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A case report of hepatic angiomyolipoma, inflammatory subtype. Clinico-pathological characterization: a diagnostic
Francisco Javier Velasco-Albendea1, María Jesús Gil-Belmonte1, Beatriz Estébanez-Ferrero2
1Department of Pathology, Torrecardenas Hospital, Almería, 04009, Spain.
Background:
Angiomyolipoma is a benign mesenchymal neoplasm of a wide histological heterogeneity belonging to the PEComa "family." The liver, after the kidney, is their second most frequent location. However, inflammatory hepatic AMLs constitute a rare entity, with only fourteen documented cases until 2020. These neoplasms can overlap morphological features of IgG4-related diseases, being of great diagnostic relevance to demonstrating myomelanocytic-lineage differentiation of the neoplastic cells.
Case Presentation:
we present a new case of an inflammatory hepatic AML resembling an IgG4-related disease in a 35-year-old woman with a subcapsular 5 cm mass confined to segment VII of the right hepatic lobe. Although having reduced its size along the tumor's natural evolution, complete tumor resection was decided due to its hypermetabolic behavior (max. SUV = 12,6) assessed by PET-CT scan. Finally, the patient underwent a right hepatectomy due to spontaneous rupture and bleeding of the lesion during the intervention. All the diagnostic and therapeutic procedures occurred in the last months of the COVID-19 pandemic.
Conclusions:
This review aims to describe inflammatory hepatic AML histological and immunohistochemical features. We further sought to establish a clinicopathological contextualization of this tumoral subtype.
Insights
Inflammatory hepatic angiomyolipomas (AMLs) are rare liver tumors. This case highlights their potential to mimic IgG4-related disease, emphasizing the need for accurate diagnosis and management.
Area of Science:
- Hepatobiliary pathology
- Tumorigenesis
- Immunopathology
Background:
- Angiomyolipomas (AMLs) are benign mesenchymal neoplasms, part of the PEComa family, with the liver being the second most common site after the kidney.
- Inflammatory hepatic AMLs are exceptionally rare, with only fourteen cases documented before 2020.
- These tumors can exhibit overlapping features with IgG4-related diseases, necessitating careful diagnostic evaluation.
Observation:
- A case of a 35-year-old woman with a 5 cm inflammatory hepatic AML in the right lobe is presented.
- The lesion showed hypermetabolic activity on PET-CT (max SUV = 12.6) despite natural size reduction.
- The patient underwent right hepatectomy due to spontaneous rupture and bleeding during intervention.
Findings:
- Histological and immunohistochemical analysis is crucial for diagnosing inflammatory hepatic AML.
- Demonstrating myomelanocytic lineage differentiation is key to differentiating AML from other conditions.
- The case underscores the diagnostic challenges posed by inflammatory hepatic AML, particularly its resemblance to IgG4-related disease.
Implications:
- Accurate clinicopathological contextualization of inflammatory hepatic AML is essential for appropriate patient management.
- Understanding the features of this rare tumor subtype can improve diagnostic accuracy and treatment strategies.
- This case report contributes to the limited literature on inflammatory hepatic AML, particularly in the context of IgG4-related disease mimicry.
