Cutaneous B-Cell Lymphoma and Lymphedema - Is There A Connection
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Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity.
Torres-Paoli D, Sanchez JL.
Department of Dermatology, University of Puerto Rico, School of Medicine, San Juan 00936-5067, USA.
This is a case report of a woman who had chronic lymphedema on one leg and who developed a primary cutaneous large B-cell lymphoma of the leg at that site. She received radiotherapy and did not show any systemic involvement thereafter. Other neoplasms may appear in a clinical setting of chronic lymphedema, namely, lymphangiosarcoma (Stewart-Treves), melanoma, and metastatic carcinoma. There are four other reports in the English literature of cutaneous lymphoma arising in an extremity with chronic lymphedema.
Publication Types:
Case Reports
Review
Review of Reported Cases
MeSH Terms:
Aged
Aged, 80 and over
Chronic Disease
Combined Modality Therapy
Female
Humans
Leg
Lymphedema/complications*
Lymphedema/pathology
Lymphedema/therapy
Lymphoma, B-Cell/etiology*
Lymphoma, B-Cell/pathology
Lymphoma, B-Cell/therapy
Lymphoma, Large-Cell/etiology*
Lymphoma, Large-Cell/pathology
Skin Neoplasms/etiology*
Skin Neoplasms/pathology
Skin Neoplasms/therapy
PMID: 10871070 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Citation
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Non-Hodgkin's lymphoma of the skin associated with chronic limb lymphoedema.
Tatnall FM, Mann BS.
Cutaneous non-Hodgkin's lymphoma developed within a leg affected by chronic lymphoedema. The lymphoedema had followed radiotherapy to bony metastases from a carcinoma of the prostate. Eighteen months after the development of the cutaneous tumours, extracutaneous involvement by the lymphoma became apparent. This is the second report of a non-Hodgkin's lymphoma appearing within a lymphoedematous limb. The possible reasons for such an unusual localization are discussed. Our case report illustrates that cutaneous tumours other than lymphangiosarcomas may localize to a lymphoedematous limb and clinically simulate the Stewart-Treves syndrome.
Publication Types:
Case Reports
PMID: 4096886 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... query_hl=6
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Primary cutaneous large B-cell lymphoma: the relation between morphology, clinical presentation, immunohistochemical markers, and survival.
Fernandez-Vazquez A, Rodriguez-Peralto JL, Martinez MA, Platon EM, Algara P, Camacho FI, Lopez-Rios F, Zarco C, Sanchez-Yus E, Fresno MF, Barthe L, Aliaga A, Fraga M, Forteza J, Oliva H, Piris MA.
Programa de Patologia Molecular, Centro Nacional de Investigaciones Oncologicas, Madrid, Spain.
The histogenesis, morphology, immunophenotype, and clinical behavior of cutaneous large B-cell lymphomas (CLBCL) are largely a matter of controversy. We performed an investigation to determine whether CLBCL have features that differentiate them from other large B-cell lymphomas and whether CLBCL is itself a heterogeneous group. To this end, we reviewed the main characteristics of a series of 32 cases of LBCL found in the skin. We reviewed the clinical findings and paraffin sections of the tumors from these 32 patients. The immunohistochemical study performed included p53, MIB1, Bcl2, Bcl6, and CD10 markers. We carried out statistical analysis of these data (univariate and multivariate), seeking an association between the features of the tumors and clinical outcome, as defined by failure-free survival time. Only one patient died as a consequence of the lymphoma. Nevertheless, the accumulated probability of survival without failure at 48 months was 0.46. The number, type, and localization of the lesions were not associated with variations in either survival or failure-free survival. The expression of p53 was negative in this group of CLBCL, whereas Bcl-2 expression or localization in the lower leg did not relate to any other significant feature. Histologic examination of the cases disclosed three different groups: Grade III follicular lymphomas (FLs), monomorphous large B-cell lymphomas (LBCL type I), and LBCL with an admixed component of small B-lymphocytes (LBCL type II). Grade III FL (11 cases) tended to be found in the head and neck and showed CD10 expression in a majority of cases. A higher probability of lymph node relapses was associated with cases located in the head and neck and with CD10+ tumors. Cutaneous large B-cell lymphomas are indolent tumors, but follow an insidious course. Our data support the interpretation that CLBCL is a heterogeneous condition; comprises some LBCL derived from CD10+ germinal center cells which manifests more frequently as tumors in the head and neck region, with an increased probability of relapse in lymph nodes [1] and has some distinctive morphologic features. The existence of a component of small B-cells within the other CLBCL could lend support to the theory that some of these tumors, more than arise de novo, may have originated in preexistent small B-cell lymphomas, but no firm evidence of this is provided in this study.
