Glomeruloid Hemangioma Research Articles

Glomeruloid hemangiomas localized to the skin of the trunk with no clinical features of POEMS syndrome

Glomeruloid hemangiomas localized to the skin of the trunk with no clinical features of POEMS syndrome

Increased expression of vascular endothelial growth factor and its receptor, Flt‐1, in glomeruloid haemangioma associated with Crow–Fukase syndrome

Increased expression of vascular endothelial growth factor and its receptor, Flt‐1, in glomeruloid haemangioma associated with Crow–Fukase syndrome

Renal Involvement in Multicentric Castleman Disease With Glomeruloid Hemangioma of Skin and Plasmacytoma

A 47-year-old man presented with fever of unknown origin, generalized weakness, edema, and renal failure. He had left-sided pleural effusion, generalized lymphadenopathy, multiple nontender cutaneous nodules, hepatomegaly, renal failure, and hypergammaglobulinemia. Axillary lymph node biopsy showed findings consistent with Castleman disease of the hyaline vascular type associated with interfollicular plasmacytosis. A renal biopsy performed in view of proteinuria and acute renal failure showed hypercellular glomeruli with capillary loop thickening and double contours consistent with membranoproliferative glomerulonephritis. Skin nodule biopsy showed a glomeruloid hemangioma characterized by dermal proliferation of capillary loops in a nodular manner resembling a glomerulus. He experienced clinical and biochemical remission with steroids. Discontinuation of steroid therapy was associated with recurrence of renal failure, reappearance of nodules, lymphadenopathy, and appearance of bony lytic lesions. Biopsy of bony lytic lesions showed plasmacytoma. The patient achieved complete remission on treatment with steroids and cyclophosphamide and is free of symptoms at the end of 40 months of follow-up. To our knowledge, this is the first case report of the occurrence of membranoproliferative glomerulonephritis, glomeruloid hemangioma of the skin, and plasmacytoma in a patient with multicentric Castleman disease without human immunodeficiency virus infection.

Glomeruloid haemangioma with cerebriform morphology in a patient with POEMS syndrome

A 40-year-old Chinese man presented with sensorimotor polyneuropathy, IgAlambda paraprotein, osteosclerotic bone lesions, hypertrichosis, and impotence with decreased testosterone and raised prolactin level. POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome was diagnosed and he was treated with melphalan and prednisolone. After chemotherapy, other manifestations of POEMS syndrome developed, such as multiple haemangiomas over the chest and neck region, splenomegaly and generalized oedema. One haemangioma had a peculiar clinical morphology, similar to the appearance of cerebral gyri. Skin biopsy confirmed the diagnosis of glomeruloid haemangioma. Chemotherapy was then switched to cyclophosphamide and prednisolone, resulting in further improvement in muscle power and hypertrichosis. To our knowledge, this is the first report on a clinically distinctive morphology of glomeruloid haemangioma, and its recognition may increase the index of suspicion for early skin biopsy.

Glomeruloid hemangiomas: A marker for POEMS

Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) is a multisystem syndrome that may accompany plasma cell dyscrasias.1,2 Polyneuropathy and a monoclonal plasmaproliferative disorder must be present along with one other minor criterion, including sclerotic bone lesions, Castleman's disease, organomegaly, edema, endocrinopathy, skin changes, or papilledema. Other associated findings may include ascites, pleural effusions, and thrombocytosis. We describe a patient with glomeruloid hemangiomas, a skin abnormality seen exclusively in POEMS.3,4 This has been reported only rarely in the neurologic literature and is a useful diagnostic criterion.5 A 58-year-old woman presented with weakness, distal sensory loss, pain, and edema in the lower extremities. The symptoms had progressed over 3 years. She was using a walker to ambulate. Neurologic examination 3 years earlier, at the time of an episode of low back pain, was normal. She was an obese woman with extensive edema of the extremities and thickening of the skin in the legs and forearms. …

Solitary glomeruloid haemangioma without POEMS syndrome

The term 'glomeruloid haemangioma' was coined by Chan et al. for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in patients without this syndrome. We report on an 86-year-old man without POEMS syndrome and with a solitary red papule on the face. A cutaneous biopsy showed histological findings consistent with a glomeruloid haemangioma. Physical examination of the skin did not show any other cutaneous lesion and laboratory and radiological studies ruled out the presence of POEMS syndrome. Glomeruloid haemangiomas could exceptionally be present as solitary vascular tumours and out of the context of POEMS syndrome. To our knowledge, this is the first case reported of glomeruloid haemangioma without POEMS syndrome. Moreover, the presentation on the face is also highly unusual.

