Glomeruloid hemangioma

A 64‐year‐old Japanese woman suffering from idiopathic thrombocytopenic purpura was treated with prednisolone. During the course of steroid withdrawal she developed parotid gland enlargement and cervical lymph node swelling with multiple dome‐shaped red papules on her trunk and upper limbs. On admission the patient was found to have numbness of her lower limbs (polyneuropathy), lymph node swelling (organomegaly), high glucose level (endocrinopathy), Bence‐Jones protein‐κ in the urine (M protein) and skin with hyperpigmentation, hypertrichosis and multiple glomeruloid hemangiomas (skin abnormalities), indicating polyneuropathy–organomegaly–endocrinopathy–M‐protein–skin abnormality (POEMS) syndrome. The patient was also found to have peripheral edema, ascites, and pleural effusion. The glomeruloid hemangiomas had intravascular capillary growth, which was composed of conglomerates of capillaries resulting in structures resembling renal glomeruli. Cells within the capillary loops were lined by endothelial cells with scant cytoplasm (CD31⁺/CD34⁺/CD68^‐/CD105⁺/UEA‐1⁺) while the outer surfaces of the loops were covered by either swollen endothelial cells containing PAS‐ and immunoglobulin‐positive eosinophilic hyaline globules (CD31⁺/CD34^‐/CD68^?/+/CD105^‐/UEA‐1^‐) or cells without globules. These two phenotypically different endothelial cells were separated by α‐smooth muscle actin‐positive pericytes. Pericytes and endothelial cells covering the outer surface of the loops were bordered by basement membrane. Biopsy of parotid gland and lymph node indicated Sjögren's syndrome and Castleman's disease of a hyaline‐vascular type, respectively. Resumed prednisolone therapy has been successful, and the patient was left with minimal residual symptoms. Glomeruloid hemangioma is a specific marker of POEMS syndrome and is related to Castleman's disease. Idiopathic thrombocytopenic purpura and Sjögren's syndrome may also be related.     

Prognostic significance of VEGF, VEGF receptors, and microvessel density in diffuse large B cell lymphoma treated with anthracycline-based chemotherapy

Vascular endothelial growth factor-mediated signaling has at least two potential roles in diffuse large B cell lymphoma: potentiation of angiogenesis, and potentiation of lymphoma cell proliferation and/or survival induced by autocrine vascular endothelial growth factor receptor-mediated signaling. We have recently shown that diffuse large B cell lymphomas expressing high levels of vascular endothelial growth factor protein also express high levels of vascular endothelial growth factor receptor-1 and vascular endothelial growth factor receptor-2. We have now assessed a larger multi-institutional cohort of patients with de novo diffuse large B cell lymphoma treated with anthracycline-based therapy to address whether tumor vascularity, or expression of vascular endothelial growth factor protein and its receptors, contribute to patient outcomes. Our results show that increased tumor vascularity is associated with poor overall survival (P=0.047), and is independent of the international prognostic index. High expression of vascular endothelial growth factor receptor-1 by lymphoma cells by contrast is associated with improved overall survival (P=0.044). The combination of high vascular endothelial growth factor and vascular endothelial growth factor receptor-1 protein expression by lymphoma cells identifies a subgroup of patients with improved overall (P=0.003) and progression-free (P=0.026) survival; these findings are also independent of the international prognostic index. The prognostic significance of overexpression of this ligand-receptor pair suggests that autocrine signaling via vascular endothelial growth factor receptor-1 may represent a survival or proliferation pathway in diffuse large B cell lymphoma. Dependence on autocrine vascular endothelial growth factor receptor-1-mediated signaling may render a subset of diffuse large B-cell lymphomas susceptible to anthracycline-based therapy.     

Potential autocrine function of vascular endothelial growth factor in head and neck cancer via vascular endothelial growth factor receptor-2.

