Radio-pathologic correlation: no pial angioma-subarachnoid varicose network drainage pathway in Sturge-Weber syndrome.

PURPOSE: The traditional imaging findings reported in Sturge-Weber syndrome (SWS) include endpoints of cortical injury-cortical atrophy and cortical calcifications-but also what has been termed a "leptomeningeal angiomatosis," the latter recognized and reported as a leptomeningeal enhancement on magnetic resonance imaging (MRI). The objective of this study is to demonstrate through neuropathological correlation that the "leptomeningeal angiomatosis" in patients with Sturge-Weber syndrome (SWS), represents a re-opened primitive venous network in the subarachnoid space that likely acts as an alternative venous drainage pathway, seen separately to abnormal pial enhancement. MATERIALS AND METHODS: Retrospective review of MR imaging and surgical pathology of patients that underwent surgery for epilepsy at a tertiary, children's hospital. A pediatric radiologist with more than 20 years of experience reviewed the MR imaging. Surgically resected brain specimens that had been sectioned and fixed in 10% paraformaldehyde for histologic processing, following processing and paraffin embedding, were cut into 5-µm unstained slides which were subsequently stained with hematoxylin and eosin (H&E). Slides were re-examined by a board-certified pediatric neuropathologist, and histologic features specifically relating to cerebral surface and vascularity were documented for correlation with MR imaging of the resected region performed prior to resection. RESULTS: Five patients were reviewed (3 boys and 2 girls; the median age at the onset of seizures was 12 months (IQR, 7 to 45 months); the median age at surgery was 33 months (IQR, 23.5 to 56.5 months)). Surgical procedures included the following: 4, hemispherotomy (right: 2, left: 2) and 1, hemispherectomy (right). A subarachnoid space varicose network was present on both MRI and histology in 4 patients. Calcifications were seen on both MRI and histology in 3 patients. Abnormal leptomeningeal enhancement was present in 5 patients and seen separately from the subarachnoid vascular network in 4 patients. CONCLUSION: Histopathology confirmed the MRI findings of a subarachnoid space varicose network seen separately from leptomeningeal enhancement and presumed to represent an alternative venous drainage pathway to compensate for maldevelopment of cortical veins, the primary abnormality in SWS. No pial-based angioma was identified.

Pseudoangiomatous stromal hyperplasia of the breast, imaging and clinical perspective: A review.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign breast pathology, which most commonly presents incidentally along with other breast pathologies. The etiology and pathogenesis of PASH are still unknown; however, there is some evidence suggesting PASH is hormone dependent. The clinical history, presentation, and imaging appearance of PASH are variable. Clinically, PASH has a wide spectrum of presentations, from being silent to gigantomastia. On imaging, PASH demonstrates various benign to suspicious features. Here we summarize PASH's clinical presentation, histopathology, imaging features, and management.

Benign Vascular Lesions of the Breast: A Clinical, Radiographic, and Pathologic Review.

Vascular lesions of the breast are uncommon, however, these are increasingly encountered now due to more frequent use of magnetic resonance imaging. They comprise a spectrum of lesions including benign, atypical, and malignant tumors. The prototype is a hemangioma, which is most often nonpalpable and is detected on routine screening. Different histopathologic subtypes of hemangioma have been described, including perilobular, venous, cavernous, and capillary hemangioma. Other benign vascular lesions include anastomosing hemangioma, a well-circumscribed proliferation of anastomosing blood vessels with lobular or diffuse growth pattern which affects a large segment of the breast, presenting as a painless slow enlarging palpable mass. Recent data suggest that benign vascular lesions diagnosed on core needle biopsy with concordant radiologic and pathologic findings do not require excision and have an excellent prognosis, except angiomatosis, which can be locally aggressive and may recur. The main focus of this study is to present the radiographic, gross, and histopathologic characteristics of benign vascular lesions of the breast and their differential diagnoses.

Diffuse cerebral angiomatosis associated to basilar apex aneurysm.

