MR imaging of spinal hemangioblastoma.
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Biomedical subjects
Publications and source records attributed to J G Smirniotopoulos.
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OBJECTIVE: We attempted to define the clinical and imaging features of cervical spine chordoma. CONCLUSION: In this series of 10 patients, cervical spine chordomas tended to be large, multilevel destructive lesions of the midcervical region that caused pain and weakness in the neck and shoulder. Typically, these tumors primarily involved the vertebral body and extended into soft tissues of the perivertebral and epidural spaces. Surprisingly, almost a third of the tumors appeared on radiographs to predominantly occupy either the epidural or the intradural space, and nearly half resembled schwannomas. Although no imaging features were pathognomonic, CT and MR imaging were valuable in identifying the tumor, revealing its extent, and defining its relationship to the intraspinal structures.
The current WHO classification greatly advances the ability to predict patient prognosis from pathologic diagnosis by introducing new tumor categories. This improved pathologic stratification is reflected in more accurate interpretations of diagnostic imaging studies. Pathology and oncology have progressed from a gross beginning, through microscopy and special stains, and into the realm of molecular biology and tumor genetics.
The "space approach" will be introduced as a means of analyzing orbital masses on imaging studies. Determination in which space an orbital mass resides, the character of its margin, the presence or absence of bony changes, and evidence of extension of the mass beyond the orbit often allows the radiologist to narrow the differential diagnosis and provides essential information to the ophthalmologist for definitive diagnosis and treatment planning.
MR imaging data were reviewed retrospectively in four male patients (32 to 74 years old) with histologically confirmed intravascular lymphomatosis (IVL), a rare, aggressive form of non-Hodgkin lymphoma. MR findings included infarct-like lesions (n = 2), focal parenchymal enhancement (n = 3), dural/arachnoid enhancement (n = 2), and, in one case, nonspecific, patchy foci of increased signal in the white matter on long-TR images. All patients had multifocal lesions. Knowledge of the spectrum of MR imaging features in this unusual disorder may aid in diagnosis and potentially enhance the role of imaging in following response to therapy.
Periorbital masses are often referred to oculoplastic surgeons. We report a 20-year-old patient presenting with a tender supertemporal mass that on gadolinium-enhanced magnetic resonance imaging (MRI) demonstrated a prominent dural enhancement adjacent to the mass, the so-called "dural tail sign." This sign has been reported to be highly specific for a meningioma; however recent literature challenges this view. In this case as well, the "dural tail sign" was not produced by a meningioma.
Neurofibromatosis type 1 (NF1) in children can produce a variety of parenchymal signal abnormalities on cranial MR. Areas of abnormal signal in these patients may represent regions of disordered myelination, "hamartomatous" change or frank neoplasia. The presence of contrast enhancement in intracranial lesions in patients with NF1 is usually strongly suggestive of tumor. We report the case of a child with NF1 and a focal enhancing brain parenchymal lesion which spontaneously resolved without specific therapy.
We reviewed the clinical records of 149 patients with pathologically proved cauda equina lesions in order to define the relative frequency and clinical presentations of the various diagnoses. The most common pathology was ependymomas (47 patients) followed in frequency by nerve sheath tumors (35 patients), metastases (27 patients), nonependymal glial neoplasms (six patients), meningiomas (six patients), lipomas (five patients), paragangliomas (five patients) and various other diagnoses (19 patients). Mean patient age at presentation for the various lesions included: metastases (51.5 years), nerve sheath tumors (49.7 years), nonependymal glial tumors (46.5 years), paragangliomas (41.2 years), ependymomas (38.3 years), meningiomas (34.7 years), and lipomas (18.4 years). ANOVA showed that the relationship between age and diagnosis for these groups to be statistically significant at a high level (P = 0.002). Low back pain was the most common symptom and occurred in 44 patients. Other symptoms included unilateral lower extremity pain or tenderness (24 patients), bilateral lower extremity pain or tenderness (16 patients), and bilateral lower extremity weakness (16 patients). No relationship between pathologic diagnosis and specific symptoms was found.
