Glomeruloid Hemangioma Research Articles

Phenomena of Intussusceptive Angiogenesis and Intussusceptive Lymphangiogenesis in Blood and Lymphatic Vessel Tumors.

Intussusceptive angiogenesis (IA) and intussusceptive lymphangiogenesis (IL) play a key role in the growth and morphogenesis of vessels. However, there are very few studies in this regard in vessel tumors (VTs). Our objective is to assess the presence, characteristics, and possible mechanisms of the formation of intussusceptive structures in a broad spectrum of VTs. For this purpose, examples of benign and malignant blood and lymphatic VTs were studied via conventional procedures, semithin sections, and immunochemistry and immunofluorescence microscopy. The results demonstrated intussusceptive structures (pillars, meshes, and folds) in benign (lobular capillary hemangioma or pyogenic granuloma, intravascular papillary endothelial hyperplasia or Masson tumor, sinusoidal hemangioma, cavernous hemangioma, glomeruloid hemangioma, angiolipoma, and lymphangiomas), low-grade malignancy (retiform hemangioendothelioma and Dabska tumor), and malignant (angiosarcoma and Kaposi sarcoma) VTs. Intussusceptive structures showed an endothelial cover and a core formed of connective tissue components and presented findings suggesting an origin through vessel loops, endothelialized thrombus, interendothelial bridges, and/or splitting and fusion, and conditioned VT morphology. In conclusion, the findings support the participation of IA and IL, in association with sprouting angiogenesis, in VTs, and therefore in their growth and morphogenesis, which is of pathophysiological interest and lays the groundwork for in-depth molecular studies with therapeutic purposes.

Glomeruloid Hemangioma in a Patient with TAFRO Syndrome.

Glomeruloid Hemangioma in a Patient with TAFRO Syndrome.

Intravascular Cutaneous Disorders. A Clinicopathologic Review.

Intravascular proliferations of the skin are clinically heterogeneous and may present with a wide range of clinical features, including violaceous papules, nodules, plaques, or other unspecific cutaneous lesions. Histopathologically, these conditions are characterized by proliferation of different cell types within the lumina of dermal vessels and endothelial cell hyperplasia. Immunohistochemistry is the best tool to identify the nature of the intravascular proliferating cells and the type of involved vessel. In this review, we analyzed the clinicopathologic and immunohistochemical characteristics of intravascular large cell lymphoma, T-cell and natural killer-cell intravascular large cell lymphoma, intralymphatic variant of CD30+ cutaneous lymphoproliferative disorders, benign atypical intralymphatic CD30+ T-cell proliferation, reactive angioendotheliomatosis, intralymphatic histiocytosis, papillary intralymphatic angioendothelioma or Dabska tumor, glomeruloid hemangioma, papillary hemangioma, intravascular papillary endothelial hyperplasia or Masson phenomenon, and the intralymphatic involvement of Merkel cell carcinoma, cutaneous metastases, and cutaneous angiosarcoma.

Glomeruloid Hemangioma as a Marker for the Early Diagnosis of POEMS Syndrome.

Glomeruloid Hemangioma as a Marker for the Early Diagnosis of POEMS Syndrome.

Contributions of Dr. Juan Rosai to the pathology of cutaneous vascular proliferations: A review of selected lesions.

Vascular proliferations in the skin have been thoroughly studied over the past 35 years, and a great deal of knowledge has been accrued regarding their pathobiological features. Dr. Juan Rosai has been a consistent contributor to the literature on this topic throughout most of his career, and this article reviews selected cutaneous endothelial lesions that he helped to characterize. They include histiocytoid-epithelioid hemangioma, targetoid-hobnail hemangioma, acquired tufted hemangioma, glomeruloid hemangioma, spindle cell hemangioma, retiform hemangioendothelioma, and angiosarcoma. Two potential simulants of angiosarcoma-papillary intravascular endothelial hyperplasia and cutaneous rudimentary meningocele-meningothelial hamartoma-are also considered.

Glomeruloid hemangioma in normal individuals

Glomeruloid hemangioma is a rare, histologically distinctive, cutaneous, benign vascular tumor, originally described by Chan et al. Glomeruloid hemangioma appears specific to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome (POEMS), and is usually, but not always, related to Castleman's disease. We herein report two cases with glomeruloid hemangioma, without any features of the POEMS syndrome. Glomeruloid hemangioma requires a high index of suspicion for diagnosis. It could rarely present as a solitary finding, without any evidence of an underlying POEMS syndrome.

