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[Stewart-Treves syndrome and lymphangiosarcoma].

Lymphangiosarcomas represent the most dangerous complication of chronic lymphedema usually following mastectomy and irradiation therapy on account of breast carcinoma, well-known as Stewart-Treves syndrome. Still more rarely documented are lymphangiosarcomas arising in chronic lymphedema due to other causes. Two cases of Stewart-Treves syndrome on the forearm and one case of lymphangiosarcoma of the hand following a fifty-year existing chronic lymphedema after burn injury in childhood are reported. Clinical description and illustrations of all three cases are given, including list of references. Therapeutic procedures are described.

Aged↗

Lymphangiosarcoma of the anterior abdominal wall: a case report.

A rare case of lymphangiosarcoma of the anterior abdominal wall arising in chronic lymphedema following radical vulvectomy with bilateral inguinal lymphadenectomy and postoperative radiotherapy is reported. This is apparently the first description of this association, and the second reported case of lymphangiosarcoma originating in the anterior abdominal wall.

Abdominal Muscles↗

Lymphangiosarcoma associated with chronic filarial lymphedema.

A case of a 19-year-old inhabitant of Southern India with chronic filarial lymphedema of the right leg and subsequent lymphangiosarcoma is reported. Two additional cases of lymphangiosarcoma occurring in patients with filarial lymphedema are reviewed from the literature.

Adult↗

Lymphangiosarcoma in two cats.

Cutaneous tumours consisting of irregular empty anastomosing spaces lined by spindle cells were diagnosed as lymphangiosarcoma in two cats. The tumour cells exhibited the characteristic lining up along pre-existing collagen and muscle fibres. Because of the small number of cases of lymphangiosarcoma in cats, conclusions regarding biological behaviour or breed incidence are not made.

Animals↗

Lymphangiosarcoma of the edematous thigh after radiation therapy for carcinoma of the vulva.

A 66-year-old patient was treated with external radiation therapy for an advanced carcinoma of the vulva. Seven years later, a lymphangiosarcoma developed in her edematous lower extremity. Lymphangiosarcomas have been reported to occur in postmastectomy patients; however, this is only the third case in a patient with a gynecologic primary malignancy. In anticipation of possible increased use of radiation therapy in vulvar carcinoma, gynecologists should be aware of this rare, highly aggressive neoplasm.

Aged↗

Lymphangiosarcoma of the hand arising in a pre-existing non-irradiated lymphangioma.

Lymphangioma is an uncommon lesion which is widely believed not to undergo malignant change. Lymphangiosarcoma is even rarer and most of the cases reported in the literature were encountered in the setting of chronic lymphoedema, although occasional cases have been reported in irradiated lymphangiomata. This brief communication describes a case of multifocal lymphangiosarcoma arising in a non-irradiated, long standing lymphangioma of the hand. To our knowledge no similar case has been reported previously in the English literature, which is briefly reviewed.

Adipose Tissue↗

Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients.

Stewart-Treves syndrome (STS) is a rare but aggressive upper extremity lymphangiosarcoma in postmastectomy patients. Unfamiliarity with this disease and the innocuous appearance of the tumor often lead to delayed diagnosis. A comprehensive search of the databases at a single tertiary-care academic institution revealed only 3 cases of STS in the last 63 years. The latency time between breast cancer treatment and diagnosis of STS was 11 to 21 years. Survival after diagnosis of STS ranged from 8 to 15 months. One patient underwent radical surgery. The extensive lymphangiosarcoma in the other 2 patients precluded surgical resection and they underwent chemotherapy. All patients had adjuvant radiation therapy at the time of the original breast cancer resection. This report includes a discussion of the epidemiology, etiology, presentation, treatment, and prognosis of STS.

Aged↗

Lymphangiosarcoma associated with lymphedema in a man with Maffucci's syndrome.

A man with Maffucci's syndrome and lifelong lymphedema in the right lower extremity had a lymphangiosarcoma in the same extremity. Despite amputation of the limb and radiotherapy, he died several months later of pulmonary metastases. Malignancies of vascular origin rarely have been reported in this syndrome. Our patient's features suggest that lymphedema may predispose patients with Maffucci's syndrome to the development of lymphangiosarcoma.

Adult↗

Lymphangiosarcoma in a young dog.

Lymphangiosarcoma was diagnosed from biopsy material obtained from an 8-week-old puppy with a progressively enlarging subcutaneous inguinal swelling. Histologically, the tumor was composed of endothelial cells immediately adjacent to large collagen bundles. Tumor cells formed irregular vascular channels which extended along the connective tissue investments of small vessels and nerves of the subcutis and deep dermis. Similar neoplastic tissue extensively infiltrated an inguinal lymph node. Neoplastic cells were immunohistochemically stained for factor 8-related antigen and were weakly positive when compared with several hemangiomas and hemangiosarcomas. Transmission electron microscopy revealed numerous micropinocytotic vesicles and a continuous basal lamina. The puppy was euthanatized at 8 months of age due to severe septic polyarthritis. Lymphangiosarcoma was documented at the site of the original tumor as well as in the axillary lymph node at necropsy.

Animals↗

Lymphangiosarcoma in a dog presenting with massive head and neck swelling.

A three-year-old, neutered male Chesapeake Bay retriever was presented for acute onset of severe, progressive swelling of the head, neck, and cranial trunk. Diffuse lymphangiosarcoma involving the superficial and deep dermis and subcutaneous tissue was observed on skin biopsies. Lymphangiosarcoma is a rarely reported tumor of the lymphatic system in dogs and cats. The importance of obtaining skin biopsies in animals with acute edema of unknown etiology is emphasized. Additionally, neoplasia should be considered as a potential diagnosis in a dog with an acute onset of edema.

