lymphangiosarcoma

Lymphangiosarcoma
Classification and external resources
ICD-O:	9170/3
DiseasesDB	29095
MeSH	D008204

リンパ管肉腫

(病名)ンパ管肉腫

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/02/04 12:20:43」(JST)

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Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.^[1]

Signs and Symptoms

The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue.^[2] It metastasizes quickly.

Causes

It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical Halstedian radical mastectomy, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment - and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as Stewart-Treves syndrome (which can be both lymphangiosarcoma and hemangiosarcoma following mastectomy). The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs.^[3] Schreiber et al. proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.^[4]^[5]

Treatment

The most successful treatment for angiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is metastatic disease. If there is no evidence of metastasis beyond the lymphedematous limb, adjuvant chemotherapy may be given anyway due to the possibility of micrometastatic disease. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as paclitaxel,^[6] doxorubicin,^[7] ifosfamide, and gemcitabine^[8] exhibit antitumor activity.

Recently, there has been interest in evaluating the effectiveness of anti-angiogenic drugs in the treatment of lymphangiosarcoma. Early evidence suggests that treatment with one such drug, Bevacizumab, may be effective in treating lymphangiosarcoma.^[9] Investigation of bevacizumab in combination with other chemotherapy agents is underway.

References

^ Hellman S, DeVita VT, Rosenberg S (2001). Cancer: principles & practice of oncology. Philadelphia: Lippincott-Raven. p. 1853. ISBN 0-7817-2229-2.
^ LYMPHEDEMA MANAGEMENT:. Academy of Lymphatic Studies 2014. 2014. pp. 26–30. ISBN 3131394838. Retrieved 25 April 2014.
^ Stewart FW, Treves N. Lymphangiosarcoma in postmas- tectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
^ Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310-313
^ Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves Syndrome: a lethal complica- tion of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.
^ Gambini D, Visintin R, Locatelli E, Galassi B, Bareggi C, Runza L, Onida F, Tomirotti M.Tumori. 2009 Nov-Dec;95(6):828-31. Paclitaxel-dependent prolonged and persistent complete remission four years from first recurrence of secondary breast angiosarcoma. Medical Oncology Unit, IRCCS Foundation "Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena", Milan, Italy.
^ Verdier E, Carvalho P, Young P, Musette P, Courville P, Joly P. Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)] Ann Dermatol Venereol. 2007 Oct;134(10 Pt 1):760-3. French.
^ Sher T, Hennessy BT, Valero V, Broglio K, Woodward WA, Trent J, Hunt KK, Hortobagyi GN, Gonzalez-Angulo AM.Primary angiosarcomas of the breast. Cancer. 2007 Jul 1;110(1):173-8.
^ M. Agulnik, S. H. Okuno, M. Von Mehren, B. Jovanovic, B. Brockstein, R. S. Benjamin and A. M. Evens. An open-label multicenter phase II study of bevacizumab for the treatment of angiosarcoma. Northwestern University, Chicago, IL; Mayo Clinic, Rochester, MN; Fox Chase Cancer Center, Philadelphia, PA; Evanston Northwestern Healthcare, Chicago, IL; University of Texas M. D. Anderson Cancer Center, Houston, TX

External links

429522968 at GPnotebook
http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiosarcoma.htm

UpToDate Contents

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1. リンパ浮腫の臨床症状および診断 clinical manifestations and diagnosis of lymphedema
2. リンパ浮腫の予防および治療 prevention and treatment of lymphedema
3. 原発性および二次性リンパ浮腫の外科的マネージメント operative management of primary and secondary lymphedema
4. 軟部および骨肉腫の病原因子 pathogenetic factors in soft tissue and bone sarcomas
5. 癌治療患者における様々なリハビリの問題 various rehabilitation issues in patients treated for cancer

English Journal

Morphoproteomic study of primary pleural angiosarcoma of lymphangioendothelial lineage: a case report.

Quesada A, Quesada J, Khalil K, Ferguson EC, Brown RE.SourceDepartment of Pathology, Internal Medicine, University of Texas Medical School at Houston, TX, USA.
Annals of clinical and laboratory science.Ann Clin Lab Sci.2013 Summer;43(3):317-22.
An unusual case of bilateral primary pleural angiosarcoma with an immunophenotype of lymphangioendothelial lineage is described. Pleural angiosarcoma is a highly malignant neoplasm for which there is currently no standard of care. A comprehensive immunophenotypic characterization established a lymph
PMID 23884228

Stewart-Treves syndrome: a case report.

McKeown DG, Boland PJ.SourceMemorial Sloan-Kettering Cancer Center, 1,275 York Avenue, Suite A342, New York, NY 10065, USA. mckeownd@mskcc.org
Annals of the Royal College of Surgeons of England.Ann R Coll Surg Engl.2013 Jul;95(5):e80-2. doi: 10.1308/003588413X13629960046110.
We present a case of chronic lymphoedema that progressed to Stewart-Treves syndrome in a 63-year-old woman with a previous modified radical mastectomy, associated lymph node dissection, chemotherapy and radiotherapy. While producing stabilisation of most cutaneous lesions initially, chemotherapeutic
PMID 23838488

A novel approach to treatment of lymphangiosarcoma in a boxer dog.

Marcinowska A, Warland J, Brearley M, Dobson J.SourceDepartment of Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge, CB3 0ES.
The Journal of small animal practice.J Small Anim Pract.2013 Jun;54(6):334-7. doi: 10.1111/jsap.12054. Epub 2013 Apr 8.
A five-year-old female boxer presented with a swelling in the area of the caudal mammary gland. The mass was surgically excised and histopathological examination revealed a poorly demarcated lesion, extending into mammary tissue and infiltrating the sinuses of adjacent lymph nodes. The diagnosis was
PMID 23560802

Japanese Journal

原発性リンパ浮腫に生じたリンパ管肉腫

桑原慎治,中原千保子,増澤幹男,江藤宏光,勝岡憲生,早川和重,岩崎純也
Skin cancer : official organ of the Japanese Society for Skin Cancer = 皮膚悪性腫瘍研究会機関誌 25(2), 132-136, 2010-09-15
患者は66歳女で，20歳頃より右上肢にむくみがあった。2007年9月頃，右前腕に紅斑と結節が出現し，近医にて血管肉腫と診断され，2008年2月当科を紹介された。右上肢浮腫と前腕に紅斑性硬結を認め，組織学的にリンパ管肉腫と診断した。外傷や手術の既往なく，原発性リンパ浮腫に生じたStewart−Treves症候群と診断した。低容量パクリタキセルの静脈点滴を開始したが，高度の骨髄抑制を認めたため，副作用 …
NAID 10027731565