BACKGROUND: Diffuse cerebral angiomatosis (DCA) is a diffuse infiltration of normal brain by complex vascular structures. It differs from arteriovenous malformations (AVMs) that are composed of a nidus of vessels through which arteriovenous shunting occurs without interposed functional brain parenchyma. A rare subgroup of AVMs is diffuse with no recognizable nidus with functional neuronal tissue interspersed within the malformed vessels. We present a rare association of DCA and cerebral arterial aneurysm, which dramatically influenced the patient's prognosis. CASE DESCRIPTION: A 43-year-old male patient with right hemispheric diffuse cerebral angiomatosis presented with a ruptured basilar tip aneurysm that was successfully embolised. Unfortunately, the patient developed a locked-in syndrome. CONCLUSION: The present report shows a possible association between diffuse cerebral angiomatosis and cerebral aneurysms, but this association appears to be less strong than it is with other AVMs.

Bilateral Diffuse Pseudoangiomatous Stromal Hyperplasia Necessitating Mastectomy.

Pseudoangiomatous stromal hyperplasia (PASH) is an uncommon, benign breast lesion often diagnosed incidentally and frequently mistaken for fibroadenoma given similar radiographic appearance. Histopathology classically reveals diffuse, dense fibrous stromal background with a complex network of spindle cells forming slit-like spaces, giving it the appearance of angiomatous proliferation. Surgical excision is generally not necessary. Here we present two unusual cases of PASH: an adolescent patient with bilateral rapid onset of symptoms, and a premenopausal patient with bilateral, diffuse, recurrent PASH. Both required mastectomy. We aim to highlight the variable nature of presentation and briefly review current management options.

Bilateral axillary pseudoangiomatous stromal hyperplasia in a premenopausal woman: A case report with imaging findings.

Pseudoangiomatous stromal hyperplasia is a benign proliferative mesenchymal lesion of the breast. To date, only a few cases of axillary pseudoangiomatous stromal hyperplasia with imaging findings have been reported in the literature. Herein, we report a rare case of pseudoangiomatous stromal hyperplasia in both axillae in a previously healthy 46-year-old woman and describe the imaging findings, including ultrasonography and computed tomography findings.

MRI features of pseudoangiomatous stromal hyperplasia with histopathological correlation.

Pseudoangiomatous stromal hyperplasia (PASH), a rare, noncancerous lesion, is often an incidental finding on magnetic resonance imaging (MRI)-guided biopsy analysis of other breast lesions. We sought to describe the characteristics of PASH on MRI and identify the extent to which these characteristics are correlated with the amount of PASH in the pathology specimens. We identified 69 patients who underwent MRI-guided biopsies yielding a final pathological diagnosis of PASH between 2008 and 2015. We analyzed pre-biopsy MRI scans to document the appearance of the lesions of interest. All biopsy samples were classified as having ≤50% PASH or ≥51% PASH present on the pathological specimen. On MRI, 9 lesions (13%) appeared as foci, 19 (28%) appeared as masses with either washout or persistent kinetics, and 41 (59%) appeared as regions of nonmass enhancement. Of this latter group, 33 lesions (80%) showed persistent kinetic features. Masses, foci, and regions of nonmass enhancement did not significantly correlate with the percentage of PASH present in the biopsy specimens (P ≥ .05). Our findings suggest that PASH has a wide-ranging appearance on MRI but most commonly appears as a region of nonmass enhancement with persistent kinetic features. Our finding that most specimens had ≤50% PASH supports the notion that PASH is usually an incidental finding. We did not identify a definitive imaging characteristic that reliably identifies PASH.

Isolated leptomeningeal angiomatosis in the sixth decade of life, an adulthood variant of Sturge Weber Syndrome (Type III): role of advanced Magnetic Resonance Imaging and Digital Subtraction Angiography in diagnosis.