Immunohistochemical Characterization of Hyaline Globules in Kaposi’s Sarcoma

Hyaline globules (HG) are frequently found in neoplastic cells of Kaposi’s sarcoma (KS). Presence of HG, characteristic of but not specific of KS, has been considered to represent erythrophagocytosis. In order to further characterize the nature of HG, we studied 10 cases of cutaneous KS with distinct HG using immunohistochemistry for hemoglobin A, Kappa and Lambda. All KS cases were of nodular type. Also included were 3 cases of glomeruloid hemangioma with similar HG in endothelial cells for the comparision. In selected cases electron microscopic examination was performed. PAS stain with diastase digestion was also done in all cases. Results: HG were diastase resistant and PAS positive. All HG in neoplastic cells of KS and endothelial cells of GH were negative for hemoglobin A. There was variable staining for light chains: focal but strong positivity for kappa, weak and variable staining for Lambda. Some HG was positive for both kappa and lambda. Electron microscopic study demonstrated HG as homogeneous electron dense inclusions with internal small vacuoles, whorled or granular materials. Conclusions: All HG in neoplastic cells and endothelial cells of GH represent phagolysosomes. Negative stain for hemoglobin A in KS and GH do not support the inclusions being fragmented RBC’s.

Vascular tumors of the skin: A selective review

Cutaneous vascular proliferations are a vast and complex spectrum. Many appear as hamartomas in infancy; others are acquired neoplasms. Some vascular proliferations are hyperplastic in nature, although they mimic hemangiomas, i.e., neoplasms. The vast majority of the vascular lesions are hemangiomas. Between the hemangiomas and frankly angiosarcomas, there is a group of neoplasms that are angiosarcomas, albeit ones of low grade histologically and, probably, biologically. The term "hemangioendothelioma" has been created to encompass these neoplasms. Vascular proliferations are, fundamentally, composed of endothelial cells. Some hemangiomas, however, contain also abundant pericytic, smooth muscle, or interstitial components, or a combination of them. These heterogeneous cellular components are present usually in hemangiomas. Some of the newly described vascular proliferations, however, are difficult to differentiate from some of the angiosarcomas. Others are markers, occasionally, of serious conditions such as Fabry's Disease (angiokeratoma) and POEM's syndrome (glomeruloid hemangioma). Kaposi's sarcoma continues to be an enigma. The demonstration of Herpes virus 8 in this condition raises doubt about its neoplastic nature. The demonstration of endothelial differentiation of its nodular lesions is tenuous and its true nature remains unresolved. While physicians have known about post-mastectomy angiosarcomas from the origin of the radical mastectomy, a new group of unusual vascular proliferations of the mammary skin are being defined. These lesions arise in the setting of breast-conserving surgical treatment with adjuvant radiation therapy. The incubation period is usually 3 to 5 years, in contrast with the 10, or more, in classical cases of post-mastectomy angiosarcoma. These lesions usually are subtle, both clinically and histologically, in contrast with the "classical," dramatic presentation of mammary angiosarcoma. The spectrum of findings ranges from "simple" lymphangiectasia-like vascular proliferations to unequivocal angiosarcomas. The pathogenesis of these lesions remains a mystery. There are very few clues that allow one to separate hemangiomas from angiosarcomas. The presence of heterologous cellular elements and, particularly, well-developed smooth muscle components tends to favor a hemangioma. Similarly, the presence of thrombosis usually supports hemangioma. Nevertheless, there are no unequivocal or reliable individual diagnostic criteria. A thorough knowledge of the different conditions and their differential diagnoses eventually leads to the proper diagnosis in most cases.