Vascular endothelial growth factor is a peptide with well-defined actions on the vasculature and fundamental role in tumor angiogenesis. Its action in vascular endothelium is exerted in a paracrine manner. The immunohistochemical expression of this protein by cancer cells in head and neck squamous cell carcinoma was correlated with increased tumor aggressiveness and poor survival in previous studies. In the past years, an increasing amount of studies demonstrated potential autocrine action of vascular endothelial growth factor in various neoplasms. However, the existence and the impact of such autocrine action in head and neck cancer have not been demonstrated yet. In this retrospective study, we evaluated the expression of vascular endothelial growth factor and its receptors in neoplastic cells, in a cohort of patients with head and neck squamous cell carcinoma, and compared this expression with tumor aggressiveness, clinicopathologic parameters and outcome. High expression of vascular endothelial growth factor was strongly correlated with high expression of vascular endothelial growth factor receptor-2 (but not vascular endothelial growth factor receptor-1) on the cancer cells (P<0.001). The co-overexpression of both the protein and vascular endothelial growth factor receptor-2 was associated with higher tumor proliferation rate (P<0.001). The above co-overexpression also correlated with worse survival (log rank P<0.05) in patients with oral-larynx squamous cell carcinoma. Our results suggest that an autocrine vascular endothelial growth factor loop, mediated via vascular endothelial growth factor receptor-2, probably exists in head and neck squamous cell carcinoma. These observations support the hypothesis that the use of vascular endothelial growth factor receptor-2 inhibitors as adjuvant antiangiogenic therapy might have beneficial effects for these patients, by disrupting both paracrine (endothelial-dependent) and autocrine actions of vascular endothelial growth factor.     

Placental expression of vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 correlates with severity of clinical preeclampsia and villous hypermaturity

Overexpression of soluble vascular endothelial growth factor receptor-1 has been linked to preeclampsia and is thought to be secondary to placental insufficiency caused by hypoxia. Villous hypermaturity, characterized by presence of increased syncytial knots, has been associated with syndromes of placental insufficiency, particularly when severe. This study was undertaken to determine whether there is a link between soluble vascular endothelial growth factor receptor-1 expression, villous hypermaturity, and clinical severity of preeclampsia. We conducted a retrospective cohort study in which 48 placentas were selected from pathology archives (hypertensive group). Of these, 6 had chronic hypertension, 15 had mild preeclampsia, 14 had severe preeclampsia, and 13 had hemolysis, elevated liver enzymes, and low platelets syndrome. These were compared with 55 placentas from normotensive patients (control group). One representative section of placental parenchyma from each case was stained with an antibody to vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 and given a score based on extent and intensity of staining, representing expression level. Assignment of staining score was done, blinded to clinical history and pathologic diagnosis. Vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 staining was seen in placental syncytiotrophoblasts and was particularly strong in syncytial knots. There was a positive association between vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 staining score and severity of clinical hypertensive state, small placental size, and villous hypermaturity. The association between vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 score and small placentas did not persist after controlling for hypermaturity. Vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 overexpression in the placenta strongly correlates with both severity of hypertensive disease and villous hypermaturity. The correlation with villous hypermaturity further links hypoxia to vascular endothelial growth factor receptor-1/soluble vascular endothelial growth factor receptor-1 production in the placenta.     

Glomeruloid microvascular proliferation follows adenoviral vascular permeability factor/vascular endothelial growth factor-164 gene delivery

Glomeruloid bodies are a defining histological feature of glioblastoma multiforme and some other tumors and vascular malformations. Little is known about their pathogenesis. We injected a nonreplicating adenoviral vector engineered to express vascular permeability factor/vascular endothelial growth factor-164 (VPF/VEGF(164)) into the ears of athymic mice. This vector infected local cells that strongly expressed VPF/VEGF(164) mRNA for 10 to 14 days, after which expression gradually declined. Locally expressed VPF/VEGF(164) induced an early increase in microvascular permeability, leading within 24 hours to edema and deposition of extravascular fibrin; in addition, many pre-existing microvessels enlarged to form thin-walled, pericyte-poor, "mother" vessels. Glomeruloid body precursors were first detected at 3 days as focal accumulations of rapidly proliferating cells in the endothelial lining of mother vessels, immediately adjacent to cells expressing VPF/VEGF(164). Initially, glomeruloid bodies were comprised of endothelial cells but subsequently pericytes and macrophages also participated. As they enlarged by endothelial cell and pericyte proliferation, glomeruloid bodies severely compromised mother vessel lumens and blood flow. Subsequently, as VPF/VEGF(164) expression declined, glomeruloid bodies devolved throughout a period of weeks by apoptosis and reorganization into normal-appearing microvessels. These results provide the first animal model for inducing glomeruloid bodies and indicate that VPF/VEGF(164) is sufficient for their induction and necessary for their maintenance.     

