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LYMPHANGIOSARCOMA 

Stewart Treves Syndrome

Angiosarcoma 

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Lymphangiosarcoma 

Related Terms: lymphangioendothelioma, hemangiosarcoma, cutaneous angiosarcoma, postmastectomy angiosarcoma, lymphangiosarcoma in postmastectomy lymphedema, postlymphedema angiosarcoma, hemangiosarcoma in chronic lymphedema, hemangiosarcoma in postmastectomy lymphedema, postmastectomy lymphedema, lymphangiosarcoma, Milroy disease, Milroy's disease, idiopathic lymphedema, congenital lymphedema, traumatic lymphedema, filarial lymphedema

Discussion

Stewart-Treves Syndrome (lymphangiosarcoma) is a rare, aggressive and cutaneous angiosarcoma often associated with long standing lymphedema. The malignancy arises from the endothelial cells of the lymphatic system.

Most cases arise from lymphedema induced by a radical mastectomy in breast cancer patients with an average onset of 5 - 15 years and an occurrence rate of <1%. Although there has also been significant reports regarding development from Milroy lymphedema and in idiopathic, congenital, traumatic, or filarial lymphedema.

Causes:

The three main indications for the development of Stewart Treves in a lymphodemous limb appear to be radiation, extensive fibrosis and recurrent or chronic infection. Another cause factor may be the immunodeficient state of a lymphodemous limb.

Pathology:

The sarcoma first appears as a bruised area or a purplish discoloration in an extremity. It progresses to an ulcer with crusting and finally to an extensive necrosis involving the skin and subcutaneous tissue. There is also often development of satellite spots from the original locale. It metastasizes widely and there is no effective therapy or cure.

Treatment:

In the past, this rapid growing sarcoma was generally fatal as treatment was difficult, if not impossible.  The malignant lesions were usually excised and/or the limb was amputated. 

In more recent years chemotherapy and radiotherapy has shown increased long term survival rates and in my opinion, should be tried before the limb is amputated.  The critical importance is in early diagnosing and intervention.

Chemotherapy with intraarterial mitoxantrone and placitaxel with ex vivo previous sensitivity test seems a current adequate complementary approach.(1)   

Diagnosis:

The appearance of  multiple "purplish" lesions, with given patient history should cause a suspiscion of lymphangiosarcoma.

"Diagnosis is finally histological. There is an admixture of spindle cells and vascular spaces deformed by pleiomorphic endothelial cells. A metastasis of the primary tumor is ruled out by electron microscopy (Weibel-palade granulations), or, more currently, by immunohistochemistry. Lymphangiosarcoma cells express positive endothelial markers (CD34, vimentin, keratine, VIII factor antigen). Kaposi's sarcoma is excluded by epidemiological context and histologic examination."(2)

Prognosis:

Poor as the malignancy spreads rapidly, metasticing to the lungs, chest walls, liver and or bone and the reoccurrence rate is high . Long term survival rates are extremely low.

Pat O'Connor

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LYMPHANGIOSARCOMA / STEWART - TREVES SYNDROME

*** Introduction to the article - please see weblink for the entire article ***

Background: Stewart-Treves syndrome is a rare, deadly cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Most commonly, this tumor is a result of lymphedema induced by radical mastectomy to treat breast cancer. Unfortunately, although the breast cancer may be cured with such radical surgery, this second primary cancer may be responsible for the patient's worsening course. The term Stewart-Treves syndrome is broadly applied to an angiosarcoma that arises in a chronically lymphedematous region due to any cause, including congenital lymphedema and other causes of secondary lymphedema unassociated with mastectomy. Lymphangiosarcoma is a misnomer because this malignancy seems to arise from blood vessels instead of lymphatic vessels. A more appropriate name is hemangiosarcoma.

In 1906, Lowenstein first described angiosarcoma in a patient's arm that had been affected by severe posttraumatic lymphedema for 5 years. In 1948, Stewart and Treves reported this rare secondary malignancy in 6 cases of angiosarcoma in postmastectomy lymphedema. They recognized that an edematous arm after radical mastectomy for breast cancer may suggest recurrent breast cancer, but that long-standing chronic edema without recurrent cancer may occasionally produce "a heretofore unrecognized and unreported sequel ... long after the malignant breast neoplasm has apparently been arrested ... a new specific tumor." Stewart and Treves suggested that these angiosarcomas were probably not observed previously because they were mistaken for recurrent, inoperable, cutaneous manifestations of breast cancer.