PMID: 11224600 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... query_hl=6
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Cutaneous B-cell lymphoma: pathological spectrum and clinical outcome in 51 consecutive patients.
Sah A, Barrans SL, Parapia LA, Jack AS, Owen RG.
HMDS, Leeds General Infirmary, Leeds, United Kingdom. anitasah@doctors.org.uk
The clinical behavior and optimal treatment of patients presenting with skin infiltration by B-cell lymphoma have not been established. To clarify this we assessed the clinical and laboratory features of 51 patients presenting with cutaneous infiltration by B-cell lymphoma. Follow-up data was available for 46 patients with a median age of 68 years (range 16-89 years) and a median follow-up of 32.5 months (range 5-123 months). Thirty-three of 51 (65%) patients had diffuse large B-cell lymphoma (DLBCL), and 15/51 (29%) had marginal zone lymphoma (MZL). The remaining 3 patients had follicular lymphoma, CLL, and post-transplant lymphoproliferative disease. Of the 33 patients with DLBCL, follow-up was available in 29; 24/29 (83%) had primary cutaneous disease, which was unifocal in 17/24 (71%). Following treatment, 8/24 (33%) of the primary cases relapsed. Of the 8 who relapsed, 7 had received local forms of treatment only. Follow-up data was available in 14/15 patients with MZL. 11/14 (79%) had primary cutaneous disease, which was unifocal in 8 (73%). Following treatment, 4 of these cases relapsed (36%); all had received local therapy only. It is evident from this study that a significant proportion ( reverse similar 20%) of patients who present with cutaneous infiltration by B-cell lymphoma have systemic disease. Staging is therefore mandatory in these patients. Approximately 1/3 patients with primary cutaneous DLBCL or MZL ultimately relapse, and relapse rates appear higher in those patients receiving local therapy only. Systemic or combined modality therapy may therefore be the most appropriate treatment at presentation. This should be assessed prospectively in randomized trials. Copyright 2004 Wiley-Liss, Inc.
PMID: 15054808 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... query_hl=6
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Squamous cell carcinoma in chronic lymphedema: case report and review of the literature.
Furukawa H, Yamamoto Y, Minakawa H, Sugihara T.
Department of Plastic and Reconstructive Surgery, Hokkaido University, School of Medicine, Sapporo, Japan. furufuru@beach.ocn.ne.jp
BACKGROUND: Squamous cell carcinoma (SCC) arising in chronic lymphedema is rare; only nine cases have been reported. OBJECTIVE: To present the evolution of SCC in chronic lymphedema. METHODS: Case report and literature review. RESULTS: The tumor was treated by wide excision and covered by a skin graft. CONCLUSION: In most of the other reported SCC cases in lymphedema, there are additional factors for carcinogenesis. There is no additional carcinogenic factor except for chronic lymphedema in our case. This strongly supports that lymphedema itself is one of the carcinogenic factors for not only angiosarcoma but also SCC.
Publication Types:
Case Reports
Review
Review of Reported Cases
PMID: 12410682 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... query_hl=6
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Chronic lymphoedema and angiosarcoma.
Azurdia RM, Guerin DM, Verbov JL.
Department of Dermatology, The Royal Liverpool University Hospital NHS Trust, UK.
Angiosarcoma has frequently been described arising within chronic lymphoedema of the upper limb following mastectomy and radiotherapy for carcinoma of the breast. We report a case of angiosarcoma arising in a lymphoedematous leg that had been subjected to radiotherapy 20 years previously for Hodgkin's disease. The diagnosis was expedited once the patient noticed the development of bleeding nodules. Prognosis of angiosarcoma is poor with treatment options being wide-excision surgery, palliative radiotherapy or chemotherapy. Unusual bruised areas or bleeding nodules developing within chronic lymphoedematous limbs should be biopsied to exclude the diagnosis.
