Stewart-Treves Syndrome

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.

Lymphangiosarcoma is a misnomer because this malignancy seems to arise from blood vessels instead of lymphatic vessels. A more appropriate name is hemangiosarcoma.

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.

Frequency:

Internationally: Currently, 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
(1)

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External Links:

Secondary angiosarcomas after conservation treatment for breast cancers 2011

http://www.ncbi.nlm.nih.gov/pubmed/22070020

Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of lymphoedema

http://www.em-consulte.com/article/274586

Fluorodeoxyglucose positron emission tomography-computed tomography scan and nuclear magnetic resonance findings in a case of Stewart-Treves syndrome.

http://www.cancerjournal.net/article.asp?issn=0973-1482;year=2011;volume=7;issue=3;spage=360;epage=363;aulast=Joshi

Cutaneous angiosarcoma arising in massive localized lymphedema of the morbidly obese: a report of five cases and review of the literature. July 2011

http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0560.2011.01703.x/abstract

Stewart-Treves syndrome and the use of positron emission tomographic scanning.

http://www.annalsofvascularsurgery.com/article/S0890-5096(11)00076-8/abstract

Two cases of Stewart-Treves syndrome observed at Sanshu hospital

http://www.ncbi.nlm.nih.gov/pubmed/21368533

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Abstracts

STEWART-TREVES SYNDROME

Angiosarcoma associated with chronic lymphedema (Stewart-Treves syndrome) of the leg: MR imaging.

Nakazono T, Kudo S, Matsuo Y, Matsubayashi R, Ehara S, Narisawa H, Yonemitsu N.

Department of Radiology, Saga Medical School Hospital, Saga City, Japan.

Magnetic resonance (MR) imaging findings of two patients with Stewart-Treves syndrome are presented. MR imaging showed edematous changes in the subcutaneous fat and skin masses that proved to be angiosarcomas. MR signal intensity of the tumor was low compared with fat on T1-weighted images and intermediate and heterogeneous on T2-weighted images. In one patient, administration of intravenous Gd-DTPA showed marked enhancement in the early phase, which persisted until the delayed phase. These finding on dynamic MR imaging may reflect the abundant vascular spaces seen in these tumors.

Publication Types:

Case Reports
Review
Review of Reported Cases

PMID: 10963428 [PubMed - indexed for MEDLINE]

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Stewart-Treves syndrome: Pathogenesis and management.

Sharma A, Schwartz RA.

Source

Dermatology and Pathology, New Jersey Medical School, Newark, New Jersey.

Abstract

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

http://www.eblue.org/article/S0190-9622(12)00451-3/abstract

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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

Acta Cytologica

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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.

Abstract

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.

ScienceDirect

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Lymphedema-induced Stewart Treves Syndrome (lymphangiosarcoma)

Arieke J. Janse, Frits van Coevorden, Hans Peterse^*, Ronald B. Keus^† and Johannes A. van Dongen

^aDepartment 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.

Science Direct

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Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients

Kevin C. 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

Abstract

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.

Science Direct

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Stewart-Treves syndrome associated with chronic lymphedema (angiosarcoma) of the leg: MR imaging

Authors

T. Nakazono¹, S. Kudo¹, Y. Matsuo¹, R. Matsubayashi¹, S. Ehara², H. Narisawa³, N. Yonemitsu⁴

¹Department 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

Abstract

Magnetic resonance (MR) imaging findings of two patients with Stewart-Treves syndrome are presented. MR imaging showed edematous changes in the subcutaneous fat and skin masses that proved to be angiosarcomas. MR signal intensity of the tumor was low compared with fat on T1-weighted images and intermediate and heterogeneous on T2-weighted images. In one patient, administration of intravenous Gd-DTPA showed marked enhancement in the early phase, which persisted until the delayed phase. These finding on dynamic MR imaging may reflect the abundant vascular spaces seen in these tumors.

Keywords

Key words Angiosarcoma · Lymphedema · Stewart-Treves syndrome · Leg · MR imaging

Springer Link

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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

Abstract Two cases of postmastectomy lymphangiosarcoma seen at our department are reported. One case, treated by amputation, died 1 year 4 months later and the other case, treated by local excision followed by amputation, died 7 months later. Twenty cases from the Japanese literature, including our 2 cases, are reviewed herein. The initial treatment for the other 18 cases was as follows: amputation in 8 cases, local excision in 2, radiotherapy in 4 and chemotherapy in 4. Fifteen of 16 cases eligible for follow up were dead within 20 months after treatment. Although the knowledge available as to the results of the treatment of postmastectomy lymphangiosarcoma is still insufficient to indicate the optimal therapeutic approach, early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.

Key Words Stewart-Treves syndrome - postmastectomy lymphangiosarcoma

Springer Link

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

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. Thepathogenesis of lymphangiosarcoma in chronic lymphedema is a combination oftwo factors. First, the edematous region responds in a manner similar to"immunologically privileged sites." Second, because of its anatomic andphysiologic properties, it is a favorable site for the development ofmutant cell populations for reasons that are not fully understood. As aresult, 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 unrestrictedtumor growth to take place, resulting in the ultimate death of the patient.Available therapeutic measures are equally unsatisfactory. Emphasis isplaced on periodic examination of the lymphedematous extremity, aggressivetreatment of established lymphedema and infections, and surgicalpreservation of lymphatic channels during breast cancer surgery.

Archives of Surgery

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Stewart-Treves syndrome arising in post-traumatic lymphedema

Bull Cancer. 1999 Mar

Laguerre B, Lefeuvre C, Kerbrat P, Hassel M.

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.

Bulletin Du Cancer

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

Rev Med Interne. 2001 Aug

Le Bouëdec G, Curé H, de Latour M, Dauplat J.

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.

Science Direct

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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.)

Science Links

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Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature.

Jan. 2012

Krishnamoorthy N, Viswanathan S, Rekhi B, Jambhekar NA.

Source

Department of Pathology, Tata Memorial Hospital, Mumbai, India.

Keywords:

chronic lymphedema;
cutaneous angiosarcoma;
filariasis;
lymphangiosarcoma

Abstract

Cutaneous angiosarcoma or lymphangiosarcoma represents an uncommon aggressive tumor known to arise on a background of chronic lymphedema secondary to various etiologies, principally following surgery or irradiation. There have been rarely reported cases of angiosarcoma following infective conditions that eventuate with lymphatic stasis. We report a case of angiosarcoma arising after 33 years within a background of filariasis. Awareness of this association can lead to early diagnosis and appropriate treatment of this potentially fatal malignant tumor. Krishnamoorthy N, Viswanathan S, Rekhi B, Jambhekar NA. Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature.

http://onlinelibrary.wiley.com/doi/10.1111/j.1600-0560.2011.01785.x/abstract

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External Links:

STEWART-TREVES SYNDROME

http://www.emedicine.com/derm/byname/stewart-treves-syndrome.htm

Stewart-Treves Syndrome

http://www.emedicine.com/derm/topic898.htm

Stewart Treves Syndrome on Congenital Lymphedema

http://www.sc.ehu.es/scrwwwsr/kirurgia/Kirurgia2003b/Linfangiosarcoma.htm

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