Cutaneous angiosarcoma of the buttock complicated by severe thrombocytopenia: A case report

  • Authors:
    • Kaoru Nagao
    • Kayo Suzuki
    • Taketoshi Yasuda
    • Takeshi Hori
    • Jun Hachinoda
    • Masahiko Kanamori
    • Tomoatsu Kimura
  • View Affiliations

  • Published online on: July 2, 2013
  • Pages: 903-907
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Angiosarcoma (AS) is an aggressive, malignant endothelial cell tumor of vascular or lymphatic origin, the presentation and clinical behavior of which may vary according to its location. This is the case report of a 56‑year‑old woman with cutaneous angiosarcoma (CAS) of the buttock complicated by severe thrombocytopenia. A review of the literature revealed that only nine cases of CAS with thrombocytopenia have been previously reported. The prognosis of CAS complicated by thrombocytopenia is poor, even after treatment with combined chemotherapy and radiotherapy (RT). The composite karyotype was 46,XX,t(12;20)(p13;p11.2)[3]/47,X,add(X)(q13),del(6)(q?),add(12)(p13),‑21,+2mar[2]/45,XX,der(1)add(1)(p36.3)del(1)(q41),‑20[1]/46,XX[13]. Only 13 cytogenetic cases of AS, including the present case, have been reported in the English literature thus far. In this case report, the clinical presentation and cytogenetic findings are described and the relevant literature on AS is reviewed.


Angiosarcoma (AS) is an aggressive, malignant endothelial cell tumor of vascular or lymphatic origin (1). The presentation and clinical behavior of AS may vary, depending on its location. Therefore, ASs are considered as several closely related tumors rather than a single entity and may be divided into several groups. Cutaneous AS (CAS) is a rare aggressive tumor of the scalp and face of elderly patients, which spreads widely, recurring locally with early metastasis (2). Cases of thrombocytopenia during tumor progression and metastasis related to intratumor sequestration have been rarely reported (38). The present study describes a case of CAS of the buttock complicated by severe thrombocytopenia and reviews the literature regarding the clinical behavior of this entity. The study was conducted following a clinical research review by our Ethics Committee. The patient was informed that data from the case would be submitted for publication and provided the required consent.

Case report

A 56-year-old female sought medical assistance due to a 2-month history of dark reddish eruptions and pain in her left buttock. There was no reported history of disease or trauma. Three discrete eruptions without edema were identified on the skin. The surface was smooth with minor inflammation and the borders were poorly defined. The lesions were tender to palpation. The radiological and laboratory findings were normal. A biopsy was performed and sheets of highly atypical, mitotically active, epithelioid endothelial cells were observed under a light microscope, with formation of irregular sinusoidal vascular channels (Fig. 1). The lesion was diagnosed as epithelioid hemangioendothelioma based on the histopathological findings.

Wide surgical resection was performed; however, the patient experienced seven local recurrences over a period of seven years. Whenever the tumor recurred, it was surgically resected. After the third resection the tumor was histopathologically re-evaluated and the diagnosis was changed to AS (Fig. 2). Immunohistochemically, tumor cells were positive for CD31 and negative for CD34. The specimen was submitted for cytogenetic analysis. Six years after the onset, the tumor had significantly enlarged. Following the seventh surgery, radiotherapy (RT, 60 Gy) and intensive multidrug chemotherapy [MAID (doxorubicin 30 mg/m2, ifosfamide 1.5 g/m2 and dacarbazine 300 mg/m2) × 3 days] were administered (9). However, one year after the RT the patient developed local recurrence, inguinal lymph node metastasis and multiple lung metastases. Lymphedema also developed due to femoral lymphatic vessel obstruction. Nine years after the initial operation, lymph node dissection and free-skin grafting were performed during the ninth resection procedure. However, the tumor subsequently recurred around the grafted skin and grew progressively, with secondary necrosis and hemorrhage. The hemorrhage persisted for 2 months and the patient developed severe anemia (5.2 g/dl) and thrombocytopenia (52,000/μl). Despite supportive care with hemorrhage control and transfusion of platelets and erythrocytes, anemia and thrombocytopenia continued to progress rapidly. The patient eventually developed disseminated intravascular coagulation and succumbed to the disease 6 weeks after the ninth surgery (9 years and 6 months after the initial consultation).

