remain the standard surgical treatments for DFSP, the authors illustrate that presurgical chemotherapeutic treatment by imatinib provides a critical adjunct to traditional therapy.
Theresa L. Williamson, Andrew Cutler, Mary I. Cobb, Shervin Rahimpour, Eric R. Butler, Stephen C. Harward, Thomas J. Cummings, and Allan H. Friedman
This study describes a patient with an autograft-derived spinal cord mass following transplantation of olfactory mucosa for treatment of cervical spine injury. The authors report the case of a 35-year-old man who suffered a complete spinal cord injury (SCI) at C5–6 in 2001. The patient underwent an olfactory mucosal cell implantation at the location of injury 4 years following initial trauma. Twelve years later, the patient presented with rapidly progressive decline in upper-extremity function as well as neuropathic pain. Imaging revealed a heterogeneously enhancing intramedullary mass from C3 to C7. At surgery, the patient was found to have a posttransplant mucinous mass. Each mucinous cyst was drained and a portion of the cyst wall was removed. Histological examination demonstrated ciliated epithelium-lined fibrous tissue, submucosal glands, and mucoid material, consistent with a transplant-derived tumor. This case report both documents a rare long-term complication of olfactory mucosal cell transplantation and serves as a cautionary tale encouraging prudent use of novel treatments in a vulnerable population of patients with severe SCI.