Publication Types:
Case Reports
PMID: 10457127 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... query_hl=6
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Non-Hodgkin's lymphoma of the skin associated with chronic limb lymphoedema.
Tatnall FM, Mann BS.
Cutaneous non-Hodgkin's lymphoma developed within a leg affected by chronic lymphoedema. The lymphoedema had followed radiotherapy to bony metastases from a carcinoma of the prostate. Eighteen months after the development of the cutaneous tumours, extracutaneous involvement by the lymphoma became apparent. This is the second report of a non-Hodgkin's lymphoma appearing within a lymphoedematous limb. The possible reasons for such an unusual localization are discussed. Our case report illustrates that cutaneous tumours other than lymphangiosarcomas may localize to a lymphoedematous limb and clinically simulate the Stewart-Treves syndrome.
Publication Types:
Case Reports
MeSH Terms:
Aged
Bone Neoplasms/radiotherapy
Bone Neoplasms/secondary
Chronic Disease
Humans
Leg
Lymphedema/complications*
Lymphedema/etiology
Lymphoma/complications*
Lymphoma/pathology
Male
Radiotherapy/adverse effects
Skin Neoplasms/complications*
PMID: 4096886 [PubMed - indexed for MEDLINE]
http://www.ncbi.nlm.nih.gov/entrez/quer ... t=Citation
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SQUAMOUS-CELL CARCINOMA ON LONG LASTING LYMPHOEDEMA.
ECHENIQUE-ELIZONDO, Miguel, MD, FACS
ELORZA, José, L. MD
Correspondence:
Echenique-Elizondo, M., MD,FACS.
Associated Professor of Surgery. Basque Country University. School of Medicine.
P. Dr. Begiristain, 105
20010 San Sebastián. Spain
Phone. +34-943017319
E-mail. gepecelm@sc.ehu.es
Key Words
Chronic
Lymphoedema
Squamous-cell
Although lymphangiosarcoma (1)is a well recognized complication of chronic lymphoedema, squamous cell carcinoma has only been reported on seven previous occasions in a recent review (2). Multiple epithelial tumor on a single limb has also been recorded (3).
Case report:
A 63 years old white male living in a rural area and complaining of congenital non previously treated lymphoedema was admitted with the aspect seen in Lymphoedema (4)may be primary or secondary to the presence of other disease and/or to the consequences of surgery. Primary lymphoedema may be congenital – Milroy´s disease – or may occur at any phase of life but it most commonly appears at puberty. Secondary lymphoedema is encountered more often. The most prevalent worldwide cause of lymphoedema is filariasis, which is particularly common in south-east Asia and Africa. In Western countries postsurgical lymphoedema of the extremity prevails. Complications of chronic limb lymphoedema include recurrent cellulitis and lymphangiosarcoma.
Although lymphangiosarcoma is a well recognized complication of chronic lymphoedema, squamous cell carcinoma has only been reported on seven previous occasions in a recent review. Multiple epithelial tumor on a single limb has been recorded. Other neplasms such as primary cutaneous B-cell lymphoma has also been registered (5) . Lymphangiosarcoma is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. Today, due to changes in the operative techniques of breast cancer, less chronic lymphoedema is seen with only a scant number of lymphangiosarcoma patients in our work place. The true incidence of lymphangiosarcoma after post-mastectomy lymphoedema remains controversial and may we underdiagnosed. One of the large reviews on the subject done between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France) (6). Of these patients, 6919 were followed for at least 1 year. Out of these, 11 presented with sarcomas thought to be induced by irradiation, 2 of which were Steward-Treves Syndrome, and 9 of which were sarcomas within the irradiated fields. The sites of these sarcomas were: parietal wall, 1 case; second costal cartilage, 1 case; infraclavicular region, 1 case; supraclavicular region, 2 cases; internal third of the clavicle, 2 cases; axillary region 2 cases; and the internal side of the upper arm (Stewart-Treves syndrome), 2 cases. The cumulative incidence of sarcoma following irradiation of breast cancer was 0.2% (0.09-0.47) at 10 years. The standardized incidence ratio (SIR) of sarcoma (observed n# of cases (Obs)/expected n# of cases (Exp) computed from the Danish Cancer Registry for the same period was 1.81 (CI 0.91-3.23). This is significantly higher than one, with a p = 0.03 (One Tailed Exact Test). The mean annual excess (Obs-Exp)/100.000 person-years at risk during the same period/(100,000) was 9.92. This study suggests that patients treated by radiation for breast cancer have a risk of subsequent sarcomas that is higher than the general population.
