Lymphangiosarcoma

A malignant tumor originating from the endothelial cells of lymphatic vessels. Most lymphangiosarcomas arise in an arm secondary to radical mastectomy but they sometimes complicate idiopathic lymphedema. The lymphedema has usually been present for 6 to 10 years before malignant changes develop. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p1866)

Also Known As:

Networked: 95 relevant articles (2 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Lymphedema (Milroy Disease)
2.	Breast Neoplasms (Breast Cancer)
3.	Neoplasms (Cancer)
4.	Carcinoma (Carcinomatosis)
5.	Neoplasm Metastasis (Metastasis)

Experts

1.	Gerding, Joseph C: 1 article (11/2015)
2.	Montgomery, Stephanie A: 1 article (11/2015)
3.	Gilger, Brian C: 1 article (11/2015)
4.	Clode, Alison B: 1 article (11/2015)
5.	Moraes, Elisa Trino de: 1 article (05/2015)
6.	Santos, Marcel Alex Soares dos: 1 article (05/2015)
7.	Pereira, Elisangela Samartin Pegas: 1 article (05/2015)
8.	Siqueira, Daniela Melo: 1 article (05/2015)
9.	Mathur, Sandeep R: 1 article (12/2014)
10.	Singh, Lavleen: 1 article (12/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Lymphangiosarcoma:

1.	Stewart Treves syndromeIBA 05/01/2015 - "Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. " 08/01/2001 - "Stewart-Treves syndrome has been defined by the eponymous authors as a lymphangiosarcoma in a setting of postmastectomy upper extremity lymphoedema. " 11/01/2000 - "Stewart-Treves syndrome (STS) is a rare but aggressive upper extremity lymphangiosarcoma in postmastectomy patients. " 11/01/2000 - "Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients." 01/01/1993 - "[Lymphangiosarcoma in postmastectomy arm lymphostasis of the arm (Stewart-Treves syndrome)]."
2.	Congenital hereditary lymphedemaIBA 10/01/1998 - "We report a case of lymphangiosarcoma of the pubic region, supported by immunohistochemical studies, in a 42-year-old woman affected by congenital, non-hereditary lymphedema of the left genital region and homolateral lower limb. " 10/01/1998 - "The literature concerning the cases of lymphangiosarcoma arising in congenital hereditary and non-hereditary lymphedema is reviewed. " 10/01/1998 - "To our knowledge, this is the first case of lymphangiosarcoma associated with congenital non-hereditary lymphedema confined to the pubic region. " 10/01/1998 - "Lymphangiosarcoma of the pubic region: a rare complication arising in congenital non-hereditary lymphedema." 03/13/1995 - "Lymphangiosarcoma in late-onset hereditary lymphedema: case report and nosological implications."
3.	CosmeticsIBA 07/01/2013 - "Patients with lymphedema sustain either cosmetic or functional problems, and several studies have indicated the potential risk, though not high, transforming lymphedema to lymphangiosarcoma. "
4.	Melphalan (Alkeran)FDA LinkGeneric 12/01/2002 - "In 10 patients with multifocal Stewart-Treves lymphangiosarcoma of the extremities, 16 ILPs with TNF plus melphalan were performed. " 12/01/2002 - "Multifocal Stewart-Treves lymphangiosarcomas in extremities with chronic lymphedema can be successfully treated by ILP with TNF and melphalan." 12/01/2002 - "Isolated limb perfusion with tumor necrosis factor and melphalan for nonresectable sSewart-Treves lymphangiosarcoma." 08/01/1989 - "The complete remissions observed in three patients with aggressive fibromatosis and in one with lymphangiosarcoma occurred after perfusion with doxorubicin combined with melphalan. "
5.	Factor VIII (Coagulation Factor VIII)IBA 01/01/2012 - "To the best of our knowledge, this is the first report of high coagulation factor VIII plasma activity in a patient with lymphangiosarcoma." 01/01/2012 - "Elevated coagulation factor VIII plasma activity in a patient with lymphangiosarcoma." 06/01/1984 - "Histogenetic classification of the tumor as hemangiosarcoma rather than lymphangiosarcoma was favored by positive immunohistochemical staining for Factor VIII. Despite high amputation and isolated perfusion with hyperthermal cytostatic infusion, she developed local recurrence and distant metastases and died 16 months after operation. "
6.	Doxorubicin (Adriamycin)FDA LinkGeneric 10/01/2007 - "Liposomal doxorubicin could be considered for the treatment of lymphangiosarcoma, particularly in elderly patients." 10/01/2007 - "We report a case of lymphangiosarcoma in an elderly woman with good response to liposomal doxorubicin, an anthracycline with lower toxicity. " 10/01/2007 - "[Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)]." 08/01/1989 - "The complete remissions observed in three patients with aggressive fibromatosis and in one with lymphangiosarcoma occurred after perfusion with doxorubicin combined with melphalan. "
7.	Desmoplakins (Desmoplakin)IBA 11/01/2005 - "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. "
8.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 11/01/2015 - "Immunohistochemically, the neoplastic cells labeled strongly positive for PROX-1, vimentin, CD-31, VEGF, weakly positive for factor VIII-related antigen, and negative for collagen IV. Based on the clinical, histological, and immunohistochemical features of this tumor, a primary ocular lymphangiosarcoma with metastasis was diagnosed. "
9.	Vascular Endothelial Growth Factor Receptor-3 (Flt 4)IBA 02/01/2010 - "Ten tumours had features suggestive of lymphatic differentiation, namely well-differentiated histology, interanastomosing channels devoid of red cells, prominent hobnailing, lymphoid aggregates, and multi-antigen expression of D2-40 (100%), Prox-1 (100%) and VEGFR-3 (60%), which might be deserving of the appellation lymphangiosarcoma. "
10.	von Willebrand FactorIBA 11/01/2015 - "Immunohistochemically, the neoplastic cells labeled strongly positive for PROX-1, vimentin, CD-31, VEGF, weakly positive for factor VIII-related antigen, and negative for collagen IV. Based on the clinical, histological, and immunohistochemical features of this tumor, a primary ocular lymphangiosarcoma with metastasis was diagnosed. "

