Paravertebral and Retroperitoneal Vascular Tumour Presenting with Kasabach-Merritt Phenomenon in Childhood, Diagnosed with Magnetic Resonance Imaging

نویسندگان

  • Gonca Keskindemirci
  • Deniz Tuğcu
  • Gönül Aydoğan
  • Arzu Akçay
  • Nuray Aktay Ayaz
  • Ali Er
  • Ensar Yekeler
  • Bilge Bilgiç
چکیده

Kasabach-Merritt phenomenon (KMP) is characterized by vascular tumour and consumptive coagulopathy with life-threatening thrombocytopenia, prolonged prothrombin time and partial thromboplastin time, hypofibrinogenemia, and the presence of high fibrin split products. We report a case of 3-year-old boy with local aggressive vascular lesions associated with KMP. Magnetic resonance imaging revealed an extensive lesion at paravertebral and retroperitoneal regions that was infiltrating vertebrae. Although we did not get any response to steroid or propranolol treatment, partial response was observed radiologically with interferon-alpha treatment. Unfortunately, the patient died because of the uncontrolled consumptive coagulopathy that led to intracranial hemorrhage which was caused by huge knee hematoma after minor trauma.

برای دانلود رایگان متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Tufted angioma associated with Kasabach-Merritt phenomenon: a therapeutic challenge.

Fig. 1. (A) Tufted angioma involving the chin in a one-month-old child. (B) The patient at 2 months of age showing a subtle increase in tumour size and decrease in platelet count. (C) A subtle residual lesion on the chin of the patient at the age of 5 years. Tufted angioma (TA) is an infrequently seen, benign vascular tumour, which was first described in 1949 (1). It is characterized clinically...

متن کامل

Sirolimus for treatment of kaposiform hemangioendothelioma associated with Kasabach-Merritt phenomenon

KHE: kaposiform hemangioendothelioma KMP: Kasabach-Merritt phenomenon INTRODUCTION Kaposiform hemangioendothelioma (KHE) was first described by Zuckerberg et al in 1992. KHE is defined as a rare, locally aggressive infiltrative vascular neoplasm that typically occurs during infancy and childhood. KHE generally originates on the skin as a distinctive cutaneous lesion with ill-defined borders, la...

متن کامل

A new successful combination therapy with atenolol and prednisolone for Kasabach-Merritt syndrome

Kasabach-merritt syndrome is a rare life-threatening syndrome associated with vascular tumors such as tufted angioma and kaposiform hemangioendothelioma. For this syndrome, there exist a few treatment modalities with variable results. For many years, corticosteroids have been employed in the first-line therapy. Nowadays, on the other hand, β blockers such as propranolol have been used owing to ...

متن کامل

[Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon].

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors app...

متن کامل

Awareness of primary spontaneous hemorrhagic angiosarcoma of the breast associated with Kasabach–Merritt syndrome in a pregnant woman by enhanced magnetic resonance imaging

INTRODUCTION Primary breast angiosarcoma with spontaneous intratumoral bleeding associating with Kasabach-Merritt Syndrome is rarely reported. CASE FINDINGS/PATIENT CONCERNS We herein present such a case in a 30-year-old pregnant woman who was initially diagnosed to hemangioma at her early gestation. However, the sudden rapid tumor growth was aware of the attention and intended for receiving ...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

عنوان ژورنال:

دوره 2015  شماره 

صفحات  -

تاریخ انتشار 2015