Lymphangiosarcoma has been described in Milroy disease and in idiopathic, congenital, traumatic, or filarial lymphedema.

http://www.emedicine.com/derm/topic898.htm

eMedicine

http://www.emedicine.com

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Angiosarcoma in a patient with congenital nonhereditary lymphedema.

Nov 2012

Shon W, Wada DA, Folpe AL, Pittelkow MR.

Source

Divison of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.

Abstract

Angiosarcoma is an uncommon but aggressive tumor of endothelial origin that may occur in the upper extremities of patients with postmastectomy lymphedema (Stewart-Treves syndrome) as well as in other regions. We present an unusual case of angiosarcoma associated with congenital nonhereditary lymphedema in an 18-year-old man. Our case underscores the need for a careful clinical examination and shows the importance of appropriate sampling and thorough pathologic examination of suspicious areas to exclude the presence of a malignant process.

PubMed

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Cutaneous angiosarcoma arising in massive localized lymphedema of the morbidly obese: a report of five cases and review of the literature.

July 2011

Shon W, Ida CM, Boland-Froemming JM, Rose PS, Folpe A.

Source

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.

Abstract

A significant proportion of angiosarcoma arises in the setting of chronic lymphedema, often following surgery for breast carcinoma (Stewart-Treves syndrome). Massive localized lymphedema (MLL), a relatively recently described and rare pseudotumor most often occurring in morbidly obese patients, is thought to be the result of derangements in lymphatic drainage secondary to excessive adipose tissue. Only a very small number of cases of angiosarcoma have been reported in patients with MLL. We reviewed the clinical and pathological features of five patients with angiosarcoma arising in this unusual clinical setting. The tumors arose in three women and two men, who were in the age range from 36 to 81 years (median: 52 years). All patients were or had recently been morbidly obese (mean body mass index = 47.2 kg/cm(2) ; range:37.6-68.5 kg/cm(2) ) and had long-standing soft tissue changes of MLL. The tumors arose in the thigh (two cases), lower leg (two cases) and abdomen/proximal thigh (one case) and ranged from 6 to 28.5 cm in size (mean: 15.9 cm). All tumors were conventional high-grade angiosarcomas and microscopically exhibited an admixture of vasoformative, spindled and epithelioid features. Microscopic changes of MLL, including dermal fibrosis, expansion of interlobular fibrous septa and lymphangiectasia, were identified in all cases. Clinical follow-up (four cases; mean: 13.8 months; range: 2-32 months) showed one patient to be dead of disease, one patient to be dead from therapy-related complications and two patients to be alive without disease. One case was too recent for meaningful clinical follow-up. We conclude that lymphedema secondary to obesity should be recognized as a significant risk factor for the development of angiosarcoma. The clinical and pathological features of angiosarcoma arising in this distinctive clinical setting appear to be identical to those of other lymphedema-associated angiosarcomas. With the continuing epidemic of obesity in Western societies, the incidence of obesity-related angiosarcoma is probably to increase. Consequently, it is critical that lymphedematous areas in obese patients be carefully examined for clinical evidence of early angiosarcoma, with the hope that timely diagnosis may lead to improved patient outcome.

Wiley Online Library

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Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)

Ann Dermatol Venereol. 

2007 Oct

Verdier E, Carvalho P, Young P, Musette P, Courville P, Joly P.

Clinique Dermatologique, CHU, Rouen, France. verdiereva@yahoo.fr

BACKGROUND: Lymphangiosarcoma is a highly malignant tumor with a poor prognosis. Anthracyclines constitute the form of chemotherapy most commonly used in these patients. Unfortunately, they are poorly tolerated. We report a case of lymphangiosarcoma in an elderly woman with good response to liposomal doxorubicin, an anthracycline with lower toxicity. 

CASE REPORT: A 70 year-old-woman with a previous history of post-mastectomy lymphedema presented a painful and bleeding lymphangiosarcoma on the arm and the chest. Because of the wide extent of the tumor, surgery was not performed. The patient was treated with liposomal doxorubicin 50 mg/m2. Marked tumor regression was observed after the first course of chemotherapy. After 5 courses, 90% regression of tumor mass was seen. Pain and bleeding also stopped. Two months after the final course of liposomal doxorubicin, relapse occurred and the patient died. 

