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[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

Long-term development of a primary lung sarcoma, probably lymphangiosarcoma--a case report.

A small primary lung lesion had remained unchanged in size over a period of 8 years. Within the ensuing 2 years rapid tumor growth occurred. On operation the surgical specimen revealed a tumor mass of 87.2 cm3. Within 6 months after resection, the tumor occurred on the same side. Extensive tumor infiltration of the chest wall prevented any further surgery. The histo-morphologic picture of both lesions was consistent with a lymphangiosarcoma without evidence of high grade malignancy. The patient died of cardio-respiratory insufficiency due to extension of the tumor mass. Unfortunately no autopsy was performed. Tumor growth rates, macroscopic reconstruction and electron microscopic evaluations are presented. This case report again points out that size and long-time stability of solitary lung nodules are not necessarily reliable criteria for assessing prognosis.

Adult

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

Electron microscopic observations of lymphangiosarcoma arising from chronic lymphedema.

Lymphangiosarcoma, so-called Stewart-Treves syndrome, originating from the edematous left leg after hysterectomy in a 53-year-old woman has been studied by light and electron microscopy. Histologically the tumor was composed of either vascular channels or solid nests of the neoplastic cells having a large hyperchromatic nucleus with a single or multiple prominent nucleoli. The inner surface of the vascular channels was lined by either single or several layers of identical cells. Erythrocytes were occasionally observed in the lumina of the vascular channels. By electron microscopy the maturation of the neoplastic cells differed considerably from one area to another even in the same section. The immature cells were clustered in groups and consisted of scanty cytoplasm and a large nucleus with prominent nucleoli, whereas some of the mature cells lining the lumina had rod-shaped tubulated bodies (Weibel-Palade bodies). Desmosomes were frequently observed between the neoplastic cells. Basal lamina was generally not continuous or not present although some vascular structures were completely surrounded by a basal lamina. An autopsy was performed but revealed no metastatic lesions in any organs or lymph nodes.

Chronic Disease

Lymphangiosarcoma in a chronic lymphedematous limb: a case report.

A 69-year-old-woman developed a lymphangiosarcoma of the limb on chronic lymphedema after radical mastectomy and radiotherapy for primary breast cancer 12 years before. Since 1948, when Stewart and Treves described this entity, 200 cases have been described. We report this case because of its extremely rare frequency. Concepts of etiology, treatment and prognosis are reviewed.

Arm

Lymphangiosarcoma in a dog.

Lymphangiosarcoma was seen in the subcutis of right chest in a 11-year-old female Poodle. No metastasis was observed clinically. Tumor cells were vimentin positive and formed irregular space or slit without erythrocytes in the tumor tissue. Lymphocytic foci and edema were seen in the stroma. Only a few tumor cells had factor VIII-related antigen. Electronmicroscopically, tumor cells did not accompany with basement membrane and intercellular junctional complex.

Animals

Chylothorax and chylous ascites in a dog with mediastinal lymphangiosarcoma.

A 10-year-old, male toy poodle presented for evaluation of a progressively worsening, harsh, nonproductive cough. Chylous pleural effusion, lymphopenia, thrombocytopenia, normoblastemia, and recurrent subcutaneous bruising were diagnosed. Surgical exploration of the cranial mediastinum revealed extensive, redundant connective tissue which was confirmed by histopathology to be mediastinal lymphangiosarcoma (LAS). During surgical placement of a fenestrated silastic mesh for passive pleuroperitoneal drainage, chylous ascites also was diagnosed. The patient was euthanized two days postoperatively due to persistent chylothorax. The etiopathogenesis of chylothorax and chylous ascites are discussed. Previous case reports of lymphatic endothelial neoplasia are reviewed.

Animals

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 in chronic lymphedema. Reports of 3 cases and review of the literature.

Lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphedema. The classic clinical picture is described and three cases are presented. Current concepts of etiology, histopathology, treatment and prognosis are discussed. Since early diagnosis may be the key to longer survival, all patients with chronic lymphedema should undergo careful examination at regular intervals throughout their lives.

Aged

Lymphangiosarcoma in chronic hereditary oedema (Milroy's disease).

Lymphangiosarcoma arising in chronic lymphoedema is extremely rare. In a reference population of about four million people, during a thirty year period (1957-1987), only four patients were treated for such a tumour. The neoplasm is almost exclusively seen in elderly patients after mastectomy but in two of our patients, reported in this paper, it arose in chronic hereditary oedema (Milroy's disease). In both these patients there was a considerable treatment delay because of wrong diagnosis. The tumour extent was difficult to assess macroscopically and ablative surgery had to be a disarticulation of the involved extremity. Macular or papular purple lesions in a lymphoedematous extremity should be a manifestation of this aggressive neoplasm.

Adult

[Lymphangiosarcoma of the leg following radiotherapy for cervical carcinoma].

A case of lymphangiosarcoma of lower extremity was observed in a female aged 68 years who had had radiotherapy for cervical carcinoma. The late therapeutic result was bad, and this indicates the necessity of radical management, that is amputation in hip joint already in an early stage of the disease, although this decision is extremely difficult for the physician.

Aged