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.

Fibroadenoma of the vulva with pseudoangiomatous stromal hyperplasia: A common neoplasm in uncommon site.

Mammary-type tissue in the vulva was first described in 1872 but has been rarely reported in the literature. This tissue was previously considered as ectopic breast tissue that occurs as a result of incomplete regression of the milk line. Similar to native breast tissue, ectopic mammary tissue is hormone-sensitive and can develop benign changes, such as fibroadenoma, as well as malignant changes. A more recent theory suggests that these benign and malignant mammary-type entities arise from mammary-like anogenital glands, which constitute normal vulvar components. We report a case of a 41-year-old woman who presented with a chronic asymptomatic cyst on the left vulva that eventually became uncomfortable, especially on standing. The cyst was located on the labium minus, measuring 1.0 × 0.5 cm, with no identified erythema or other skin abnormalities. Excision of the lesion and subsequent microscopic examination showed a circumscribed mass with a nodular overgrowth of epithelial and stromal components, resembling a mammary fibroadenoma with pseudoangiomatous stromal hyperplasia. We bring to attention this rare diagnosis and the importance of considering it in the presence of a vulvar lesion. The malignant and recurrence potential of mammary-type tissue necessitates excision with clear margins and close monitoring of these patients.

Vertebral Angiomatosis in a Dog.

A 3 yr old spayed female Cavalier King Charles spaniel was referred for insidious ataxia and paraparesis. A thoracolumbar lesion was suspected. Computed tomography showed focal osteolysis of the vertebral body and pedicles of T5. In addition, a hyperdense, extradural material within the vertebral canal, causing spinal cord compression on the right side, was present. The lesion was confirmed with magnetic resonance imaging. A T4-T5 hemi-dorsal laminectomy was performed to decompress the spinal cord. Histopathological examination was consistent with vertebral angiomatosis. After the surgery, the dog rapidly improved; however, 5 mo later the clinical signs relapsed. Vertebral angiomatosis is a vasoproliferative disorder, rarely reported as a cause of myelopathy in cats. This condition has not previously been reported in dogs. This case report describes the clinical features, the diagnostic findings, and the follow-up of a young dog with vertebral angiomatosis.

Arterial spin-labeled (ASL) perfusion in children with Sturge-Weber syndrome: a retrospective cross-sectional study.

PURPOSE: Sturge-Weber syndrome (SWS) is a developmental disorder with venous hypertension and associated tissue responses including pial angiomatosis, cortical calcifications, and cerebral atrophy. Arterial spin-labeled (ASL) perfusion is an advanced MR sequence which can assess perfusion, without the need for contrast. We systematically evaluated the potential benefits of using ASL in Sturge-Weber syndrome, to determine the extent of intracranial perfusion abnormality and stage of disease, relevant for prognostication and surgical planning. METHODS: Two pediatric neuroradiologists retrospectively evaluated ASL perfusion imaging of 31 children with confirmed SWS and recorded the presence of hyper-perfusion, hypo-perfusion, or normal perfusion. The presence and distribution of ASL abnormality were compared against the presence and side of atrophy/calcification and pial angiomatosis on standard MR sequences. RESULTS: Thirty-one children (52% female, median age 16.7 months) with SWS had ASL imaging. Seven (23%) had hyper-perfusion, 15 (48%) had hypo-perfusion, and 9 (29%) had no perfusion abnormalities. ASL perfusion abnormality matched the location of SWS findings on conventional imaging in 86% (19/22). ASL demonstrated statistically significant increased perfusion in the early stage of the disease and decreased perfusion when there was atrophy. The parietal lobe was involved in 86% of cases. CONCLUSION: ASL perfusion imaging is an advanced technique which may contribute to earlier diagnosis and more accurate prognostication of Sturge-Weber syndrome, helping guide management and potential surgical planning.

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.

Pseudoangiomatous stromal hyperplasia of the breast: Clinical evaluation.

BACKGROUND: Pseudoangiomatous stromal hyperplasia is a rare benign breast stromal proliferative lesion of the breast. Clinical presentation ranges from rapidly growing mass to incidental identification in routine screening. This difference in manifestation and its rarity makes it difficult to be a standard treatment protocol. Therefore, we aimed to share our clinical experience in Pseudoangiomatous stromal hyperplasia. METHODS: The files of patients who underwent core biopsy or surgical excision due to a breast mass and resulted in pseudoangiomatous stromal hyperplasia between January 2013 and December 2021 were included in the study. RESULTS: 17 patients with a median age of 37 (22-68) were found Pseudoangiomatous stromal hyperplasia confirmed by surgical excision or core biopsy. Chosen treatment option was observation in 8 patients (47.1%), while surgical excision was used in 9 (52.9%) patients. The mean follow-up period was 55.24 ± 26.72 (13-102) months. None of the patients observed the Malignant transformation during the follow-up period. CONCLUSION: For Pseudoangiomatous Stromal Hyperplasia of the breast, surgical excision with clean margins or close follow-up after diagnosis confirmation by tissue biopsy is sufficient. Pseudoangiomatous Stromal Hyperplasia is not a risk factor for developing breast cancer.

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.

Cutaneous angiomatosis-like presentation in koi carp (Cyprinus carpio koi): Clinical-pathological investigations.