DISCUSSION: A dramatic response to liposomal doxorubicin was noted in the present case, as previously reported in a patient with an angiosarcoma of the scalp. Liposomal doxorubicin could be considered for the treatment of lymphangiosarcoma, particularly in elderly patients.

Elsevier Masson

For Information of Doxorubicin: MedMaster Patient Drug Information

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Radiotherapy in multilocalized lymphedema-associated angiosarcoma

Eur J Dermatol. 2005 Nov-Dec

Danz B, Hellmann A, Stadie V, Dunst J, Richter C, Marsch WCh, Helmbold P.

Department of Dermatology Martin Luther University Halle-Wittenberg, Klinik und Poliklinik für Hautkrankheiten Ernst-Kromayer-Str. 5 D-06097 Halle (Saale), Germany.

Keywords : interferon β, lymphangiosarcoma, lymphedema, radiotherapy, Stewart Treves Syndrome

We report an 80-year-old woman, suffering from a recurrence of a multilocalized lymphedema-associated angiosarcoma of the right arm. The tumor consisted of solid tumor cell formations and "classical" spongiform tumor complexes. In the tumor periphery, pathological endothelial cell proliferates on pre-existing dilated lymphatic capillaries were detectable, which, together with immunohistology (CD 31+/Desmoplakin-1-2.17+/CD 34-), supported the diagnosis of lymphangiosarcoma. Complete remission was achieved under radioimmunotherapy (54 Gy/Interferon beta). A further recurrence 3 months later outside the primary therapy fields was successfully treated with radiotherapy alone. During a follow-up observation period of 3 years, there was neither local recurrence nor metastasis. This case demonstrates for the first time the long-lasting efficacy of photon radiation in a case of histologically-defined lymphangiosarcoma. Further studies should elucidate the suitability of radio monotherapy as first-line therapy in lymphedema-associated angiosarcoma with lymphatic endothelium-like immunohistology.

European Journal of Dermatology

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Upper Limb Lymphangiosarcoma Following Breast Cancer Therapy

Journal of Clinical Oncology, Vol 24, No 9 (March 20), 2006: pp. 1477-1478
© 2006 American Society of Clinical Oncology.
DOI: 10.1200/JCO.2005.01.8473

Alberto Ocaña, Carlota Delgado, Cesar A. Rodríguez, Lorena Bellido, Noelia Izquierdo, Rebeca Martín, Juan J. Cruz

Departments of Medical Oncology, Dermatology, and Pathology, Hospital Universitario de Salamanca, Salamanca, Spain

A 75-year-old woman with a history of hypertension, diabetes mellitus, and cardiac failure was referred to our institution for a second opinion after a diagnosis of lymphangiosarcoma of the upper left limb. In 1997, she was diagnosed with invasive ductal carcinoma of the left breast. Treatment consisted of breast-conserving surgery followed by axillary node dissection and local radiotherapy. No additional systemic treatment was administered. The patient did well except for residual lymphedema of the arm. Three months before admission to our hospital, edema and pain of the left arm was observed, which was associated with fever during the last 2 weeks. The patient was diagnosed with erysipelas and systemic antibiotic treatment was administered without resulting in any improvement. Physical examination showed striking lymphedema with purple-colored multifocal raised lesions, several of them progressing to ulceration (Fig 1). As no improvement was observed with antibiotic treatment, histologic study was performed. Biopsy showed a lymphovascular invasive pattern compatible with lymphangiosarcoma. There was proliferation of spindle cells and multiple vascular spaces (Fig 2). Immunohistochemistry was positive for endothelial cell markers (vimentin, keratin, and CD34). After carefully evaluating the extent of the disease, including thoracic and abdominal computed tomography that showed no involvement of chest wall or visceral metastasis, radical surgery was performed followed by a reconstructive procedure.

