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PubMed1985

Basement membrane in Kaposi's sarcoma: an immunohistochemical and ultrastructural study.

Basement membranes were investigated in early angiomatous and late sarcomatoid stages of Kaposi's sarcoma (KS). Seven frozen skin biopsies of KS from five elderly Mediterranean people and one renal allograft recipient were labelled, using an immunoperoxidase technique, for basement membrane-specific macromolecules, laminin and type IV-collagen. Twenty-seven other frozen cutaneous lesions including haemangio and lymphangiosarcomas, benign vascular tumours, and various epithelial, melanocytic, fibrohistiocytic, fibrosarcomatous and muscular tumours were processed in the same way. In addition an ultrastructural study was performed in two cases of KS, one haemangiosarcoma and one lymphangiosarcoma. Intense labelling was observed for both type IV-collagen and laminin, which appeared closely co-distributed, in all areas of KS. Staining pattern was often regular and continuous around neoplastic vessels in early lesions of KS, as in benign vascular lesions, whereas in late nodular lesions large amounts of basement membrane components were present in intercellular spaces between densely aggregated spindle cells. In contrast, ultrastructural examination disclosed early disruption of basement membranes around neoplastic vessels, and occasional fragments of external lamina were seen at the interface between KS spindle cells and collagen. Similar results were obtained in angiosarcomas both by immunohistological and ultrastructural study. In the comparative group, laminin and type IV-collagen were present, in significantly fewer quantities and in various distinctive patterns, in epithelial, melanocytic, fibrohistiocytic and muscular tumors. This study deals with basement membrane modifications in early and late lesions of KS and provides further evidence in favour of the endothelial nature of the spindle cells of KS.

Adult↗

PubMed1991

Vascular soft-tissue sarcomas. An analysis of tumor-related mortality.

Between 1982 and 1990, 69 adult patients were admitted to Memorial Sloan-Kettering Cancer Center with a diagnosis of angiosarcoma, lymphangiosarcoma, or malignant hemangiopericytoma. The existing literature regarding sarcomas focuses on individual histologic conditions or site-specific activity and includes pediatric patients, which makes estimates of survival difficult. We describe the clinical course of all vascular sarcomas, the survival without distant recurrence of patients with vascular sarcomas, and the overall survival of patients with vascular sarcomas. Using Cox's stepwise regression model, histologic characteristics of the tumors, tumor grade, tumor size, and other factors were assessed to determine their prognostic significance. Noncurative treatment and the presence of metastases were the only two factors influencing survival. Tumor grade approached significance. Disease recurrence was common among curatively treated patients (37% of such patients), and more than half of these recurrences involved distant sites. The survival of these patients depends on complete surgical resection and is independent of individual histologic characteristics of the tumor.

Adolescent↗

PubMed1982

Surgical management and reconstruction of extensive chest wall malignancies.

Seven patients aged 8 to 77 years underwent massive resection for chest wall malignancies. Two had chondrosarcoma, one recurrent breast cancer, one malignant hemangioepithelioma, one embryonal cell sarcoma, one metastatic osteogenic sarcoma, and one lymphangiosarcoma. The smallest surgical defect was 17 by 19 cm, the largest 35 by 45 cm. Closure was done with Marlex mesh, full-thickness muscle flaps, or free island pectoralis or latissimus dorsi flaps. The rotation of myocutaneous island flaps (bilateral in two patients) greatly facilitated reconstruction. No infection, pulmonary compromise, or operative morbidity or mortality was encountered. The age of the patients and the location or size of the lesions were not significant factors. Designing a surgical strategy which provides adequate full-thickness margins and immediate reconstruction is critically important. Massive chest wall resection for malignancy should be pursued aggressively whenever these lesions are encountered. The operations can be performed safely and can be curative, and the benefits to patients in terms of comfort and prolonged survival justify this extensive surgery.

Adolescent↗

PubMed1991

Long-term risk of sarcoma following radiation treatment for breast cancer.

Between 1954 and 1983, 7620 patients were treated for breast carcinoma at Institut Gustave Roussy (France). 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.

Adult↗

PubMed2001

[Stewart-Treves syndrome following mastectomy for breast cancer: a case report].

