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.

Management of Gynecomastia and Male Benign Diseases.

Gynecomastia is a common benign breast disease involving abnormally increased mammary gland tissue that can affect men of all ages. It is usually due to a hormonal imbalance without a definitive underlying cause (idiopathic), or secondary to medications/drugs, systemic disorders, or malignancy. Gynecomastia is often self-limiting, and its management is watchful waiting. Other male benign breast diseases, such as cysts, lipomas, seromas, infections, and pseudoangiomatous stromal hyperplasia, should be worked up in a similar manner and often require surgical drainage or excision.

Diffuse dermal angiomatosis of the breast: an emerging entity in the setting of cutaneous reactive angiomatoses.

New and emerging types of cutaneous vascular (capillary) proliferations have been described or better categorized in the last few years. They include reactive angioendotheliomatosis, acroangiodermatitis (pseudo-Kaposi sarcoma), diffuse dermal angiomatosis, intravascular histiocytosis, glomeruloid angioendotheliomatosis, and angiopericytomatosis (angiomatosis with cryoproteins). Clinically, they are characterized by multiple, red violaceous, and purpuric patches and plaques, sometimes evolving toward necrosis and ulceration with a wide distribution but a propensity to involve the extremities. Histologically, they are characterized by different patterns of intravascular or extravascular lobular or diffuse hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. Although these angioproliferations can histologically have a pseudoangiosarcomatous pattern, they are reactive in that they originate from the (sub)occlusion of vascular lumina by different localized or systemic disorders. The vascular proliferation stops after the inducing hypoxic stimulus has been withdrawn. Among them, diffuse dermal angiomatosis of the breast is a variant of diffuse dermal angiomatosis involving middle-aged women with macromastia, obesity, smoking, and vasculopathic disorders, considered a distinct disorder in the spectrum of cutaneous reactive angiomatoses. It presents with reticulated erythematous to purple patches with sometimes a tendency to ulcerate and bleeding, appearing on large, pendulous breasts. The pathogenesis is related to tissue hypoxemia resulting from subclinical torsion, compression, and increased venous hydrostatic pressure due to the macromastia, aggravated by the associated ischemic conditions such as hypertension and diabetes. There is no evidence-based therapy, but reduction mammoplasty is a viable treatment option. This should be evaluated in all patients who fail conservative therapy.

Clinical variability in multifocal lymphangioendotheliomatosis with thrombocytopenia: a review of the literature.

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a recently recognized disorder characterized by vascular lesions marked by distinct endothelial proliferation. Lesions affect multiple tissues, and MLT can be associated with refractory thrombocytopenia resulting in life-threatening bleeding. Diagnosing MLT may be challenging given its rarity and phenotypic variability. There is no consensus on the optimal management or treatment duration. We report a 4-month-old male who presented with multiple vascular malformations involving the gastrointestinal tract, lung, bones, choroid plexus, and spleen, with minimal cutaneous involvement and no thrombocytopenia. Wedge resection of a pulmonary nodule was strongly positive for lymphatic vessel endothelial hyaluronan receptor 1 favoring MLT despite the lack of thrombocytopenia. The patient's clinical symptoms and vascular lesions improved on sirolimus therapy. We review the literature to highlight the clinical variability of MLT and discuss the diagnostic and therapeutic options for MLT.

The Spectrum of Cutaneous Reactive Angiomatoses in End Stage Renal Failure.

ABSTRACT: Cutaneous reactive angiomatoses encompass a spectrum of conditions driven by underlying occlusive vasculopathy. We present 2 cases of reactive angioproliferation in the context of end-stage renal failure (ESRF) manifesting as painful cutaneous ulceration. The first case demonstrates histologic features of diffuse dermal angiomatosis. The second case illustrates a nonspecific pattern of vascular proliferation which does not conform to any of the classically described subtypes, therefore reinforcing the concept of a spectrum of histopathologic changes in reactive angioproliferations. Diffuse dermal angiomatosis has been described in ESRF alone and in association with calciphylaxis, a life-threatening condition. Recognizing the patterns of reactive angioproliferation in the context of ESRF therefore has clinical relevance. Earlier identification may facilitate mitigation of risk factors and improve prognosis.

Meningioangiomatosis: Multimodal Analysis and Insights From a Systematic Review.