Cytogenetic analysis was performed on G-band by trypsin banding using surgical specimens. Standard culture and harvesting procedures were used, as previously described (10). The karyotypes were expressed in accordance with the International System for Human Cytogenetic Nomenclature (11). Twenty cells were analyzed and the composite karyotype was 46,XX,t(12;20)(p13;p11.2)[3]/47,X,add(X)(q13),del(6)(q?),add(12)(p13),−21,+2mar[2]/45,XX,der(1)add(1)(p36.3)del(1)(q41),−20[1]/46,XX[13] (Fig. 3).


ASs are, collectively, one of the rarest types of soft tissue tumors. They comprise <1% of all sarcomas. Among all cases of AS, one third occur in the skin, one fourth in soft tissue and the remainder in other sites. Since the clinical behavior of AS varies depending on its location, ASs are divided into several clinical groups. CAS without lymphedema is the most frequently encountered type of AS. It usually develops after the age of 60 years and more commonly affects females. Due to the fact that AS readily invades the surrounding tissues, complete resection is difficult. The prognosis of CAS is poor, with a 5-year survival rate of 10–34% (1214).

Only 9 cases of CAS with thrombocytopenia, including the present case, have been described in the English literature thus far (38) (Table I). The mean age at presentation was 72.7 years (range, 56–87 years) and it occurred more commonly among males. The tumors were located in the scalp (5/9, 55.6%), face (2/9, 22.2%) and trunk (2/9, 22.2%). The prognosis of CAS complicated by thrombocytopenia is poor, even following treatment with combined chemotherapy and RT. The average period from the initial diagnosis to the development of thrombocytopenia was 4.1 months (range, 0–10 months) and the average survival was 9.7 months (range, 3–36 months). Of the 9 reported cases of CAS with thrombocytopenia (including the present case), all the patients succumbed to the disease within 4 months after the onset of thrombocytopenia. Our patient survived for 76 months subsequent to the initial consultation but succumbed to the disease 2 months following tumor necrosis. These findings suggest that local control of the tumor is critical.

Table I

Clinical characteristics of cutaneous angiosarcoma with thrombocytopenia.

Table I

Clinical characteristics of cutaneous angiosarcoma with thrombocytopenia.

CaseAge (years)/genderLocationTreatmentDurationa (months)Outcome (months)Authors (Refs.)
166/FFaceRE0DOD (3)Arcomano et al(3)
259/MScalpCT, RT7DOD (10)Satoh et al(4)
379/MFaceRT, CT3DOD (4)
479/MChestCT2DOD (6)
569/MScalpRE, RT, CT3DOD (4)Salameh et al(5)
667/MScalpCT, RT10DOD (36)Imafuku et al(6)
776/MScalpRT, CT7DOD (9)Kluger et al(7)
887/MScalpRT, CT1DOD (3)Tan et al(8)
956/FButtockRE, CT, RT72DOD (76)Present case

a Duration indicates the time from the first consultation to the development of thrombocytopenia.

{ label (or @symbol) needed for fn[@id='tfn2-mco-01-05-0903'] } F, female; M, male; RE, resection; CT, chemotherapy; RT, radiotherapy; DOD, dead of disease.

The present case describes the development of thrombocytopenia in a patient with metastatic AS of the buttock. The mechanism of thrombocytopenia is considered to be related to the Kasabach-Merritt syndrome (KMS) (15). KMS has been used to describe various tumors that broadly fit the initial description (16), such as thrombocytopenia associated with giant hemangioendothelioma (5), intestinal angioma (17), hepatic epithelioid hemangioendothelioma (18), occult visceral hemangiomatosis (19) and AS (20). KMS is defined as consumption of platelets within tumors due to the accumulation of blood in the tumor vasculature and platelet accretion to the abnormal vascular endothelium (16,20). In our case, tumor growth and recurrence led to KMS secondary to hyperplasia of the abnormal blood vessels and continuous bleeding due to tumor necrosis.

Only a few cases of AS have been cytogenetically investigated (2129). The cytogenetic findings of AS are occasionally complex and limited. However, certain cytogenetic changes reported in tumors from different locations revealed similarities (Table II). The most common changes were gain of 5pter-p11, 8p12-qter and 20pter-q12, losses of 4p, 7p15-pter and Y and abnormalities in 22q (1). In our case, t(12;20)(p13;p11.2) including a site of 20pter-q12 was observed.

Table II

Chromosomal changes in angiosarcoma.

Table II

Chromosomal changes in angiosarcoma.

