Developmental Disorders of the Lymphatics

An information blog for disorders of the lymphatics. For all articles, please click on "Archives" - Due to spammers, I will no longer allow comments, sorry.

Monday, January 28, 2013

Nonspecific granulomatous inflammation in Crohn's disease.


Nonspecific granulomatous inflammation in Crohn's disease.


2012

[Article in Russian]
[No authors listed]

Abstract


A comparative morphological study of intestinal wall tissues in such chronic colonic diseases, such as Crohn's disease, ulcerative colitis, and catarrhal rectal fistulas, allows the formation of giant cells of foreign bodies and their granulomas and sarcoid-type ones to be nonspecific. Their spread through and outside the colon is due to the migration of foreign bodies along the lymphatic vessels. Foreign inclusions of different shapes and structures in the cytoplasm of giant cells suggest that the colon contains the multiple particles of varying antigenic nature, which induce a unified morphological response medicated by innate and adaptive immunity cells. Consequently, the universally accepted substantiation of the diagnosis of Crohn's disease by the presence of granulomas is unconvincing.

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Saturday, January 19, 2013

Hemangioendothelioma.


Hemangioendothelioma.


Feb 2013

Source

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain. Electronic address: lrequena@fjd.es.

Abstract


Hemangioendothelioma is the term used to name those vascular neoplasms that show a borderline biological behavior, intermediate between entirely benign hemangiomas and highly malignant angiosarcomas. Although originally spindle cell hemangioendothelioma was proposed as a specific clinicopathologic variant of hemangioendothelioma, currently, it is considered as an entirely benign lesion, and thus, the name spindle cell hemangioma seems to be the most accurate for this lesion. Authentic hemangioendotheliomas involving the skin and soft tissues include papillary intralymphatic angioendothelioma (also known as Dabska tumor), retiform hemangioendothelioma, kaposiform hemangioendothelioma, epithelioid hemangioendothelioma, pseudomyogenic hemangioendothelioma (also known as epithelioid sarcoma-like hemangioendothelioma), and composite hemangioendothelioma. Each of these neoplasms exhibit characteristic histopathologic features. The most characteristic finding of papillary intralymphatic hemangioendothelioma consists of papillary tufts, with a central hyaline core lined by hobnail-like endothelial cells protruding into the lumina. Retiform hemangioendothelioma is an infiltrative neoplasm composed of elongated arborizing vessels, arranged in an anastomosing pattern that resembles that of the rete testis, and lined by a single layer of hobnail-like endothelial cells that protrude within the narrow lumina. Kaposiform hemangioendothelioma is composed of several solid poorly circumscribed nodules, and each nodule is composed of a mixture of small capillaries and solid lobules of endothelial cells arranged in a glomeruloid pattern. A frequent finding consists of the presence of areas of lymphangiomatosis adjacent to the solid nodules. Epithelioid hemangioendothelioma is composed of cords, strands, and solid aggregates of round, oval, and polygonal cells, with abundant pale eosinophilic cytoplasm, vesicular nuclei, and inconspicuous nucleoli, embedded in a fibromyxoid or sclerotic stroma. Many neoplastic cells exhibit prominent cytoplasmic vacuolization as an expression of primitive vascular differentiation. Pseudomyogenic hemangioendothelioma is a poorly circumscribed, fascicular lesion with infiltrative borders composed of round or oval neoplastic cells, with vesicular nuclei and inconspicuous nucleoli, and ample homogeneous eosinophilic cytoplasm, giving them a rhabdomyoblastic appearance. Finally, composite hemangioendothelioma is the term used to name locally aggressive vascular neoplasms of low-grade malignancy showing varying combinations of benign, low-grade malignant, and high-grade malignant vascular components. From the immunohistochemical point of view, proliferating cells of all hemangioendotheliomas express a lymphatic endothelial cell immunophenotype. Most hemangioendotheliomas are low-grade vascular neoplasms, with a tendency to recur locally and a low metastatic potential, mostly to regional lymph nodes. Epithelioid hemangioendothelioma, especially large lesions and those located in deep soft tissues, seems to have a more aggressive biological behavior.

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Saturday, January 12, 2013

Bilateral renal lymphangiomatosis: imaging and histopathologic findings.


Bilateral renal lymphangiomatosis: imaging and histopathologic findings.


Jan 2013

Source

Service d'Anatomie et de Cytologie Pathologiques, Université Paris-Sud 11, APHP, Hôpital de Bicêtre, CHU de Bicêtre, 78 rue du Général Leclerc, 94275, Le Kremlin-Bicêtre, France, r.karkouche@gmail.com.

Abstract


Renal lymphangiomatosis is an extremely rare disease characterized by developmental malformation of the lymphatic system surrounding the kidneys. We present the case of a 22-year-old pregnant female discovered because of worsening. Ultrasound, computed tomography, and magnetic resonance imaging studies were performed. An 18 × 11 × 10 cm voluminous cystic subcapsular lesion compressing the left kidney and subcapsular cysts of the right kidney were found. After the delivery, marsupialization was performed and the pathological analysis confirmed the diagnosis of lymphangiomatosis. A review of the literature is proposed.

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Saturday, January 05, 2013

Relationship between angiogenesis and lymphangiogenesis in recurrent pterygium.


Relationship between angiogenesis and lymphangiogenesis in recurrent pterygium.


2012

Source

Department of Ophthalmology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, Guangdong Province, China.

Abstract


AIM:

To examine the relationship between angiogenesis and lymphangigenesis in recurrent pterygia.

METHODS:

Tissues from 34 excised recurrent pterygia (including 12 Grade 1, 10 Grade 2, and 12 Grade 3) were involved in the study and tissues from 7 nasal epibulbar conjunctivae segments were used as controls. Sections from each pterygium were immunostained with CD(31) and LYVE-1 monoclonal antibodies to evaluate lymphatic microvessel density (LMVD) and blood microvessel density (BMVD), and the relationship between LMVD and BMVD in the pterygium was examined.

RESULTS:

There was a large number of CD(31) ((+))LYVE-1((-)) blood vessels but only a few CD(31) ((+))LYVE-1((+))lymphatic vessels in grades 1 and 2 pterygium. However, lymphatic vessels were dramatically increased in grade 3 pterygium. LMVD correlated closely with BMVD in all pterygia, including grades 1, 2 and 3 peterygium patients . Although both the density of blood and lymphatic vessels increased in recurrent pterygia, lymphatic vessels developed much faster than blood vessels, especially in grade 3 pterygia.

CONCLUSION:

 There is a significant but not parallel relationship between angiogenesis and lymphangiogenesis in recurrent pterygium. The outgrowth of blood and lymphatic vessels provide evidence that immunological mechanism may play a role in the development and recurrence of pterygium.


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