POEMS syndrome with idiopathic portal hypertension: Autopsy case and review of the literature

Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome is a rare multi‐system disease. Reported herein is an autopsy case of POEMS syndrome in a subject who developed idiopathic portal hypertension (IPH). The patient was a 38‐year‐old woman who was initially admitted to the Saiseikai Central Hospital because of polyneuropathy and edema. Diagnosis of POEMS syndrome was established on additional symptoms (splenomegaly and papilloedema) and serum M‐protein. Corticosteroid was given for 10 years. The patient was admitted again at the age of 48 years because of gastrointestinal bleeding due to portal hypertension. The patient died of hepatoencephalopathy at 58 years of age. The liver at autopsy demonstrated dense portal fibrosis and obliteration of small portal vein branches, which are characteristic histological findings of IPH. Portal hypertension is a rare symptom in POEMS syndrome. Only three cases of IPH associated with POEMS syndrome (including the present one) have been reported so far. In the previous two reports, liver biopsy failed to determine the cause of portal hypertension. This is the first report on the occurrence of histological findings compatible with IPH in the liver. Although it is not confirmed whether IPH is related to POEMS syndrome, elevated serum cytokines such as vascular endothelial growth factor and coagulation abnormality could have contributed to the development of IPH in the present case.     

血管内皮生长因子与促血管生成素1对大鼠血管内皮细胞的作用

背景：血管内皮生长因子、促血管生成素1是血管形成过程中始动并且使之持续的重要因子,研究其对血管内皮细胞的作用具有重要的意义。 目的：观察血管内皮生长因子与促血管生成素1对培养血管内皮细胞迁移与增殖能力的影响,并探讨其在血管生成方面的作用机制。 方法：在大鼠脐静脉内皮细胞内单独或联合加入血管内皮生长因子、促血管生成素1后,划痕实验和MTT检测对细胞迁移与增殖的影响,观察内皮细胞形态、活性、迁移能力。 结果与结论：划痕实验显示单独血管内皮生长因子作用时,与空白对照组细胞迁移无明显差异,单独促血管生成素1作用时,不仅不能增加细胞的迁移作用,反较空白对照组有所减弱,当血管内皮生长因子与促血管生成素1联合作用时,细胞迁移较空白对照组明显增强;MTT实验结果表明：单纯加入血管内皮生长因子或促血管生成素1,均不能起到有效促进内皮细胞增殖的作用;联合应用血管内皮生长因子及促血管生成素1可有效促进增殖。结果可见当血管内皮生长因子与促血管生成素1联合应用时,才能有效促内皮细胞迁移与增殖,发挥促血管生成作用。     

血小板反应素-1对猪胸导管内皮细胞增殖和凋亡的影响

目的探讨血小板反应素-1(thrombospondin-1,TSP-1)对淋巴管内皮细胞(lymphatic endothelial cells,LECs)增殖和凋亡的影响。方法取健康猪的胸导管内皮细胞进行分离、培养;应用凝血因子VIII,血管内皮生长因子受体-3(VEGFR-3)抗体联合对淋巴管内皮细胞进行鉴定;采用MTT法检测TSP-1对LEC的生长抑制作用;采用Hoechst33258荧光染色检测淋巴管内皮细胞凋亡。结果培养的淋巴管内皮细胞呈第VIII因子和VEGFR-3阳性反应,为典型淋巴管内皮细胞。MTT法证实,当浓度为0.8μg/ml￣2.0μg/ml时,TSP-1能明显抑制淋巴管内皮细胞的增殖,且抑制作用呈现剂量依赖关系。Hoechst33258证实,经TSP-1处理后淋巴管内皮细胞,可观察到其核周围有凋亡小体。结论TSP-1对淋巴管内皮细胞增殖有明显的剂量依赖性抑制作用,诱导淋巴管内皮细胞凋亡。     