A Case of Glomeruloid Hemangioma in a Patient with Multicentric Castleman's Disease

A Case of Glomeruloid Hemangioma in a Patient with Multicentric Castleman's Disease

Association of Kaposi's sarcoma and leishmaniasis in a HIV patient

This second part of our review about vascular proliferations summarizes the clinicopathologic features of the cutaneous vascular hyperplasias and benign neoplasms. Hyperplasias comprise a heterogeneous group of vascular proliferations that eventually show a tendency to regression. Angiolymphoid hyperplasia with eosinophilia is included within the group of hyperplasias because of its historical denomination and its reactive nature, probably as a consequence of an arteriovenous shunt, although usually the lesions do not regress. Pyogenic granuloma, bacillary angiomatosis, intravascular papillary endothelial hyperplasia, and pseudo–Kaposi's sarcoma qualify as vascular hyperplasias because they regress when the stimulus that initiated them is removed. Benign neoplasms form a large group of hemangiomas with distinctive clinicopathologic characteristics, although some of them are of recent description and may produce diagnostic difficulties. We classified cutaneous benign vascular neoplasms according to their cell lineage of differentiation, for example, endothelial, glomus cell, and pericytic differentiation. Subsequent categories are established according to the size of the involved vessels (capillaries, venules and arterioles, or veins and arteries) or the nature of the proliferating vessels (blood or lymphatic vessels). Capillary and cavernous hemangiomas have been the terms classically used to name the most common variants of benign vascular neoplasms (i.e., infantile hemangiomas), but they are not the most appropriate denominations for these lesions. First, these names are not contrasting terms. Furthermore, most of the so-called "cavernous" hemangiomas are not hemangiomas (neoplasms) at all, but venous malformations. The most important conceptual issue is that, at any point in time, a particular hemangioma has its own histopathologic pattern throughout the depth of the lesion. For these reasons, we classified hemangiomas into superficial and deep categories. Some of the lesions reviewed have been recently described in the literature, and they may histopathologically mimic lesions of Kaposi's sarcoma; these include targetoid hemosiderotic hemangioma, microvenular hemangioma, tufted hemangioma, glomeruloid hemangioma, kaposiform hemangioendothelioma, spindle-cell hemangioendothelioma, and benign lymphangioendothelioma. In each of these lesions, we update and emphasize those clinical and histopathologic features that are helpful for differential diagnosis with lesions of authentic Kaposi's sarcoma in any of its three stages of development (patch, plaque, or nodule). (J Am Acad Dermatol 1997;37:887-920.) Learning objective: At the conclusion of this learning activity, participants should be able to describe the clinicopathologic characteristics of the cutaneous vascular hyperplasias and benign neoplasms and their classification according to their cell lineage of differentiation. The differential diagnosis between some of the more recently described vascular neoplasms and cutaneous lesions of Kaposi's sarcoma should be comprehensible to the participants in terms of the histopathologic features.

Cutaneous vascular proliferations. Part II. Hyperplasias and benign neoplasms

This second part of our review about vascular proliferations summarizes the clinicopathologic features of the cutaneous vascular hyperplasias and benign neoplasms. Hyperplasias comprise a heterogeneous group of vascular proliferations that eventually show a tendency to regression. Angiolymphoid hyperplasia with eosinophilia is included within the group of hyperplasias because of its historical denomination and its reactive nature, probably as a consequence of an arteriovenous shunt, although usually the lesions do not regress. Pyogenic granuloma, bacillary angiomatosis, intravascular papillary endothelial hyperplasia, and pseudo–Kaposi's sarcoma qualify as vascular hyperplasias because they regress when the stimulus that initiated them is removed. Benign neoplasms form a large group of hemangiomas with distinctive clinicopathologic characteristics, although some of them are of recent description and may produce diagnostic difficulties. We classified cutaneous benign vascular neoplasms according to their cell lineage of differentiation, for example, endothelial, glomus cell, and pericytic differentiation. Subsequent categories are established according to the size of the involved vessels (capillaries, venules and arterioles, or veins and arteries) or the nature of the proliferating vessels (blood or lymphatic vessels). Capillary and cavernous hemangiomas have been the terms classically used to name the most common variants of benign vascular neoplasms (i.e., infantile hemangiomas), but they are not the most appropriate denominations for these lesions. First, these names are not contrasting terms. Furthermore, most of the so-called "cavernous" hemangiomas are not hemangiomas (neoplasms) at all, but venous malformations. The most important conceptual issue is that, at any point in time, a particular hemangioma has its own histopathologic pattern throughout the depth of the lesion. For these reasons, we classified hemangiomas into superficial and deep categories. Some of the lesions reviewed have been recently described in the literature, and they may histopathologically mimic lesions of Kaposi's sarcoma; these include targetoid hemosiderotic hemangioma, microvenular hemangioma, tufted hemangioma, glomeruloid hemangioma, kaposiform hemangioendothelioma, spindle-cell hemangioendothelioma, and benign lymphangioendothelioma. In each of these lesions, we update and emphasize those clinical and histopathologic features that are helpful for differential diagnosis with lesions of authentic Kaposi's sarcoma in any of its three stages of development (patch, plaque, or nodule). (J Am Acad Dermatol 1997;37:887-920.) Learning objective: At the conclusion of this learning activity, participants should be able to describe the clinicopathologic characteristics of the cutaneous vascular hyperplasias and benign neoplasms and their classification according to their cell lineage of differentiation. The differential diagnosis between some of the more recently described vascular neoplasms and cutaneous lesions of Kaposi's sarcoma should be comprehensible to the participants in terms of the histopathologic features.

