Similarities and differences of human and experimental mouse lymphangiomas.

Lymphangioma is a disfiguring malformation of early childhood. A mouse lymphangioma model has been established by injecting Freund's incomplete adjuvant (FIA) intraperitoneally, but has not been compared with the human disease. We show that, in accordance with studies from the 1960s, the mouse model represents an oil-granuloma, made up of CD45-positive leukocytes and invaded by blood and lymph vessels. Several markers of lymphatic endothelial cells are expressed in both mouse and human, like CD31, Prox1, podoplanin, and Lyve-1. However, the human disease affects all parts of the lymphovascular tree. We observed convolutes of lymphatic capillaries, irregularly formed collectors with signs of disintegration, and large lymph cysts. We observed VEGFR-2 and -3 expression in both blood vessels and lymphatics of the patients, whereas in mouse VEGFR-2 was confined to activated blood vessels. The experimental mouse FIA model represents a vascularized oil-granuloma rather than a lymphangioma and reflects the complexity of human lymphangioma only partially.

Sclerosing therapy as first line treatment for low flow vascular lesions of the orbit.

PURPOSE: To evaluate the outcome of sodium morrhuate 5% injections in patients with low flow vascular lesions, which consist of orbital lymphangiomas, and in one patient with intraosseous cavernous hemangioma. DESIGN: Prospective, interventional consecutive case series. METHODS: Intralesional sodium morrhuate 5% was injected under direct visualization or under radiographic guidance to six patients with orbital lymphangiomas and one patient with intraosseous cavernous hemangioma. Comprehensive eye examination and follow-up imaging studies were performed. main outcome measures: Lesion size was evaluated by orbital imaging and clinical examination, visual acuity, exophthalmos, and posttreatment complications. RESULTS: Seven patients (four female, three male; average age, 33 years) were included. Six patients were diagnosed with orbital lymphangioma, and one patient was diagnosed with intraosseous cavernous hemangioma. Patients received an average of 2.6+/-2 intralesional injections of sodium morrhuate, with a range of one to six injections and a mean volume of 0.9+/-0.8 ml (range, 0.2 to 2.1 ml). Lesions showed a decrease in size an average of 50% (33%) and ranged from minimal (10%) to near total resolution (85%). Visual acuity and intraocular pressure remained unchanged; exophthalmos decreased an average of 1.5+/-1.8 mm. Complications included one case of orbital hemorrhage that resolved spontaneously and transient keratopathy in all patients with anterior orbital lesions. CONCLUSION: Intralesional sclerosing therapy with sodium morrhuate 5% is effective in tumor debulking in patients with orbital lymphangioma and is not associated with vision-threatening complications. It may be a better alternative to surgery for low flow orbital tumors, which includes lymphangioma.

Immunohistochemical co-localization of lymphatics and blood vessels in oral squamous cell carcinomas.

BACKGROUND: Differentiating lymphatic vessels from blood vessels is difficult, partly due to the lack of a specific method for identifying lymphatics. A new lymphatic vessel-reactive antibody, D2-40 has recently become commercially available. We examined the selectivity of D2-40 for lymphatics in oral neoplastic lesions for discrimination from blood vessels. METHODS: Formalin-fixed, paraffin-embedded sections of oral lymphangiomas (n = 3), oral hemangiomas (n = 7), and oral squamous cell carcinomas (OSCC, n = 46) were double immunostained with D2-40 and anti-CD34 monoclonal antibodies (MoAb) using ENVISION-polymer technique with 5-bromo-4-chloro-3-indoxyl-phosphate (BCIP)/nitroblue tetrazolium chloride (NBT) and 3,3'-diaminobenzidine (DAB) as color reagents, respectively. RESULTS: In the oral lymphangiomas and hemangiomas D2-40 was detected in all lymphatics, while all blood vessels were positive for CD34. In OSCC, number of vessels for lymphatics (P < 0.01) and for blood vessels in the perineoplastic areas were significantly greater than those in intratumoral areas. CONCLUSIONS: These results indicate that lymphatic proliferation might be much more extensive in the peritumoral area than intratumoral.

CT features of mediastinal lymphangiohemangioma associated with superior vena cava ectasia. A case report.

