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At least 19 recordsLinked to original sources

Postmastectomy lymphangiosarcoma: experience with three patients and electron microscopic observations in one.

The clinical features are presented of three patients in whom lymphangiosarcoma developed after radical mastectomy followed by local irradiation. The median time between radical mastectomy and the diagnosis by biopsy of lymphangiosarcoma was 9.9 years. No form of therapy, including high-dose combination chemotherapy, appeared able to control the disease. All three patients died with residual lymphangiosarcoma and in two it was the direct cause of death. These results stress that prevention of postmastectomy lymphangiosarcoma is vital. This can be accomplished by avoiding radical mastectomy followed by local irradiation. Ultrastructural observations in one case suggest that the tumour has a primitive vasoformative origin.

Adult

Lymphangiosarcoma arising in congenital lymphedema.

An 85-year-old woman with congenital lymphedema of the right upper extremity developed a small purplish papule on the forearm, which was the first clinical evidence of lymphangiosarcoma. The lesion grew rapidly and became necrotic and ulcerated. The patient experienced severe pain. A mid-arm amputation was performed. Microscopical examination of the amputated limb showed widespread histological involvement of skin, muscle, and subcutaneous tissue by lymphangiosarcoma. The need for continuous monitoring of patients with both primary and secondary lymphedema is emphasized, since early diagnosis of the developing lymphangiosarcoma and rapid surgical intervention provide the best prognosis for survival.

Aged

Postmastectomy lymphangiosarcoma: a reappraisal of the concept--a critical review and report of an illustrative case.

The syndrome of postmastectomy lymphangiosarcoma (LAS) has been universally accepted since it was first outlined by Stewart & Treves (1948), except for a small number of authors who concluded that the neoplasms arising in the chronic lymphoedematous arms were in fact due to retrograde spread from the original breast carcinoma (Laffargue, Pinet & le Go 1960, Giannardi, Pelù & Zampi 1960, Giannardi & Pelù 1961, Delarue 1962, Salm 1963, Laugier, Olmos, Hunziker & Orusco 1973), but their views have been largely ignored. A case is reported in whom neoplastic arm lesions appeared 27 years after mastectomy and were due, in our opinion, to recent metastases from a new primary Carcinoma of the lung. The validity of the entire concept of LAS is re-examined.

Adenocarcinoma

Cutaneous lymphangiosarcoma of Stewart-Treves.

Lymphangiosarcoma of an edematous extremity is a highly malignant cutaneous neoplasm seen most often ten years after a successful radical mastectomy. The sarcoma presents with an ecchymosis and/or purplish cutaneous nodules on the edematous arm; it spreads through the bloodstream and is fatal in most cases. A case is presented which shows some typical and some unusual features of this disease.

Aged

Lymphangiosarcoma arising from lymphangioma circumscriptum.

A lymphangiosarcoma arose at the site of a preexisting lymphangioma circumscriptum on the skin of the anterior part of the abdominal wall. To our knowledge, this is only the second such case to be reported, and in both patients, the preexisting lymphangioma circumscriptum had been exposed to substantial x-ray therapy. Since it is possible that x-irradiation may play a role in the development of this unusual malignant neoplasm, it seems advisable that lymphangioma circumscriptum not be exposed to substantial amounts of such radiation, if feasible.

Adult

Lymphangiosarcoma arising in a congenitally lymphoedematous arm: case report.

A malignant endothelial tumour arising in a congenitally lymphoedematous extremity is a sufficiently rare tumour to deserve the reporting of a new case. This tumour presented as small nodules on the back of the hand and in spite of midhumeral amputation widely scattered pulmonary metastases were present at the autopsy 28 months after the original presentation.

Arm

Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency.

