Selected case from the Arkadi M. Rywlin International Pathology Slide Series: Leiomyomatosis peritonealis disseminata: report of 3 cases with extensive review of the literature.

We present the clinicopathologic features of 3 cases of leiomyomatosis peritonealis disseminata (LPD). The patients were 33, 34, and 41 years old at the time of diagnoses. The 3 women had undergone laparoscopic removal of multiple uterine leiomyomas between 1 and 6 years before the diagnoses of LPD. Laparoscopic uterine leiomyomectomies were performed on 3 occasions in patient 1, and once in patients 2 and 3 by the time a diagnosis of LPD was made. In patients 2 and 3, one of the multiple uterine leiomyomas had been qualified as mitotically active. Patients 1 and 2 received hormonal treatment before LPD was diagnosed. Malignancy was clinically and/or pathologically suspected in all the 3 cases. Patients 1 and 2 were managed conservatively. Patient 3 underwent radical hysterectomy with bilateral adnexectomy and omentectomy. Patients 1 and 2 belong to a rare subset of LPD that have fewer tumor nodules larger (5 to 10 cm) than typically seen. Patient 3 was classic in that she exhibited innumerable nodules measuring between a few millimeters and 1.5 cm, intraoperatively mimicking peritoneal carcinomatosis. Histopathologically, patients 1 and 2 were diagnosed as pure LPD, whereas patient 3 was diagnosed as LPD associated with endometriosis (adenomyosis type). Patients 1 and 3 had incipient foci of leiomyomatous changes in the blood vessel walls, at the site of the LPD tumors, supporting the hypothesis that these are de novo lesions arising locally and not migrated or disseminated from the previously excised or concurrent uterine smooth muscle tumors, usually seen in this context. Conceivably, laparoscopic leiomyomectomy with morcellation may play a role in the pathogenesis of this rare condition, at least in hormonally susceptible patients. Alternatively, LPD may derive from metaplastic submesothelial cells, a condition analogous to gliomatosis peritonei.

Solitary fibrous tumor of the central nervous system: report of an additional 5 cases with comprehensive literature review.

Solitary fibrous tumor (SFT) of the central nervous system was first described in 1996. A number of cases have been reported since. The authors present 5 new cases: 4 intracranial and 1 intraspinal. All patients were adults (age range, 47 to 75 years); 4 were male and 1 female; 4 cases were primary tumors; and 1 was a second tumor recurrence. All patients were surgically treated with gross total removal. All cases were histologically examined with immunohistochemical confirmation; 2 tumors exhibited diffuse classic histology, 1 tumor was a cellular variant, 1 tumor was myxoid, and 1 was predominantly classic with focal myxoid features and focally pleomorphic. The postoperative course was uneventful in all. The patient with the cellular variant experienced 2 local recurrences and eventually died of disease 10 years after the initial diagnosis. The patient with the myxoid variant--the tumor studied--which was the second recurrence of a previously misdiagnosed fibrous meningioma surgically treated 15 years earlier, had a recurrence after 2 years for the third time and eventually died of disease. Three patients are alive and well 11.6, 6, and 4 years after surgery. SFT is a rare tumor that needs to be differentiated from some mimickers, mainly fibrous meningioma, hemangiopericytoma, and with regard to the myxoid variant, also adult-onset myxochordoid meningioma and myxoid peripheral nerve sheath tumor. Immunohistochemistry is crucial for the correct diagnosis of SFT. The authors also performed a review of the literature and found a little more than 200 cases on record.

Solitary fibrous tumor of the central nervous system: a 15-year literature survey of 220 cases (August 1996-July 2011).

We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.

Improvement of migraine after patent foramen ovale percutaneous closure in patients with subclinical brain lesions: a case-control study.