An unusual presentation of glomeruloid hemangioma in a patient with VHL syndrome: A case report and review of literature

Von Hippel-Lindau (VHL) is an inherited neoplasia syndrome caused by inactivation of the VHL tumor suppressor gene, characterized by the development of sporadic clear cell renal carcinoma, pheochromocytomas, retinal angioma, pancreatic cysts, and CNS hemangioblastomas. Glomeruloid hemangioma is a vascular lesion, previously considered to be specifically associated with POEMS (polyneuropathy, organomegaly, endocrinopathy/edema, M-protein and skin abnormalities) syndrome. However, there are reports of solitary glomeruloid hemangioma in patients without POEMS syndrome. We report the case of a 39-year-old male with VHL disease, with known bilateral clear cell renal carcinomas, CNS hemangioblastoma and pancreatic cysts. The patient presented with a0.35 cmred papule on the left lateral neck, which was easily irritated, and bleed frequently. Histopathologically, there were irregular areas of ectatic vascular channels of small capillaries, resembling renal glomeruli, surrounded by actin-positive pericytes, within the dermis. These findings were consistent with a glomeruloid hemangioma. Fluorescent in-situ hybridization studies confirmed a deletion in the 3p25.3 region. As per clinical tests, no evidence of POEMS syndrome was found in this patient. Only six reports of glomeruloid hemangioma have been previously reported in patients without POEMS syndrome and this constitutes the first report of glomeruloid hemangioma in a patient with VHL.

Glomeruloid Hemangiomas in the Absence of POEMS Syndrome

Journal of Cutaneous PathologyVolume 39, Issue 4 p. 401-401 Glomeruloid Hemangiomas in the Absence of POEMS Syndrome Erick Jacobson-Dunlop MD, Erick Jacobson-Dunlop MD Department of Pathology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorHelen Liu MD, Helen Liu MD Department of Dermatology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorEric L. Simpson MD, MCR, Eric L. Simpson MD, MCR Department of Dermatology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorClifton R. White Jr. MD, Clifton R. White Jr. MD Departments of Dermatology and Pathology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorKevin P. White MD, Corresponding Author Kevin P. White MD Departments of Dermatology and Pathology, Oregon Health and Science University, Portland, ORe-mail: whiteke@ohsu.eduSearch for more papers by this author Erick Jacobson-Dunlop MD, Erick Jacobson-Dunlop MD Department of Pathology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorHelen Liu MD, Helen Liu MD Department of Dermatology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorEric L. Simpson MD, MCR, Eric L. Simpson MD, MCR Department of Dermatology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorClifton R. White Jr. MD, Clifton R. White Jr. MD Departments of Dermatology and Pathology, Oregon Health and Science University, Portland, ORSearch for more papers by this authorKevin P. White MD, Corresponding Author Kevin P. White MD Departments of Dermatology and Pathology, Oregon Health and Science University, Portland, ORe-mail: whiteke@ohsu.eduSearch for more papers by this author First published: 23 March 2012 https://doi.org/10.1111/j.1600-0560.2012.01898_1.xCitations: 2Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article.Citing Literature Volume39, Issue4April 2012Pages 401-401 RelatedInformation

Glomeruloid Hemangiomas in the Absence of POEMS Syndrome

Glomeruloid Hemangiomas in the Absence of POEMS Syndrome

Congenital eccrine angiomatous hamartoma associated with glomeruloid hemangioma without POEMS syndrome

Congenital eccrine angiomatous hamartoma associated with glomeruloid hemangioma without POEMS syndrome

Hemangioma glomeruloide e a síndrome POEMS: relato de dois casos e revisão da literatura

Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.

Uterine Glomeruloid Hemangioma in a Patient Without POEMS Syndrome

Cutaneous glomeruloid hemangioma is a hallmark of POEMS syndrome. These patients have elevated serum levels of vascular endothelial growth factor. The authors report an extracutaneous uterine glomeruloid hemangioma in an 82-year-old woman with a history of breast and endometrial carcinomas. Within the lumen of myometrial vessels, a lobular, glomeruloid proliferation of capillary-like CD31 and vascular endothelial growth factor receptor-1-positive endothelial cells was found. The capillary loops were lined by endothelial cells, most of them containing PAS-positive and immunoglobulin-positive eosinophilic hyaline globules (thanatosomes). This vascular proliferation was consistent with a glomeruloid hemangioma. Although an extracutaneous glomeruloid vascular proliferation has been found in the retroperitoneal adipose tissue in a patient with POEMS syndrome, this study reports what seems to be the first case of visceral glomeruloid hemangioma in a patient without POEMS syndrome. The authors hypothesize that the glomeruloid endothelial cell proliferation with vascular endothelial growth factor receptor-1 expression may be a paraneoplastic phenomenon.

