Sporadic Cutaneous Angiosarcomas

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Sporadic Cutaneous Angiosarcomas

Postby patoco » Sat Jan 12, 2008 7:15 am

Sporadic Cutaneous Angiosarcomas: A Proposal for Risk Stratification Based on 69 Cases.

Am J Surg Pathol. 2008 Jan

Deyrup AT, McKenney JK, Tighiouart M, Folpe AL, Weiss SW.
*Department of Pathology ‡Department of Biostatistics, Winship Cancer Institute, Emory University, Atlanta GA †Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR §Department of Pathology, The Mayo Clinic, Rochester, MN.


Angiosarcomas have traditionally been considered high-grade lesions for which histologic features and grading have played no role in prognostication and, consequently, they have been excluded from the American Joint Committee on Cancer staging system. We have, therefore, analyzed 69 cutaneous angiosarcomas seen in consultation and not associated with lymphedema or prior radiation therapy to determine if a combination of histologic and clinical parameters could be used to differentiate indolent from aggressive tumors.

The clinical features analyzed included patient age, location, size, depth, and focality of the lesion; the histologic features studied included pattern of growth (vasoformative vs. solid), nuclear grade (high vs. low), necrosis (present/absent), cell type (epithelioid or spindled), extent of inflammatory infiltrate (minimal vs. marked), and mitotic rate. Lesions occurred on the head/neck (49), extremities (15), and trunk (5) of adults (21 to 94 y) and predominated in males (41 males; 28 females). Tumors ranged in size from 0.3 to 15 cm (average 3.1 cm) and involved the papillary (n=9), reticular (n=16), or deep dermis/ subcutis (n=30). They could be predominantly vasoformative to solid (greater than 50% solid, n=41). Most lesions were of high (n=65) as opposed to low (n=4) nuclear grade, were mitotically active (0 to 99/10 high power fields), and occasionally displayed necrosis (n=14) and epithelioid features (n=21). Inflammatory infiltrates were minimal in most cases. Follow-up information was obtained for all patients. Recurrences developed in 18 patients (26%) and metastasis in 15 (22%) to the following sites: lung (6), lymph node (7), liver (2), bone (2), and parotid gland (1). Forty-seven patients died (30 of disease) and 22 were alive at last known follow-up (range, 16 to 158 mo; mean, 65; median, 36).

Five-year disease-specific survival was 48%. By univariate analysis only older age, anatomic site, necrosis, and epithelioid features correlated with increased mortality. Tumors were stratified into low (n=41) or high risk groups based on necrosis and/or epithelioid features. By multivariable analysis, high-risk group (hazard ratio 4.07, P=0.0004) and age >70 (hazard ratio 2.79, P=0.012) were associated with increased mortality, and tumor depth (P=0.048) correlated with the risk of local recurrence. The high-risk group had a significantly worse prognosis than the low-risk group with 3-year survival of 24% and 77%, respectively. No patients with high-risk features survived 5 years.

In conclusion, we report that a combination of clinical and histologic features allows stratification of angiosarcoma patients into 2 risk groups that are strongly associated with marked differences in clinical course. These features seem to diminish in importance with increased tumor size and are probably most useful in tumors less than 5 cm in maximum dimension.

Lippincott Williams & Wilkins, Inc.

http://www.ajsp.com/pt/re/ajsp/abstract ... 29!8091!-1

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Angiosarcoma arising in an unirradiated breast with subsequent pituitary metastasis

Clin Breast Cancer. 2007 Oct

Rozen WM, Mann GB.
Department of Surgery, Royal Melbourne Hospital, Parkville, Victoria, Australia. warrenrozen@hotmail.com