Therapies and Procedures

1.	Radical Mastectomy 02/01/2000 - "In 1948, Stewart and Treves first described six cases of lymphangiosarcoma after radical mastectomy. " 11/01/1979 - "The median time between radical mastectomy and the diagnosis by biopsy of lymphangiosarcoma was 9.9 years. " 11/01/1979 - "The clinical features are presented of three patients in whom lymphangiosarcoma developed after radical mastectomy followed by local irradiation. " 02/01/1965 - "LYMPHANGIOSARCOMA ARISING IN A CHRONICALLY EDEMATOUS ARM FOLLOWING RADICAL MASTECTOMY." 01/01/1963 - "Lymphangiosarcoma following radical mastectomy."
2.	Radiotherapy 03/01/1990 - "We believe that only in early cases can the radiotherapy give a good palliation in postmastectomy lymphangiosarcoma." 03/01/1990 - "After radiotherapy she lived four years from first lesion of lymphangiosarcoma. " 03/01/1990 - "Response of postmastectomy lymphangiosarcoma to radiotherapy: report of four cases." 01/01/2009 - "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." 12/01/2014 - "Lymphangiosarcoma of mons pubis following radiotherapy for carcinoma cervix."
3.	Drug Therapy (Chemotherapy) 12/15/2012 - "Clinical management that included medical and surgical treatments and chemotherapy resulted in improved quality of life and extended survival time in a dog with metastatic lymphangiosarcoma." 02/01/2004 - "Lymphangiosarcoma in a dog treated with surgery and chemotherapy." 11/01/2000 - "The extensive lymphangiosarcoma in the other 2 patients precluded surgical resection and they underwent chemotherapy. " 03/01/1981 - "Chemotherapy for postmastectomy lymphangiosarcoma." 04/01/2000 - "Facing the poor track record of both irradiation and chemotherapy in this highly malignant lymphangiosarcoma, amputation was recommended but refused by the patient. "
4.	Mastectomy (Mammectomy) 05/01/1972 - "Lymphangiographic studies in a case of post-mastectomy lymphangiosarcoma." 02/01/1999 - "Treatment of post-mastectomy lymphangiosarcoma is still unsatisfactory. " 02/01/1999 - "A case of post-mastectomy lymphangiosarcoma is reported. " 05/01/1993 - "Lymphangiosarcoma after mastectomy was first described by Stewart and Treves in 1948. " 11/01/1988 - "[A case of post-mastectomy lymphangiosarcoma]."
5.	Amputation 10/01/1981 - "At present, early amputation appears to be the treatment of choice for patients with lymphangiosarcoma." 04/01/2000 - "Facing the poor track record of both irradiation and chemotherapy in this highly malignant lymphangiosarcoma, amputation was recommended but refused by the patient. " 06/01/1985 - "[Lymphangiosarcoma of the lyphedematous arm following breast amputation (Stewart-Treves syndrome)]." 07/01/1960 - "[Lymphangiosarcomas developing on brawny arms after amputation of the breast for cancer (Stewart-Treves syndrome)]." 06/01/1984 - "Histogenetic classification of the tumor as hemangiosarcoma rather than lymphangiosarcoma was favored by positive immunohistochemical staining for Factor VIII. Despite high amputation and isolated perfusion with hyperthermal cytostatic infusion, she developed local recurrence and distant metastases and died 16 months after operation. "