Excessive Endothelial Cell Proliferation Occurring in an Organizing Thyroid Hematoma: Report of a Case and Review of the Literature

The authors report a case of a thyroid hematoma containing an exuberant endothelial cell proliferation. No underlying vascular neoplasm was apparent and the hematoma occurred in a substernal goiter from a 90-yr-old male. Review of the literature revealed four similar previously reported cases of endothelial cell proliferation found in thyroid hematomas for a total of five cases. A fine needle aspiration or biopsy procedure antedated the definitive surgery in four patients and may be responsible for the formation of the hematoma in these cases. Areas of the endothelial proliferation resembled papillary endothelial hyperplasia (Masson’s hemangioma) in four cases and cavernous hemangioma in three. However, these lesions occurred outside of vascular lumina and instead were intimately associated with fibrinous material of the hematoma. In addition, two cases contained foci of endothelial cells with nuclear atypia or intracytoplasmic lumens requiring distinction from angiosarcoma. These vigorous endothelial proliferations most likely represent alterations in the organization of a thyroid hematoma, a process similar to papillary endothelial hyperplasia, which is usually intravascular. Primary vascular neoplasms of the thyroid are exceedingly rare. Nevertheless, awareness of exuberant endothelial cell proliferation as a potential, albeit rare, manifestation of a thyroid hematoma should prompt careful attention to distinguish this reactive process from vascular neoplasms such as hemangioma or angiosarcoma.     

Infantile hemangiomas are arrested in an early developmental vascular differentiation state.

Infantile hemangiomas, the most common tumors of infancy, are vascular tumors characterized by rapid proliferation of endothelial cells during the first few months of postnatal life followed by slow spontaneous involution, whose molecular pathogenesis remains unclear. The recent identification of developmental expression of vascular lineage-specific markers prompted us to characterize infantile hemangiomas for the expression of lymphatic endothelial hyaluronan receptor-1 (LYVE-1), Prox-1, CD31 and CD34. We found that LYVE-1, a specific marker for normal and tumor-associated lymphatic vessels, was strongly expressed in tumor cells of infantile hemangiomas (n=28), but not in other vascular tumors including pyogenic granulomas (n=19, P<0.0001) or intramuscular hemangiomas (n=9), using LYVE-1/CD31 double immunostains. Whereas LYVE-1 expression was detected on the endothelial cells of all proliferating infantile hemangiomas, this lymphatic marker was absent from the lesional capillaries during involution in the majority of cases (P=0.0009). The majority of LYVE-1(+) endothelial cells also expressed CD34, but were negative for the lymphatic-specific homeobox protein Prox-1. Based on coexpression of both LYVE-1 and the blood vascular marker CD34, we propose that the endothelial cells in proliferating infantile hemangioma are arrested in an early developmental stage of vascular differentiation. The immature, incompletely differentiated immunophenotype of proliferating infantile hemangiomas may contribute to their rapid growth during the first few months of life.     

Angiogenic Factor with G-patch and FHA Domain 1 (AGGF1) Expression in Human Vascular Lesions

Angiogenic factor with G-patch and FHA domain 1 (AGGF1) is a novel angiogenic factor that was first described in Klippel-Trenaunay syndrome, a congenital vascular disease associated with capillary and venous malformations. AGGF1, similar to vascular endothelial growth factor (VEGF), has been shown to promote strong angiogenesis in chick embryos in vivo. Blocking AGGF1 expression prevented vessel formation, which suggests AGGF1 is a potent angiogenic factor linked to vascular malformations. So far, AGGF1 expression studies in human vascular lesions have not been performed. Here, we immunohistochemically investigated AGGF1 expression in venous, arteriovenous or capillary malformations, and infantile or congenital hemangioma. We found that AGGF1 was mostly expressed in endothelial cells with plump morphology. Moreover, the majority of mast cells strongly expressed AGGF1. Notwithstanding our incomplete knowledge of the molecular mechanism of AGGF1 in angiogenesis, our results show for the first time that AGGF1 is expressed in plump endothelial cells and mast cells.     

