Angiosarcomas recurrence after surgical excision and radiother- Elisa Cinotti, Franco Rongioletti apy. In one case, the accompanying dense infl am- matory infi ltrate was attributable to a superimposed Cutaneous angiosarcoma is a rare, aggressive infection by Pseudomonas aeruginosa . vascular sarcoma that occurs in three main differ- Pathology : It is characterized by the same ent clinical settings: classic cutaneous angiosar- atypical vessels of the classical angiosarcoma, coma arising in sun-damaged skin of elderly with the addition of a prominent infl ammatory patients, cutaneous angiosarcoma associated lymphoid infi ltrate between the vessels, obliterat- with chronic lymphedema, and post radiation ing some or most of the channels (Fig. 2 ). The angiosarcoma. Recent studies have shown that infi ltrate can be diffuse or can be organized in high-level amplifi cation of MYC oncogene seems lymphoid follicles with germinal centers scat- to be specifi c for radiation and lymphedema- tered within the diffuse lymphoid infi ltrate. associated angiosarcoma. A new histological Vessels are poorly circumscribed, irregularly variant has been named pseudolymphomatous dilated, and anastomosing, lined by prominent, cutaneous angiosarcoma. In general, cutaneous atypical endothelial cells (Fig. 3 , 4 ) that usually angiosarcoma carries a poor prognosis, associ- express CD31 (Fig. 5 ), CD34, and D2-40. Most ated with 5-year overall survival rates between 10 of the cells of the lymphoid infi ltrate express and 30 %. strong immunoreactivity for CD3, CD4, CD5, Pseudolymphomatous angiosarcomas and CD45 markers, whereas only scattered cells Synonyms: Angiosarcoma with prominent express CD8. Most of the lymphocytes of the lymphocytic infi ltrate. germinal centers are positive for CD20, CD21, Introduction: Pseudolymphomatous cutane- CD79a, and Bcl-6 whereas Bcl-2 can be detected ous angiosarcoma, described by Requena et al . in in the cells of the mantle zone. 2007, is characterized by a prominent infl amma- Differential diagnosis: The main histological tory lymphoid infi ltrate that can mask the under- differential diagnoses are: (1) cutaneous B-cell lying vascular malignant proliferation and mimic follicle center lymphoma, (2) pseudolymphoma, a lymphomatous process. (3) Kimura's disease/angiolymphoid hyperplasia Clinical features: It presents with the same with eosinophilia (ALHE), and (4) Kaposi clinical features as the classical angiosarcoma, as sarcoma. a bruise-like areas or erythematous- violaceous Pseudolymphomatous angiosarcoma can nodule or plaque of the scalp, face and breast mimic both cutaneous lymphoma and pseudo- (Fig. 1 ). Three cases resembled facial rosacea, lymphoma, and the recognition of irregular anas- one case developed seven years after breast radio- tomosing vascular spaces lined by prominent therapy. Rongioletti el al . described a collision endothelial cells among the lymphocytic infi l- tumor with a basal cell carcinoma, and a transfor- trate allows the correct diagnosis. In particular, mation of an histologically- classical cutaneous among lymphomas the differential diagnosis is angiosarcoma in a pseudolymphomatous type in a mainly with cutaneous follicle center B-cell F. Rongioletti et al. (eds.), Rare Malignant Skin Tumors, DOI 10.1007/978-1-4939-2023-5, 335 © Springer Science+Business Media New York 2015 336 Angiosarcomas Fig. 1 Pseudolymphomatous cutaneous angiosarcoma. Fig. 4 Pseudolymphomatous cutaneous angiosarcoma An erythematous-violaceous plaque with bruise-like fea- The atypical cells are obscured by the dense lymphoid tures on the forehead infi ltrate Fig. 2 Pseudolymphomatous cutaneous angiosarcoma. A Fig. 5 Pseudolymphomatous cutaneous angiosarcoma prominent infl ammatory lymphoid infi ltrate between the The atypical endothelial cells are outlined by CD31 vessels, obliterating some or most of the channels expression lymphoma where the infi ltrate can be diffuse and/ or organized in follicles. The demonstration of a clonal rearrangement of heavy-chain immuno- globulin can help the diagnosis. Kimura's dis- ease/ALHE shows a more or less diffuse lymphoid infi ltration with lymphoid follicles and germinal centers in the presence of thick-walled blood vessels lined with “epithelioid” or “histio- cytoid” endothelial cells protruding into the lumen. The presence of eosinophils and the absence of atypical cytologic features of the endothelial cells are clues for Kimura's disease/ ALHE. A spindle-cell component and clusters of Fig. 3 Pseudolymphomatous cutaneous angiosarcoma At higher magnifi cation, vessels are poorly circumscribed, eosinophilic hyaline globules may help to distin- irregularly dilated, and anastomosing guish Kaposi sarcoma. Angiosarcomas 337 Prognosis: Cutaneous angiosarcoma is believed ous angiosarcoma: a rare variant of cutaneous angio- to have the worst prognosis quoad vitam among sarcoma readily mistaken for cutaneous lymphoma. Am J Dermatopathol. 