Lymphangiosarcoma secondary to upper extremity edema following postmastectomy was first described by Stewart and Treves in 19481 and since then, this association has been called Stewart-Treves syndrome. Lymphangiosarcoma following breast cancer surgery is a rare entity. The incidence among white women in the United States is 1.6 of 100,000 and only 25% of these sarcomas are located in the upper extremity.2 Since the original description, approximately 300 cases of Stewart-Treves syndrome have been reported worldwide. The pathogenic mechanism of this syndrome is unknown, but several hypotheses have been postulated. As proliferation of lymphatic vessels is often seen in areas of affected edematous tissue,3 it has been suggested that the block of the lymphatics represent a stimuli for growth factors and cytokines and for, thus, a proliferation of vessels and lymphatics. The association between radiotherapy and chest wall sarcomas is well known,4 but it is unclear if radiation therapy contributes to this entity, since most reported postradiation sarcomas are not lymphangiosarcomas. Although postmastectomy is the main predisposing factor for lymphedema development, other factors such as hypertension and cardiovascular disease have been described.5 In our patient, the association of cardiovascular disease could contribute to the lymphedema. The prognosis of these tumors is poor and wide surgical resection is the treatment of choice.6 Although amputation is usually indicated with a curative intention, there is also a role for palliation in bleeding, fungation, and severe pain.7 The role of chemotherapy is not clear and the response rate in advanced disease is poor.8 Stewart-Treves Syndrome is a rare entity that must be taken into account when skin abnormalities appear in an edematous extremity secondary to primary breast cancer surgery, especially if postoperative radiotherapy was used. Early diagnosis and radical surgical treatment appear to confer the best chance for improved survival.

Authors' Disclosures of Potential Conflicts of Interest

The authors indicated no potential conflicts of interest.

REFERENCES

  1. Stewart FW, Treves N: Lymphangiosarcoma in postmastectomy lymphedema: A report of six cases in elephantiasis chirurgica. Cancer 1:64-81, 1948 [CrossRef]
  2. Mack TM: Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum and spleen. Cancer 75:211-245, 1995 [CrossRef][Medline]
  3. Woodward AH, Ivins JC, Soule EH: Lymphangiosarcoma arising in chronic lymphedematous extremities. Cancer 30:562-572, 1972 [CrossRef][Medline]
  4. Wiklund TA, Blomqvist CP, Raty J, et al: Postirradiation sarcoma: Analysis of nationwide cancer registry material. Cancer 68:524-531, 1991 [CrossRef][Medline]
  5. Bohler FK, Rhomberg W, Doringer W: Hypertention as a risk factor for increased rate of side effects in the framework of breast cancer irradiation. Strahlenther Onkol 168:344-349, 1992 [Medline]
  6. Stewart NJ, Pritchard DJ, Nascimento AG, et al: Lymphangiosarcoma following mastectomy. Clin Orthop Relat Res 320:135-141, 1995 [Medline]
  7. Clark MA, Thomas JM: Amputation for soft-tissue sarcoma. Lancet Oncol 4:335-342, 2003 [Medline]
  8. Kuten A, Sapir D, Cohen Y, et al: Postirradiation soft tissue sarcoma occurring in breast cancer patients: Report of seven cases and results of combination chemotherapy. J Surg Oncol 28:168-171, 1985 [Medline]

Journal of Clinical Oncology

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Lymphangiosarcoma
Peter P. Sordillo, MD *, Robert Chapman, MD, Steven I. Hajdu, MD, Gordon B. Magill, MD, Robert B. Golbey, MD
Departments of Medicine and Pathology, Memorial Sloan-Kettering Cancer Center, Cornell University School of Medicine, New York, New York

*Correspondence to Peter P. Sordillo, Memorial Hospital, 1275 York Avenue, New York, NY 10021

Abstract
Forty-four cases of lymphangiosarcoma treated at Memorial Sloan-Kettering Cancer Center were reviewed. With the exception of four patients with primary lymphangiosarcoma of the scalp, all patients had had chronic lymphedema of the involved limb for many years, usually following mastectomy for breast carcinoma, but occasionally due to other causes. Although chronic lymphedema could be implicated in the etiology of lymphangiosarcoma in all patients with neoplasms of an extremity, a significant number of patients did not have a history of radiation therapy at the site where their tumor developed. Histologically, although there were no differences in the lymphangio-sarcomas between any of the groups of patients, the morphology of the tumors was influenced in the individual patient by the size and anatomic site of the sarcoma. Early amputation seemed to give the best chance of long-term survival, with responses to wide resection, chemotherapy, and radiation therapy generally only of short duration. Amputation after local recurrence was ineffective in preventing pulmonary metastases and death. At present, early amputation appears to be the treatment of choice for patients with lymphangiosarcoma.

Wiley Interscience

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LYMPHANGIOSARCOMA  ON   CONGENITAL  LYMPHOEDEMA.