OBJECTIVES: We sought to evaluate the benefits on frequency and severity of migraine recurrence after patent foramen ovale (PFO) closure in patients with subclinical brain lesions at magnetic resonance imaging (MRI). BACKGROUND: Migraine improvement has been reported after PFO closure in patients with cerebrovascular symptomatic events. Subclinical brain MRI lesions are detectable in patients with PFO and in migraineurs. METHODS: A total of 82 patients with moderate/severe migraine, PFO, large right-to-left shunt, and subclinical brain MRI lesions were prospectively examined for a 6-month period. Patients were subdivided into closure (n = 53) and control (n = 29) group according to their consent to undergo percutaneous PFO closure. In controls, therapy for migraine was optimized. Six-month frequency and severity of migraine recurrence were compared with baseline. RESULTS: The number of total attacks decreased more in the closure group (32 +/- 9 to 7 +/- 7, p < 0.001) than in the control group (36 +/- 13 to 30 +/- 21, p = NS) (p < 0.001). A significant reduction in disabling attacks was observed only in the closure group (20 +/- 12 to 2 +/- 2, p < 0.001; controls: 15 +/- 12 to 12 +/- 12, p = NS). Migraine disappeared in 34% of the closure group patients and 7% of controls (p = 0.007); >50% reduction of attacks was reported by 87% and 21%, respectively (p < 0.001). Disabling attacks disappeared in 53% of closure group patients and 7% of controls (p < 0.001); >50% reduction occurred in 89% and 17%, respectively (p < 0.001). CONCLUSIONS: In migraineurs with a large PFO and subclinical brain MRI lesions, a significant reduction in frequency and severity of migraine recurrence can be obtained by PFO closure when compared with frequency and severity in controls.

Proton MR spectroscopy of cerebral gliomas at 3 T: spatial heterogeneity, and tumour grade and extent.

This study aimed to evaluate the usefulness of proton MR spectroscopic imaging ((1)H-MRSI) at 3 T in differentiating high- from low-grade gliomas, and tumour from necrosis, oedema or normal tissue. Forty-four patients with brain gliomas and four with meningiomas were retrospectively reviewed. The normalised metabolites choline (nCho), N-acetylaspartate (nNAA), creatine (nCr) and lactate/lipids (nLL), and the metabolite ratios Cho/NAA, NAA/Cr and Cho/Cr were calculated. Necrotic-appearing areas showed two spectroscopic patterns: "necrosis" with variable nCho and high nLL, and "cystic necrosis" with variable nLL or nonevident peaks. Peri-enhancing oedematous-appearing areas showed three spectroscopic patterns ("tumour" with abnormal Cho/NAA, "oedema" with normal Cho/NAA and "tumour/oedema" with normal nCho and abnormal Cho/NAA) in gliomas, and one ("oedema") in meningiomas. Peri-enhancing or peri-tumour normal-appearing areas showed two patterns ("infiltrated" with abnormal nCho and/or Cho/NAA and "normal" with normal spectra) in gliomas and one ("normal") in meningiomas. Discriminant analysis showed that classification accuracy between high- and low-grade glioma masses was better with normalised metabolites or all parameters together than metabolite ratios and that among peri-enhancing areas was much better with normalised metabolites. The analysis of spatial distribution of normalised metabolites by 3-T (1)H-MRSI helps to discriminate among different tissues, offering information not available with conventional MRI.

Clinical and brain magnetic resonance imaging follow-up after percutaneous closure of patent foramen ovale in patients with cryptogenic stroke.

Patent foramen ovale (PFO) closure is reported to result in fewer episodes of clinically manifest recurrent cerebral ischemia than medical treatment. We evaluated by means of magnetic resonance imaging (MRI) whether silent cerebral ischemic episodes are also decreased by PFO closure. Seventy-one patients with PFO were selected for percutaneous closure of PFO at our center. All had PFO with large right-to-left shunt documented by transcranial Doppler ultrasound and transesophageal echocardiography, > or =1 previous stroke or transient ischemic attack with MRI documentation at the index event, and no alternative cause for cerebral ischemia. MRI studies were performed in all patients 24 hours before the procedure and at 1-year follow-up (or before in the case of a suspected new neurologic event). Eight patients (11%) had >1 clinical event before the procedure. Comparing the 2 MRI studies before the procedure, silent ischemic lesions were observed in 14 other patients (20%). Thus, considering clinical and silent events together, >1 event was present at baseline in 22 patients (31%). After PFO closure (follow-up 16 +/- 7 months), 1 recurrent neurologic event occurred (1%, p = 0.02 vs preprocedural clinical events); however, urgent brain MRI results were negative. Moreover, only 1 patient showed 1 new silent lesion at brain MRI at follow-up (1%, p <0.001 vs preprocedural silent brain lesions). Considering clinical and silent events, relapses occurred in 2 patients only (p <0.001 vs before procedure). Recurrent events were limited to those with incomplete PFO closure at postprocedural transcranial Doppler ultrasound (p = 0.02). In conclusion, percutaneous PFO closure results in few clinical or silent events after 1-year follow-up, especially when complete PFO closure is successfully accomplished.

