ARTICLE
Auteur(s) : David Aguiar
Bujanda1, Rafael Camacho Galán2, Jesús
Bastida Iñarrea3, José Aguiar Morales1,
Alicia Conde Martel4, Pino Rivero Suárez3,
Fernando de Armas Diaz5, Uriel Bohn
Sarmiento1, Miguel Ángel Cabrera Suárez1
1Servicio de Oncología Médica,
2Servicio de Anatomía Patológica,
3Servicio de Dermatología,
4Servicio de Medicina Interna,
5Servicio de Cirugía Plástica, Hospital Universitario de
Gran Canaria Dr. Negrín.Barranco de la ballena s/n. 35020 Las
Palmas de Gran Canaria. Spain
accepté le 14 Decembre 2005
Angiosarcoma is a rare and highly malignant tumor, accounting for
less than 1% of all sarcomas. It has predilection for skin and its
underlying superficial soft tissues, mainly in the head and neck
region and in the extremities. However it has been described in
multiple different locations such as breast, liver, spleen,
deep-soft tissues, bones, and heart [1].Many conditions have been
described in association with angiosarcoma, including exposure to
thorotrast (contrast for angiography), arsenic (insecticides),
vinyl chloride (plastics), radiation (particularly radiotherapy for
breast cancer), long-term exposure to foreign materials (synthetic
blood vessel grafts, material lost during surgical procedures,
metal fragments), and chronic lymphedema [2-5]. Cutaneous
angiosarcoma secondary to chronic lymphedema usually arises in the
extremities, most of them occurring in the upper extremities of
postmastectomy patients, which is termed Stewart-Treves syndrome
(STS) [6].We report a unique case of angiosarcoma arising in the
abdominal wall of a man, complicated by morbid obesity and chronic
abdominal lymphedema, eight years after extensive dermolipectomy
and in the absence of any other known predisposing factors.
Case report
In December 2003, a 64-year-old white man was admitted to our
hospital because of a presumed episode of erysipelas of the
abdominal wall. Personal history comprised morbid obesity from the
age of 16, with a maximum weight of 160 kg at the age of 40. He was
noticed to have lymphedema at the anterior abdominal and thoracic
wall for the first time in 1994. The following year he underwent
abdominal dermolipectomy and one year later thoracoplasty with
bilateral resection of the subaxillary fat tissue and bilateral
subcutaneous mastectomy. In 1998 he presented an extensive
cellulitis of the abdominal and thoracic wall which was resolved
with penicillin-based antibiotic therapy. In August 2003 an ulcer
was noted in the inferior abdominal wall fold with lack of response
to any topical medication. Over the next 3 months the skin
surrounding the ulcer became thick, hard, erythematous, and
painful, with edema of the anterior abdominal wall which progressed
up to the inframammary fold.
At admission the patient was afebrile and had no other symptoms
except for those related to the skin lesions. Physical examination
revealed pendular lower abdomen with an ulcer showing sharp
anfractuous margins and whitish exudation over the scar of
abdominoplasty. There was painful lymphedema and peau d’orange
thickening of the skin involving all the abdominal wall regions,
bilateral subaxillary area, and inframammary folds. The skin was
erythematous with wide hemorrhagic areas, centrally dark, and red
spotted at the periphery. Scattered reddish nodules 0.5-1 cm
were present over the abdominal surface (figure 1). Biopsy of the
lesions revealed a neoplastic proliferation of gaping vascular
channels, lined by atypical endothelial cells with large nuclei and
prominent nucleoli (figure 2). Marked apoptosis
and a high mitotic index were also observed. Tumor cells stained
strongly positive for CD31 and CD34 antigens, whereas Factor
VIII-related antigen showed focal staining. S100 protein and
cytokeratin (AE1/AE3) were negative (figure 3). Based on these
findings, a diagnosis of angiosarcoma was made.
Laboratory studies were normal except for a mild anaemia and
thrombopenia.
A computed tomography scan of the thorax and abdomen showed
extensive heterogeneous thickening of the skin and subcutaneous
cellular tissue, with infiltration of thoracic and abdominal
musculature. Small round nodules in both lower-lung lobes were also
noted suggesting metastatic disease. Surgery was not felt to be
possible and the patient begun palliative chemotherapy with
doxorubicin and ifosfamide which led to partial response of the
skin involvement and good symptomatic palliation. The patient died
in May 2004 from progressive disease with lung and pleural
involvement.
Discussion
Angiosarcoma arising in chronic lymphedema usually occurs in the
upper extremities of women with chronic lymphedema after mastectomy
for breast carcinomas with or without radiotherapy, termed STS [6].