CaseAge (years)/genderLocationKaryotype (no. of cells)Authors (Refs.)
152/FSoft tissue 41,XY,−3,−6,−6,+der(7)t(6;7)(p21.1;p22),−17,−17,−20[1]/45,XX,−1,+2,−22[1]/45,XX,−6[1]/45,XX,−10[1]/45, XX,−13[1]/45,XX,−14[1]/45,XX,−16[1]/45,XX,−20[1]/45,XX,−22[1]/45,X,−X[1]/46,X,+del(1)(q21), t(6:8) (q22–23;q24),−X[1]/46,XXt(1;12)(p34.1–34.3;q13)[1]/46,XX,t(5;?)(p13;?)[1]/46,X,t(5;19)(q21–22;p13.3), −X,+mar[1]/46,XX,t(10;17)(q11.2;p11.2)[1]/47,XX,t(3;21)(p21;p13),del(7)(q21),+19[1]/47,XX, +3,t(X;10)(q28;q11.2)[1]/48,XX,+2,+7[1]/46,XX[13]Kindblom et al(21)
234/FOvary 48,XX,t(1;3)(q11;p11),+3,+12Fletcher et al(22)
361/FBreast 47,X,der(X)t(X;9)(q11;q12),t(1;3)(p13;q29),t(1;17)(p13;p13),−2,−2,del(4)(q25q31), +der(4)del(4)(p14)t(2;4)(p13;q35),del(7)(p11),add(8)(q13),+12,der(15)t(5;15)(p11;p11), +der(20)t(6;20)(q13;q23),der(22)t(6;22)(q15;q13)Gil-Benso et al(23)
4NASoft tissue 46,XX,add(1)(p36),t(1;3)(q32;q21),del(2)(p21),del(7)(p15),add(12)(q13),add(17)(q24)Van den Berg et al 24)
567/MKidney 46,XY,inv(7)(p15q11)[2]/46–47,X,−Y,del(1)(q25),add(4)(q25),add(5)(q31),+mar1[cp15]/46,XY[3]Cerilli et al(25)
641/MSoft tissue 39–43,XY,der(1;11)(q10;q10),−4,t(5;15)(q11;q24),+der(8)t(8;?17)(p12;q11)add(8)(q24), −9,−11,−13,−17,−17,−18,add(18)(p11),+del(20)(q12–13),+der(?)t(?;10)(?;q22)[cp9]/46,XY[2]Schuborg et al(26)
769/FSoft tissue 45–47,XX,add(8)(q22),+add(22)(q12),inc[cp9]
875/MSoft tissue 46,XY,der(7)del(7)(p15)del(7)(q11q22)[3]/47,XY,der(7),+mar[2]/45,X, −Y[13]/47,XY,+8[7]/48,XY,+X,+8[6]
9a71/FSoft tissue 73–79,XX,−X,add(1)(q11),−3,+5,t(5;14)(q13;p13),+6,+7,+8,+9,+9, (primary) der(9;10)(q10;q10)x2,+11,−13,+20,−21,−22,inc[cp3]/46,XX[11]
9b71/FSoft tissue (recurrence) 77–79,XXX,add(1),−3,i(5)(q10),t(5;14),+6,+6,+7,der(9;10)x2, +11,add(15)(p11),+16,+20,inc[2]/46,XX[p]Wong et al(27)
1034/FNasopharynx 43,XX,?dup(3)(q21q29),del(4)(p11p16),−8,der(9;17)(q10;q10),−13,add(14)(q32), −16,add(16)(q22),der(18)t(1;18)(q21;q21),−19,−20,add(22)(q13),+r,+mar1,+mar2[4]/43, idem, −14,+20[3]/33–43,idem,−3,−4,−5,−6,−7,−10,−11,−16,−18,−20[cp10]/61–84,XXX,?dup(3),+?dup(3), del(4),+del(4),+5,+7,−8,der(9;17),?i(9)(q10),+10,+11,+12,+14,+15,−16,add(16)x2,−17,add(18), add(19)(p13),−20,add(22),+add(22),+rx2,+mar1×2,+mar2×2[cp6]/46,XX[3]
1129/MHeart 55,XY,+der(1;17)(q10;q10),+2,+7,+8,+8,+19,+20,+21,+22Zu et al(28)
1279/FBone 46,XX,t(1;14)(p21;q24)[16]/46,XX[3]Dunlap et al(29)
1356/FCutaneous 46,XX,t(12;20)(p13;p11.2)[3]/47,X,add(X)(q13),del(6)(q?),add(12)(p13),−21, +2mar[2]/45,XX,der(1)add(1)(p36.3)del(1)(q41),−20[2]/46,XX[13]Present case

[i] F, female; M, male, NA, not available.