PURPOSE: To determine the computed tomographic (CT), magnetic resonance (MR) imaging, and angiographic findings of papillary endolymphatic sac tumors. MATERIALS AND METHODS: Clinical and imaging studies in 20 patients (aged 17-65 years) with histopathologically proved papillary endolymphatic sac tumors were retrospectively reviewed. Patients underwent CT (n = 18), MR imaging (n = 15), or angiography (n = 12). CT scans were evaluated for bone erosion and calcification; MR images, for signal intensity, enhancement patterns, and flow voids; and angiograms, for tumoral blood supply. RESULTS: All tumors were destructive and contained calcifications centered in the retrolabyrinthine region at CT. The MR imaging appearance varied with lesion size; 12 of 15 tumors showed increased signal intensity at T1-weighted imaging. The high-signal-intensity area was circumferential in lesions 3 cm or smaller and was scattered throughout the lesion in advanced tumors. Only tumors larger than 2 cm had flow voids. The blood supply arose predominantly from the external carotid artery. Large tumors had additional supply from the internal carotid and posterior circulation. CONCLUSION: Papillary endolymphatic sac tumors are destructive, hypervascular lesions that arise from the temporal bone retrolabyrinthine region. Increased signal intensity at unenhanced T1-weighted MR imaging is common and may help distinguish these lesions from more common, aggressive temporal bone tumors.
This article is a brief review of the radiologic-pathologic correlation of central nervous lesions occurring in patients with AIDS. The major discussions of the imaging appearance and radiologic differential diagnosis have been presented elsewhere in this issue. Our emphasis is on the gross pathologic correlations that are only possible with autopsy materials. We will illustrate the opportunistic neoplasms such as primary CNS lymphoma. This article also discusses the imaging and pathology of the common opportunistic infections. Toxoplasmosis, an obligate intracellular protozoan, is the most common CNS infection producing a mass lesion in AIDS. However, AIDS encephalitis, a direct infection of the brain by the HIV-1 virus itself, may actually be more prevalent. Other viral infections occurring in AIDS include progressive multifocal leukoencephalopathy. Fungal diseases infecting the central nervous system of AIDS patients include cryptococcus, aspergillosis, and mucormycosis. The primary purpose of this article is to demonstrate how the gross pathology correlates with the radiologic images.
Primary tumors of the spine are relatively infrequent lesions compared with metastatic disease, multiple myeloma, and lymphoma. However, when a solitary lesion of the spine occurs, these neoplasms represent an important group of entities for diagnostic consideration. A wide variety of benign neoplasms can involve the spine, including osteoid osteoma, osteoblastoma, aneurysmal bone cyst, giant cell tumor, enostosis, and osteochondroma. Common primary nonlymphoproliferative malignant neoplasms of the spine include chordoma, chondrosarcoma, Ewing sarcoma or primitive neuroectodermal tumor, and osteosarcoma. The imaging features of these lesions of the spine are often characteristic. These changes include a small sclerotic focus with irregular thorny margins in the vertebral body (enostosis), a small radiolucent nidus with central calcification in the posterior elements of the vertebral body (osteoid osteoma), a large expansile lesion with multiple fluid-fluid levels (aneurysmal bone cyst), and an aggressive mineralized mass (chondroid or osteoid) with osseous and soft-tissue involvement (chondrosarcoma or osteosarcoma). Radiologists should be aware of the appearance of these unusual neoplasms in order to provide a complete differential diagnosis and to guide clinical colleagues in patient treatment.