Preferential responses in amygdala and insula during presentation of facial contempt and disgust.

Some authors consider contempt to be a basic emotion while others consider it a variant of disgust. The neural correlates of contempt have not so far been specifically contrasted with disgust. Using functional magnetic resonance imaging (fMRI), we investigated the neural networks involved in the processing of facial contempt and disgust in 24 healthy subjects. Facial recognition of contempt was lower than that of disgust and of neutral faces. The imaging data indicated significant activity in the amygdala and in globus pallidus and putamen during processing of contemptuous faces. Bilateral insula and caudate nuclei and left as well as right inferior frontal gyrus were engaged during processing of disgusted faces. Moreover, direct comparisons of contempt vs. disgust yielded significantly different activations in the amygdala. On the other hand, disgusted faces elicited greater activation than contemptuous faces in the right insula and caudate. Our findings suggest preferential involvement of different neural substrates in the processing of facial emotional expressions of contempt and disgust.

Multiparametric 3T MR approach to the assessment of cerebral gliomas: tumor extent and malignancy.

INTRODUCTION: Contrast-enhanced MR imaging is the method of choice for routine assessment of brain tumors, but it has limited sensitivity and specificity. We verified if the addition of metabolic, diffusion and hemodynamic information improved the definition of glioma extent and grade. METHODS: Thirty-one patients with cerebral gliomas (21 high- and 10 low-grade) underwent conventional MR imaging, proton MR spectroscopic imaging ((1)H-MRSI), diffusion weighted imaging (DWI) and perfusion weighted imaging (PWI) at 3 Tesla, before undergoing surgery and histological confirmation. Normalized metabolite signals, including choline (Cho), N-acetylaspartate (NAA), creatine and lactate/lipids, were obtained by (1)H-MRSI; apparent diffusion coefficient (ADC) by DWI; and relative cerebral blood volume (rCBV) by PWI. RESULTS: Perienhancing areas with abnormal MR signal showed 3 multiparametric patterns: "tumor", with abnormal Cho/NAA ratio, lower ADC and higher rCBV; "edema", with normal Cho/NAA ratio, higher ADC and lower rCBV; and "tumor/edema", with abnormal Cho/NAA ratio and intermediate ADC and rCBV. Perienhancing areas with normal MR signal showed 2 multiparametric patterns: "infiltrated", with high Cho and/or abnormal Cho/NAA ratio; and "normal", with normal spectra. Stepwise discriminant analysis showed that the better classification accuracy of perienhancing areas was achieved when regarding all MR variables, while (1)H-MRSI variables and rCBV better differentiated high- from low-grade gliomas. CONCLUSION: Multiparametric MR assessment of gliomas, based on (1)H-MRSI, PWI and DWI, discriminates infiltrating tumor from surrounding vasogenic edema or normal tissues, and high- from low-grade gliomas. This approach may provide useful information for guiding stereotactic biopsies, surgical resection and radiation treatment.

3.0 T magnetic resonance in neuroradiology.

Ever since the introduction of magnetic resonance (MR), imaging with 1.5 T has been considered the gold standard for the study of all body areas. Until not long ago, higher-field MR equipment was exclusively employed for research, not for clinical use. More recently, the introduction of 3.0 T MR machines for new and more sophisticated clinical applications has yielded in important benefits, especially in neuroradiology. Indeed, their high gradient power and field intensity allow adjunctive and more advanced diagnostic methodologies to be applied with excellent resolution in a fraction of the time of acquisition compared with earlier machines. The numerous advantages of these machines in terms of higher signal, increased spatial resolution and greater sensitivity, and their few limitations, which can be overcome and anyway do not adversely affect diagnostic efficacy, will make 3.0 T MR systems the gold standard for morphological and functional studies of the brain.