See also: lymphangiosarcoma
Stewart-Treves Syndrome is a rare, aggressive and cutaneous angiosarcoma often associated with long standing lymphedema.
Most cases arise from lymphedema induced by a radical mastectomy in breast cancer patients. Although there has also been significant reports regarding development from Milroy lymphedema and in idiopathic, congenital, traumatic, or filarial lymphedema.
The three main indications for the development of Stewart Treves in a lymphedemous limb appear to be radiation, extensive fibrosis and recurrent or chronic infection.
The sarcoma first appears as a bruised area or a purplish discoloration in an extremity. It progresses to an ulcer with crusting and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes widely and there is no effective therapy.
In 1906, Lowenstein first described angiosarcoma in a patient’s arm that had been affected by severe posttraumatic lymphedema for 5 years. In 1948, Stewart and Treves reported this rare secondary malignancy in 6 cases of angiosarcoma in postmastectomy lymphedema. They recognized that an edematous arm after radical mastectomy for breast cancer may suggest recurrent breast cancer, but that long-standing chronic edema without recurrent cancer may occasionally produce “a heretofore unrecognized and unreported sequel … long after the malignant breast neoplasm has apparently been arrested … a new specific tumor.” Stewart and Treves suggested that these angiosarcomas were probably not observed previously because they were mistaken for recurrent, inoperable, cutaneous manifestations of breast cancer.
approximately 400 cases are reported in the world literature. In 1962,
Schirger calculated that the incidence of this disease is 0.45% in
patients who survive at least 5 years after radical mastectomy. Others
have noted a much lower incidence of 0.07% in patients after mastectomy.
Lymphangiosarcomas are extremely aggressive tumors with a high local recurrence rate and a tendency to metastasize early to many areas.
Metastatic angiosarcoma to the lungs and chest wall are the most common
cause of death in patients with Stewart-Treves syndrome.
Metastases to the liver and bones can also occur.
Lymphangiomas are associated with a high rate of local recurrence and metastasis, even after aggressive surgical treatment
PMID: 10963428 [PubMed - indexed for MEDLINE]
Stewart-Treves syndrome: Pathogenesis and management.
Sharma A, Schwartz RA.
Dermatology and Pathology, New Jersey Medical School, Newark, New Jersey.
Key words: breast cancer, Kaposi sarcoma, lymphangiosarcoma, lymphedema, postmastectomy angiosarcoma, Stewart-Treves syndrome
Stewart-Treves syndrome is a malignancy that arises within chronic lymphedema. Although classically described as a consequence of radical mastectomy, this lymphangiosarcoma has been documented to occur in cases of congenital and other causes of chronic secondary lymphedema. The development of this aggressive lymphangiosarcoma at sites of chronic lymphedema renders it a possible model for Kaposi sarcoma. Because of the increase in conservative treatment for breast carcinoma and improvement of operative and radiation therapy techniques, the prevalence of Stewart-Treves syndrome has decreased. Regardless, this malignancy significantly worsens patients' outcomes and needs to be diagnosed and treated early. Chemotherapy and radiation therapy have not improved survivorship significantly. Early amputation or wide local excision offers the best chance for long-term survival. Yet, overall prognosis remains dismal. Untreated patients usually live 5 to 8 months after diagnosis.
2012 American Academy of Dermatology
Postmastectomy Stewart-Treves Syndrome (Angiosarcoma))
Michio Shimizu, M.D., F.I.A.C., Mitsuyoshi Hirokawa, M.D., F.I.A.C., Takashi Fukuya, M.D., Noriko Osugi, C.T.(IAC), Isao Koshima, M.D., and Toshiaki Manabe, M.D.