BACKGROUND: Meningioangiomatosis is a poorly studied, rare, benign, and epileptogenic brain lesion. OBJECTIVE: To demonstrate that surgical resection and a short-time interval to surgery improves epileptic seizure control, we performed a systematic review and meta-analysis of meningioangiomatosis cases. METHODS: Using PRISMA-IPD guidelines, the authors performed a systematic review and meta-analysis of histopathologically-proven meningioangiomatosis cases. Literature search in French and English languages (PubMed, Embase, the Cochrane Library, and the Science Citation Index) including all studies (January 1981 to June 2020) dealing with histopathologically-proven meningioangiomatosis, without age restriction. We assessed clinical, imaging, histomolecular, management, and outcome findings of patients with meningioangiomatosis. RESULTS: Two-hundred and seven cases of meningioangiomatosis from 78 studies were included. Most meningioangiomatosis was sporadic, preferentially concerned male patients, younger than 20 years old, and allowed a functionally independent status. Epileptic seizure was the main symptom, with 81.4% of patients having uncontrolled seizures at the time of surgery. Meningioangiomatosis mainly had frontal (32.3%) or temporal (30.7%) locations. Imaging presentation was heterogeneous, and the diagnosis was often missed preoperatively. The histopathologic pattern was similar whatever the clinical presentation, and immunohistochemistry had limited diagnostic value. On molecular analysis, allelic loss at 22q12 was more frequent in samples of meningioangiomatosis-associated meningioma (37.5%) than in isolated meningioangiomatosis (23.1%). Time interval from diagnosis to surgery (p = 0.011) and lack of surgical resection of the meningioangiomatosis (p = 0.009) were independent predictors of postoperative seizure control. CONCLUSIONS: Owing to low scientific evidence, a multicentric prospective study should help refining the management of meningioangiomatosis.

[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?

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.

Atypical pseudoangiomatous stromal hyperplasia showing rapid growth of the breast: Report of a case.

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a benign lesion manifesting as myofibroblastic proliferation and anastomosing slit-like spaces. Atypical PASH is an extremely rare lesion characterized by cytological alteration of myofibroblast, presenting as myofibroblastic sarcoma arising from PASH. To our knowledge, only one other case has been reported since the first report of Rosen. We present a case of atypical PASH. A 39-year-old female presented with a round, elastic hard, painless mass in the left breast. Mammography and ultrasonography revealed no definitive sign of malignancy. Core needle biopsy report was suggestive of atypical PASH. Five months later, the mass had grown rapidly with pain. Considering the clinicopathological features, excision was performed. Pathological examination revealed the spindle cells proliferation in collagenous stroma. The spindle cell involved the adipose tissue and lobules and lined peudoangiomatous spaces. These cells exhibited marked cytological atypia and mitotic activity. Immunohistochemically, these spindle cells were positive for SMA, CD10, and bcl-2, and negative for podoplanin, p63, CD31, ERG and cytokeratins. The final diagnosis was atypical PASH. She is tumor-free on 12 months follow-up. The nature of atypical PASH remains unknown. Further studies are required for a clear definition, a new histological entity and diagnostic criteria.

Anesthetic Considerations in Cobb Syndrome: A Case Report.

Cutaneomeningospinal angiomatosis, popularly known as Cobb syndrome, is a rare clinical disorder predominantly presenting with a vascular skin lesion and a spinal angioma at the same dermatomal level. Several case reports and case series have reported on the surgical management of this syndrome, but we failed to find any information about its anesthetic considerations in the Medline database. Our case report describes the perioperative anesthetic management of a 46-year-old man with Cobb syndrome.

Eruptive cherry angiomas and skin melanoma: a fortuitous association?

The aim of the study was to assess the association between eruptive cherry angiomas (CAs) and malignant melanoma (MM). Secondary objectives included investigating (i) this association in different age subgroups, and (ii) the association of eruptive CAs with other variables such as malignant tumours, in general, and immunosuppressive treatments. This cross-sectional study involved all patients referred to the outpatient Dermatology-Oncology Units of the universities of Ferrara and Bologna, Italy, and submitted to total body skin examination. These patients were included in a previously collected series. We recorded age, sex, cutaneous and noncutaneous malignancies, immunosuppressive treatments, and presence of CAs. CAs were arbitrarily considered as 'eruptive' when more than or equal to 10. Variables significantly associated with eruptive CAs were included in the logistic regression analysis, also stratified by age. A total of 1693 patients were included in the present study: 500 patients had malignancies, 460 malignant skin tumours, 263 had MM; 150 patients were immunosuppressed; 804 (47.49%) patients had eruptive CAs. In the whole study population, age, immunosuppressive treatment, MM, other skin and nonskin malignant tumours were significantly associated with eruptive CAs at the multivariate analysis. Multivariate analysis in each age subgroup revealed that the association between MM and eruptive CAs was highly significant in younger patients (≤50 years), significant in the 51-70 year-old subgroup, whereas it lost significance in older patients. These findings suggest an association between MM and eruptive CAs, particularly in the lower and intermediate age groups. Both the nature of this association and its possible impact in clinical practice, especially in MM screening, are yet to be established.