INTRODUCTION: Stewart-Treves syndrome has been defined by the eponymous authors as a lymphangiosarcoma in a setting of postmastectomy upper extremity lymphoedema. EXEGESIS: The clinical record of one patient with Stewart-Treves syndrome is analyzed. The primary angiosarcoma of the skin represented by a purple nodule occurred on a chronic lymphoedematous arm following radical mastectomy and axillary lymph node dissection for breast carcinoma performed 9 years earlier. Immunohistochemistry tests formally eliminated epithelial cutaneous metastasis and produced evidence in favour of conjunctive vascular tissue origin of the tumor. CONCLUSION: Conservative surgery for breast cancer, application of axillary sentinel node biopsy in the lymphatic staging and prevention of arm lymphoedema should reduce the incidence of this syndrome.

Arm↗

PubMed1996

[Stewart-Treves syndrome after breast carcinoma].

The Stewart-Treves syndrome is an angiosarcoma that occurs due to chronic lymphoedema which in most cases is a complication after mastectomy with axillary node dissection and postoperative radiation. Prognosis for this rare tumour is poor. The best therapy is early and radical excision. Chronic lymphoedema seems to be an important pathogenetic factor. We report on a 70-year old patient with early stage lymphangiosarcoma who had a lumpectomy with axillary node dissection and postoperative radiation five years ago.

Aged↗

PubMed2002

Long-term survival after radical operations for cancer treatment-induced sarcomas: how two survivors invite reflection on oncologic treatment concepts.

Extent and radicality of surgical oncologic treatment has changed in the past 30 years. Two patients with node-positive breast cancer are presented, who underwent (total or radical) mastectomy with lymphadenectomy and postoperative radiation 24 and 40 years ago. A radiation-associated sarcoma of the parascapular soft tissue developed in one patient 9 years after treatment; the other one sought treatment for a lymphedema-associated Stewart-Treves lymphangiosarcoma 16 years after initial therapy. Both patients underwent a forequarter amputation for their treatment-associated high-grade sarcoma. Both are currently alive and cancer-free 15 and 24 years after amputation. These reports remind us that radical locoregional treatment can cure some solid cancers in the absence of systemic therapy; that such extensive treatment may induce significant disability or secondary malignancies long-term; that even advanced treatment-associated sarcomas can be cured with aggressive resection; that today's multimodality therapy approaches and appropriate patient selection have rendered such extensive locoregional treatment for many tumors obsolete or unnecessary; and that if no effective alternative treatment exists and organ or limb preservation is not feasible, an aggressive resection approach for high-grade cancer should not be discounted unless systemic failure is certain or imminent.

Amputation, Surgical↗

PubMed2000

Intra-arterial mitoxantrone and paclitaxel in a patient with Stewart-Treves syndrome: selection of chemotherapy by an ex vivo ATP-based chemosensitivity assay.

We report on a 72-year-old patient developing Stewart-Treves syndrome (STS) of the right arm 9 years after curative irradiation for ipsilateral stage III breast cancer. Facing the poor track record of both irradiation and chemotherapy in this highly malignant lymphangiosarcoma, amputation was recommended but refused by the patient. Therefore, limb conserving-therapy using three courses of intra-arterial mitoxantrone (MX) and paclitaxel (PTX) was attempted. This novel chemotherapy protocol was selected by pretherapeutic ex vivo ATP-based chemosensitivity testing of autologous tumor tissue. The patient experienced complete response, which was subsequent histologically confirmed by compartment resection. When developing recurrent STS outside of the perfused area 6 months after primary therapy, the patient was retested and reinduced with three other courses of intraarterial MX/PTX which again produced durable complete remission. This case demonstrates the benefit of indivdualized therapy in this prognostically desperate disease allowing both limb conservation and maintained quality of life.

Adenosine Triphosphate↗

PubMedDate not supplied

[Surgical treatment of irradiation-induced sarcoma after treatment for breast carcinoma, personal experience: 4 case reports] ].

In the Department of Plastic Surgery of "Dubrava" University Hospital from 1993 to 1999 four patients were treated for radiation induced sarcoma. All of the patients were formerly operated for breast cancer and irradiated postoperatively. The mean time span between radiotherapy and development of sarcoma was 4.75 years. Four patients were treated with wide excision and immediate reconstruction with local flaps. One of the patients had fibrosarcoma, two patients had lymphangiosarcoma, and one patient had osteosarcoma. Two patients died within two years. Radiation induced sarcomas are therapy resistant, and the review of literature did not show large controlled investigations which would offer the most optimal treatment. Most frequently a rapid progression of the disease is seen.