Treatment of CAS is challenging and an optimal treatment protocol has not yet been determined. The treatment usually consists of surgical excision with wide safety margins and postoperative radiation (2,30), although radiation monotherapy appears to be insufficient. In addition, the role of chemotherapy remains undetermined and definitive application of adjuvant modalities has not yet been established (2,30). Additional comprehensive studies on the treatment of CAS are required.


This study was supported in part by Grants-in-Aid for Scientific Research (C) 24592227 (KAKENHI).



Weiss SW and Goldblum JR: Angiosarcoma. Enzinger and Weiss's Soft Tissue Tumors. Weiss SW and Goldblum JR: Mosby; St. Louis: pp. 703–720. 2007


Mendenhall WM, Mendenhall CM, Werning JW, Reith JD and Mendenhall NP: Cutaneous angiosarcoma. Am J Clin Oncol. 29:524–528. 2006. View Article : Google Scholar : PubMed/NCBI


Arcomano MA, Shulkin BL, Petry NA and Wahl RL: Metastatic angiosarcoma with thrombocytopenia and intratumoral indium-111-platelet deposition. J Nucl Med. 32:2278–2280. 1991.PubMed/NCBI


Satoh T, Takahashi Y, Yokozeki H, Katayama I and Nishioka K: Cutaneous angiosarcoma with thrombocytopenia. J Am Acad Dermatol. 40:872–876. 1991. View Article : Google Scholar


Salameh F, Henig I, Bar-Shalom R and Maza I: Metastatic angiosarcoma of the scalp causing Kasabach-Merritt syndrome. Am J Med Sci. 333:293–295. 2007. View Article : Google Scholar : PubMed/NCBI


Imafuku S, Hosokawa C, Moroi Y and Furue M: Kasabach-Merritt syndrome associated with angiosarcoma of the scalp successfully treated with chemoradiotherapy. Acta Derm Venereol. 88:193–194. 2008. View Article : Google Scholar : PubMed/NCBI


Kluger N, Girard C, Boissier E, Sibille L, Mariano-Goulart D and Guillot B: Metastatic cutaneous angiosarcoma complicated with severe thrombocytopenia. Eur J Dermatol. 20:662–663. 2010.PubMed/NCBI


Tan SM, Tay YK, Liu TT and Mancer K: Cutaneous angiosarcoma associated with the Kasabach-Merritt syndrome. Ann Acad Med Singapore. 39:941–942. 2010.PubMed/NCBI


Elias A, Ryan L, Sulkes A, Collins J, Aisner J and Antman KH: Response to mesna, doxorubicin, ifosfamide, and dacarbazine in 108 patients with metastatic or unresectable sarcoma and no prior chemotherapy. J Clin Oncol. 7:1208–1216. 1989.PubMed/NCBI


Yasuda T, Nishio J, Sumegi J, et al: Aberrations of 6q13 mapped to the COL12A1 locus in chondromyxoid fibroma. Mod Pathol. 22:1499–1506. 2009. View Article : Google Scholar : PubMed/NCBI


Shaffer LG and Tommerup N: An International System for Human Cytogenetic Nomenclature. S Karger; Basel: 2005


Holden CA, Spittle MF and Jones EW: Angiosarcoma of the face and scalp, prognosis and treatment. Cancer. 59:1046–1057. 1987. View Article : Google Scholar : PubMed/NCBI


Mark RJ, Poen JC, Tran LM, Fu YS and Juillard GF: Angiosarcoma. A report of 67 patients and a review of the literature. Cancer. 77:2400–2406. 1996. View Article : Google Scholar : PubMed/NCBI


Morgan MB, Swann M, Somach S, Eng W and Smoller B: Cutaneous angiosarcoma: a case series with prognostic correlation. J Am Acad Dermatol. 50:867–874. 2004. View Article : Google Scholar : PubMed/NCBI


Kasabach H and Merritt K: Capillary hemangioma with extensive purpura. Am J Dis Child. 59:1063–1070. 1940. View Article : Google Scholar


Hall GW: Kasabach-Merrit syndrome: pathogenesis and management. Br J Haematol. 112:851–862. 2001. View Article : Google Scholar : PubMed/NCBI


Bhandari B, Gupta BM and Agarwal SS: Giant haemangioendothelioma with purpura (Kasabach-Merrit syndrome). (A case report). Indian Pediatr. 9:718–720. 1972.PubMed/NCBI


Eugene C, Fingerhut A, Quevauvilliers J, et al: Microangiopathic anaemia with thrombocytopaenia. Cure following excision of an angioma of the small intestine (author's transl). Nouv Presse Med. 7:735–737. 1978.(In French).