However in the current of epidermoid carcinoma arising on long lasting lymphoedema case there is not experience large enough in treatment and follow-up because we are dealing with a few isolated clinical reports and no large series registered exists to set the appropriate management. Treatment options include radical ablative surgery, radiation therapy, and chemotherapy. Early recognition and radical ablative surgery must be considered on the changes arising on chronic long lasting lymphoedema.
References.
1.- Heitmann C, Ingianni G. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.
Ann Plast Surg. 2000.44(1).72-75
2.- Lister RK, Black MM, Calonje E, Burnand KG.
Squamous cell carcinoma arising in chronic lymphoedema.
Br J Dermatol. 1997 Mar;136(3):384-7.
3.- Dandurand M, Bernard F, Barneon G, Guilhou E, Guillot B, Guilhou JJ.Multiple spinocellular carcinomas on chronic lymphedema. Association with vitiligo.
Ann Dermatol Venereol. 1990;117(12):953-6.
4.- Szuba A, Rockson SG.
Lymphedema: classification, diagnosis and therapy.
Vasc Med. 1998;3(2):145-56.
5.- Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity.
Am J Dermatopathol. 2000 Jun;22(3):257-60.
6.- Taghian A, de Vathaire F, Terrier P, Le M, Auquier A, Mouriesse H, Grimaud E, Sarrazin D, Tubiana M.Long-term risk of sarcoma following radiation treatment for breast cancer.
Int J Radiat Oncol Biol Phys. 1991 Jul;21(2):361-7.
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Arm and Leg Swelling After Cancer
With the advent of better and more effective cancer treatments, the survival rate for all cancers has risen dramatically. With this progress, a new and often misunderstood and misdiagnosed complication has arisen.
Many cancer survivors , having overcome cancer, find themselves with sudden and often unexplained swelling, usually of the arms or of the legs.
This swelling occurs because of one of several factors.
First, the swelling begins after lymph nodes have been removed for cancer biopsies.
Second, the swelling may start as a result of radiation damage to either the lymph nodes and/or the lymph system.
Due to either the removal of lymph nodes or damage to the lymph system, your body is no longer able to rid itself of excess fluids. The fluids collect in the limbs effected and swelling beings.
This swelling is called lymphedema. The swelling that occurs is permanent, and while it is not curable it is treatable.
Permanent Leg or Arm Swelling
****In the situation of any permanent leg swelling whether the cause is known or unknown, the diagnoses of lymphedema must be considered****
There are several groups of people who experience leg or arm swelling from known causes, but it doesn't go away or unknown causes where the swelling can actually get worse as time goes by.
Group One
This group includes those who have had the injuries, infections, insect bites, trauma to the leg, surgeries or reaction to a medication. When this swelling does not go away, and becomes permanent it is called secondary lymphedema.
Group Two
Another extremely large group that experiences permanent leg or arm swelling are cancer patients, people who are morbidly obese, or those with the condition called lepedema. What causes the swelling to remain permanent is that the lymph system has been so damaged that it can no longer operate normally in removing the body's waste fluid.
In cancer patients this is the result of either removal of the lymph nodes for cancer biopsy, radiation damage to the lymph system, or damage from tumor/cancer surgeries.
This is also referred to as secondary lymphedema.
Group Three
Group three consists of people who have leg or arm swelling from seemingly unknown reasons. There may be no injury, no cancer, no trauma, but for some reason the leg simply is swollen all the time.
The swelling may start at birth, it may begin at puberty, or may begin in the 3rd, 4th or even 5th decade of life or sometimes later.
This type of leg or arm swelling is called primary lymphedema. It can be caused by a genetic defect, malformation or damage to the lymph system while in the womb or at birth or be part of another birth condition that also effects the lymph system.
This is an extremely serious medical condition that must be diagnosed early, and treated quickly so as to avoid painful, debilitating and even life threatening complications. Treatment should NOT include the use of diuretics.
What is Lymphedema?
Lymphedema is defined simply as an accumulation of excessive protein rich fluid in the tissues of the leg. The accumulation of fluid causes the permanent swelling caused by a defective lymph system.