Aged↗

PubMed1986

Lymphangiogenesis and lymphologic syndromes.

A unifying concept linking disorders of lymphatic dysplasia, hyperplasia and neoplasia is presented. The central role of disturbed lymphangiogenesis is illustrated in a wide variety of lymphologic syndromes characterized by lymphedema, lymphangiectasia, lymphangioma, and lymphangiosarcoma.

Adolescent↗

PubMed1994

Chylous ascites in cats: nine cases (1978-1993).

Medical records of 9 cats with chylous ascites that underwent exploratory celiotomy were reviewed. In 7 cats, chylous ascites was associated with intra-abdominal neoplasia: 4 cats had an unresectable tumor (hemangiosarcoma, 3 cats; paraganglioma, 1 cat) within the mesenteric root; 2 had malignant lymphoma of the small intestine and mesenteric lymph nodes; and 1 had lymphangiosarcoma of the abdominal wall. In 2 cats, chylous ascites was associated with nonneoplastic diseases: 1 cat had severe biliary cirrhosis and an extrahepatic portosystemic shunt; the other had steatitis caused by vitamin E deficiency. Three cats were euthanatized or died at the time of surgery, and 5 cats were euthanatized within 3 months of surgery. One cat with malignant lymphoma responded well to chemotherapy and lived for 14 months after surgery.

Animals↗

PubMed1978

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↗

PubMed1989

Results of regional isolation perfusion with cytostatics in patients with soft tissue tumors of the extremities.

From 1975 to 1986, 26 patients with soft tissue tumors of the extremities underwent a total of 29 perfusions. The cytostatics used were doxorubicin (Adriamycin, Adria Laboratories, Columbus, OH) (19 perfusions), melphalan (two perfusions), and a combination of these agents (eight perfusions). Before perfusion most patients had been treated by surgical excision(s), radiotherapy, or systemic chemotherapy. Of 17 patients perfused because of local inoperable tumor, four showed prolonged complete remission of the tumor mass, stable disease was seen in three, and ten showed progression. The complete remissions observed in three patients with aggressive fibromatosis and in one with lymphangiosarcoma occurred after perfusion with doxorubicin combined with melphalan. Doxorubicin added to the perfusate as the sole cytostatic was not effective. Local recurrence was observed in five of nine patients treated by adjuvant perfusion, always after dubiously radical tumor excision. Toxicity was high, especially in the first few years. Tissue necrosis necessitated amputation in three cases (in two after perfusion with doxorubicin and melphalan and in one after repeated perfusion with doxorubicin only). This complication was no longer seen after adjustment of the dosage and dose distribution of doxorubicin, but the morbidity after perfusion with doxorubicin remained considerable.

Adolescent↗

PubMed1986

Incidence of soft-tissue cancers in blacks and whites in New York State.

Incidence rates for several histologic types of soft-tissue cancers were compared between black and white residents of New York State during 1976-81, using data from the population-based New York State Cancer Registry. Incidence rates for leiomyosarcoma, neurofibrosarcoma and malignant neurolemmoma were higher for blacks than for whites in almost all age groups. Black-white differences in malignant mesenchymoma, while not statistically significant, were similar to those from other population-based studies. There was no evidence for black-white differences in certain other histologic types (e.g., blood-vessel cancers, lymphangiosarcoma). Black-white differences in leiomyosarcoma included higher rates for the uterus in black than in white females, and higher rates for the stomach in blacks of both sexes. Possible explanations for these differences in soft-tissue cancer incidence were discussed, along with comparisons with limited data on black populations in Africa.

Adult↗

PubMed2002

[Tumors of the lymphatic vessel of the skin and soft tissue].

Tumours of lymphatic vessels comprise only a small group in the heterogeneous spectrum of vascular neoplasms of skin and soft tissues. However, this discrepancy between haemangiomas/angiosarcomas and lymphangiomas/lymphangiosarcomas probably represents the present inability to reliably differentiate between lymphatic and capillary vascular endothelium. Histologically, neoplastic lymphatic vessels tend to be lined by endothelial cells with plumper and more prominent, matchstick-like nuclei (in contrast to vascular spaces in haemangiomas that are lined by flat or epithelioid endothelial cells), often show variations in the thickness of the vessel walls and are not completely surrounded by actin-positive (myo)pericytes. Endothelial cells in lymphatic neoplasms tend to be negative for CD34 or stain only focally positive for this marker and an expression of lymphatic markers as vascular endothelial growth factor-C receptor (VEGRF-3), podoplanin and M2A oncofetal antigen has been reported most recently. In addition to "traditional" lymphatic neoplasms including lymphangioma circumscriptum, cavernous lymphangioma/cystic hygroma, benign lymphangioendothelioma, lymphangiomatosis and the rare lymphangiosarcoma, histological and immunohistochemical features of a group of vascular neoplasms with a hobnail cytomorphology (hobnail haemangioma, retiform haemangioendothelioma, papillary intralymphatic angioendothelioma, benign lymphangiomatous papule following radiotherapy) suggest that these lesions also belong to the spectrum of tumours of lymphatic vessels.