Frider B, Bruno A, Selser J, Vanesa R, Pascual P and Bistoletti R: Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association. J Hepatol. 42:282–283. 2005. View Article : Google Scholar : PubMed/NCBI


Schulz AS, Urban J, Gessler P, Behnisch W, Kohne E and Heymer B: Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas. Eur J Pediatr. 158:379–383. 1999. View Article : Google Scholar : PubMed/NCBI


Kindblom LG, Stenman G and Angervall L: Morphological and cytogenetic studies of angiosarcoma in Stewart-Treves syndrome. Virchows Arch A Pathol Anat Histopathol. 419:439–445. 1991. View Article : Google Scholar : PubMed/NCBI


Fletcher JA, Kozakewich HP, Hoffer FA, et al: Diagnostic relevance of clonal cytogenetic aberrations in malignant soft-tissue tumors. N Engl J Med. 324:436–442. 1991. View Article : Google Scholar : PubMed/NCBI


Gil-Benso R, Lopez-Gines C, Soriano P, Almenar S, Vazquez C and Llombart-Bosch A: Cytogenetic study of angiosarcoma of the breast. Genes Chromosomes Cancer. 10:210–212. 1994. View Article : Google Scholar : PubMed/NCBI


Van den Berg E, Van Oven MW, de Jong B, et al: Comparison of cytogenetic abnormalities and deoxyribonucleic acid ploidy of benign, borderline malignant, and different grades of malignant soft tissue tumors. Lab Invest. 70:307–313. 1994.PubMed/NCBI


Cerilli LA, Huffman HT and Anand A: Primary renal angiosarcoma: a case report with immunohistochemical, ultrastructural, and cytogenetic features and review of the literature. Arch Pathol Lab Med. 122:929–935. 1998.PubMed/NCBI


Schuborg C, Mertens F, Rydholm A, et al: Cytogenetic analysis of four angiosarcomas from deep and superficial soft tissue. Cancer Genet Cytogenet. 1:52–56. 1998. View Article : Google Scholar : PubMed/NCBI


Wong KF, So CC, Wong N, Siu LL, Kwong YL and Chan JK: Sinonasal angiosarcoma with marrow involvement at presentation mimicking malignant lymphoma: cytogenetic analysis using multiple techniques. Cancer Genet Cytogenet. 129:64–68. 2001. View Article : Google Scholar


Zu Y, Perle MA, Yan Z, Liu J, Kumar A and Waisman J: Chromosomal abnormalities and p53 gene mutation in a cardiac angiosarcoma. Appl Immunohistochem Mol Morphol. 9:24–28. 2001.PubMed/NCBI


Dunlap JB, Magenis RE, Davis C, Himoe E and Mansoor A: Cytogenetic analysis of a primary bone angiosarcoma. Cancer Genet Cytogenet. 194:1–3. 2009. View Article : Google Scholar : PubMed/NCBI


Abraham JA, Hornicek FJ, Kaufman AM, et al: Treatment and outcome of 82 patients with angiosarcoma. Ann Surg Oncol. 14:1953–1967. 2007. View Article : Google Scholar : PubMed/NCBI

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Nagao, K., Suzuki, K., Yasuda, T., Hori, T., Hachinoda, J., Kanamori, M., & Kimura, T. (2013). Cutaneous angiosarcoma of the buttock complicated by severe thrombocytopenia: A case report. Molecular and Clinical Oncology, 1, 903-907.
Nagao, K., Suzuki, K., Yasuda, T., Hori, T., Hachinoda, J., Kanamori, M., Kimura, T."Cutaneous angiosarcoma of the buttock complicated by severe thrombocytopenia: A case report". Molecular and Clinical Oncology 1.5 (2013): 903-907.
Nagao, K., Suzuki, K., Yasuda, T., Hori, T., Hachinoda, J., Kanamori, M., Kimura, T."Cutaneous angiosarcoma of the buttock complicated by severe thrombocytopenia: A case report". Molecular and Clinical Oncology 1, no. 5 (2013): 903-907.