Endovascular papillary angioendothelioma (Dabska tumor)

The histological diagnosis of endovascular papillary angioendothelioma (EPA) (Dabska tumor) is controversial although the tumor is included in the World Health Organization classification. Papillary endothelial proliferation with a central hyaline core is one of the most characteristic features of EPA; however, this type of proliferation has been observed in other vascular tumors, such as angiosarcoma, retiform hemangioendothelloma and glomeruloid hemangioma. Several vascular tumors have EPA-like foci and EPA is not well defined generally. Endovascular papillary angioendothelioma may not be a distinct entity and may well include a heterogeneous group of lesions.     

Recently characterized vascular tumours of skin and soft tissues

This review summarizes the clinicopathological features of a number of vascular tumours that have been characterized only in recent years. These include: glomeruloid haemangioma, a multifocal vascular lesion associated with POEMS syndrome: Kaposi-like infantile haemangioendothelioma, a borderline malignant tumour occurring in the retroperitoneum of infants, mimicking Kaposi's sarcoma histologically; giant cell angioblastoma, characterized by proliferated vessels with a granuloma-like appearance; benign lymphangioendothelioma (progressive lymphangioma), slowly-growing macule or plaque over the trunk or limb, mimicking low-grade angiosarcoma histologically; targetoid haemosiderotic haemangioma, a benign lesion with a distinctive annular appearance and histologically overlapping with benign lymphangioendothelioma; spindle cell haemangioendothelioma, a lesion located mostly in the distal extremities, characterized by cavernous vascular spaces, spindle cells with interspersed narrow vascular channels and scattered plump vacuolated endothelial cells; acquired tufted angioma, characterized by 'cannon-ball' involvement of the dermis by lobules of pericyte-rich capillaries; sinusoidal haemangioma, a distinctive variant of cavernous haemangioma which may be confused with angiosarcoma; and epithelioid angiosarcoma, a highly aggressive tumour of deep soft tissue mimicking metastatic carcinoma and co-expressing endothelial and epithelial markers.     

血管内皮生长因子及其受体家族在角膜组织及角膜病变中的作用和研究进展

BACKGROUND: Vascular endothelial growth factors are a family of multifunctional cytokines that can enhance vascular permeability, induce angiogenesis, promote endothelial cell growth and migration, and inhibit cell apoptosis. OBJECTIVE: To elaborate the latest progress in the role of vascular endothelial growth factor and its receptors in the corneal tissue. METHODS: A computer-based search of PubMed databases was performed for relevant articles published from 2005 to 2015. The key words were “vascular endothelial growth factor, cornea”. According to the inclusion and exclusion criteria, 43 articles were included in result analysis. RESULTS AND CONCLUSION: Vascular endothelial growth factor and its receptors are involved in the regulation of corneal neovascularization by causing Tip cell activation that affects the Notch signaling pathways. Corneal lymphatic regeneration mainly relies on macrophages to secrete vascular endothelial growth factor-C or vascular endothelial growth factor-D that further activate vascular endothelial growth factor receptor-3 in the lymphatic endothelial cells to cause cell proliferation and migration, and eventually lead to the formation of new lymphatic vessels. But herpes simplex keratitis HSK induces the corneal lymphatic regeneration by vascular endothelial growth factor-A/vascular endothelial growth factor receptor-2 pathway. Vascular endothelial growth factor family can significantly improve the damaged corneal nerve endings, epithelium and corneal sensitivity, has the function of nerve nutrition and promote restoration of the corneal epithelium.       

