Breast Hemangioma: Unique Presentation in a Patient with Klippel-
Trenaunay-syndrome

Ayesha   Shamim   Siddiqui; Ibtesam      Zafar; Ayesha   Isani   Majeed; Ramish      Riaz

doi:10.2174/1871529X21666210924150832

Abstract

Background: Klippel-Trénaunay-Syndrome (KTS) is characterized by a triad of varicose veins, port-wine stain and soft tissue or bony hypertrophy, and the diagnosis of KTS can be made if any two of these three features are present. Hemangiomas in various locations, e.g., skull, brain, epidural and vertebral hemangioma, mediastinal, colonic hemangioma, intraneural/intramuscular hemangiomas, are reported with KTS.

Case Presentation: Benign vascular tumors may rarely develop malignant transformation as Bugarin- Estrada et al. reported breast angiosarcoma in a patient diagnosed as Klippel-Trenaunay-Syndrome. We reported a case of a 40-year-old female with a known case of Klipple-Trenaunay-Syndrome with left leg varicosities, cutaneous nevus, as well as unfortunate development of deep venous thrombosis and markedly enlarged right breast hemangioma. Due to low incidence or lack of early detection of breast hemangioma, its diagnosis is challenging.

Conclusion: The history of the patient and multi-modality imaging utilization can help in early and accurate diagnosis of diseases leading to better prognosis.

Keywords: Klippel-trenaunay-syndrome, deep venous thrombosis, breast hemangioma, mammogram, sonomammogram, magnetic resonance imaging.

[1] 
Klippel M, Trenaunay P. Du naevus variqueux osteohypertrophique. Arch Gen Med  1900; 185: 641-72.
[2] 
Kundzina L, Lejniece S. Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome: a case report. J Med Case Reports  2017; 11(1): 243.
[http://dx.doi.org/10.1186/s13256-017-1413-1] [PMID: 28823249] 
[3] 
al-Salman MM. Klippel-Trénaunay syndrome: clinical features, complications, and management. Surg Today  1997; 27(8): 735-40.
[http://dx.doi.org/10.1007/BF02384987] [PMID: 9306589] 
[4] 
Kharat AT, Bhargava R, Bashi V, Goyal A. Klippel-trenaunay syndrome: A case report with radiological review. Med J DY Patil Univ  2016; 9: 522-6.
[http://dx.doi.org/10.4103/0975-2870.186069] 
[5] 
Vahidnezhad H, Youssefian L, Uitto J. Klippel-Trenaunay syndrome belongs to the PIK3CA-related overgrowth spectrum (PROS). Exp Dermatol  2016; 25(1): 17-9.
[http://dx.doi.org/10.1111/exd.12826] [PMID: 26268729] 
[6] 
Sung HM, Chung HY, Lee SJ, et al. Clinical Experience of the Klippel-Trenaunay Syndrome. Arch Plast Surg  2015; 42(5): 552-8.
[http://dx.doi.org/10.5999/aps.2015.42.5.552] [PMID: 26430625] 
[7] 
Tsaridis E, Papasoulis E, Manidakis N, et al. Management of a femoral diaphyseal fracture in a patient with Klippel-Trenaunay-Weber syndrome: a case report. Cases J  2009; 2: 8852.
[http://dx.doi.org/10.4076/1757-1626-2-8852] [PMID: 19918405] 
[8] 
Susan H Cha, Michael A. Romeo, Janet A. Neutze. Visceral Manifestations of Klippel-Trénaunay Syndrome 2005; 25(6): 1694-7.
[http://dx.doi.org/10.1148/rg.256055042] 
[9] 
Krishnappa Santosh Kumar, Nanjappa Mohan Kumar. Klippel Trenaunay Syndrome: a rare case report in a neonate 2017; 4(1): 294-6.
[http://dx.doi.org/10.18203/2349-3291.ijcp20164624] 
[10] 
Święcicka-Klama A, Drożdż K, Sapian-Raczkowska B, Szuba A, Chachaj A. Klippel-Trenaunay syndrome: case report and literature review. Acta Angiologica  2019; 25(2): 120-5.
[http://dx.doi.org/10.5603/AA.2019.0009] 
[11] 
Asghar F, Aqeel R, Farooque U, Haq A, Taimur M. Presentation and management of klippel-trenaunay syndrome: A review of available data. Cureus  2020; 12(5): e8023.
[http://dx.doi.org/10.7759/cureus.8023] [PMID: 32528762] 
[12] 
van der Loo LE, Beckervordersandforth J, Colon AJ, Schijns OE. Growing skull hemangioma: first and unique description in a patient with Klippel-Trénaunay-Weber syndrome. Acta Neurochir (Wien)  2017; 159(2): 397-400.
[http://dx.doi.org/10.1007/s00701-016-3012-0] [PMID: 27822901] 
[13] 
Gourie-Devi M, Brahm Prakash MCh. Vertebral and epidural hemangioma with paraplegia in Klippel-Trenaunay-Weber syndrome. Case report. J Neurosurg  1978; 48(5): 814-7.
[http://dx.doi.org/10.3171/jns.1978.48.5.081] 
[14] 
Bugarin-Estrada, Emmanuel & Villanueva-Castro, Eliezer & Medina-Franco, Heriberto. Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome. Cir Cir  2018; 86: 566-9.
[http://dx.doi.org/10.24875/CIRU.18000306] 

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DOI https://dx.doi.org/10.2174/1871529X21666210924150832	Print ISSN 1573-4056
Publisher Name Bentham Science Publisher	Online ISSN 1875-6603

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Breast Hemangioma: Unique Presentation in a Patient with Klippel- Trenaunay-syndrome

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