A conservative estimate is that there may be 1-2 million people in the United States with some form of primary lymphedema and two to three million with secondary lymphedema.
What are the symptoms of Lymphedema?
If you are an at risk person for arm lymphedema there are early warning signs you should be aware of. If you experience any or several of these symptoms, you should immediately make your physician aware of them.
1.) Unexplained aching, hurting or pain in the arm
2.) Experiencing "fleeting lymphedema." This is where the limb may swell, even slightly, then return to normal. This may be a precursor to full blown arm lymphedema.
3.) Localized swelling of any area. Sometimes lymphedema may start as swelling in one area, for example the hand, or between the elbow and hand. This is an indication of early lymphatic malfunction.
4.) Any arm inflammation, redness or infection.
5.) You may experience a feeling of tightness, heaviness or weakness of the arm.
How is Lymphedema Treated?
The preferred treatment today is decongestive therapy. The forms of therapy are complete decongestive therapy (CDT) or manual decongestive therapy (MDT), there are variances, but most involve these two type of treatment.
It is a form of massage therapy where the leg is very gently massaged to actually move the fluid out of the leg and into an area where the lymph system still functions normally.
With these massage treatments, swelling is reduced and then the patient is fitted with a pre-measured custom pressure garment to keep the swelling down and/or is taught to use compression wraps to maintain the leg size.
What are some of the complications of lymphedema?
1. Infections such as cellulitis, lymphangitis, erysipelas. This is due not only to the large accumulation of fluid, but it is well documented that lymphodemous limbs are localized immuno-deficient.
2. Draining wounds that leak lymphorrea which is very caustic to surrounding skin tissue and acts as a port of entry for infections.
3. Increased pain as a result of the compression of nerves usually caused by the development of fibrosis and increased build up of fluids.
4. Loss of Function due to the swelling and limb changes.
5. Depression - Psychological coping as a result of the disfigurement and debilitating effect of lymphedema.
6. Deep venous thrombosis again as a result of the pressure of the swelling and fibrosis against the vascular system. Also, can happen as a result of cellulitis, lymphangitis and infections.
7. Sepsis, Gangrene are possibilities as a result of the infections.
8. Possible amputation of the limb.
9. Pleural effusions may result if the lymphatics in the abdomen or chest are to overwhelmed to clear the lung cavity of fluids.
10. Skin complications such as splitting, plaques, susceptibility to fungus and bacterial infections.
11. Chronic localized inflammations.
Can lymphedema be cured?
No, at the present time there is no cure for lymphedema. But it can be treated and managed and most of the complications can be avoided. Life with lymphedema can still be active and full, with proper treatment, patient education, and patient life style adaptation.
For extensive information on lymphedema, please visit our home page:
Lymphedema People
http://www.lymphedemapeople.com
(c) Copyright 2005 by Pat O'Connor and Lymphedema People. Use of this information for educational purpose is encouraged and permitted. It must be available free and without charge and not used for financial renumeration or gain. Please include an acknowledgement to the author and a link to Lymphedema People.
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Good Information on B-Cell Lymphomas:
Lymphoma, B-Cell
Last Updated: January 10, 2005 Rate this Article
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Synonyms and related keywords: non-Hodgkin lymphoma, NHL, lymph node, lymphoid tissue, indolent lymphomas, aggressive lymphomas, Burkitt lymphoma, B-cell neoplasms, T/NK-cell neoplasms, Hodgkin lymphoma, Hodgkin's lymphoma, B-cell chronic lymphocytic leukemia, CLL, B-cell CLL, B-cell small lymphocytic lymphoma, lymphoblastic lymphomas, T-cell acute lymphocytic leukemias, diffuse large B-cell lymphoma, follicular lymphoma, follicular large cell lymphoma, mantle cell NHL, small lymphocytic lymphoma, splenic marginal zone lymphoma, extranodal B-cell lymphoma of mucosa-associated lymphoid tissue, MALT, mediastinal diffuse large B-cell lymphoma, indolent NHL, lymphoproliferative malignancy, lymphoproliferative disease, Revised European-American Lymphoma Classification, REAL Classification, immunocytoma, mycosis fungoides, Sézary syndrome, lymphadenopathy, Waldeyer ring, bcl-2 apoptotic inhibitor oncogene
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For information about Lymphedema
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