BACKGROUND: Sturge-Weber syndrome (SWS) is primarily diagnosed in pediatric population, but clinical presentation in late adulthood is rarely reported. Evolution of radiological findings in the adulthood variant of SWS with isolated leptomeningeal angiomatosis has never been reported to our knowledge. CASE PRESENTATION: We report here a case of an isolated temporo-parieto-occipital leptomeningeal angiomatosis on the right cerebral hemisphere in a sixty-two-year-old male who presented with generalized seizure, GCS score 14/15 (E4 V4 M6) with equal and reacting pupils, psychomotor slowing, left hemineglect and grade 4 power in the left upper and lower limbs. Over a period of 48 h his neurological status deteriorated, but recovered spontaneously over a week on titration with anticonvulsants. He had a prior history of treatment for focal leptomeningitis, three years ago. Cerebrospinal fluid (CSF) analysis showed glucose of 75 mg/dL, proteins of 65 mg/dL and culture grew no organisms. On follow-up, he had intermittent episodes of focal seizure for two years. Initial, computed tomography of brain showed hyperdense lesion in the parieto-occipital convexity subarachnoid space on the right cerebral hemisphere mimicking subarachnoid hemorrhage and computed tomography angiography showed no significant abnormality. Magnetic resonance imaging (MRI) of brain showed intense pial enhancement in the right temporo-parieto-occipital region with a subtle T2W hyperintense signal in the underlying subcortical white matter without edema or infarct or mass effect. Digital subtraction cerebral angiography (DSA) showed hypertrophy of the cerebral arteries, arteriolo-capillary bed and venules in the right temporo-parieto-occipital territory associated with early arterio-capillary and venous opacification. Serial MRI done after six months, one and two years showed increase in the T2W hyperintense signal in the subcortical white matter and cortical atrophy with no changes in the pial enhancement. MR perfusion imaging showed reduced cerebral blood flow (CBF) and cerebral blood volume (CBV) in the right parieto-temporo-occipital cortical and subcortical regions and increased perfusion in the leptomeninges with reduction of the NAA / Cr ratios in spectroscopy. CONCLUSION: Conglomeration of various radiological findings in MRI, Perfusion, MRS and DSA with the clinical presentation can aid in establishing the diagnosis of this rare presentation of SWS-type 3 variant in late adulthood.

Management of recurrent bilateral multifocal pseudoangiomatous stromal hyperplasia (PASH).

Pseudoangiomatous stromal hyperplasia (PASH) is a benign hyperplastic condition of the breast that can lead to macromastia. The standard treatment for PASH is focal excision or rarely reduction mammoplasty. We present a rare case of postpartum bilateral rapid breast enlargement and axillary growth that was refractory to reduction mammoplasty. Ultimately, the patient required bilateral mastectomy and two-stage implant-based breast reconstruction. This more extensive form along with its management represents one of the few reported cases in the literature. The decision to pursue bilateral mastectomy was undertaken after exhausting more conservative options. Excellent aesthetic outcome and pain relief was obtained following definitive extirpative and reconstructive surgery.

Successful management of diffuse bilateral pseudoangiomatous stromal hyperplasia of the mammary glands by reductive mammoplasty.

Pseudoangiomatous stromal hyperplasia (PASH) is a benign breast condition commonly presented as an incidental microscopic finding. However, it can also manifest as a mass-forming lesion (tumorous PASH) or as gigantomastia (diffuse PASH). Most of the previously reported cases are unilateral tumorous PASH treated by mastectomy. In this article, we reported a rare case of diffuse bilateral PASH. A 21-year-old woman presented with a two-year history of bilateral breast enlargement and neck pain. Physical examination revealed asymmetrical bilateral macromastia with profound ptosis. Breast ultrasound demonstrated no cysts or masses in both breasts. The decision was made to perform an inverted T bilateral reductive mammoplasty which was performed successfully through a superior-central pedicle approach. Microscopic examination of the specimens confirmed the diagnosis of PASH without any evidence for malignancy. Nine months postoperatively, the size for both breasts was optimal the patient was satisfied. Recognition and reporting of this rare form of PASH is essential for proper investigation, pathology understanding, risk factors recognition, prognosis assessment and treatment methods selection.

[Recurrent stroke, acrocyanosis and livedo racemosa: is it always Sneddon's syndrome?] / Ictus recidivante, acrocianosis y livedo racemosa: ¿siempre síndrome de Sneddon?

TITLE: Ictus recidivante, acrocianosis y livedo racemosa: ¿siempre síndrome de Sneddon?