We present a case of mediastinal mesenchymal tumor in a 53-year-old woman with a 1-month history of chest tightness. A histological diagnosis of lymphangiohemangioma was obtained by excisional biopsy. CT findings are reported with emphasis on its association with intratumoral superior vena cava ectasia.

Immunoelectron microscopic characterization of human dermal lymphatic microvascular endothelial cells. Differential expression of CD31, CD34, and type IV collagen with lymphatic endothelial cells vs blood capillary endothelial cells in normal human skin, lymphangioma, and hemangioma in situ.

We performed a comparative investigation of the immunomorphological characteristics of lymphatic and blood microvascular endothelial cells in normal human skin, cutaneous lymphangiomas, and hemangiomas, employing a pre-embedding immunogold electron microscopic technique. We stained for cell membrane proteins that are commonly used for light microscopic characterization of blood endothelial cells. With blood microvascular endothelial cells, we observed uniform labeling of the luminal cell membranes with monoclonal antibodies (MAbs) JC70 (CD31), EN-4 (CD31), BMA120, PAL-E, and QBEND-10 (CD34), and strong staining of the vascular basal lamina for Type IV collagen under normal and pathological conditions. In contrast, lymphatic microvascular endothelial cells in normal human skin and in lymphangiomas displayed, in addition to a luminal labeling, pronounced expression of CD31 and CD34 along the abluminal cell membranes. Moreover, CD31 was preferentially detected within intercellular junctions. The expression of CD34 was mostly confined to abluminal endothelial microprocesses and was upregulated in lymphangiomas and hemangiomas. Type IV collagen partially formed the luminal lining of initial lymphatics and occasionally formed bridges over interendothelial gaps. Our findings suggest a function of transmigration protein CD31 in recruitment of dendritic cells into the lymphatic vasculature. CD34 labeling may indicate early endothelial cell sprouting. The distribution of Type IV collagen also supports its role as a signal for migration and tube formation for lymphatic endothelial cells.

A case of acquired lymphangioma of the vulva.

A 46-year-old woman developed multiple papules on the right labium majus. A histological examination revealed acanthotic epidermis and dilated vessels in the papillary dermis. The clinical and histological features were compatible with those of lymphangiectasis or acquired lymphangioma of the vulva, which occurs after surgery or irradiation for cervical cancer. This patient, however, had no such past history. Acquired lymphangioma of the vulva arising without obvious causes seems to be unusual.

Giant cystic hygroma associated with venous aneurysm.

Complete excision of giant cystic hygroma requires meticulous dissection of the multilocular lymphatic cysts from the adjacent vascular and neural structures. The association of venous aneurysms with cystic hygroma is extremely rare. We report two infants with cystic hygroma in whom preoperative diagnosis of venous aneurysm was helpful in planning complete excision of the lymphatic lesions.

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma. Analysis of 39 cases and delineation of two new entities.

Primary vascular tumors of lymph nodes other than Kaposi's sarcoma are very rare, as attested to by only a handful of case reports in the literature. Based on an analysis of 39 such cases, we could distinguish five major groups. Hemangiomas of capillary/cavernous, lobular capillary, and cellular types were composed of compact aggregates of blood-filled vessels, variable in size, that replaced the nodal architecture partly or almost completely; some appeared to have originated in the hilum or medulla. These hemangiomas either represented incidental findings in lymph nodes or were seen with solitary lymph node enlargement; the evolution was benign with no recurrence. A distinctive benign lesion occurring exclusively in inguinal lymph nodes, which we propose designating "angiomyomatous hamartoma," showed replacement of the nodal parenchyma by smooth muscle cells and fibrous tissue, in continuity with exuberant proliferation of muscular vessels in the hilum. Epithelioid vascular tumors, characterized by plump endothelial cells with dense eosinophilic cytoplasm and numerous vacuoles, exhibited a range of differentiation, from hemangioma with well-formed vascular channels (with or without tissue eosinophilia) to hemangioendotheliomas composed predominantly of cords and sheets of tumor cells lying in a hyaline-myxoid matrix. Epithelioid hemangioendothelioma was particularly likely to be mistaken for metastatic carcinoma, and local recurrence could occur. A variant, the spindle and epithelioid hemangioendothelioma, was characterized by the presence of an additional component of spindle cells. Another tumor we found, polymorphous hemangioendothelioma, is a previously uncharacterized borderline malignant vascular tumor exhibiting solid, primitive vascular and angiomatous patterns and relatively bland cytologic features. Lymphangiomas of lymph nodes usually showed simultaneous multifocal and extra-nodal involvement and were characterized by cystic endothelium-lined spaces filled predominantly with lymph fluid. It is important to recognize these primary vascular tumors of lymph nodes to avoid mistaking them for a variety of benign vasoproliferative lesions, Kaposi's sarcoma, angiosarcoma, and metastatic cancer.