SOTO, Jorge.,MD

ECHENIQUE-ELIZONDO, Miguel, MD, FACS

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. +34-943-017319  - Fax.  +34-943-017330

E-mail. gepecelm@sc.ehu.es

Key Words  - Chronic Lymphoedema; Lymphangiosarcoma; Milroy disease

Case report: 

A 31 years old white male complaining of congenital – familial form-   non previously treated lymphoedema  - Milroy disease - was admitted with the aspect seen - Images  1,2,3 -. A biopsy  revealed the presence of lymphangiosarcoma – Images 4,5,6 -. No evidence of metastatic disease was clinically evident. High AK amputation was indicated and performed followed by prosthetic extremity replacement. No complementary treatment was done. Patient is free of the disease three years after operation and no evidence recurrence has been noticed so far.. 

Lymphoedema (1) may be primary or secondary to the presence of other disease and/or to the consequences of surgery or trauma (2). Primary lymphedema may be congenital – Milroy´s disease – or may occur at any phase of life but it most commonly appears at puberty – Meig’s disease -. 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 albeit other tumors such squamous-cell carcinoma (3,4), b-cell lymphoma (5)  and angiosarcoma  (6,7,8,9) has been reported. 

In cases of long-lasting or congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade lymphangiosarcoma developing in a patient with congenital hereditary lymphoedema (Milroy's disease) in a familial form (10). Hereditary lymphoedemas that are not associated with other malformations usually affect the lower limbs and are inherited in an autosomal dominant fashion. These non-syndromic hereditary lymphoedemas are categorized by their age of onset, being either congenital (Milroy disease) or having an onset in childhood or around puberty (Meige disease) (11). Development of  lymphangiosarcoma is  usually associated with post-mastectomy lymphoedema, has  been described in late-onset hereditary lymphoedema. There is a high incidence of multiple primary tumors in association with lymphangiosarcoma in the literature (12). 

Lymphangiosarcoma is a rare, aggressive, vascular neoplasm arising in chronic congenital or acquired lymphedema. Although it is most frequently associated with post-mastectomy lymphedema (Stewart-Treves's syndrome), lymphangiosarcoma can exceptionally arise in congenital hereditary lymphedema (11)(Milroy's syndrome and Meige's syndrome) and non-hereditary lymphoedema (congenital, praecox or forme tarde lymphoedemas) (13).  

 The risk of appearance of lymphangiosarcoma following mastectomy and radiation therapy has been recently analyzed. Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France) (14). 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. All histological slides were reviewed and a comparison with those of breast cancer was done. 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 median age of these 11 patients at the diagnosis of sarcomas was 65.8 (49-83). The mean latent period was 9.5 years (4-24). Three patients underwent radical mastectomy and nine modified radical mastectomy. Only one patient received chemotherapy. The radiation doses received at the site of the sarcoma were 45 Gy/18 fr. for 10 cases and 90-100 Gy for 1 case (due to overlapping between two fields). The histology was as follows: malignant fibrous histiocytoma, 5 cases; fibrosarcoma, 3 cases; lymphangiosarcoma, 2 cases; and osteochondrosarcoma, 1 case. The median survival following diagnosis of sarcoma was 2.4 years (4 months-9 years). Two patients are still alive: one with recurrence of her breast cancer, the other in complete remission, with 7 and 3 years follow-up, respectively. All other patients died from their sarcomas. 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, the benefit from adjuvant radiation therapy in the treatment of breast cancer exceeds the risk of second cancer; therefore, the potential of radiation-induced sarcomas should not be a factor in the selection of treatment for patients with breast cancer. 

Moreover, we emphasized the importance of regular clinical controls in all patients affected by chronic lymphoedema (15,16). In fact, although the prognosis of this neoplasm is very poor, a prompt diagnosis and a rapid, ablative surgery associated with radiation therapy can increase the possibility of survival of these patients (17,18). Chemotherapy with intraarterial mitoxantrone and placitaxel with ex vivo previous sensitivity test seems a current adequate complementary approach (19).

 http://www.sc.ehu.es/scrwwwsr/kirurgia/Kirurgia2003b/Linfangiosarcoma.htm

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Chronic lymphoedema and angiosarcoma.

Clin Exp Dermatol. 1999

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.

Blackwell Synergy

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Histological differentiation, histogenesis and prognosis of cutaneous angiosarcoma. June 2011

Histological differentiation, histogenesis and prognosis of cutaneous angiosarcoma.

Kamo R, Ishii M.