Animals↗

Ocular lymphangiosarcoma in a cow.

Ocular lymphangiosarcoma was diagnosed in a Holstein cow with a progressively enlarging limbal mass. The cow was treated by exenteration. The cow survived for 2 years without clinical signs of recurrence. Lymphangiosarcoma is a vascular neoplasm that rarely has been reported in domestic animals. In other species, it has a high rate of metastasis and is associated with poor long-term survival.

Animals↗

[Lymphangiosarcoma with postmastectomy edema of the arm].

A case of a lymphangiosarcoma (LAS) in a chronic postmastectomy lymphedematous arm in a 67 y.o; women is reported. The LAS was found 8 years after a radical left mastectomy for cancer and 7 years after the development of a lymphedema in the left arm. Because of a rapid spread of the disease no surgical treatment was done. The patient died 14 months after the diagnosis. Lymphangiosarcoma is a rare neoplasia that usually arise in chronic lymphedematous limbs mostly in post-mastectomy lymphedema of the arms. Chronic lymphedema is an important neoplastic stimuli decreasing the local immunity, as well leading to lymphoproliferative and degenerative changes of collagenous and fat tissues. Usually it appears as a multicentric lesion like bluish nodules, sclerotic plaques, bullous lesions. Lungs, pleura and thoracic wall are the most common sites of metastatic disease. The DD should be done with Kaposi sarcoma, hemangioma, hemangiopericytoma. The prognosis is always poor and after treatment the mean survival time is 18 months. The best treatment that gives a temporary result is the ablative surgery. Therefore because of unsuccessful therapeutic procedures a particular care should be paid to avoid postmastectomy lymphedema leaving, when possible, a reasonable lymphatic drainage of the arm.

Aged↗

[A case of post-mastectomy lymphangiosarcoma].

Postmastectomy lymphedema and lymphangiosarcoma. A case of 73-year-old woman with postmastectomy lymphedema of the right arm and subsequent lymphangiosarcoma is reported. The diagnosis has been confirmed immunohistologically. The patient had to undergo an amputation of the arm. Presently this treatment offers the greatest chance for survival.

Adenocarcinoma↗

Chemotherapy for postmastectomy lymphangiosarcoma.

Twenty-two patients with postmastectomy lymphangiosarcoma have been seen at M. D. Anderson Hospital during the past 20 years. Of these, 13 received chemotherapy, either regionally, systemically, or both. Six patients were treated with regional chemotherapy using either methotrexate alone, a combination of melphalan, nitrogen mustard, and actinomycin D, or a combination of melphalan with nitrogen mustard or actinomycin D; 3 achieved a partial or complete response. Eleven patients received 19 trials of systemic chemotherapy; one complete and seven partial responses were observed, giving an overall response rate of 42% (8/19). Responses occurred with 5-fluorouracil, methotrexate, a combination of vincristine, actinomycin D, and cyclophosphamide, and a combination of Adriamycin and dacarbazine with or without vincristine. The median survival time of the six patients who responded to at least one chemotherapeutic trial was 26.5 months compared with four months for the five patients who failed to respond. These data indicate that chemotherapy may play a significant role in the treatment of patients with this rare but distinctive tumor.

Adult↗

The coexistence of lymphangiosarcoma and Kaposi's sarcoma in a renal transplant recipient.

A case of coexisting lymphangiosarcoma and Kaposi's sarcoma that occurred in a female renal transplant recipient is presented. Both sarcomas were localized to the skin and were slowly progressive over several years. The coexistence of these two sarcomas may indicate that they arose from a common precursor endothelial cell and that systemic immune suppression may be important in the pathogenesis of both these malignancies.

Female↗

Lymphangiosarcoma in postmastectomy lymphedema (Stewart-Treves syndrome): ultrastructural and immunohistologic characteristics.

Two cases of lymphangiosarcoma arising in a chronic lymphedematous extremity following mastectomy (Stewart-Treves syndrome) were reported with not only standard histology, but also special study on the ultrastructure and immunohistology. These cancers developed as a result of chronic lymphedema of the involved limb following mastectomy 15 and 16 years ago, respectively, for breast carcinoma. Immunohistologic and electron microscopic examinations proved that this tumor originates in the vascular endotheliocytes, even though they are clinically chronic lesions apparently derived from the lymphatic vessels. This sarcoma develops multicentrically in an edematous arm and spreads out rapidly, so prognosis is quite discouraging in those patients. They need to be radically surgically treated without hesitation; hence, amputation, not limb-saving surgery, should be indicated.

Adenocarcinoma, Scirrhous↗

Lymphangiosarcoma and haemangiosarcoma in a cat.

Ultrastructural findings in a feline ventral abdominal vascular tumour showed lack of basal lamina, few micropinocytotic vesicles and intercellular junctions and a discontinuous endothelial cell layer. A splenic cyst had a continuous basal lamina, numerous micropinocytotic vesicles and intercellular junctions and a continuous endothelial cell layer. These findings were compatible with diagnosis of lymphangiosarcoma (ventral abdomen and metastases) and haemangiosarcoma (splenic cyst).

Animals↗

Lymphangiosarcoma. Reports of 3 cases and review of the literature.

Three cases of lymphangiosarcoma have been followed recently. All were finally treated by amputation. Only one is actually disease-free. The other two developed local recurrence at the thoracic cage. An extensive review of the literature follows the case presentation.

Combined Modality Therapy↗