Glomeruloid hemangioma occuring in Castleman's disease

Glomeruloid hemangioma occuring in Castleman's disease

Glomeruloid Hemangioma

Glomeruloid hemangioma is a histologically distinctive cutaneous hemangioma that recently has been associated with POEMS syndrome. We report a further case in which POEMS syndrome was signaled by multiple eruptive angiomas of the glomeruloid type. Histopathology showed multiple dilated vascular spaces containing a conglomerate of capillaries resulting in a structure resembling renal glomeruli. Eosinophilic globules, probably representing immunoglobulins, were found within the cytoplasm of some endothelial and stromal cells. The finding of focal histologic features of tufted angioma (angioblastoma) in the same lesion suggests that a spectrum of hemangiomatous lesions may be seen in POEMS syndrome. Alternatively, glomeruloid hemangioma may represent a reactive endothelial proliferation rather than a neoplastic lesion.

GLOMERULOID HEMANGIOMA

Glomeruloid hemangioma is a histologically distinctive cutaneous hemangioma that recently has been associated with POEMS syndrome. We report a further case in which POEMS syndrome was signaled by multiple eruptive angiomas of the glomeruloid type. Histopathology showed multiple dilated vascular spaces containing a conglomerate of capillaries resulting in a structure resembling renal glomeruli. Eosinophilic globules, probably representing immunoglobulins, were found within the cytoplasm of some endothelial and stromal cells. The finding of focal histologic features of tufted angioma (angioblastoma) in the same lesion suggests that a spectrum of hemangiomatous lesions may be seen in POEMS syndrome. Alternatively, glomeruloid hemangioma may represent a reactive endothelial proliferation rather than a neoplastic lesion.

Acquired Benign and “Borderline” Vascular Lesions

In recent years, the classification of vascular lesions has been expanded and modified with the addition of several newly described entities, the redefinition of others, the recognition of lesions of borderline biologic behavior, and the need to avoid misdiagnosis with early Kaposi's sarcoma. This review clarifies the nomenclature, updates information on previously known lesions, and summarizes data on several recently discovered, lesser-known entities such as glomeruloid hemangioma, microvenular hemangioma, and multinucleate cell angiohistiocytoma. Clinicopathologic features and differential diagnosis are emphasized.

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.

Glomeruloid Hemangioma A Distinctive Cutaneous Lesion of Multicentric Castlemanʼs Disease Associated with POEMS Syndrome

A histologically distinctive cutaneous hemangioma occurring in two patients with biopsy-proven multicentric Castleman's disease associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome are reported. The lesions were multiple, and appeared as red to purple papules over the trunk and proximal limbs. Microscopically, ecstatic dermal vascular spaces were seen filled with aggregates of capillaries, resulting in structures reminiscent of renal glomeruli. Interspersed between the blood-filled capillary loops were plump "stromal" cells possessing clear vacuoles and periodic acid-schiff-positive eosinophilic globules. These cells had the immunohistochemical profile of endothelial cells (positivity for factor VIII-related antigen, and negativity for leukocyte common antigen, macrophage marker, and muscle-specific actin), and probably represented immature elements that had accumulated immunoglobulins and other proteinaceous material from the circulation. Because vascular lesions may appear before the full-blown POEMS syndrome develops, we suggest careful evaluation and follow-up of all patients presenting with glomeruloid hemangioma or cherry-type capillary hemangioma with focal glomeruloid features for potential development of this syndrome.