Source

Department of Dermatology, Osaka City University, Graduate School of Medicine, 1-4-3 Asahimachi, Abeno-ku, Osaka 545-8585, Japan. m8701131@med.osaka-cu.ac.jp

Abstract

BACKGROUND:

Cutaneous angiosarcoma (CAS) is a rare, extremely malignant vascular tumor. The optimum treatment for patients with CAS has not been defined because of its exremely rarity. As prognostic factors in patients with CAS, tumor less than 5 cm in size has a better prognosis. Although tumor differentiation in other sarcoma is an important prognostic factor, tumor differentiation in CAS is not a prognostic factor. CAS is thought as a collection of hemangiosarcoma,lymphangiosarcoma, tumors which cannot be classified as of vascular and lymphatic origin, or mixed tumor of both. Histogenesis of CAS have not been clarified yet. We tried to classify histogenesis by immunohistochemistry and evaluate the prognosis among histogeneses.


METHODS:

Using immunohistochemistry, we classified histogenesis of CAS in 20 patients who visited Osaka City University Hospital between 1998 and 2008.


ESULTS:

From the results of immunohistochemical staining with CD34 and D2-40, histogenesis of CAS can be divided into vascular type (CD34 positive D2-40 negative), mixed type (CD34 positive D2-40 positive), and lymphatic type (CD34 negative D2-40 positive). Vascular type was found in 2 cases, mixed type in 5 cases, and lymphatic type in 13 cases. Survival rates were not significantly affected by histogenesis, however, survival rate of mixed type was better than those of others.


CONCLUSIONS:

CAS can be divided into vascular type, mixed type, and lymphatic type based on immunohistochemistry. Because of a small group, we did not suggest that histogenesis of CAS was related with prognosis. We speculate that antiangiogenic agents might be important in the treatment based on histogeneses in CAS. In the future, further accumulation of chemotherapeutic cases might upgrade histogenesis classification as an important prognostic factor in the treatment of CAS. 

PubMed
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Lymphangiosarcoma of the arm presenting with lymphedema in a woman 16 years after mastectomy: a case report.

Sept 2009

Source

Department of Surgery (Orthopedics), Aga Khan University Medical College P.O. Box 3500, Karachi-74800 Pakistan. jamalyasir@gmail.com

Abstract


Lymphangiosarcoma following breast cancer is a relatively rare entity, with around 300 cases so far reported worldwide. Affecting the long term survivors of breast cancer, lymphangiosarcoma (Stewart-Traves Syndrome) has a high mortality rate. Since lympedema following radical mastectomy or axillary clearance and radiotherapy seems to be the main predisposing factor, further research regarding modifications in the surgical technique of axillary nodes dissection as well as the development of new chemotherapeutic agents effective in lymphangiosarcoma are required.

Cases Journal

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Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature. 

Jan. 2012

Krishnamoorthy N, Viswanathan S, Rekhi B, Jambhekar NA.

Source

Department of Pathology, Tata Memorial Hospital, Mumbai, India.

Keywords:

  • chronic lymphedema;
  • cutaneous angiosarcoma;
  • filariasis;
  • lymphangiosarcoma



Abstract:

Cutaneous angiosarcoma or lymphangiosarcoma represents an uncommon aggressive tumor known to arise on a background of chronic lymphedema secondary to various etiologies, principally following surgery or irradiation. There have been rarely reported cases of angiosarcoma following infective conditions that eventuate with lymphatic stasis. We report a case of angiosarcoma arising after 33 years within a background of filariasis. Awareness of this association can lead to early diagnosis and appropriate treatment of this potentially fatal malignant tumor. Krishnamoorthy N, Viswanathan S, Rekhi B, Jambhekar NA. report on a case of Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature.

http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0560.2011.01785.x/abstract

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External Links

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Elevated Coagulation Factor VIII Plasma Activity in a Patient with Lymphangiosarcoma

2012


KeyWords: cervucak cabcerm chronic lymphedema, lymphangiosarcoma, factor VIII-related antigen

JStage

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Use of surgery and mitoxantrone chemotherapy in a dog with disseminated lymphangiosarcoma

Dec 2012 


Journal of the American Veterinary Medical Association

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Lymphangiosarcoma of the thorax and thoracic vertebrae in a 16-year-old girl. 

Oct 2012 


Journal of Clinical Oncology

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Plasma-cell rich lymphangiosarcoma: an under-recognized variant and potential diagnostic pitfall. 

Jun 2012 


Wiley Online

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Lymphangiosarcoma of the vocal cord: a rare entity defined by a D2-40 immunohistochemical and ultrastructural study. 