Key words: Breast-conserving surgery, Mastectomy, Radiation therapy

Angiosarcoma of the breast is a rare condition with known risk factors. The Stewart-Treves syndrome describes angiosarcoma of the arm or breast in the setting of adjuvant radiation therapy (RT) after breast conservation surgery. Angiosarcoma of the breast, in the absence of RT, is rarer still. We present a unique case of angiosarcoma of the breast, diagnosed 6 years after conservative surgery for carcinoma of the breast when no adjuvant RT was given. Chronic postoperative lymphoedema in the breast delayed the investigation of skin changes, with an eventual diagnosis of angiosarcoma. An isolated pituitary metastasis 9 months from the diagnosis, the first such reported case, adds to the complexity of the case. The diagnosis and management of angiosarcoma of the breast is discussed. Angiosarcoma is a rare occurrence after breast conservative surgery. Postoperative lymphoedema can impede early diagnosis. The disease can display unique metastatic potential.

http://www.cigjournals.com/CIG/c.abs/cl ... icle206768

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Cutaneous angiosarcoma following conservative surgery and radiotherapy for breast carcinoma. A case report

Cesk Patol. 2007 Apr

Kajo K, Lúcan J, Macháleková K, Beratsová Z.
Ustav patologickej anatómie, Jesseniovej lekárskej fakulty, Martinskej fakultnej nemocnice, Martin. karol.kajo@post.sk

Breast angiosarcomas (AS) are very rare neoplasms, which can be divided into primary (or sporadic), and secondary AS, the latter arising either on the base of lymphoedema after mastectomy (so called AS with Stewart-Treves syndrome- ASSTS), or skin AS after breast conservation surgery with subsequent radiotherapy for breast cancer (KPRAS). The authors present a case of a 55-year-old female patient with a 17mm tumour in nipple region, developing 8 years after primary diagnosis of tubulolobular carcinoma of the breast which was treated by breast conservation surgery and radiotherapy. A probatory bioptic examination of the lesion proved well-differentiated KPRAS. The patient underwent mastectomy. The authors analyse the basic clinical and morphological features of KPRAS, which distinguish it from other forms of AS, e.g. occurrence in older age, shorter period of latency after radiation therapy when compared to ASSTS, absence of lymphoedema, and rare involvement of the breast tissue. Prognosis of this entity is very poor; today the most reliable prognostic marker is histological grading. However, it will be needed to assess in the future new indicators of prognosis of patients with this rare disease.

http://www.ncbi.nlm.nih.gov/pubmed/1762 ... d_RVDocSum

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Cutaneous angiosarcoma arising after radiotherapy treatment of a breast carcinoma. Description of a case and review of the literature

Pathologica. 2003 Aug

Di Tommaso L, Fabbri A.
Dipartimento di Oncologia, Sezione di Anatomia Patologica Marcello Malpighi, Ospedale Bellaria, Università di Bologna, via Altura 3, 40139 Bologna. ldito2001@yahoo.it

Cutaneous angiosarcomas occurring as a complication of radiation therapy for breast cancer are rare. To the best of our knowledge 37 cases have been reported in English literature during the last two decades. We describe a case of angiosarcoma arising in the skin of a breast previously irradiated for breast carcinoma. The patient, a 74-year-old woman, had been treated with conservative surgery and radiotherapy for a pT1N0 infiltrating ductal carcinoma of the left breast in 1993. Nine years later a skin lesion, which was interpreted as a recurrence, arose in the same site. A biopsy obtained from the lesion, and initially considered to be negative for cancer, showed istological features consistent with those of the atypical vascular lesion. Seven months later the same lesion transformed in a 5 x 3 reddish mass which at histological examination was diagnosed as an high grad post-irradiation angiosarcoma of the skin of the breast. Data on previously reported post iradiation angiosarcoma of the breast are reviewed; further consideration on differential diagnosis between those lesions and atypical vascular lesions are presented.

http://www.ncbi.nlm.nih.gov/pubmed/1457 ... PlusDrugs2

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See also:

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Angiosarcoma and Long Term Lymphedema

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Lymphedema After Cancer - How Serious Is It?

http://www.lymphedemapeople.com/wiki/do ... ious_is_it

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Re: Sporadic Cutaneous Angiosarcomas

Postby patoco » Thu Nov 17, 2011 2:00 pm

Sporadic cutaneous angiosarcomas generally lack hypoxia-inducible factor 1alpha: a histologic and immunohistochemical study of 45 cases.

