A I5-month-old girl was admitted to hospital with the complaints of bruising around the eyes for 10 days. She had one month history of abdominal pain and accompanied by fever and vomiting for one week. Physical examination demonstrated pallor, periorbital ecchymoses (raccoon eyes) and bilaterally mild proptosis (Fig. 1). She also had a left sided abdominal mass, which was 5 × 8 cm in diameter. The laboratory investigation revealed hemoglobin level 6g/dL, white cell count 10,000/mm3 and platelet count 34,000/mm3 . Urinary vanillymandelic acid level was high. Bilateral bone marrow aspiration revealed infiltration with neuroblasts. Abdominal CT showed a left supra-renal mass (5 × 7 cm in diameter), which was diagnosed as a neuroblastoma on histo-pathologic examination.
- The metastatic involvement of the periorbital tissues. has been described and the resultant proptosis and orbital ecchymosis has been given the tag of ‘raccoon eyes’.
- Orbital metastases can be found in up to 20% of children with stage IV neuroblastoma.
- The characteristic "raccoon eyes" appearance associated with neuroblastoma and metastasis to the skull is probably related to obstruction of the palpebral vessels (branches of the ophthalmic and facial vessels) by tumor tissue in and around the orbits .
differential diagnosis:The correct diagnosis of this condition is sometimes delayed because of workup for child abuse or trauma. There are a multitude of differential diagnoses for the presentation of periorbital edema and ecchymosis,
- child abuse or trauma,
- infection of the soft tissues associated with a spreading dental infection and an allergic reaction.
- Other systemic causes to be considered include myxoedema,
- other neoplasias such as lymphoma or
- haematological coagulopathies such as haemophilia.
- Neuroblastoma has been called the great mimicker because of its myriad clinical presentations related to the site of the primary tumor, metastatic disease, and its metabolic tumor by-products.
- Pepper syndrome occurs in infants with overwhelming metastatic neuroblastoma of the liver that results in respiratory compromise. Pepper syndrome was identified as a localized primary tumor and metastatic disease limited to the skin, liver, and bone marrow in infants. Pepper syndrome has since been associated with stage 4S neuroblastoma, a unique entity that occurs only in infants younger than 1 year. Pepper syndrome generally confers a better prognosis, as it is associated with spontaneous regression.
- "Blueberry muffin" babies are infants in whom neuroblastoma has metastasized to random subcutaneous sites. When provoked, the nodules become intensely red and subsequently blanch for several minutes thereafter. The response is probably secondary to the release of vasoconstrictive metabolic tumor by-products. These nodules can be diagnostic of neuroblastoma, but leukemic infiltrates that metastasize to the skin should be considered in the differential diagnoses when these children are evaluated.
- Widespread metastasis of neuroblastoma to the bone may result in Hutchinson syndrome, which results in bone pain with consequent limping and pathologic fractures. Neuroblastomas that arise in the paraspinal ganglia may invade through the neural foramina, compress the spinal cord, and subsequently cause paralysis
- The most common finding upon physical examination is a nontender, firm, irregular abdominal mass that crosses the midline. In contrast, children who present with Wilms tumor have a smooth mobile flank mass that typically does not cross the midline.