Lymphangiomatosis
What is:
A syndrome of Lymphatic Dysplasia
Lymphangiomatosis (limf-an-jee-oh-mah-TOE-sis). In medical terminology lymphangiomatosis means: lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis). There is no standard definition describing this disease that has been agreed to by the medical/scientific community. (1)
Tumors (lymphangiomas) grow in the lymph system itself. These are benign in nature but they spread throughout the body invading other body tissues. They can spread to any area, but are more commonly found in the lungs (pulmonary), bones (skeletal), kidney (renal), the thoracic cavity, connective tissue and spleen.
While the tumors are not malignant, they can quickly interfere with organ function causing extreme pain, difficulty in breathing, destroy other tissue, thereby causing other serious potential complications.
Causes:
The specific cause is not yet known.
Diagnosis:
Diagnosis will be made on the basis of the symptoms and multiple lymphangiomas in the body. Radiology can be of assistance also. The two most commonly used are CT (computed tomography) scan and magnetic resonance imaging (MRI).
If needed or necessary a lymphoscintigraphy exam can be used to determine lymph flow and/or blockage.
Signs and Symptoms:
Treatment:
Treatment is usually multi-focal and depends on where the location of the tumor is and the complications they may present.
There are three basic methods of treating lymphangiomatosis.
First is the use of a medication that stops tumor growth. The medication is Interferon Alfa-2b. This drug (Intron® A) is a man-made protein. Natural interferons are produced to help the immune system fight viral infections and certain cancer growths. Interferon alfa-2b has similar actions to natural interferons and is used to treat AIDS-related Kaposi's sarcoma, certain types of hepatitis, and leukemia or other cancers. Generic interferon alfa-2b injections are not yet available.
Chemotherapy may be used and radiation targeting specific tumor as well.
Other targeted treatment modalities are:
| Cardiothoracic | Pleurodesis, ligation of thoracic duct, pleurperitoneal shunt, radiation therapy, pleurectomy, surgical resection, thalidomide, Interferon Alpha2b, TPN nutrition, thoracocentesis, medium chain triglyceride and high protein diet, chemotherapy, sclerotherapy, transplantion | |
| Gastrointestinal | Interferon Alpha2b, sclerotherapy, resection, percutaneous drainage | |
| Kidneys | Percutaneous drainage | |
| Liver | Resection, transplantation | |
| Skeleton | Interferon Alpha2b, bisphosphonates (i.e. pamidronate), surgical resection, radiation therapy, sclerotherapy, percutaneous bone cement, bone grafts, prosthesis, surgical stabilization | |
| Spleen | Interferon Alpha2b, splenectomy (1) |
The thoaracic duct is the central location in the body where the lymph system literally dumps excess fluid back into the cardiovascular system, to befinally removed throughthe kidneys. Should this become blocked, for example by a tumor, the patient may experience lung fluid somewhat like a malignant effusion. This means the fluid is extreme and rapidly reoccurs. If a thoracentesis is not sufficient, a procedure called a pleurodesis. In pleurodesis, an irritant (such as Bleomycin, Tetracyclines, or talc powder) is instilled inside the space between the pleura (the two layers of tissue lining the lungs) in order to create inflammation which tacks the two pleura together. This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid.
The lymphedema treatment program would include: Manual lymphatic drainage; compression wraps or compression bandages (using short stretch bandages), compression garments, compression sleeves.
=================================
A very special page dedication
In memory of and with respect to: Marcus Petersen
September 10, 1995 - September 7, 2004
He fought a courageous battle for years with lymphangiomatosis and Gorham's Disease. Please visit the family website at:
http://www.gorhams.dk/index.html
You will be remembered by many, Marcus.
=========================
Lymphangiomatosis
Definition: (1)
Multiple intraosseous lymphangiomas occurring as a diffuse form as part of a spectrum of either cystic haemangiomatosis or massive osteolysis (Gorhams disease). For a general description, see lymphangiomatosis.In this disorder, the lymphangiomas may involve two or more widely separated bones, several bones in the same region of the body or many bones in a diffuse pattern; in some cases lymphangiomas also occur in the soft tissues or viscera. Most often the long tubular bones and flat or irregular bones are affected. Lymphangiography is valuable in making a specific diagnosis through uptake of contrast agent in intraosseous tumours
---------------------------------
Skeletal Lymphangiomatosis
This rare congenital disorder can affect the bones or the visceral organs. Skeletal involvement leads to pathological fracture, joint deformity and may cause pain. The bones are filled with a network of interconnected lymph channels containing fluid.
The pattern of skeletal involvement varies, with multiple contiguous bones heavily involved and others completely normal. Other patients present with multiple cystic lesions in non-contiguous bones combined with lymph fluid collections in the chest and abdominal cavities (chylothorax and chyloperitoneum). Generalized lymphedema of the extremity may occur.
There have been isolated cases of association with other mesenchymal dysplasias, such as Mafucci syndrome and osteochondromas. Gorham's disease (idiopathic massive osteolysis, dissapearing bone disease) may be an aggressive form of lymphangiomatosis. A familial cluster of cases with an autosomal dominant pattern of inheritance has been reported.
