Cases reported "lymphangiosarcoma"

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1/48. lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome.

    A case of post-mastectomy lymphangiosarcoma is reported. lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphoedema. Our patient was treated by amputation and died 6 months later. Treatment of post-mastectomy lymphangiosarcoma is still unsatisfactory. Early recognition and radical ablative surgery seem to provide best chance for survival. ( info)

2/48. Chronic lymphoedema and angiosarcoma.

    Angiosarcoma has frequently been described arising within chronic lymphoedema of the upper limb following mastectomy and radiotherapy for carcinoma of the breast. We report a case of angiosarcoma arising in a lymphoedematous leg that had been subjected to radiotherapy 20 years previously for Hodgkin's disease. The diagnosis was expedited once the patient noticed the development of bleeding nodules. prognosis of angiosarcoma is poor with treatment options being wide-excision surgery, palliative radiotherapy or chemotherapy. Unusual bruised areas or bleeding nodules developing within chronic lymphoedematous limbs should be biopsied to exclude the diagnosis. ( info)

3/48. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.

    lymphangiosarcoma (LAS) is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. patients with LAS demonstrate a history of breast cancer treated by radical mastectomy in the majority of patients. In the 1960s the incidence of LAS in patients with a 5-year survival after radical mastectomy varied from 0.07 to 0.45%. Today, due to changes in the operative techniques of breast cancer, less chronic lymphedema is seen with only a scant number of LAS patients. The etiology of this enigmatic tumor is not yet completely understood. Histologically, LAS arises from vascular endotheliocytes, and all vascular sarcomas originating in the setting of a chronic lymphedema are categorized as LAS. There is no standard treatment of LAS. The treatment options include radical ablative surgery, radiation therapy, and chemotherapy. The prognosis of LAS is poor; long-term survival is the exception. Only early recognition and radical surgery offer a chance of cure. ( info)

4/48. hemangiosarcoma of the left hand in a patient with the rare combination of Maffucci's and Stewart Treves syndrome.

    We describe a patient with the previously unseen combination of Maffucci's and Stewart Treves syndrome who presented with an angiosarcoma of the hand. Maffucci's syndrome is characterized by the presence of multiple enchondroma and soft tissue hemangioma. The syndrome is a rare nonhereditary condition with a usual onset in childhood. Malignant transformations are a common feature of this syndrome. In 1948, Stewart and Treves first described six cases of lymphangiosarcoma after radical mastectomy. This syndrome is an unusual form of angiosarcoma occuring as a complication of lymphedema. Chronic lymphedema and lymphangiectasia preceding lymphangiosarcoma may not only be induced by radical mastectomy with axillary lymph node dissection and postoperative radiation therapy. Posttraumatic, congenital or spontaneous chronic lymphedema may also be associated with lymphangiosarcoma. A time interval of many years seems to be required before malignant transformation develops. Generally the syndrome has a very poor prognosis. Both syndromes described above are of a rare frequency. We report this case because of prior unknown coincidence of both syndromes. ( info)

5/48. 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. ( info)

6/48. 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. ( info)

7/48. Two cases of Kaposi's sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive.

    Although angiosarcoma is the most frequent tumor arising in the clinical setting of chronic lymphedema, as in Stewart-Treves syndrome, Kaposi's sarcoma has also been reported in this setting, although rarely. We describe two women who developed Kaposi's sarcoma in the lymphedematous arm many years after surgery for breast cancer. Case 1 is a 92-year-old and Case 2 is an 81-year-old; they underwent left total mastectomy and axillary node dissection for infiltrating breast carcinoma in 1981 and 1982 respectively. At that time, neither patient received further treatment. Except for persistent lymphedema, both women did well until over fourteen years later when each noted the development of several purple asymptomatic plaques on the edematous arm. In both, the clinical diagnosis at the time of biopsy was angiosarcoma. However, histologic findings in both cases were typical for Kaposi's sarcoma. In addition, a nested polymerase chain reaction (PCR) for the detection of a 233bp segment of KSHV/HHV8 was performed on dna extracted from the paraffin-embedded specimens and both cases were positive for this sequence. Histologic sections of both cases were also tested for KSHV by in situ hybridization and demonstrated a positive signal in the lesional cells in each case. ( info)

8/48. 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. ( info)

9/48. Stewart-Treves syndrome as a rare complication of a hereditary lymphedema.

    lymphangiosarcoma (LAS) may occur as a rare complication of primary lymphedema. A case of LAS in hereditary lymphedema of the lower extremity in a 36-year old female is reported. Despite of chemotherapy, local hyperthermia and later amputation of the extremity the patient died of progressive disease due to pulmonary metastasis. In respect to this case, the different therapeutic concepts, as reported in the literature, and their results are presented and discussed. ( info)

10/48. platelet-derived growth factor receptor-beta in Gorham's disease.

    BACKGROUND: A 17-year-old male presented with pain in his lower-left chest. He had no significant medical history and was previously in good health. He had a fractured ninth left anterior rib and the tenth, eleventh and twelfth ribs were absent, which was thought to be a congenital anomaly. Several months later, he presented again with back pain, an enlarging mass in the lower-left chest wall, erosion of the lateral pedicles of the lower thoracic vertebrae and pleural effusion. INVESTIGATIONS: physical examination, chest X-ray, MRI of the spine, incisional biopsy, serial CT imaging of the hemithorax, immunohistochemistry, flow cytometry, and enzyme-linked immunosorbent assays. DIAGNOSIS: Gorham's lymphangiomatosis with expression of platelet-derived growth factor receptor-beta and elevated circulating platelet-derived growth factor-BB. MANAGEMENT: spine stabilization, thalidomide, celecoxib, interferon-alpha2b, pamidronate, zoledronate, thoracotomy, pleurectomy, talc pleurodesis, and imatinib mesylate. ( info)
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