The skin represents an indicator of an animal's health status. Causes of cutaneous diseases in fish most often trace back to biological agents. However, fish skin diseases can also arise from a complex interaction of infectious and non-infectious causes, making it more difficult to identify a specific aetiology. In the period between April and September of the years 2019-2022, four koi carp (Cyprinus carpio koi) from two European countries presented with multifocal, irregularly round, few mm to 1 cm, variably raised cutaneous reddened areas. The fish displayed good general condition. Cutaneous samples, investigated by microbiological and molecular methods and microscopy, did not indicate a primary pathogenic agent. Gross and histological findings of the cutaneous biopsies were consistent with a multifocal/reactive process centred on dermal vessels. The histological features were reminiscent of angiomatosis, a benign proliferative condition affecting the dermal vessels of mammals, including human patients. The clinical-pathological presentation and the dermatologic condition that affected the koi carp are discussed and compared with the veterinary and human literature.

Clinicopathological Analysis of Sturge-Weber Syndrome with Focal Cortical Dysplasia FCD IIIc.

Objective: To investigate the clinicopathological features of children with Sturge-Weber syndrome and to analyze the correlation between the distribution area of leptomeningeal angiomatosis, the degree of cerebral cortical calcification, and the degree of cerebral atrophy associated with epileptic seizures. Methods: 10 children were diagnosed with SWS with FCD IIIc by histopathology and immunohistochemistry. Spearman correlation analysis was used to calculate the association of SWS with FCD IIIc and seizures in children. Results: The leptomeningeal angiomatosis area was markedly positively correlated with the degree of brain atrophy in 10 children with SWS (r = 0.783, p = 0.007). The distribution of leptomeningeal hemangiomatosis, the degree of cortical calcification, and brain atrophy were not significantly correlated with epilepsy. Conclusion: SWS may be accompanied by FCD IIIc. The more extensive the cerebral lobes of leptomeningeal angiomatosis in SWS, the more pronounced the brain atrophy.

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.

Pseudoangiomatous stromal hyperplasia: A clinical perspective and a brief review.

ABSTRACT: Pseudoangiomatous stromal hyperplasia (PASH) is a benign entity of the breast and typically found incidentally or as a palpable mass. It usually affects women in the reproductive age group. In men, it is associated with gynecomastia. While the exact etiology is unknown, PASH is considered hormonally responsive. The mammography and ultrasonography findings are nonspecific. Grossly, PASH is a well-circumscribed, firm, rubbery mass with solid, homogenous, gray-white cut surface. On histologic examination, it is characterized by the presence of open slit-like spaces in dense collagenous stroma. The spindle cells express progesterone receptors and are positive for vimentin, actin, and CD34. PASH lesions should be surgically excised if increasing in size or associated with symptoms, also in cases of suspicious imaging findings or other lesions are synchronously diagnosed that require excision. In other small incidentally detected asymptomatic cases observation with clinicoradiological follow-up can be considered. It is associated with a good prognosis with no increased risk to develop breast cancer. Hence, the operating surgeon and pathologist should be aware of this not so uncommon entity so that patients are not subjected to unnecessary excision and subsequent physical and mental morbidity associated with it. In this article, we present a case report and also provide a brief review of the sparse literature on PASH, discussing clinical presentation, etiology and pathogenesis, radiopathological features, differential diagnosis, and the implications for further patient management and prognosis.

Tumoral pseudoangiomatous stromal hyperplasia (PASH) of the male breast: A systematic analysis.

<b><br>Aim:</b> The study was conducted to systematically analyze the recent peer-reviewed literature related to tumoral pseudoangiomatous stromal hyperplasia (PASH) in males.</br> <b><br>Materials and methods:</b> Articles published in peer-reviewed journals of repute from 2012 to 2023 were evaluated for eight variables: 1) the age of the patient, 2) clinical presentation, 3) laterality (unilateral or bilateral), 4) associated breast disorder, 5) the presence of systemic co-morbidity, 6) imaging modalities aiding diagnosis, 7) treatment, and 8) recurrence.</br> <b><br>Results:</b> There were 10 patients ranging in age from 1 year to 67 years (mean: 34.5 years). In 8 cases (80%), there were breast lumps; in 2 cases (20%), there were axillary lumps at presentation. Pain was not a prominent symptom. Six patients (60%) had another breast disorder at presentation and 3 (30%) had significant systemic co-morbidities, including diabetes and renal insufficiency. The greatest dimension of the lumps ranged from 2 cm to 12 cm, with 80% of the lumps being below 5 cm in size. Imaging modalities utilized in evaluation included breast ultrasound (USG), mammograms, computerized tomography (CT), and magnetic resonance imaging (MRI). All the cases were treated surgically, and the final diagnosis was made only after the analysis of the surgical specimen. Recurrence was recorded in 2 (20%) cases.</br> <b><br>Conclusions:</b> Tumoral PASH is a benign myofibroblastic proliferation disorder rarely reported in males. This condition should be considered a differential diagnosis of breast or axillary lumps, and an evaluation should be initiated accordingly. The recommended course of treatment is adequate surgical excision with regular clinical and radiological follow-up.</br>.