Acquired Progressive Lymphangioma Research Articles

Lymphatic Malformation of Inguinal region in an adult: A Case Report.

Lymphatic malformation are most commonly located in regions of confluence of major lymphatic channels, including the neck ( 75%), axilla ( 20%), mediastinum, retroperitoneum, pelvis and groin. We are reporting here a rare case in which a 35year old female presented with swelling over right groin and thigh. A provisional diagnosis was made on radiological investigations. Wide Local Excision with Tensor Fascia Lata Flap Reconstruction with Split Skin Graft was done and the diagnosis was confirmed on histopathology and immunohistochemistry. Keywords: Lymphatic malformation, Acquired Progressive Lymphangioma, Benign lymphangioendothelioma

BENIGN LYMPHANGIOENDOTHELIOMA

The authors describe modern data relating to the definition, clinical, pathological and immunohistochemical features of a rare vascular tumor — benign lymphangioendothelioma. The problems of differential diagnosis with Kaposi’s sarcoma and angiosarcoma are discussed. This is the first description of this tumorin domestic literature.

Men in red

Cutaneous vascular tumours are plentiful and represent one of the most common groups of soft tissue proliferations presenting in the skin. In the majority of cases the histological diagnosis of a cutaneous vascular is straightforward and most lesions are benign. Pathologists face three very challenging tasks when confronted with a skin biopsy containing a vascular proliferation. The first pertains to benign vascular lesions that are often confused with malignancy. This group of lesions includes lesions such as symplastic haemangioma, cutaneous angiomatous nodule, benign lymphangioendothelioma and the atypical vascular lesion arising after radiotherapy amongst others. The second relates to the recognition and accurate diagnosis of low-grade malignant vascular tumours. These tumours are rare and difficult to diagnose and some have been identified recently including pseudomyogenic haemangioendothelioma. The third refers to the accurate diagnosis of poorly differentiated malignant vascular tumours. This task is usually greatly aided by the judicious use of immunohistochemistry including fairly specific vascular markers such as CD31 and the more recently described FLI-1 and particularly ERG. ERG gene fusions occur in subsets of prostatic carcinoma, acute myeloid leukaemia and Ewing sarcoma. ERG is a highly specific marker of endothelial cell differentiation.

Benign lymphangioendothelioma: a clinical, histopathologic and immunohistochemical analysis of four cases

Benign lymphangioendothelioma represents a rare lymphatic vascular proliferation characterized by proliferation of irregular and thin-walled vessels dissecting amongst dermal collagen. Immunohistochemical analysis has been lacking in most previously reported cases. Herein, we report the clinical and histopathologic characteristics of four cases of benign lymphangioendothelioma. Immunohistochemical study was completed for all lesions. All lesions presented as large, red to brown patches or plaques. Three lesions were located on the thigh and one lesion was located on the neck. Histopathologically, all lesions showed proliferation of anastomotic or retiform thin-walled vessels with a single layer of endothelial cells that dissect the dermis. D2-40 and Prox1 immunostains were positive and Wilms tumor 1 (WT-1) immunostain was negative in all cases. Benign lymphangioendothelioma represents a lymphatic vascular proliferation. A lack of expression of WT-1 suggests it represents a lymphatic vascular malformation.

Giant benign lymphangioendothelioma

Benign lymphangioendothelioma is a rare locally infiltrative vascular neoplasm, presenting as a slow-growing, asymptomatic, reddish-violaceous plaque. Histopathologically, it is characterized by irregular and thin-walled vascular spaces, lined by a single and discontinuous layer of flat endothelial cells, dissecting dermal collagen bundles. We present the case of a 75-year-old man with a giant benign lymphangioendothelioma, to our knowledge, the largest example described in the literature. The immunohistochemical expression of Wilms tumor 1 gene is useful in vascular lesions to differentiate malformations from proliferative endothelial lesions. In our case, the positivity for WT1 supports the neoplastic nature of this lesion.

Acquired progressive lymphangioma in an HIV‐positive patient

Acquired progressive lymphangioma (APL) is a rare condition characterized by benign proliferation of thin-walled vessels lined by flattened endothelial cells.(1-4) Although benign, the acquired nature of this tumor may lead to misdiagnosis as a malignant vascular tumor. This is especially true if the patient has risk factors, such as immunodeficiency. In this article, the authors present a case of APL in an HIV-positive man.