Astrocytic tumors are divided into two basic categories: circumscribed (grade I) or diffuse (grades II-IV). All diffuse astrocytomas tend to progress to grade IV astrocytoma, which is synonymous with glioblastoma multiforme (GBM). GBMs are characterized by marked neovascularity, increased mitosis, greater degree of cellularity and nuclear pleomorphism, and microscopic evidence of necrosis. Several genetic abnormalities have been associated with the development of GBM: In some cases, the abnormality is inherited (e.g., Li-Fraumeni syndrome); in others, genetic alteration appears to result from mutation into an oncogene or deterioration of the tumor-suppressor gene p53. A common, distinctive histopathologic feature of GBM is pseudopalisading. The most common imaging appearance of GBM is a large heterogeneous mass in the supratentorial white matter that exerts considerable mass effect. Less frequently, GBM can occur near the dura mater or in the corpus callosum, posterior fossa, and spinal cord. GBM typically contains central areas of necrosis, has thick irregular walls, and is surrounded by extensive, vasogenic edema, but the tumor may also have thin round walls, scant edema, or a cystic appearance with a mural nodule. GBMs most commonly metastasize from their original location by direct extension along white matter tracts; however, cerebrospinal fluid, subependymal, and hematogenous spread also can occur. Given the rapidly growing body of knowledge about GBM, the radiologist's role is more important than ever in accurate and timely diagnosis.
The structural organization of the dura and leptomeninges is reflected in its magnetic resonance (MR) imaging appearance in normal and disease states. Two distinct enhancement patterns are characterized: dura-arachnoid enhancement and pia-subarachnoid space enhancement. The dura-arachnoid pattern consists of curvilinear enhancement overlying the brain and immediately deep to the inner table of the calvaria, as well as along the falx and tentorium. Pial enhancement closely follows the brain surface into sulci and outlines the basal cisterns. Recognition of these enhancement patterns and other MR imaging characteristics may enhance the role of MR imaging in the detection, diagnosis, and follow-up of neoplastic and nonneoplastic disorders affecting the meninges. The focus of this article (Part I) is the MR appearance of the normal meninges and nonneoplastic causes of meningeal disease. Part II of this series will discuss neoplastic conditions affecting the coverings of the brain and spinal cord with an emphasis on pathways of disease spread.
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PURPOSE: To describe the MR and CT imaging features of hemangiopericytoma and to identify the characteristics that might distinguish them from meningioma. METHODS: We retrospectively reviewed the CT and MR findings in 34 pathologically proved cases of hemangiopericytoma. We evaluated the size, shape, and location of the tumor; the presence of hydrocephalus, edema, and mass effect; the type of dural attachment (broad-based or narrow-based) and bone changes (erosion, hyperostosis); and the tumor's density, signal, and contrast-enhancement characteristics. RESULTS: Thirty of 34 tumors were 4 cm or more in greatest dimension, 32 were lobular, and only seven were in the posterior fossa. Hydrocephalus was present in 18, edema in 30, and mass effect in 33. Twenty-three had broad-based dural attachment and 11 had narrow-based attachment. All 26 unenhanced CT scans showed hyperdense tumors; 19 were heterogeneous and seven homogeneous. All 27 contrast-enhanced CT scans showed enhancement; 17 were heterogeneous and 10 homogeneous. Bone erosion was present in 17 of 29 hemangiopericytomas imaged with CT. None had hyperostosis or tumor calcifications. On T1-weighted MR images, 13 of 17 tumors were isointense with cortical gray matter; on T2-weighted image, 10 of 17 were isointense. All 14 tumors imaged with contrast enhanced T1-weighted MR imaging showed enhancement, and 13 of these were heterogeneous; eight of the 14 had a "dural tail" sign. CONCLUSION: Intracranial hemangiopericytomas are multilobulated, extraaxial tumors, sometimes associated with narrow-based dural attachment and bone erosion. Unlike with meningiomas, hyperostosis and intratumoral calcification are not present.
