DefinePK

Research Objective

To present a case of very rare childhood Epithelioid Hemangioendothelioma of the liver in a ten-year-old boy and discuss its characteristics, diagnosis, and management challenges.

Methodology

Case report detailing a 10-year-old boy presenting with abdominal pain and abnormal liver function tests. Diagnostic methods included physical examination, blood investigations, contrast-enhanced CT scans of the abdomen, chest, and pelvis, bone scans, histopathological examination, and immunohistochemical panel. Treatment was palliative due to widespread metastatic disease and lack of facilities for surgical resection, liver transplant, or radiofrequency ablation.

Methodology Flowchart

                        graph TD;
    A["Patient Presentation"] --> B["Clinical Examination & Blood Tests"];
    B --> C["Imaging Studies"CT, Bone Scan""];
    C --> D["Histopathological & Immunohistochemical Analysis"];
    D --> E["Diagnosis: Epithelioid Hemangioendothelioma"];
    E --> F["Multidisciplinary Consultation"];
    F --> G["Treatment Decision Palliative"];
    G --> H["Patient Management"];
    H --> I["Outcome Death"];                    

Discussion

Epithelioid Hemangioendothelioma (HEHE) is an infrequent vascular tumor with borderline malignant potential, extremely rare in the pediatric population. It is a multi-organ disease with non-specific clinical signs. Definitive diagnosis relies on imaging, biopsy, and molecular confirmation. Management guidelines are lacking due to the disease's refractory nature. The case highlights the challenges in managing widespread metastatic HEHE in children, especially in resource-limited settings.

Key Findings

The patient, a 10-year-old boy, presented with symptoms suggestive of liver involvement. Radiologically, hepatic, pulmonary, and bony lesions were identified. Histopathological examination confirmed Epithelioid Hemangioendothelioma. Due to the advanced and widespread nature of the disease, palliative treatment was administered. The patient died seven months after diagnosis.

Conclusion

This case report underscores the rarity and aggressive nature of metastatic hepatic epithelioid hemangioendothelioma in children. The lack of specific management protocols and the refractory nature of the disease contribute to poor outcomes.

Fact Check

1. Patient Age: The case report concerns a 10-year-old boy. This is confirmed by the title and the case description.
2. Diagnosis: The diagnosis of Epithelioid Hemangioendothelioma was confirmed by histopathological examination and an immunohistochemical panel. This is stated in the abstract and case report.
3. Patient Outcome: The patient died at the seventh-month follow-up due to widespread disease. This is reported in the abstract and case report.

Mind Map