To the Editors: Since the first report by Stewart and Treves,5 more than 220 cases of postmastectomy angiosarcoma, also called Stewart-Treves syndrome, have been reported. The histology of postmastectomy angiosarcoma has been well published4; however, to our knowledge, its cytology has not been described in detail. We describe a case of angiosarcoma occurring in the lymphedematous upper right arm 12 years after mastectomy for cancer of the right breast. A 76-year-old Japanese female was admitted to the hospital for evaluation of edema in the right arm and anterior aspect of the chest. She had had a right radical mastectomy with regional lymph node dissection for cancer of the right breast 12 years earlier at another hospital. Biopsy and imprint cytology of the right arm and anterior chest wall were performed. Cytology from both the right arm and anterior chest wall revealed spindle-shaped, elongated, crescent-shaped cells with delicate nuclear chromatin and collagen fragments. There was little cellular cohesion, and the background was clear (Figure 1). At higher magnification, spindle-shaped and oval nuclei with prominent nucleoli were observed (Figure 2). Although variation in nuclear shape and size was seen, neither mitosis nor marked atypia was found. Because of the moderate cellularity with mild atypia, elongated or spindle-shaped cells, prominent nucleoli and overall lack of cohesion, we concluded that these were atypical cells of probable mesenchymal origin, and malignancy was suspected.
Keywords: angiosarcoma, breast neoplasms, mastectomy
Stewart-Treves syndrome after treatment for breast cancer
Y. M. Kirova, F.
Feuilhade, E. Calitchi, Y. Otmezguine and
J. P. Le Bourgeois
Department of Cancerology, Henri Mondor University Hospital, Creteil, France
Available online 12 April 2002.
This study reviews 3 cases of angiosarcoma of the upper extremity after mastectomy and radiotherapy for breast cancer (Stewart-Treves syndrome). Angiosarcoma was diagnosed an average 14 years (from 6.5 to 26 years) after treatment for breast cancer. Presenting signs included a red raised lesion, a palpable mass, a blister appearance (in one case). Two of our three patients underwent surgical treatment: one patient underwent local excision followed by chemotherapy, and the other patient wide excision, followed by external beam radiotherapy. Local recurrence occured in one of these two patients and was followed by the development of lung metastases. The second patient who had treatment is free of disease without problems. The third patient refused any treatment and died 5 months later. The purpose of this article is to add to the literature 3 new cases of Stewart-Treves syndrome and to discuss some specific problems of this rare tumour.
Lymphedema-induced Stewart Treves Syndrome (lymphangiosarcoma)
of Surgery, The Netherlands Cancer
Institute/Antoni van Leeuwenhoek ziekenhuis, Amsterdam, The Netherlands
Department of Pathology, The Netherlands Cancer Institute/Antoni van Leeuwenhoek ziekenhuis, Amsterdam, The Netherlands
†Department of Radiotherapy, The Netherlands Cancer Institute/Antoni van Leeuwenhoek ziekenhuis, Amsterdam, The Netherlands
Accepted 20 October 1994. Available online 13 October 2004.
A series of eight patients with chronic lymphedema-related lymphangiosarcoma is presented. All but one case showed a typical rapid progression and fatal outcome, as has been reported in other series. In one patient the lymphangiosarcoma developed on the chest wall, the axilla and the arm where persisting lymphedema and fibrosis occurred after bilateral mastectomy and bilateral postoperative radiotherapy. In this patient an asymptomatic course and slow locoregional progression of lesions was seen. The clinical picture, the etiological considerations and the therapeutic options of the lymphedema-induced lymphangiosarcoma with regard to the literature are discussed.
Author Keywords: lymphangiosarcoma; angiosarcoma; Stewart-Treves syndrome; breast carcinoma; lymphedema
Correspondence to: F. van Coevorden, MD, PhD, Department of Surgery, The Netherlands Cancer Institute/Antoni van Leeuwenhoek ziekenhuis, Plesmanlaan 121, 1066 CX Amsterdam, The Netherlands.
Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients
Chung MD, Huyi Jin Elizabeth Kim BA and Lynn L. C. Jeffers MD
From the Section of Plastic and Reconstructive Surgery, Department of Surgery, The University of Michigan Medical Center.
Received 27 December 1999. Available online 8 May 2002
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. (J Hand Surg 2000;25A:1163-1168.