2007;29:342–50. malignant skin tumors, with an overall 5-year sur- 3. Panizzon R, Schneider BV, Schnyder UW. Rosacea- vival rate ranging from 12% to 20% (median sur- like angiosarcoma of the face. Dermatologica. vival 18–28 months). Two cases series have showed 1990;181:252–4. a better prognosis of the pseudolymphomatous 4. Mentzel T, Kutzner H, Wollina U. Cutaneous angio- sarcoma of the face: clinicopathologic and immuno- variant with either longer survival or increased dis- histochemical study of a case resembling rosacea ease-free intervals, and less frequently metastases. clinically. J Am Acad Dermatol. 1998;38:837–40. However, further studies are necessary to confi rm 5. Rongioletti F, Albertini A-F, Fausti V, Cinotti E, the relatively better prognosis of pseudolymphoma- Parodi A, Fraitag S. Pseudolymphomatous cutane- ous angiosarcoma: a report of 2 new cases arising in tous variant of cutaneous angiosarcoma. an unusual setting. J Cutan Pathol. 2013;40: Treatment: Early wide surgical excision of the 848–54. tumour is the treatment of choice. The effi ciency 6. Diaz-Cascajo C, de la Vega M, Rey- Lopez A. Superinfected cutaneous angiosarcoma: a highly of adjuvant radiotherapy has not been established. malignant neoplasm simulating an infl ammatory pro- cess. J Cutan Pathol. 1997;24:56–60. 7. Donghi D, Kerl K, Dummer R, Schoenewolf N, Cozzio A. Cutaneous angiosarcoma: own experience References over 13 years. Clinical features, disease course and immunohistochemical profi le. J Eur Acad Dermatol 1. Mentzel T. Sarcomas of the skin in the elderly. Clin Venereol. 2010;24:1230–4. Dermatol 2011;29:80-90. 8. Maddox JC, Evans HL. Angiosarcoma of skin and 2. Requena L, Santonja C, Stutz N, Kaddu S, Weenig soft tissue: a study of forty-four cases. Cancer. RH, Kutzner H, et al. Pseudolymphomatous cutane- 1981;15;48:1907–21. Index A ES , 150 Acantholytic squamous cell carcinoma (ASCC) PH , 169 differential diagnosis , 4–5 Apocrine adenocarcinoma. See Primary cutaneous pathology , 3–4 apocrine adenocarcinoma prognosis , 5 Apocrine gland carcinoma , 63 pseudoacantholysis , 3, 5 Array comparative genomic hybridization (aCGH) , 234 vs. SCC , 3–5 ASC. See Adenosquamous carcinoma (ASC) treatment , 5 ASCC. See Acantholytic squamous cell carcinoma Adenoacanthoma of the sweat glands , 3 (ASCC) Adenosquamous carcinoma (ASC) ATLL. See Adult T-cell lymphoma/leukemia (ATLL) vs. ASCC , 4 Atrophic DFSP , 139 clinical features , 15 Atypical cellular blue nevus (ACBN) , 241 differential diagnosis , 16–17 Atypical fi broxanthoma (AFX) and mucoepidermoid carcinoma , 16 clinical features , 141–142 pathology , 15–16 differential diagnosis , 144–145 prognosis , 17 pathology , 143–144 treatment , 17 prognosis , 145 Adjuvant radiotherapy treatment , 145 CS , 24 Atypical intradermal smooth muscle neoplasm , 185, 186 DM , 208 Atypical spindle cell lipomatous tumor , 175, 176 fi brosarcoma , 135 MCC , 252 MPNST , 260 B PC , 55 Balloon cell malignant melanoma (BCMM) trichoblastoma , 43 clinical features , 223 Adnexal CS , 23–25 differential diagnosis , 224–225 ADPC. See Digital papillary adenocarcinoma (ADPC) pathology , 223–224 Adult T-cell lymphoma/leukemia (ATLL) prognosis , 225 clinical features , 279, 280 treatment , 225 differential diagnosis , 280 Basal cell carcinoma (BCC) HTLV-1 infection , 279 FEP , 33 pathology , 279, 280 matrical differentiation prognosis , 280–281 clinical features , 37 T-cell non-Hodgkin lymphoma , 279 differential diagnosis , 38 treatment , 281 pathology , 37–38 AFX. See Atypical fi broxanthoma (AFX) prognosis , 38 Aggressive digital papillary adenocarcinoma , 103, 105 treatment , 38 Aggressive pilomatricoma , 55 with a sarcomatous component , 24 Alveolar rhabdomyosarcoma , 189–191 SCC , 9 Amputation with shadow cells , 37, 38 F. Rongioletti et al. (eds.), Rare Malignant Skin Tumors, DOI 10.1007/978-1-4939-2023-5, 339 © Springer Science+Business Media New York 2015 340 Index BCMM. See Balloon cell malignant melanoma (BCMM) clinical features , 7 Bednar tumor , 139 differential diagnosis , 9 Benign trichoblastoma , 42, 43 pathology , 7–9 Berti lymphoma , 291 prognosis , 9 Blastic plasmacytoid dendritic cell neoplasm (BPDCN) treatment , 9 clinical features , 307, 308 Colloid carcinoma , 115 pathology , 307, 308 Complete surgical excision. See also Surgical excision PDC , 307 CS , 24 prognosis , 308 DFSP , 140 treatment , 308 EHE , 159 Borderline melanoma (BM) , 227 FEP , 35 Borrelia infection , 284 malignant mixed tumor of the skin , 79 Bowen’s disease , 17 MEC , 21 BPDCN. See Blastic plasmacytoid dendritic cell myxoid LPS , 179–180 neoplasm (BPDCN) PC , 110 Breast carcinoma pilomatrix