Sporadic Meningioangiomatosis Presenting as a Middle Cranial Fossa Arachnoid Cyst.

BACKGROUND: Meningioangiomatosis is an extremely rare meningovascular disease of the central nervous system that is characterized by the proliferation of leptomeninges, cortical vessels, and perivascular spindled cells. Although it is a benign, neoplastic disorder that carries a good prognosis after surgical excision, initial diagnosis may be challenging as radiologic findings are often variable and nonspecific. CASE DESCRIPTION: In this report, we describe an unusual presentation of meningioangiomatosis presenting as a symptomatic middle cranial fossa arachnoid cyst. CONCLUSIONS: In view of the unexpected diagnosis and infrequency of this condition, the case is discussed in collaboration with current literature and management strategies.

Diffuse cerebral angiomatosis: a case report with fatal outcome.

Diffuse cerebral angiomatosis is a rare disorder of the brain and only 5 cases have been reported to date. We report a 16-year-old female patient who presented to the emergency department with seizures. The patient had no hereditary syndromes, no epistaxis, no skin lesions and no telangiectasia. There was no livedo reticularis. The ophthalmologic examination was within normal limits. Magnetic resonance angiography (MRA) showed multiple diffuse tortuous vessels all around the brain tissue and ventricular system. Brain CT showed multiple calcifications. Digital subtraction angiography (DSA) demonstrated multiple arteriovenous shunts and diffuse arteriovenous malformations (AVM) without a typical nidus, draining into dilated cerebral veins. Multimodal ultrasound showed increased flow velocities in all cerebral arteries. We report the case of a patient with diffuse cerebral angiomatosis. This is the third study in which hemodynamic indices were evaluated through transcranial Doppler. Unlike all other case reports our patient had a bad outcome with important disability, dementia and drug resistant epilepsy that lead to the patient's death.HighlightsDiffuse cerebral angiomatosis is a rare disorder of the brain and only 5 cases have been reported to date.Unlike all other case reports our patient had a bad outcome with disability, dementia and drug resistant epilepsy that lead to the patient's death.

Rare Finding of Bilateral Pseudoangiomatous Stromal Hyperplasia of the Breast: A Case Report.

A 49-year-old woman, with a medical history of rheumatism, was admitted to our hospital with chief complaints of bilateral enlargement and redness of breasts. She underwent weekly breast examinations. Mammography findings were reported as category 3 for both breasts. Breast ultrasonography, magnetic resonance imaging, and chest contrast computed tomography revealed a massive tumor in the left BD region, however, there were no findings for suspected malignancy. Needle biopsy did not yield histologically malignant cells in both breasts. Mammary interstitium was edematous, and capillary-like slit structures were observed. The stroma stained with alcian blue and destained with hyaluronidase treatment. Since the stroma tested positive for vimentin, calponin, and CD34 and negative for CD31, the patient was diagnosed as (PASH). Because both breasts had similar diagnosis based on histopathologic findings, bilateral mastectomy was performed. Details about the origin of bilateral PASH are unknown but it may be related to the development of rheumatoid arthritis. Additionally, systemic autoimmune diseases like rheumatism may be the reason for repeated contraction and enlargement of PASH.

Benign spindle cell lesions of the breast: a diagnostic approach to solitary fibrous tumour, nodular pseudoangiomatous stromal hyperplasia and nodular fasciitis.

Benign spindle cell lesions of the breast include neoplastic and reactive entities that are diagnostically challenging given their rarity and similar histomorphology. Accurate diagnosis on percutaneous core biopsy within this category is essential as some lesions require excision and surveillance, whereas others may be observed. We present three cases of rare benign spindle cell lesions of the breast that reflect the diversity of this group: solitary fibrous tumour, nodular pseudoangiomatous stromal hyperplasia and nodular fasciitis. Through these cases, we discuss the associated differential diagnosis and demonstrate how emerging ancillary studies can be integrated into a diagnostic approach. We highlight distinctive clinical and histopathological features and summarise recent updates to the clinical management of these lesions. An organised approach to the broad differential of spindle cell lesions is essential for appropriate diagnosis and treatment.