The Wunderlich syndrome found after the rupture of primitive renal Ewing’s sarcoma is not a situation that we find often in everyday practice. The clinical findings are not specific, which is why the differential diagnosis must be made with a multitude of benign and malignant renal masses until the correct diagnosis can be made by the pathologist. The CT and MRI images are not characteristic. One treatment option is the multidisciplinary approach; however, the prognosis remains poor for patients with metastatic disease.
A Wunderlich syndrome caused by the rupture of Ewing’s sarcoma of the kidney is a rare entity and represents a life-threatening emergency. In the beginning, PNET (primitive peripheral neuroectodermal tumours) and Ewing’s sarcoma were considered different but today, because of their similar histologic and cytogenetic characteristics, they are included in the same family with Ewing’s sarcoma.
A 40-year-old woman presented to the emergency room for left flank pain lasting for 2 hours and irradiating to the groin. Her medical and surgical history included aortic regurgitation, left kidney stones, stress urinary incontinence surgically treated, and obesity (BMI = 40.8). On physical examination, the patient was found to be afebrile and in good clinical status except for a positive Giordano’s sign. Her blood haemoglobin was 12.7 g/dL with a normal coagulation profile and normal renal function. She was first treated with anti-inflammatory agents, which lead to a decrease of the clinical symptoms. A computed tomography scan showed a large solid heterogeneous mass measuring 7 × 6.7 cm, without calcifications, located in the left kidney, as well as a perinephric hematoma (Figure
(a) The coronal reconstructed CT image shows a large solid heterogeneous mass without calcifications in the left kidney and also a perirenal hematoma. (b) The axial CT image of the upper abdomen reveals a large solid heterogeneous mass measuring 7 × 6.7 in the left kidney and also a perirenal hematoma.
(a) Small blue cells arranged in Homer-Wright rosettes; (b) tumor cells and rosettes, numerous atypical mitoses; (c) tumor cells labeled with anti-S100 protein antibody.
Ewing’s sarcoma is a rare primary malignant renal tumor and it is characterized by an aggressive biological behaviour. The first Ewing’s sarcoma was reported by Mor and colleagues. Later, John Ewing presented this type of tumor in the diaphysis of long bones [
The Wunderlich syndrome was described for the first time in 1856 as a spontaneous renal bleeding confined to the perinephric or subcapsular space [
Ewing’s sarcomas have obvious metastases at the time of diagnosis in 20–25% of the cases, being the main indicator of prognosis. Furthermore, after local therapy, 80–90% of the cases develop in time systemic relapse, suggesting that the metastases had been present subclinically at the moment of diagnosis [
Wunderlich syndrome after the rupture of a renal Ewing’s sarcoma is a rare entity, which represents a diagnostic challenge and already aggressive tumor. The CT and MRI images are not specific that is why the true diagnosis will be made by the anatomopathologist after molecular, immunohistochemistry, and histologic tests. It is important to have a definite diagnosis in order to choose a good multimodal treatment, due to the tumor’s poor prognosis and to the young age of most of the patients.
The authors declare that there is no conflict of interests regarding the publication of this paper.