Increased Tie2 Expression, Enhanced Response to Angiopoietin-1, and Dysregulated Angiopoietin-2 Expression in Hemangioma-Derived Endothelial Cells

Infantile hemangiomas are endothelial tumors that grow rapidly in the first year of life and regress slowly during early childhood. Although hemangiomas are well-known vascular lesions, little is known about the mechanisms that cause the excessive endothelial cell proliferation in these most common tumors of infancy. To investigate the molecular basis of hemangioma, we isolated endothelial cells from several proliferative-phase lesions and showed that these cells are clonal and exhibit abnormal properties in vitro (E. Boye, Y. Yu, G. Paranya, J. B. Mulliken, B. R. Olsen, J. Bischoff: Clonality and altered behavior of endothelial cells from hemangiomas. J Clin Invest 2001, 107:745-752). Here, we analyzed mRNA expression patterns of genes required for angiogenesis, including members of the vascular endothelial growth factor (VEGF)/VEGF receptor family and the angiopoietin/Tie family, in hemangioma-derived and normal endothelial cells. KDR, Flt-1, Tie1, Tie2, and angiopoietin-2 (Ang2) were strongly expressed in cultured hemangioma-derived endothelial cells and in hemangioma tissue. In contrast, there was little expression of angiopoietin-1 (Ang1) or VEGF. We found Tie2 mRNA and protein up-regulated with a concomitant increase in cellular responsiveness to Ang1 in most hemangioma-derived endothelial cells. Ang2 mRNA was down-regulated in response to serum in hemangioma-derived endothelial cells, but not in normal endothelial cells, suggesting altered regulation. These findings implicate Tie2 and its ligands Ang1 and Ang2 in the pathogenesis of hemangioma.     

Vascular endothelial growth factor confers a growth advantage in vitro and in vivo to stromal cells cultured from neonatal hemangiomas.

Neonatal hemangioma is a common benign proliferation of unorganized structures containing stromal and capillary endothelial cells. We tested the hypothesis that such cell proliferation might result from the release by stromal cells of endothelial cell mitogens. Stromal cells cultured from biopsies of surgically removed life-threatening hemangiomas released an endothelial cell mitogen in vitro that was indistinguishable from vascular endothelial growth factor (VEGF) based on independent criteria such as affinity chromatography for heparin or anti-VEGF IgG and radioreceptor assay. A functional product of the KDR gene encoding a cognate VEGF receptor was also expressed by these stromal cells. Transient transfection with antisense oligonucleotides targeted on the translation initiation codon of KDR abolished its tyrosine phosphorylation and mitogenic response of neonatal hemangioma cells to VEGF, confirming the existence of an autocrine loop of proliferation. When grafted in nude mice, these stromal cells elicited an angiogenic response that was blocked by neutralizing anti-VEGF IgG. These results might provide a clue to the importance of stromal cells in the pathogeny of neonatal hemangiomas.     

BILATERAL OVARIAN HEMANGIOMAS ASSOCIATED WITH DIFFUSE HEMANGIOENDOTHELIOMATOSIS: A CASE REPORT

A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome".     

Bilateral ovarian hemangiomas associated with diffuse hemangioendotheliomatosis: a case report

A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome."     

Papillary hemangiomas and glomeruloid hemangiomas are distinct clinicopathological entities

The author reviews and compares the clinicopathological features of papillary hemangiomas and glomeruloid hemangiomas, 2 rare, cutaneous intravascular capillary-type vascular lesions with overlapping morphological details. Immunostaining for collagen IV highlighted discriminating features in these lesions. Thin basement membranes and glomeruloid architecture are typical of glomeruloid hemangiomas, whereas papillae with thick mantles of a basement membrane- like matrix enveloping pericytes are prominent in papillary hemangiomas. Thus, collagen IV staining patterns provide further evidence that papillary and glomeruloid hemangiomas represent distinct histopathological entities. This additional technique should allow pathologists to readily distinguish between the lesions and make a proper diagnosis. What is important is that glomeruloid hemangiomas-often presenting in a spectrum of multiple cutaneous vascular lesions, including cherry hemangiomas-are a hallmark of POEMS (acronym for polyneuropathy, oganomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and/or multicentric Castleman's disease, whereas papillary hemangiomas clinically present as innocent solitary cutaneous hemangiomas in otherwise healthy individuals.