Individuals without visceral involvement have a better prognosis. The skeletal lesions may involute or inprove after skeletal maturity. The author is not aware of any report of sarcomatous degeneration in these lesions.
Treatment is centered on minimizing skeletal complications and the associated deformity. Bisphosphonate medications may limit the bone loss and the risk of pathologic fracture associated with expansion of the lesions. The optimum dosage and pharmacokinetics of the oral bisphosphonate medicines available for these conditions have not yet been fully established in children, but there use is under active investigation.
Surgical intervention is necessary for certain pathological fractures and to correct symptomatic deformity. Fractures treated with non-surgical techniques can be expected to heal. Other minimally invasive interventions that have been used to treat related lesions, such as percutaneous osteoplasty as used for hemangioma of bone, may hold some promise. One report of the use of radiation to control these lesions in an inoperable location has been published.
Since the condition is very rare, each case must be evaluated and treated as a unique entity.
In the case illustrated here, severe involvement of the pelvis and the resulting pathological collapse of the articular surface of the femoral head lead to disabling pain. This was treated by Girdlestone-type resection arthroplasty of the hip.
At surgery, the lymph fluid seeps and runs from the cut bone surface, with the fluid welling up and drawing back into the bone in response to slight changes of the central venous pressure.
Ref:
Mirra, Bone Tumors Lea and Febinbger, pp1426
11/2002 HD
================================================
Diffuse lymphangiomatosis of bone
| Australasian Radiology Volume 43 Issue 4 Page 535 - November 1999 doi:10.1046/j.1440-1673.1999.00726.x |
|
| Abstract | |
| Case Report | |
| Diffuse lymphangiomatosis of bone | |
| Daniel D Maki, Mark E Nesbit & Harry J Griffiths1Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania,, 2Department of Pediatric Oncology, University of Minnesota Medical School, Minneapolis, Minnesota and, 3Department of Radiology, University of Missouri Health Sciences Center, Columbia, Missouri, USA | |
|
Two cases of lymphangiomatosis of bone, a very rare systemic condition characterized by both skeletal and parenchymal lesions, are presented. The skeletal changes have an appearance similar to haemangiomas in the spine, and soap-bubbly lesions in the flat bones. One case carried the diagnosis of eosinophilic granuloma for 18 years. The findings on MRI, which have not been previously well-established, are discussed. |
|
 |
....................
Renal lymphangiomatosis: Sonographic findingsJ Clin Ultrasound. 2008 May
Department of Radiology, Shiraz University of Medical Sciences, Zand Street, Shiraz, Iran.
Renal lymphangiomatosis is an exceedingly rare disorder characterized by developmental malformation of the lymphatic system surrounding the kidneys. We report a case of bilateral renal lymphangiomatosis in a 21-year-old man who underwent abdominal sonographic examination that revealed numerous cystic areas of varyious sizes around both kidneys with extension along the renal hilum. Subsequent abdominal CT examination demonstrated bilateral, multilocular, fluid-filled cystic masses with thin walls in the perirenal and peripelvic region. MRI of the patient revealed bilaterally enlarged kidneys with multiple hyperintense lesions in both perirenal spaces and the peripelvic area on T2-weighted images. These cystic spaces appeared hypointense on T1-weighted images with no enhancement in postcontrast images. The diagnosis of renal lymphangiomatosis was made based
....................
Bilateral renal lymphangiomatosis: conservative management
Sept 2007
Servicio de Urología, Hospital Universitario Virgen de la Arrixaca, Murcia, España. ignaciotorne@hotmail.com
OBJECTIVE: Lymphangiomatosis is a benign disease of difficult diagnosis. The bilateral form is very rare, being in some cases an incidental finding.
METHODS: We report the case of a female patient with the incidental radiological diagnosis of bilateral lymphangiomatosis, who did not present symptoms in relation to the disease.
RESULTS: After a meticulous study with ultrasound, abdominal CT scan and the MRI the diagnosis was asymptomatic bilateral lymphangiomatosis and expectant management was decided with good outcome.
CONCLUSIONS: Most cases reported have been treated by nephrectomy for diagnosis, but in our case it was possible to do precise diagnosis adding the experience of the radiologist and the urologist and she had a good outcome with conservative management.
....................
Department of Radiotherapy, The Royal Marsden Hospital, Downs Road, Sutton, Surrey SM2 5PT, UK
http://adc.bmjjournals.com/cgi/content/full/archdischild;83/2/138
....................
a
Section of Cardiothoracic Surgery, Mayo Medical
Center, Jacksonville, Jacksonville, Florida, USA
b Department of General Surgery, Mayo Medical
Center, Jacksonville,
Jacksonville, Florida, USA
Accepted for publication May 1, 2000.
http://ats.ctsnetjournals.org/cgi/content/abstract/71/2/694
....................
Splenic lymphangiomatosis in
children.
Wadsworth DT, Newman B, Abramson SJ, Carpenter BL, Lorenzo RL.
Mallinckrodt Institute of Radiology, St Louis, Mo, USA.
PURPOSE: To study the clinical and imaging features of splenic
lymphangiomatosis.