Jan 2011 

Journal of Clinical Oncology 

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FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations.  Nov 2011

Wiley OnLine

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Angiosarcoma (Stewart-Treves syndrome): palliative role of Mohs' ointment. Sept 2010 

Wiley Online

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Lymphangiosarcoma complicating extensive congenital mixed vascular malformations. Sept 2010

Mary Ann Liebert

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Can lymphangiosarcoma be resurrected? A clinicopathological and immunohistochemical study of lymphatic differentiation in 49 angiosarcomas. Feb 2010 

Wiley Online

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Fatal lymphangiosarcoma revealed by lymphoedema of the neck. 2009

ACTA Dermato Venereikiguca

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Excerpt from Stewart-Treves Syndrome

http://www.emedicine.com/derm/byname/stewart-treves-syndrome.htm

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Angiosarcoma in a Chronically Lymphedematous Leg: An Unusual Presentation of Stewart-Treves Syndrome

Posted
09/09/2003

Andrzej L. Komorowski, MD, Wojciech M. Wysocki, MD, Jerzy Mitus, MD, PHD

http://www.medscape.com/viewarticle/460522_1

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Lymphangiosarcoma of the pubic region: a rare complication arising in congenital non-hereditary lymphedema.

Cerri A, Gianni C, Corbellino M, Pizzuto M, Moneghini L, Crosti C.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?holding=npg&cmd=Retrieve&db=PubMed&list_uids=9854166&dopt=Abstract

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Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients.

http://www.jhandsurg.org/article/S0363-5023(00)65102-X/abstract

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Lymphangiosarcoma of Stewart-Treves (2)

European Journal of Dermatology

http://www.john-libbey-eurotext.fr/en/revues/medecine/ejd/e-docs/00/01/87/84/article.md?type=text.html

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Diagnostic Images

Google Images

http://images.google.com/images?hl=en&q=lymphangiosarcoma&gbv=2

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Lymphedema People Cancer Information Pages

Cervical, ovarian Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=152

Kidney and Renal Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=150

Hodgkins Disease or Hodgkins Lymphoma

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=149

Gynecological Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=977

Leg Lemphedema After Gynecological Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=979

Kaposi’s Sarcoma

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=641

Skin Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=278

Testicular Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=153

Primary Lymphedema and Cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=277

Cutaneous T-cell Lymphoma

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=147

Cutaneous B-cell Lymphoma

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=146

My Life with Lymphedema and Lymphoma

http://www.lymphedemapeople.com/wiki/doku.php?id=my_life_with_lymphedema_and_lymphoma

Lymphedema Affects Quality of Life

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=980

Angiosarcoma and Long Term Lymphedema

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=564

Colon Cancer

http://www.lymphedemapeople.com/thesite/lymphedema_and_colon_cancer.htm

Prostate Cancer

http://www.lymphedemapeople.com/thesite/lymphedema_and_prostate_cancer.htm

Melanoma

http://www.lymphedemapeople.com/thesite/lymphedema_and_melanoma.htm

Male Breast Cancer

http://www.lymphedemapeople.com/thesite/lymphedema_and_male_breast_cancer.htm

Leg Swelling

http://www.lymphedemapeople.com/thesite/leg_swelling.htm

Arm Swelling

http://www.lymphedemapeople.com/thesite/arm_swelling.htm

Swelling

http://www.lymphedemapeople.com/thesite/swelling.htm

Breast Cancer

http://www.lymphedemapeople.com/thesite/lymphedema_and_breast_cancer.htm

Lymphedema After Cancer - How Serious Is It?

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_after_cancer_-_how_serious_is_it

Secondary Lymphedema in the Cancer Patient

http://www.lymphedemapeople.com/wiki/doku.php?id=secondary_lymphedema_in_the_cancer_patient

Complications of Breast Cancer Radiotherapy

http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_breast_cancer_radiotherapy

Complete decongestive therapy lymphedema in breast cancer

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=792

Patient self-massage for breast cancer-related lymphedema

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=332

Predictive Factors of Response to Intensive Decongestive Physiotherapy in Upper Limb Lymphedema After Breast Cancer Treatment: a Cohort Study

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=283

Lymphedema Therapy and the Quality of Life for Breast Cancer Patients

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_therapy_and_the_quality_of_life_for_breast_cancer_patients

Cancer Associated with Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=cancer_associated_with_lymphedema

Pseudolymphomatous Cutaneous Angiosarcoma: A Rare Variant of Cutaneous Angiosarcoma Readily Mistaken for Cutaneous Lymphoma.