Generalized retiform purpura as a presenting sign of diffuse dermal angiomatosis.

A 49-year-old man presented with progressive, painful, ulcerative, retiform purpuric patches on the torso and extremities. Multiple skin biopsies revealed a prominent pan-dermal vascular proliferation but no occlusive vasculopathy or cutaneous vasculitis. Diffuse dermal angiomatosis should be considered in the differential diagnosis of retiform purpura, especially in patients with atherosclerotic disease or underlying hypercoagulable states.

Sporadic Meningioangiomatosis: Bystander or Curious Culprit.

In this report, we describe two cases of sporadic meningioangiomatosis (MA) - a rare condition of the central nervous system known to cause headaches, seizures and other focal neurologic deficits. Both patients presented with headache and vision change, somewhat suggestive of migraine. The combination of magnetic resonance imaging (MRI) and computerized tomography (CT) can establish the diagnosis of MA.

Eruptive pseudoangiomatosis - cherry angiomas with perilesional halo.

Eruptive pseudoangiomatosis is a rare viral exanthem characterized by acute onset of hemangiomata-like lesions, however, histological findings are distinct from that of true angiomas. This entity has been reported from Europe, North America, Japan, and Korea till date. Here, we report 12 cases of eruptive pseudoangiomatosis from a tertiary care hospital in Punjab.

Angiomatosis of the uterus, cervix and fallopian tubes: a rare and benign cause of intractable, heavy menstrual bleeding.

Angiomatosis of the uterus, cervix and fallopian tubes is a rare and benign entity that has not been reported in the literature previously. We present a case of a 27-year-old patient with severe and intractable heavy menstrual bleeding unresponsive to all conservative and conventional forms of treatment. Following a laparoscopic hysterectomy, the histopathological finding of angiomatosis, a vascular abnormality in the uterus, cervix and fallopian tubes, provided a plausible explanation in this situation.

Incidence and risk factors of massive subretinal hemorrhage in retinal angiomatous proliferation.

OBJECTIVE: To evaluate the incidence and associated risk factors of massive subretinal hemorrhage (SRH) in patients with retinal angiomatous proliferation (RAP). METHODS: A total of 187 eyes of 135 treatment-naive patients diagnosed with RAP were evaluated retrospectively. Clinical records including the time between the initial visit, last anti-vascular endothelial growth factor (VEGF) treatment, last stable examination, and the date of massive SRH were reviewed. Imaging findings including indocyanine green angiography (ICGA) and optical coherence tomography (OCT) were analyzed. RESULTS: Massive SRH developed in 18 eyes (9.6%) a median of 20 months after the initial presentation. Kaplan-Meier survival analysis revealed that the incidence (2.8, 5.8, 13.1, and 21.0% after 1,2,5 and 10 years, respectively) continuously increased. Among 14 eyes with discernable vascular anastomosis on baseline ICGA, 13 (92.8%) showed retinal arteriole involvement. On spectral-domain OCT imaging of the last visit prior to the massive SRH, a layered lamellar tissue complex was noted under the retinal pigment epithelium in 9 of 13 eyes, which was significantly associated with massive SRH[hazard ratio(HR),5.883;P = .010]. The average time between the last stable examination/last injection and the massive SRH was 2 and 5 months, respectively. The patients were treated with anti-VEGF, gas and recombinant tissue plasminogen activator injection; however, all except one eye had visual acuity worse than 20/1000 at the final visit. CONCLUSIONS: Massive SRH can occur in RAP in the course of anti-VEGF treatment, resulting in severe vision loss. A proactive dosing regimen may be more appropriate for these RAP eyes.

68Ga-PSMA Expression in Pseudoangiomatous Stromal Hyperplasia of the Breast.

Ga-labeled prostate-specific membrane antigen ligand PET-CT has emerged as a promising technique to evaluate the extent of disease in patients with prostate carcinoma. We are reporting a 63-year-old man with prostatic carcinoma subjected to Ga-prostate-specific membrane antigen ligand PET-CT for initial staging. In addition to the radiotracer uptake in known primary site (prostate), focal increased radiotracer uptake was also noticed in the left breast. Subsequent biopsy of the breast lesion revealed PASH (pseudoangiomatous stromal hyperplasia), which is a benign mesenchymal proliferative lesion that may present clinically as palpable mass requiring further evaluation to rule out malignancy.