Post‐Irradiation Vascular Proliferations: Potential Mimic of Kaposi’s Sarcoma

A variety of benign and malignant cutaneous vascular lesions occur in irradiated skin. We report two cases of vascular lesions that developed on the breast within a year after radiation therapy for breast carcinoma. Both cases mimicked Kaposi’s sarcoma (KS) histologically. We compare these with one case of post‐irradiation angiosarcoma and 3 cases of acquired progressive lymphangioma (APL) unrelated to radiation therapy. The post‐irradiation vascular lesions showed slit‐like vessels dissecting through superficial dermal collagen, leading to an initial diagnosis of KS by one contributing pathologist. Immunohistochemically, the vessels in both cases expressed CD31 and CD34, stained only partially for actin, and had a low Ki67 proliferation index. Neither case expressed HHV8. The post‐irradiation angiosarcoma occurred three years after radiation, and also exhibited a focal slit‐like growth pattern resembling KS. However, there was marked cytologic atypia, a high proliferation rate, and negative staining for HHV8. The APL were characterized by more ectactic vascular spaces and deeper involvement of the dermis than was present in the post‐irradiation lesions. These cases also failed to stain for HHV8. Recognition of the phenomenon of post‐irradiation vascular proliferations is important, especially because such lesions may resemble KS, yet invariably follow a benign clinical course.

Benign vascular proliferations in irradiated skin.

Several types of cutaneous vascular proliferations have been described in areas of irradiated skin, including both benign lesions, such as benign lymphangiomatous papules, atypical vascular lesions, or benign lymphangioendothelioma, and malignant neoplasms such as high-grade angiosarcomas. This report describes the clinicopathologic features of 15 cases of different types of benign cutaneous vascular proliferations arisen within irradiated skin. All patients were female ranging in age from 33 to 72 years, and they had received postoperative external radiotherapy for treatment of breast carcinoma (14 cases) or ovarian carcinoma (one case). In those cases in which the latency interval period between radiotherapy and the development of the vascular lesion was known from the clinical records, the latency interval period elapsed between radiotherapy and diagnosis of the vascular lesion ranged from 3 to 20 years. The most common clinical presentation of the cutaneous lesions consisted of papules, small vesicles, or erythematous plaques on the irradiated field. Histopathologically, most lesions consisted of irregular dilated vascular spaces, with a branching and anastomosing pattern, thin walls, and lymphatic appearance involving the superficial dermis. A discontinuous single layer of endothelial cells with flattened nuclei lined these vascular channels, and numerous small stromal papillary formations also lined by endothelial cells projected into the lumina of the dilated lymphatic vessels. These cases were classified as benign lymphangiomatous papules or plaques. Two cases showed different histopathologic findings because they consisted of poorly circumscribed and focally infiltrating irregular jagged vascular spaces involving the entire dermis and lined by inconspicuous endothelial cells. In some areas these irregular slit-like vascular spaces dissected collagen bundles of the dermis. These cases were classified as atypical vascular proliferations mimicking benign lymphangioendothelioma or patch-stage Kaposi's sarcoma. All cases showed similar immunohistochemical findings and the endothelial cells lining the vascular spaces expressed immunoreactivity for CD31, but they stained only focally positive for CD34 or were negative for this marker. Immunohistochemical investigations for alpha-smooth muscle actin failed to demonstrate a complete peripheral ring of actin-positive pericytes in most of the neoformed vascular structures. This immunohistochemical profile also supported the lymphatic nature of these vascular proliferations developed in irradiated skin. Although some of these lesions may mimic histopathologically patch-stage Kaposi's sarcoma or well-differentiated angiosarcoma, the follow-up of the patients of this series demonstrated that the vascular proliferations arisen in irradiated skin invariably showed a benign biologic behavior.

Tumors of the lymphatic vessel of the skin and soft tissue

Tumours of lymphatic vessels comprise only a small group in the heterogeneous spectrum of vascular neoplasms of skin and soft tissues. However, this discrepancy between haemangiomas/angiosarcomas and lymphangiomas/lymphangiosarcomas probably represents the present inability to reliably differentiate between lymphatic and capillary vascular endothelium. Histologically, neoplastic lymphatic vessels tend to be lined by endothelial cells with plumper and more prominent, matchstick-like nuclei (in contrast to vascular spaces in haemangiomas that are lined by flat or epithelioid endothelial cells), often show variations in the thickness of the vessel walls and are not completely surrounded by actin-positive (myo)pericytes. Endothelial cells in lymphatic neoplasms tend to be negative for CD34 or stain only focally positive for this marker and an expression of lymphatic markers as vascular endothelial growth factor-C receptor (VEGRF-3), podoplanin and M2A oncofetal antigen has been reported most recently. In addition to "traditional" lymphatic neoplasms including lymphangioma circumscriptum, cavernous lymphangioma/cystic hygroma, benign lymphangioendothelioma, lymphangiomatosis and the rare lymphangiosarcoma, histological and immunohistochemical features of a group of vascular neoplasms with a hobnail cytomorphology (hobnail haemangioma, retiform haemangioendothelioma, papillary intralymphatic angioendothelioma, benign lymphangiomatous papule following radiotherapy) suggest that these lesions also belong to the spectrum of tumours of lymphatic vessels.

Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.

The clinicopathologic features of 12 cases of benign lymphangioendothelioma (acquired progressive lymphangioma) are reported. There were five male and seven female patients. Age at diagnosis ranged from 17 to 90 years (median age, 54 yrs). Development of a single macular/papular hemangiomatous or pigmented lesion was the main presenting symptom. Symptom duration before diagnosis ranged from 2 months to 20 years (median, 5.5 yrs). Tumor size ranged from 0.3 cm to 10 cm (median. 1.5 cm). Location included skin of the head and neck (n = 5), back (n = 1), breast (n = 1), shoulder (n = 1), forearm (n = 1), plantar aspect of the foot (n = 2), and oral mucosa (n = 1). No patient had any other concomitant vascular anomaly (for example, lymphangiomatosis) or was suspected to have acquired immunodeficiency syndrome. Treatment consisted of excisional biopsy in nine patients, incisional biopsy in two, and wide excision in one. Follow-up information on nine patients (range, 4-40 mos; median, 12 mos) showed two local recurrences in one patient. Microscopically, the lesions consisted of anastomosing, often widely dilated vascular structures developing in the superficial dermis. As the lesion grew within deeper dermis, the vascular spaces collapsed and dissected the dermal collagen in an angiosarcoma-like pattern. The lining endothelium was flat and monolayered, with little or no cytologic atypia and no evident mitoses. Some vascular structures contained stromal papillary projections resembling papillary endothelial hyperplasia, and intravascular red blood cells were present occasionally. Immunohistochemistry performed in eight specimens showed variable endothelial cell reactivity for CD31 (7 of 8), CD34 (7 of 7), and factor VIII-related antigen (4 of 6). A smooth muscle cell layer was observed focally around the vascular spaces in six lesions. Benign lymphangioendothelioma (acquired progressive lymphangioma) is an uncommon benign lesion that, in view of major differences in treatment and prognosis, should be distinguished from well-differentiated angiosarcoma and Kaposi's sarcoma, especially the patch stage and lymphangioma-like variants of the latter.

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.

Lymphangioma-like variant of Kaposi's sarcoma: clinicopathologic study of seven cases with review of the literature.

The clinical and pathological features of seven cases of lymphangioma-like Kaposi's sarcoma (KS) are reported. As with the other subtypes of KS, the lymphangioma-like variant occurs more often in men aged 59-80 years. Clinically, the lesion appears intermingled with the classical forms of KS, but a "bulla-like" appearance recognized in seven of 13 cases has been considered as a clinical hallmark of this variant. Although occasional cases have shown aggressive behavior, the most frequent clinical course is slowly progressive with localized or diffuse involvement of lower limbs. The histological pattern, characterized by permeation of dermal collagen by labyrinthine vascular channels lined by a flattened endothelium, must be differentiated from spindle cell hemangioendothelioma, low-grade angiosarcoma, targetoid hemosiderotic hemangioma, and benign lymphangioendothelioma.

Acquired progressive lymphangioma occurring following femoral arteriography

Our case report concerns an unusual case of acquired progressive lymphangioma (APL) which developed in a 52-year-old Japanese man following femoral artery catheterization for angiography. The histological examination revealed many irregularly shaped and dilated lymphatic channels that were lined by a single layer of endothelial cells. Immunohistochemical staining for von Willebrand factor was negative in the endothelial cells of the dilated channels. Electron microscopic study demonstrated no Weibel-Palade bodies in the cytoplasm of the endothelial cells. The endothelial cells had no distinct basement membrane.

Beningn lymphangioendothelioma: report of 2 cases

Benign lymphangioendothelioma is an acquired vascular proliferation clinically appearing as well-demarcated, pink to red-brown macules or plaques. Histologically, numerous endothelial-lined channels are found infiltrating throughout the dermis. Recognition and differentiation from malignant vascular tumors is essential. We report 2 cases of benign lymphangioendothelioma. One of these lesions is unique in its presentation as a subcutaneous nodule. The other is also unusual, as it resolved spontaneously.

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.

Benign lymphangioendothelioma

We have studied eight cases of an acquired lymphatic endothelial lesion for which we propose the name "benign lymphangioendothelioma." The lesions developed as solitary, slowly extending, erythematous macules and plaques, usually occurring on the extremities or the shoulders in adolescents or adults. The characteristic histopathologic feature is permeation of the dermal collagen by flattened, endothelium-lined channels and spaces. Hemorrhage, iron deposition, and inflammation were not part of the lesion. Ulex europaeus agglutinin I labeled the lesional endothelial cells consistently, but factor VIII-related antigen labeling was negative. This histologic pattern and the special studies suggested a lymphatic lesion. Surgical excision, performed in six patients, was not followed by recurrence.