BACKGROUND: Psammomatoid ossifying fibromas represent a unique subset of fibro-osseous lesions of the sinonasal tract. They have distinctive histomorphologic features and a tendency toward locally aggressive behavior, including invasion and destruction of adjacent anatomic structures. METHODS: Seven cases of psammomatoid ossifying fibromas of the sinonasal tract were identified in the files of the Otolaryngic Tumor Registry at the Armed Forces Institute of Pathology. Medical records, including the clinical history, location of the lesions, radiographs, treatment, and follow-up were reviewed in each case. Follow-up information was available in all of the cases. RESULTS: Four of the patients were male and three were female. The patient's ages ranged from 5 to 54 years (median age, 33 years). Symptoms included facial swelling, nasal obstruction, pain, sinusitis, headache, and proptosis. Radiographic studies confirmed the presence of an osseous and/or soft tissue mass varying in appearance from well demarcated without invasion or erosion to invasive with bone erosion and intracranial extension. Sites of involvement included the nasal cavity and all paranasal sinuses, particularly the ethmoid and maxillary sinuses. Often, more than one sinus was involved and extension of disease included involvement of the orbit, nasopharynx, palate, and anterior cranial fossa. The histologic appearance was characterized by the presence of small mineralized (psammomatoid) bodies admixed with a cellular stroma with a variable amount of myxomatous material and scattered giant cells. Confusion with other osseous and soft tissue tumors may occur resulting in too limited or too aggressive management. En bloc surgical excision is the treatment of choice and may prove curative. Aggressive behavior with recurrence(s) or invasion into adjacent structures occurred. At the time of this writing, the patients are alive over follow-up periods ranging from 6 months to 7 years. CONCLUSIONS: Gnathic and midfacial fibro-osseous proliferations are a diverse group of lesions. A subset of these fibro-osseous lesions with predilection for the sinonasal tract were identified. These lesions are characterized by their distinctive histology, including psammomatoid ossicles and their locally aggressive growth. Complete surgical removal is the treatment of choice.
Dermoids and epidermoids are ectoderm-lined inclusion cysts that differ in complexity: Epidermoids have only squamous epithelium; dermoids contain hair, sebaceous and sweat glands, and squamous epithelium. Both arise from trapped pouches of ectoderm, near normal folds, or from failure of surface ectoderm to separate from the neural tube. These slowly expanding, unilocular, cystic masses may produce only mild symptoms. They commonly occur in the orbit, calvarial diploic space, and intracranially (the posterior and middle fossae). They may be complicated by rupture leading to chemical meningitis, and dermoids with a fistulous tract can become infected. Craniofacial teratomas are true neoplasms arising from misplaced embryologic germ cells. They contain a medley of heterogeneous tissues, typically reflecting more than one of the three embryonic germ layers. They are usually multiloculated masses, often large, with complex radiologic characteristics. Craniofacial teratomas may manifest prenatally with macrocrania or polyhydramnios, during a difficult delivery, or postnatally as a life-threatening mass causing brain herniation, hydrocephalus, respiratory distress, or feeding difficulty. In infancy, they can be biologically benign, even when their histologic characteristics are immature. Surgery is the treatment of choice for all three masses and may be curative.
PURPOSE: To correlate the magnetic resonance (MR) imaging features of and histologic findings in Lhermitte-Duclos disease (LDD) (dysplastic gangliocytoma). MATERIALS AND METHODS: MR imaging and histologic data from eight patients with the diagnosis of LDD were retrospectively reviewed. Gross pathologic specimens were also available in one case. Contrast material-enhanced MR images were obtained in seven of the eight patients. RESULTS: At MR imaging, a non-enhancing mass in the cerebellar hemisphere was characterized by a striated pattern of hyperintensity on T2-weighted images and corresponding hypointensity on T1-weighted images, which represented the abnormally thickened folia. None of the masses demonstrated enhancement. CONCLUSION: Most other cerebellar masses destroy the folial pattern and show enhancement. The nonenhancing striated MR appearance of thickened folia in patients with LDD is distinctive and should suggest this diagnosis.