MATERIALS AND METHODS: The clinical and abdominal imaging data of 10
children
with splenic lymphangiomatosis were retrospectively reviewed. The
modalities
used in the study included computed tomography (CT) (10 patients),
sonography
(five patients), and magnetic resonance (MR) imaging (two patients).
Pathologic
confirmation of lymphangiomatosis was obtained in nine patients.
RESULTS:
Splenic lymphangiomatosis was discovered incidentally in all cases and
was a key
finding in enabling the correct diagnosis in six children with
extrasplenic
disease as well. Only two children had clinical splenomegaly. Sonograms
and MR
images showed multiple, wEll-defined cysts. Multiple, low-attenuation
lesions
that did not enhance with intravenous administration of contrast
material (n =
8) or a mottled spleen (n = 2) were seen at CT. One of the mottled
spleens had
target lesions on an early (arterial) image obtained after
administration of a
bolus of contrast material. One child underwent a splenectomy; one
child
underwent therapeutic embolization. Eight patients remained
asymptomatic with
respect to the spleen 1-20 years later. CONCLUSION: Splenic
lymphangiomatosis is
often an incidental imaging finding that frequently has a
characteristic imaging
appearance. The recognition of this appearance helps in diagnosis of
this
disease and may prevent the need for further invasive procedures.
Splenic
changes can be isolated or can coexist with bone or soft-tissue
lymphangiomas.
PMID: 8988208 [PubMed
- indexed for
MEDLINE]
....................
Definition
Synonyms
Pulmonary lymphangiectasis1,2,3, generalized lymphangiectasis4, (intra)thoracic lymphangiomatosis5,6, and diffuse pulmonary angiomatosis7. The confusing nomenclature of pulmonary lymphatic disorders has been reviewed8.
Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection8.
Patients present with "asthma", dyspnea or haemoptysis8,12. Pleural effusions are common8,12.
There are increased interstitial markings. CT shows smooth thickening of the interlobular septa, major fissures, central airways and pleura, perihilar and mediastinal fat11. There are often patchy pulmonary ground glass changes11.
The lymphatic proliferation renders the bronchovascular bundles, pleura and septa prominent.
Endothelium-lined lymphatic spaces permeate pleura, interlobular septa and bronchovascular sheaths12. There may be bland spindle cells between the vascular channels. There is often eosinophilic acellular material. The lung parenchyma may contain iron-laden macrophages12 but the lung architecture is preserved8.
|
cells lining lymphatics |
spindle cells |
|||
|
Factor VIIIRA |
positive0,10,12 |
|
||
|
Ulex europaeus |
positive12 |
|
||
|
CD31 |
positive0,8,10 |
|
||
|
Vimentin |
|
positive12 |
||
|
Desmin |
|
positive12 |
||
|
Actin |
|
positive12 |
||
|
PR |
|
positive12 |
||
|
ER |
|
negative12 |
||
|
Cytokeratins |
|
negative12 |
||
|
HMB-45 |
|
negative12 |
||
Lymphangiectasis: the lymphatics are dilated but not increased in number and do not show an anastomosing pattern. There is no spindle cell proliferation8.
Lymphangioleiomyomatosis: there are cysts and the spindle cells are positive for HMB45, disrupting the lung architecture.
Kaposi sarcoma: lacks anastomosing lymphatic channels.
Diffuse pulmonary haemangiomatosis: the spaces are blood-filled.
Interstitial emphysema: the spaces are air-filled and lack smooth muscle.
Percutaneous sclerotherapy with doxycycline has been employed with good results9.
The condition is commonly fatal and tends to be more aggressive in younger children12.
*Link no longer available
===========================
External Links:
.................
Lymphangiomatosis (1)
http://www.amershamhealth.com/medcyclopaedia/medical/Volume%20III%201/LYMPHANGIOMATOSIS.ASP
Skeletal Lymphanagiomatosis with Chylous Pleural Effusion
www.jradiology.com/arts/56.pdf
Splenic lymphangiomatosis: a rare cause of splenomegaly.
May 1987 PubMed
Diffuse pulmonary lymphangiomatosis: imaging findings
Margraf, L. R.. 1996. Thoracic lymphangiomatosis. Pediatr. Pathol. Lab. Med. 16: 155-160
Canny GJ, Cutz E, MacLusky IB, et al. Diffuse pulmonary angiomatosis. Thorax 1991; 46:851-3
===========================
Support Groups
.................
The Lymphangiomatosis Foundation
http://www.lymphangiomatosis.org/
...........
The Lymphangiomatosis & Gorham's Disease Alliance (1)
===========================
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Lymphedema People / Advocates for Lymphedema
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Lymphatic
Disorders Support Group @ Yahoo Groups
While we have a number of support groups for lymphedema... there is
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comprehensive information sites on all lymphatic disorders, I thought
perhaps,
it is time that one be offered.
DISCRIPTION
Information and support for rare and unusual disorders affecting the
lymph
system. Includes lymphangiomas, lymphatic malformations,
telangiectasia,
hennekam's syndrome, distichiasis, Figueroa
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......................
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......................
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