Stewart-Treves syndrome associated with chronic lymphedema (angiosarcoma) of the leg: MR imaging
of Radiology, Saga Medical School
Hospital, 5-1-1 Nabeshima, Saga City, Saga, 849-8501 Japan
2Department of Radiology, Iwate Medical University Hospital, Morioka, Iwate, 020-8505 Japan
3Department of Dermatology, Saga Medical School Hospital, Saga, Japan
4Department of Pathology, Saga Medical School Hospital, Saga, Japan
Stewart-treves syndrome. A report of two cases with a review of Japanese literature
Masakuni Noguchi1 , Hajime Hasegawa1, Kiyoshi Tajiri1, Xabier De Aretxabala1, Itsuo Miyazaki1, Shintaro Terahata2 and Katsuro Tomita
The Department of Surgery (II), School of Medicine, Kanazawa University, Takara-machi, 13-1, 920 Kanazawa, Japan; Department of Clinical Pathology, School of Medicine, Kanazawa University, Takara-machi, 13-1, 920 Kanazawa, Japan; Department of Orthopedics, School of Medicine, Kanazawa University, Takara-machi, 13-1, 920 Kanazawa, Japan
Key Words Stewart-Treves syndrome - postmastectomy lymphangiosarcoma
H. Schreiber, F. M. Barry, W. C. Russell, W. L. Macon 4th, J. L. Ponsky and W. J. Pories
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.
Stewart-Treves syndrome arising in post-traumatic lymphedema
Bull Cancer. 1999 Mar
Centre Eugène-Marquis, Département d'oncologie médicale, rue de la Bataille-Flandres-Dunkerque, BP 6279, 35062 Rennes Cedex.
The authors report a case-history of lymphangiosarcoma or Stewart-Treves syndrome which occurred in a patient with posttraumatic lymphedema of the arm. He presented two recurrences after surgery which led to the decision of amputation. He is now disease free nine years after this treatment. Initially defined as "lymphangiosarcoma arising in chronic lymphedematous extremities after mastectomy for breast cancer", Stewart-Treves syndrome complicates rarely traumatic, postsurgical, postradiation, idiopathic, congenital or filarial lymphedema. Clinical diagnosis is based on nodular, purple and frequently multiple skin lesions on chronic lymphedema of the limb. A proliferating malignant endothelium with affinity for anti-factor-VIII is found on histological slides. The tumor aggressivity explains the short survival if no treatment is given. Stewart-Treves syndrome prognosis is poor, with the occurrence of multiple local recurrences and pulmonary metastasis. On account of its rarity, there is no therapeutic consensus. In localized stage, radical surgery is usually performed. Conservative treatment with complete tumour removal and postoperative radiotherapy has not been yet evaluated. In metastatic or locally advanced tumours, it is necessary to study the benefit of cytotoxic drugs like anthacyclins and ifosfamide known to be effective on soft tissue sarcomas.
Stewart-Treves syndrome following mastectomy for breast cancer: a case report
Rev Med Interne. 2001 Aug
Service de chirurgie, centre Jean-Perrin, centre de lutte contre le cancer, 58, rue Montalembert, B.P. 392, 63011 Clermont-Ferrand, France. Recherche@cjp.u-clermont1.fr
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.
A case of Stewart-Treves syndrome.
Abstract;We report a case of Stewart-Treves syndrome. A 71-year-old Japanese woman visited us with a reddish nodule on the right upper arm. She had radical mastectomy with lymph node dissection for the right breast cancer at the age of 53. On physical examination, a solitary darkreddish nodule measured 3.0*2.5*0.8cm with subcutaneous infiltration was found on the right upper arm showing postmastectomy lymph edema. Histology of the nodule revealed numerous round cells with marked cellular atypia forming slit-like space. The diagnosis of Stewart-Treves syndrome was confirmed. The surgical excision then was performed. After the surgical resection, she was treated with electron beam irradiation and intravenous recombinant human IL-2 therpapy. The patient had no local recurrence or metastasis for 12 months after the excision. (author abst.)
Stewart Treves Syndrome on Congenital Lymphedema
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