Nodule de sœur Marie-Josèphe

We here report the case of a 6-year old female patient with no pathological history, presenting with diffuse abdominal pain and abdominal distension evolving in the last 2 months. The patient had a deterioration in her general condition. Clinical examination showed distended abdomen, average abundance ascites, venous collateral circulation and multiple mobile hard abdominal masses measuring 3-4 cm in diameter at the level of the iliac fossa and of the left hypochondriac region associated with a well circumscribed, hard, rounded umbilical mass measuring 3cm in diameter lifting the skin with inflammatory signs (A). The remainder of the physical examination was normal. Thoraco-abdominal CT scan showed subcutaneous umbilical nodule (B), with homogeneous enhancement measuring 36x29x19mm, associated with mesenteric lymphadenopathy magmas, pelvic mass and extended jejunum thickening. Fine needle aspiration biopsy of the mass showed the presence of several naked nuclei and a few blasts. Biopsy showed Burkitt lymphoma. Therefore, the diagnosis of Burkitt lymphoma revealed by sister Marie-Josèphe nodule was retained and the patient underwent chemotherapy according the LMB01 protocol. Outcome was favorable, marked by the regression of the nodule and of the abdominal masses. Sister Mary Joseph nodule is an umbilical metastasis from a tumor (usually an abdomino pelvic tumor) mainly an adenocarcinoma, exceptionally a lymphoma. It is a rare sign. It accounts for 1-3% of all abdomino-pelvic neoplasms and it is associated with poor prognosis. It is essential to know that this nodule is a lesion secondary to solid tumor in order to avoid delayed management of the underlying neoplasm.