Diagnosis, Differential↗

PubMed1985

Angiosarcomas arising in edematous extremities: immunostaining for factor VIII-related antigen and ultrastructural features.

Immunostaining for Factor VIII-related antigen was seen in deparaffinized sections from 19 of 20 postmastectomy angiosarcomas and from four of four sarcomas that arose in chronically edematous tissue unrelated to breast carcinoma. Staining was also seen in sections from two malignant hemangioendotheliomas, four capillary hemangiomas, and one granulation tissue specimen. Sections from two lymphangiomas were immunonegative for Factor VIII-related antigen in the endothelium of lymphatic channels, whereas staining was observed in the surrounding normal blood vessels. Electron microscopic study of four postmastectomy angiosarcomas disclosed ultrastructural features (fenestrae, intense pinocytotic activity, cell junctions, and Weibel-Palade bodies) supporting the blood vascular endothelial nature of the neoplastic cells. It is concluded that a neoplastic blood vessel component is present in sarcomas that arise in chronically edematous tissues. It is questionable whether a lymphatic component is also present. These tumors, therefore, should be regarded as angiosarcomas rather than lymphangiosarcomas.

Adult↗

PubMed2002

Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas.

There is controversy over the histogenesis of Kaposi's sarcoma (KS) from lymphatic or blood vessel endothelium. D2-40 is a novel monoclonal antibody to an Mr 40,000 O-linked sialoglycoprotein that reacts with a fixation-resistant epitope on lymphatic endothelium. We sought to establish the selectivity of D2-40 for lymphatic endothelium in normal tissues and compare its reactivity with the expression of the widely used vascular endothelial marker CD31 in a series of 62 formalin-fixed and paraffin-embedded vascular lesions including KS. In normal tissues, D2-40 stained the endothelium of lymphatic channels but not of blood vessels, including arteries and capillaries defined by reactivity with the blood vessel endothelial marker PAL-E. In our series of vascular lesions, D2-40 stained lymphangiomas (10/10), benign tumors of undisputed lymphatic origin, but not benign neoplasms or tumorlike lesions of blood vessel origin, including hemangiomas (0/10), glomus tumors (0/3), angiolipomas (0/2), pyogenic granulomas (0/2), vascular malformations (0/2), hemangiopericytoma (0/1), or hemangioendothelioma (0/1). D2-40 stained all cases of cutaneous KS (24/24) at all stages of progression, including patch, plaque, and nodular stages, supporting the concept that this disease originates from a cell type capable of undergoing lymphatic differentiation. D2-40 also stained three of seven angiosarcomas, indicating that a subset of these tumors can undergo at least partial differentiation along the lymphatic endothelial lineage and could be classified as lymphangiosarcomas. In comparison, CD31 was expressed in all benign and malignant vascular lesions, except for glomus tumors (0/3) and 5/10 lymphangiomas, in which staining was absent. We conclude that D2-40 is a new selective marker of lymphatic endothelium in normal tissues and vascular lesions and is valuable for studying benign and malignant vascular disorders in routinely processed tissue specimens.

Antibodies, Monoclonal↗

PubMed2000

Primary cutaneous B-cell lymphoma of the leg in a chronic lymphedematous extremity.

This is a case report of a woman who had chronic lymphedema on one leg and who developed a primary cutaneous large B-cell lymphoma of the leg at that site. She received radiotherapy and did not show any systemic involvement thereafter. Other neoplasms may appear in a clinical setting of chronic lymphedema, namely, lymphangiosarcoma (Stewart-Treves), melanoma, and metastatic carcinoma. There are four other reports in the English literature of cutaneous lymphoma arising in an extremity with chronic lymphedema.

Aged↗