Ann Diagn Pathol. 2010 Feb

Abedalthagafi M, Rushing EJ, Auerbach A, Desouki MM, Marwaha J, Wang Z, Fanburg-Smith JC.


Department of Pathology, Georgetown University Medical Center, Washington, DC 20007, USA.


Cutaneous angiosarcoma (AS) is a rare malignant neoplasm of dermis composed of infiltrating cells of endothelial phenotype with overall poor prognosis. Although autocrine stimulation by vascular endothelial growth factor secretion may play a role in the pathogenesis of angiosarcoma, its mechanism has not been fully established. Hypoxia-inducible factor-1 (HIF-1) is a transcription factor that mediates cellular and systemic homeostatic responses to hypoxia.. The stability of HIF can regulate key proteins in angiogenesis and the alpha-subunit has been found in epithelial tumors, only 1 case of human retroperitoneal angiosarcoma, and rare vascular proliferations and tumors in knockout mice. We wanted to observe the utility of HIF-1alpha as a marker or explanatory factor in AS. Cases coded as "angiosarcoma" of dermis were culled and re-reviewed for inclusion as AS, based on patient folder, slides, and obtained immunohistochemistry including CD31 and smooth muscle actin (SMA). Hypoxia-inducible factor-1alpha was performed on a subset of cases, with additional available material. Forty-five cases met the criteria for AS; there were 17% females and 83% males, with a mean age at presentation of 67 years (range, 27-88 years). Tumors presented most commonly in the skin of the scalp followed by the left lower leg, face, nose, lower arm, neck, thigh, eyelid, ear, and temple. Associated basal cell carcinoma was noted in 1 patient; no others had other neoplasms or unrelated surgeries. There was no history of other primary, lymphedema, radiation, breast-associated, or thorotrast-induced angiosarcoma. The tumors ranged in size from 0.4 up to 9.5 cm, with a mean size of 2.4 cm. Histopathologically, most tumors were vasoformative, with either solid architecture (n = 35) or papillary endothelial hyperplasia-like foci (n = 7). All cases demonstrated infiltrative growth pattern, cytologic atypia, and mitotic activity, including atypical forms. Surface ulceration was present in 44% and solar elastosis in the most evaluable cases. Epithelioid morphology was present in 29% (n = 13) cases. Mild to moderate lymphocytic inflammatory response was noted in 62% (n = 28) cases. CD31 highlighted malignant endothelial cells. SMA (for pericytes) was generally absent. Hypoxia-inducible factor 1alpha was focally positive in cytoplasm of 3 of 18 (17%) cases studied. Treatment and follow-up data were only available on 4 cases: 2 died of disease within 4 years, 2 others had known recurrence within 2 years. Cutaneous angiosarcoma is largely found on the scalp of older individuals. Requirement for diagnosis includes extravascular proliferation of atypical endothelial cells with mitotic activity in vasoformative, solid, and papillary patterns. Absence of SMA can prove extravascular extension of tumor, outside their normal vessel confines. Cutaneous angiosarcoma generally lacks HIF-1alpha expression. Accordingly, the hypoxic response pathway is not thought to be a documentable common mechanism of angiogenesis in this entity.


Annals of Diagnostic Pathology
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Re: Sporadic Cutaneous Angiosarcomas

Postby patoco » Thu Nov 17, 2011 2:03 pm

A Bruise-Like Patch to the Forehead and Scalp

Ravneet Ruby Kaur, Hilary Mandel
Authors and Disclosures

Posted: 09/10/2009; Dermatology Nursing. 2009

History: An 82-year-old man with a history of nonmelanoma skin cancer presented for a skin check.

Description: He had an enlarging purpuric ovoid patch of the right hairline for 2.5 months. On initial examination, the lesion was 4 cm x 1.5 cm. The patient denied pain, pruritus, bleeding, trauma, or previous radiation to the area. The patient was not on any anti-coagulants, corticosteroids, or aspirin, but was taking non-steroidal antiinflammatory drugs daily for joint pain.