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=1178

Lymphomatoid Papulosis

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=265

Papillomatosis cutis carcinoides

Related Terms: Verrucous Carcinoma, Squamous Cell Carcinoma, Epithelioma cuniculatum, Carcinoma cuniculatum

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=108

Cutaneous lymphomas assoc with lymphoproliferative disorders

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=992

Aqua Lymphatic Therapy for Postsurgical Breast Cancer Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=aqua_therapy_for_postsurgical_breast_cancer_arm_lymphedema

Sporadic Cutaneous Angiosarcomas

http://www.lymphedemapeople.com/phpBB2/viewtopic.php?t=1265

Axillary node biopsy

http://www.lymphedemapeople.com/wiki/doku.php?id=axillary_node_biopsy

Sentinel Node Biopsy

http://www.lymphedemapeople.com/wiki/doku.php?id=sentinel_node_biopsy

Small Needle Biopsy - Fine Needle Aspiration

http://www.lymphedemapeople.com/wiki/doku.php?id=small_needle_biopsy

Extraperitoneal para-aortic lymph node dissection (EPLND)

also includes (1) Retroperitoneal Lymph Node Dissection and (2) Laparoscopic Retroperitoneal Lymph Node Dissection

http://www.lymphedemapeople.com/wiki/doku.php?id=extraperitoneal_para-aortic_lymph_node_dissection_eplnd

Lymphoscintigraphy

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy

Magnetic Resonance Imaging

http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging

Cancer Glossary

http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=36

Skin Glossary

http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=42

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Lymphedema People Online Support Groups

MEN WITH LYMPHEDEMA

If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.

http://health.groups.yahoo.com/group/menwithlymphedema/

Subscribe: menwithlymphedema-subscribe@yahoogroups.com

Pat O'Connor

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Join us as we work for lymphedema patients everywehere:

Advocates for Lymphedema

Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.

http://health.groups.yahoo.com/group/AdvocatesforLymphedema/

Subscribe: AdvocatesforLymphedema-subscribe@yahoogroups.com

Pat O'Connor

Lymphedema People / Advocates for Lymphedema

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For information about Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema\

For Information about Lymphedema Complications

http://www.lymphedemapeople.com/wiki/doku.php?id=complications_of_lymphedema

For Lymphedema Personal Stories

http://www.lymphedemapeople.com/phpBB2/viewforum.php?f=3

For information about How to Treat a Lymphedema Wound

http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound

For information about Lymphedema Treatment 

http://www.lymphedemapeople.com/wiki/doku.php?id=treatment

For information about Exercises for Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=exercises_for_lymphedema

For information on Infections Associated with Lymphedema

http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema

For information on Lymphedema in Children

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children

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Lymphedema Glossary

http://www.lymphedemapeople.com/wiki/doku.php?id=glossary:listing

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Lymphedema People

Online Support Groups

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Join us as we work for lymphedema patients everywehere:

Advocates for Lymphedema

Dedicated to be an advocacy group for lymphedema patients. Working towards education, legal reform, changing insurance practices, promoting research, reaching for a cure.

http://health.groups.yahoo.com/group/AdvocatesforLymphedema/

Subscribe: AdvocatesforLymphedema-subscribe@yahoogroups.com

Pat O'Connor

Lymphedema People / Advocates for Lymphedema

================================================

Children with Lymphedema

The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.

http://health.groups.yahoo.com/group/childrenwithlymphedema/

Subscribe: childrenwithlymphedema-subscribe@yahoogroups.com

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Lipedema Lipodema Lipoedema

No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.

Come join, be a part of the family!

http://health.groups.yahoo.com/group/lipedema_lipodema_lipoedema/?yguid=209645515

Subscribe: lipedema_lipodema_lipoedema-subscribe@yahoogroups.com

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MEN WITH LYMPHEDEMA

If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.

http://health.groups.yahoo.com/group/menwithlymphedema/

Subscribe: menwithlymphedema-subscribe@yahoogroups.com

......................

All About Lymphangiectasia

Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.

http://health.groups.yahoo.com/group/allaboutlymphangiectasia/

Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com

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Lymphatic Disorders Support Group @ Yahoo Groups

While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most 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
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.

http://health.groups.yahoo.com/group/lymphaticdisorders/

Subscribe: lymphaticdisorders-subscribe@yahoogroups.com

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lPage Updated Jan. 5, 2012