Yellow tumor disease, also known as Hand-Schuller-Christian disease, is one of the diseases of the reticuloendothelial system and is a hereditary lipid storage disease, not a tumor. It is most commonly seen in children under 10 years old, particularly in boys aged 3-5, and occasionally occurs in adults. The cause of the disease is unknown, and it typically affects the skull, with other bones rarely involved. Classic clinical manifestations include map-like skull defects, exophthalmos, and diabetes insipidus, along with possible symptoms such as low-grade fever, anemia, and muscle pain. The main treatment methods are radiotherapy and surgery, and timely treatment can lead to symptom relief in most patients.

bubble_chart Clinical Manifestations

1. Localized swelling and pain in the head, such as invasion of the orbit leading to exophthalmos, visual field defects, and decreased vision; invasion of the skull base resulting in diabetes insipidus, manifesting as polydipsia, polyuria, and developmental disorders. 2. Systemic symptoms include low-grade fever, muscle pain, weight loss, fatigue, and weakness.

bubble_chart Diagnosis

1. Localized swelling and pain in the head, with larger areas accompanied by skull defects. 2. Systemic symptoms such as low-grade fever, muscle and joint pain, weight loss, fatigue, and lack of strength. 3. Typical cases present with three major symptoms: map-like skull defects, exophthalmos, and diabetes insipidus. 4. Blood tests show eosinophilia and elevated blood lipids, but normal cholesterol levels. 5. X-ray skull films reveal typical map-like defects, single or multiple, varying in size, with clear, sharp edges and no sclerotic margins.

1. Surgical treatment: For patients with large skull defects, cranial repair surgery is performed concurrently. 2. Radiotherapy.

The cause of this disease is unknown, with no clear familial predisposition. Most children are discovered by parents during bathing due to a head lump or initially present with diabetes insipidus. Systemic symptoms are generally mild and often overlooked. If a child exhibits the above manifestations, parents should take it seriously and seek early diagnosis and treatment. Currently, there is no specific medication for this disease, with the main approaches being surgery and radiotherapy. Patients with diabetes insipidus are treated with antidiuretic drugs. Although neither surgery nor radiotherapy can cure the disease, they can alleviate the condition.

bubble_chart Cure Criteria

1. Cure: The mass disappears; symptoms disappear or significantly improve; intracranial pressure returns to normal. 2. Improvement: The mass shrinks; symptoms are relieved or stabilized. 3. No recovery: The mass continues to grow, and symptoms worsen.