One month later, the examination showed a 9.5 x 1.5 cm smooth, linear purpuric patch at the right hairline and frontal scalp. Figure 1 shows the lesion at the time of initial presentation. The lesion did not appear to be consistent with a melanocytic process, such as a nevus or melanoma, and had a vascular appearance. Laboratory analysis including a complete blood count with platelets, chemistry panel, and liver function tests were unremarkable. The chest x-ray was negative. A shave biopsy was performed. Figure 2 shows the pathology specimen at a magnification of 4X.

Differential Diagnoses: Post-traumatic bruise, senile purpura, hobnail hemangioendothelioma, Kaposi’s sarcoma (arguably a form of angiosarcoma), radiationinduced vascular proliferation, bacillary angioimatosis.

Diagnosis: Low-grade cutaneous angiosarcoma.

Microscopic Findings and Clinical Course: Histopathologic examination of the biopsy specimen revealed a vascular neoplasm favoring low-grade angiosarcoma, although hobnail hemangioendothelioma was also considered in the differential. The diagnosis was confirmed by the Armed Forces Institute of Pathology.

Excision with split thickness skin graft was performed. Microscopic examination from the excision specimen showed focal atypical vessels and vascular proliferation with rare atypical endothelial cells at the deep margin. The lateral margin was clear. Figure 3 shows the pathology slides specimen at a magnification of 20X. Focal atypical vessels and vascular proliferation with rare atypical endothelial cells can be seen. Of note, actinic keratosis was seen in the non-neoplastic skin. Staging computed tomographic studies of the head, neck, chest, and abdomen were negative for masses, lymphadenopathy, metastases, or hemorrhage. The Tumor, Nodes, Metastasis (TNM) classification of tumor staging was: T2, N0, M0. Six weeks postoperatively the STSG and left thigh donor site had healed well.

Discussion: Angiosarcoma is a rare, aggressive vascular neoplasm (Eiling et al., 2002). Fewer than 5% of soft tumor sarcomas occur in the head and neck, with only approximately 10% classified as angiosarcomas (Pawlik et al., 2003). Most occur in the scalp and face with a strong predilection for elderly White men. Pawlik and colleagues (2003) found that angiosarcoma can occur in the settings of chronic lymphedema, chronic radiation dermatitis, or as a result of severe trauma. Lesions tend to be multifocal, violaceous patches; nodules or plaques with extensive local growth are common. Asgari, Cockerell, and Weitzul (2007) described a useful diagnostic tool in cutaneous angiosarcoma: the Head Tilt Maneuver which highlights the vascular nature of these lesions. The maneuver involves lowering the patient’s head below the level of the heart, which serves to intensify the erythema of the vascular lesions. This easy noninvasive maneuver can help facilitate early diagnosis and gain a better appreciation of the clinical extent of the tumor.

Elder, Elenitsas, Johnson, and Murphy (2005) found that metastasis to regional lymph nodes and lungs may occur. Five-year survival is between 12% and 33%. Zanetta and colleagues (2005) suggested that a decrease in cell adhesion molecules such as cadherins, which are calcium-ion dependent adhesive proteins located at intercellular adherens junctions, may contribute to the local invasiveness and potential metastasis of angiosarcoma. Histopathology demonstrates irregular anastomosing vascular channels lined by atypical endothelial cells with frequent mitotic figures. Nuclear atypia is present with large atypical hyperchromatic cells (Weedon, 2002).

Elder and colleagues (2005) found that hobnail hemangioendothelioma, in contrast, shows ectatic or dilated vascular channels lined by plump endothelial cells in the upper dermis with intraluminary papillary projections. Distinguishing features of Kaposi sarcoma include benign-appearing endothelial cells in the vascular channels with absence of mitoses, slit-like vascular spaces, eosinophilic globules, and a plasma cell infiltrate.