Other conditions leading to chronic lymphedema of the extremities
such as post-traumatic lymphedema, burn injuries, lymphatic
filariasis, and congenital lymphedema are also related to the
development of angiosarcoma, as well as Kaposi’s sarcoma,
carcinomas, melanomas and B-cell lymphomas [7-10].
Chronic lymphedema represents a risk factor for the development
of tumors, especially angiosarcoma. Several explanations have been
proposed for this phenomenon, but the exact pathogenesis remains
unclear [11]. The lymphedematous extremity becomes an
immunologically vulnerable area because immune surveillance is down
regulated by the lack of antigen presentation and immunocompetent
cells. Moreover, lymphedema stimulates angiogenesis and development
of a new vascular network. This continuous angiogenic stimulus on
an “immunocompromised” area can predispose to the induction of
neoplasia.
Only 2 cases of abdominal wall angiosarcoma associated with
morbid obesity have been previously reported [12, 13]. In both,
morbid obesity and subsequent chronic lymphedema of the abdominal
wall were postulated to be the etiological factors implicated in
the development of the angiosarcoma. So, we consider that those two
and our patient share the same pathogenesis compared to patients
with classic STS of the extremities. We believe that our case is
unique, since neither of these other 2 patients had a history of
surgery for abdominal dermolipectomy as had our patient.
The rarity of this association may be explained by the sequence
of events needed to induce the development of the tumor. In our
case, long-term morbid obesity as well as the surgical procedures
certainly all contributed to the impairment of the lymph drainage,
chronic lymphedema, and finally induction of angiosarcoma.
Standard treatment of angiosarcoma is surgical resection with
wide margins. Little information is available about the utility of
other treatment modalities such as radiotherapy or chemotherapy.
However in our case, systemic chemotherapy with doxorubicin and
ifosfamide resulted in a partial response and a good palliation for
the patient.
In conclusion, chronic lymphedema predisposes to malignancy,
specially angiosarcoma in the extremities. Although exceptionally
observed, angiosarcoma may arise in the abdominal wall of morbidly
obese patients. Perhaps in the future more cases similar to these
will be reported because of the increasing prevalence of obesity in
the general population. The skin of the abdomen of obese patients,
especially those with morbid obesity, should be thoroughly
explored, and any suspicious cutaneous lesion be considered for
biopsy in order to establish a prompt diagnosis.
References
1 Enzinger FM, Weiss SW. Malignant vascular tumors. In:
In: Soft tissues tumors. 3rd ed. St louis: Mo: Mosby-Year Book Inc,
1995: 641-77.
2 Baxter PJ, Langlands AO, Anthony PP,
et al. Angiosarcoma of the liver: a marke tumor for the late
effect of thorotrast in Great Britain. Br J Cancer 1980; 41:
446-53.
3 Salgado M, Sans M, Forns X, et al. Hepatic
angiosarcoma: a report of a case associated with treatment with
arsenic salts and a review of the literature. Gastroenterol Hepatol
1995; 18: 132-5.
4 Pendlebury SC, Bilous M, Langlands AO. Sarcomas
following radiation therapy for breast cancer: a report of three
cases and a review of the literature. Int J Radiat Oncol Biol Phys
1995; 31: 405-10.
5 Jennings TA, Peterson L, Axiotis CA,
et al. Angiosarcoma associated with foreign body material. A
report of three cases. Cancer 1988; 62: 2436-44.
6 Stewart FW, Treves N. Lymphangiosarcoma in
postmastectomy lymphedema: a report of six cases with elephantiasis
chirurgica. Cancer 1948; 1: 64-81.
7 Peyron N, Dandurand M, Guillot B. Malignant
tumors as complications of lymphedema. J Mal Vasc 1993; 18:
293-8.
8 Ron IG, Amir G, Marmur S, et al. Kaposi’s
sarcoma on a lymphedematous arm after mastectomy. Am J Clin Oncol
1996; 19: 87-90.
9 Torres-Paoli D, Sanchez JL. Primary cutaneous B-cell
lymphoma of the leg in a chronic lymphedematous extremity. Am J
Dermatopathol 2000; 22: 257-60.
10 Lister RK, Black MM, Calonje E,
Burnand KJ. Squamous cell carcinoma arising in chronic
lymphoedema. Br J Dermatol 1997; 136: 384-7.
11 Ruocco V, Schwartz RA, Ruocco E. Lymphedema:
an immunologically vulnerable site for development of neoplasm. J
Am Acad Dermatol 2002; 47: 124-7.
12 Krause KI, Hebert AA, Sanchez RL,
Solomon AR. Anterior abdominal wall angiosarcoma in a morbid
obese woman. J Am Acad Dermatol 1986; 15: 327-30.
13 Azam M, Saboorian H, Bieligk S, et al.
Cutaneous angiosarcoma complicating morbid obesity. Arch Pathol Lab
Med 2001; 125: 531-3.
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