Lymphangiosarcoma is a fatal complication of postmastectomy lymphedema. The pathogenesis of lymphangiosarcoma in chronic lymphedema is a combination of two factors. First, the edematous region responds in a manner similar to "immunologically privileged sites." Second, because of its anatomic and physiologic properties, it is a favorable site for the development of mutant cell populations for reasons that are not fully understood. As a result, these mutant cells, with their genetically nonidentical antigens, escape recognition by the host's impaired immune surveillance mechanism. The failure to promote a sufficient immune response allows unrestricted tumor growth to take place, resulting in the ultimate death of the patient. Available therapeutic measures are equally unsatisfactory. Emphasis is placed on periodic examination of the lymphedematous extremity, aggressive treatment of established lymphedema and infections, and surgical preservation of lymphatic channels during breast cancer surgery.

Aged

[Classification of vascular neoplasms].

Neoplasms of blood and lymph vessels differ from angiectatic and angiokeratotic nevi by real proliferating growth. According to their features of growth and their wall structures, they are classified into three main groups: angiomas, glomangiomas and malignant vascular tumors. Within the angiomas on the one hand, capillary angiomas are classified into: planotuberous and tuberonodous angiomas of childhood and Kasabach-Merritt syndrome, multilocular hemangiomatosis, progressive multiple angiomas, tardive ("senile") angiomas, eruptive angiomas (granulomata pediculata), papular angioplasia, gemmangioma, and benign juvenile hemangioendothelioma. On the other hand, cavernous angiomas, i.e. arterial and venous cavernomas, as well as blue rubberbleb nevus, Mafucci's syndrome, angioleiomyoma, benign juvenile hemangiopericytoma and cavernous lymphangioma, form thick walled structures without involution. Glomangiomas occur as solitary, multiple systematized, and multiple disseminated and familiar forms. Within the group of malignant vascular tumors--Kaposi sarcoma, lymphangiosarcoma in lymphedema, hemangioendothelioma and angioplastic reticulosarcoma, hemangio- or lymphangiosarcoma, angioendotheliomastosis proliferans, rarity and increasing loss of characteristic differentiated structures give rise to difficulties in nosologic classification.

Diagnosis, Differential

[Malignant tumors of lymphatic vessels in children].

Lymphangiosarcomas were found in 5 out of 82 observations of vascular malignant tumours in children, according to the data of the pathological department of Children's Clinical Hospital No. 1, for 60 years. Differential diagnosis of relapsing lymphangiomas and rarely found malignant lymphangioendetheliomas presents considerable difficulties for morphologists because of poorly manifest cell polymorphism in the primary node of neoplasia and the presence of areas of lymphangioma and malignant hemangioendothelioma among fields of lymphangiosarcoma. Cytological studies assist in specification of the degree of malignancy and histologenesis of the neoplasias.

Child, Preschool

Lymphangioma circumscriptum following radical mastectomy and radiation therapy.

Lymphangioma circumscriptum, a rare long-term complication of chronic lymphedema, has been described only twice following radical mastectomy. The case discussed in this report developed 25 years after radical mastectomy and radiation therapy. Both this condition and lymphangiosarcoma, its malignant counterpart, may arise in lymphedematous extremities regardless of the etiology of the lymphatic obstruction.

Aged

Cutaneous lymphangioma.

Cutaneous lymphangiomas from 158 patients were studied clinically and pathologically. Lymphangiomas show a predilection for the neck and axilla, breasts and chest, and buttocks and thighs, but may occur on almost any area of skin. They show highest incidence of onset in infancy, the majority are present by age 5 years, but they may appear spontaneously in adolescence or adult life. No correlation among cutaneous lymphangioma, mucous membrane lymphangioma, internal lymphangioma, or lymphangiosarcoma was found. No familial histories of lymphangioma were elicited. Single surgical excision cured 75% of cutaneous lymphangiomas and reexcision cured an additional 12%. The cutaneous lymphangiomas are divided into superficial "lymphangioma circumscriptum" and deep "lymphangioma cavernosum." No specific histologic criteria could be found to differentiate lymphangioma from "bloodless" hemangioma, primary from secondary lymphangioma (lymphangiectasia), or cystic cavernous lymphangoma from cystic hygroma.

Adolescent