Primary lymphangioma of the thyroid gland.

A 21-year-old woman presented with a solitary, hypofunctioning right thyroid nodule. Findings of fine-needle aspiration biopsy were consistent with a thyroid cyst, but a subsequent biopsy demonstrated changes suggestive of a papillary neoplasm. Surgery was performed and a well-circumscribed 12-mm nodule was identified within the thyroid parenchyma. Microscopic examination confirmed the presence of a thyroid lymphangioma. To our knowledge, this is the first case of a primary thyroid lymphangioma reported in the English literature.

MR gated subtraction angiography: evaluation of lower extremities.

We report the first clinical experience with a new method for projective imaging of blood vessels (angiography) using magnetic resonance. Vascular contrast is produced noninvasively by the phase response of moving protons. Diastolic and systolic gated images produce, respectively, flow signal and flow void; the difference image is a map of the pulsatile flow: an arteriogram. Preliminary studies are presented of the lower extremities of one healthy volunteer and four patients (one each with occlusive disease, soft-tissue tumor, arteriovenous malformation, and venous femoral-popliteal graft). Patient data are compared with accompanying conventional arteriograms, and the new method is discussed.

Vascular tumors of the breast. III. Angiomatosis.

This report describes three female patients who were treated for diffuse vascular lesions of the breast. The lesion was congenital in one patient. Two were adults (40 and 59 years old). Each presented with a mass within the breast. Microscopically, the tumors were composed of vascular channels growing diffusely in breast parenchyma. The vessels were lined by flat, inconspicuous endothelium with sparse supporting mural tissue virtually devoid of smooth muscle. The lesions consisted of hemangiomatous (erythrocyte-containing) and lymphangiomatous (seemingly empty) channels with lymphoid aggregates. This combination of a mixed pattern of vessels and diffuse growth is similar to that seen in lesions that have been called angiomatosis in other anatomic sites. Because the microscopic distinction between very orderly angiosarcomas and angiomatosis may be difficult, complete excision of the tumor is necessary. For bulky lesions, this may require mastectomy. In one patient congenital angiomatosis has persisted and recurred twice in the mammary region over 39 years. The other two patients remain well 3 years after local excision and 2 years and 4 months after mastectomy, respectively. Angiomatosis of the breast has not exhibited clinically malignant behavior since no metastases have been observed.

Hemangiomas and vascular malformations.

The classification, natural history, and treatment of hemangiomas, arteriovenous fistulas, lymphangiomas, and port-wine stains are in many respects as puzzling now as they were fifty years ago. A review of the literature assesses the progress made and addresses the questions yet to be answered.

Angioimmunoblastic lymphadenopathy.

Forteen cases of angioimmunoblastic lymphadenopathy were reviewed retrospectively, and histologic material was examined by various staining and histochemical techniques. The predominant morphologic features were effacement of nodal architecture, microvascular proliferation, and a polymorphous cellular infiltrate of immunoblasts, plasma cells, lymphocytes, and eosinophils. Immunofluorescence studies suggested the presence of cells elaborating a variety of immune globulins with an unpredictable pattern. The clinical course and treatment varied, perhaps partly reflecting the initial spectrum of diagnoses made in these cases before angioimmunoblastic lymphadenopathy was defined. Because systemic infection appears to be a major factor in the deaths of these patients whose immune competence is uncertain, the usual chemotherapy for malignant lymphomas may be too vigorous for this condition. At present, the cause of the condition is unknown.

[Cystic lymphangiomyomas of the uterus]. / Le lymphangiomyome kystique de l'uérus

About 7 cases the author describes a uterine tumor made up of serous cysts with endotheliform covering and small areas of immature leio-myocytes.