Elder and colleagues (2005) agreed with Ohsawa and colleagues (1995) that CD31 is a specific immunohistochemical marker for endothelial cell differentiation and CD34 is also sensitive. Other useful endothelial cell markers include Factor VIII-Related Antigen and Ulex europaeus lectin type 1. Also, the absence of von-Willebrand Factor labeling and a decrease in Weibel-Pallade bodies favor a lymphatic endothelial origin of angiosarcoma (Elder et al., 2005).

Pawlik and colleagues (2003) contended that the gold standard of treatment is excision although complete removal is difficult because of extension beyond the apparent clinical lesion. It is difficult to obtain negative surgical margins, only seen in less than 21.4% in one study. Eiling and colleagues (2002) found that recurrence is frequent, requiring close clinical followup. Other treatment options include radiation therapy and chemotherapy with liposomal doxorubicin. Pestoni, Paredes-Saurez, Peteiro, and Toibio (2005) found paclitaxel to be effective as well. In a recent study based on 69 cases of sporadic cutaneous angiosarcoma, estimated 5-year disease-specific survival was 48% (Deyrup, McKenney, & Tighiouart, 2008).

Conclusion: It is important not to overlook ecchymotic lesions of recent onset, especially without a history of trauma. Skin biopsies should be performed on these lesions with a high index of suspicion for angiosarcoma. Since the disease is rare, standard protocols have not been developed. We recommend that patients diagnosed with cutaneous angiosarcoma be followed closely every 3 to 6 months for the first 2 years and then every year for life after. Special attention should be paid to lymph nodes and other sites for metastasis.

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Re: Sporadic Cutaneous Angiosarcomas

Postby patoco » Thu Nov 17, 2011 2:05 pm

Pediatric Cutaneous Angiosarcomas: A Clinicopathologic Study of 10 Cases

American Journal of Surgical Pathology:
January 2011 - Volume 35 - Issue 1 - p 70–75

Deyrup, Andrea T. MD, PhD*; Miettinen, Markku MD†; North, Paula E. MD, PhD‡; Khoury, Joseph D. MD§; Tighiouart, Mourad PhD∥; Spunt, Sheri L. MD¶,♯; Parham, David M. MD**; Shehata, Bahig M. MD††; Weiss, Sharon W. MD††


Cutaneous angiosarcomas are rare tumors, which predominantly arise in the sun-exposed skin of the head and neck of adult and elderly patients. Rarely, these tumors can be seen in children. We identified cutaneous angiosarcomas in 10 children and assessed clinical (patient age, tumor site, tumor size, and tumor focality) and histologic features including growth pattern (vasoformative vs. solid), mitotic rate (mitotic figures per 10 high power field), necrosis (present vs. absent), and cell shape (epithelioid vs. nonepithelioid). Tumors predominated in the lower extremities (6 of 10) of female patients (2 male and 8 female); age at diagnosis ranged from 1.5 months to 15 years. Four patients had preexisting conditions: congenital hemihypertrophy of the contralateral limb, the Aicardi syndrome, congenital lymphedema, and congenital hemangioma treated with radiation therapy. Tumors were located in the lower extremity (6), flank (1), elbow (1), and buccal mucosa (1), and ranged in size from 0.6 to 6.5 cm. Eight cases showed predominantly epithelioid morphology, 1 case showed mixed epithelioid and spindled morphology and 1 case was entirely spindled. Mitotic activity ranged from 1 to 55 mitotic figures per 10 high power field. Necrosis was seen in 5 cases. Clinical follow-up was obtained for 9 patients: 4 died of disease (range, 12 to 49 mo; mean, 25 mo) and 5 patients were alive without disease (18 mo to 28 y). Five patients had metastatic disease; sites of involvement included the lung, soft tissue, lymph node, pleura, liver, and bone. Cutaneous angiosarcomas in children are rare tumors, which are commonly associated with a preexisting condition, suggesting a greater role for genetics as opposed to environmental factors in the pathogenesis of these tumors.

American Journal of Surgical Pathology

http://journals.lww.com/ajsp/Abstract/2 ... __A.8.aspx
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