Acquired facial atrophy: A neglected clinical sign of POEMS syndrome

I read with great interest the review on POEMS syndrome by Angela Dispenzieri [1]. As indicated by the author, skin lesions are frequent in this syndrome, some characteristic, and considered as minor criteria of POEMS syndrome. These include hyperpigmentation, hypertrichosis, and glomeruloid hemangioma, requiring a whole skin examination. However, the author does not mention acquired facial atrophy observed in more than half of patients in a recent dermatologic series including 21 patients conducted at our institution [2]. This clinical manifestation is similar to the lipoatrophy observed in HIV-infected patients under highly active antiretroviral therapy with protease inhibitors and nucleoside reverse transcriptase inhibitors as a part of the metabolic or lipodystrophy syndrome [3], or in patients with autoimmune diseases and alternate complement pathway abnormalities [4], including lupus panniculitis [5]. In our institution, we consider that POEMS syndrome should be evoked in patients not under antiretroviral treatment presenting with acquired facial lipoatrophy not preceded by a deep skin infiltration as observed in lupus panniculitis, when associated with polyradiculoneuropathy and monoclonal gammopathy. Claude Bachmeyer*, * Service de Médecine Interne, CHU Tenon (AP-HP), 4 rue de la Chine, 75020 Paris, France.

Glomeruloid hemangioma as a very early presenting sign of POEMS syndrome

Glomeruloid hemangioma as a very early presenting sign of POEMS syndrome

Síndrome de POEMS con lesión lítica aislada y hemangiomas glomeruloides: Caso clínico

POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.

Glomeruloid haemangioma is not always associated with POEMS syndrome

Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. We report a 78-year-old man who presented with a single, red-violet soft nodule with superficial telangiectases on the scalp. Histopathologically, the lesion consisted of lobules of coiled aggregated capillaries that involved the lumina of dilated vascular structures, mimicking renal glomeruli. A collagenous stroma separated the capillary lobules, and eosinophilic, periodic-acid-Schiff positive globules of varying sizes and shapes were seen within the cytoplasm of endothelial cells. Immunohistochemical studies with antibodies against IgA and IgG, and against the kappa and lambda light chains of immunoglobulins showed immunoreactivity within the eosinophilic globules. Results of complete blood count, liver, renal and thyroid function tests, fasting blood sugar measurement, serum levels of oestradiol, testosterone, prolactin and cortisol, serum protein electrophoresis, immunoelectrophoresis and immunofixation yielded normal or negative results. No Bence-Jones proteinuria was found in a sample from a 24-h urine collection. To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.

Eosinophilic Globules in 3 Cases of Glomeruloid Hemangioma of the Head and Neck: A Characteristic Offering More Evidence for Thanatosomes With or Without POEMS

Glomeruloid hemangiomas (GHs) are glomeruli-like capillary tufts lined by endothelial cells that contain periodic acid-Schiff (PAS) positive eosinophilic globules (EGs). These hemangiomas are characteristic cutaneous manifestation of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes). Hemangiomas histologically identical to GHs but not associated with POEMS have recently been designated as papillary hemangiomas. In this report, we present solitary head and neck GHs in 3 patients, 2 without POEMS, with particular attention to the characteristic EGs. We performed immunostains for hemoglobin A, kappa and lambda light chains, factor VIII-related antigen, CD31 and CD34, PAS stain after diastase digestion (PASD), and electron microscopic examinations on routinely fixed tissues containing EGs. Eosinophilic globules stained uniformly positive for PASD but only peripherally positive for hemoglobin and light chains on surfaces, with interiors negative for antigens. Factor VIII-related antigen and CD31 and CD34 confirmed cells containing EGs to be endothelial. Electron microscopic examination suggested that EGs are enlarged secondary lysosomes (thanatosomes). These features fail to support red blood cells or immunoglobulins as EG constituents. Glomeruloid hemangiomas may be vascular proliferations stimulated by endothelial cells' protein phagocytosis but not by phagocytosis of either hemoglobin-containing red blood cells or immunoglobulins. The vascular lesions in POEMS syndrome appear identical to papillary hemangioma in cases without the other syndromic manifestations.

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.

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-kappa 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 alpha-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.

Glomeruloid hemangiomas without POEMS syndrome: series of three cases

Glomeruloid hemangiomas without POEMS syndrome: series of three cases