Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized

Autor: Jie Liu, De-Fei Hong, Minjie Shang, Cheng-Wu Zhang, Yuan Chen, Ran Tao, Yu-Hua Zhang
Rok vydání: 2016
Předmět:
Adult
Male
Pathology
medicine.medical_specialty
Angiolipoma
Biopsy
03 medical and health sciences
0302 clinical medicine
MART-1 Antigen
Predictive Value of Tests
Retrospective Study
hemic and lymphatic diseases
medicine
Biomarkers
Tumor
Humans
Diagnostic Errors
Neoplasm Metastasis
Aged
Retrospective Studies
medicine.diagnostic_test
integumentary system
business.industry
Disease progression
Epithelioid Cells
Liver Neoplasms
Gastroenterology
General Medicine
Middle Aged
medicine.disease
Immunohistochemistry
Magnetic Resonance Imaging
Tumor Burden
HMB-45
Tomography x ray computed
Treatment Outcome
030220 oncology & carcinogenesis
Disease Progression
Epithelioid angiomyolipoma
030211 gastroenterology & hepatology
Female
Neoplasm Recurrence
Local
business
Tomography
X-Ray Computed
Epithelioid cell
Melanoma-Specific Antigens
gp100 Melanoma Antigen
Zdroj: World journal of gastroenterology. 22(20)
ISSN: 2219-2840
Popis: To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma (HEAML).Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from PubMed and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years (ranging from 31 to 56 years). The mean tumor size was 7.3 ± 5.5 cm (ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45 (HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from PubMed and MEDLINE. The majority of the papers were published as case reports. Only 5 (5/75, 6%) cases were associated with tuberous sclerosis complex (TSC). More than half (35/66) were discovered incidentally upon physical examination. Approximately 65% (22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10% (8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.
Databáze: OpenAIRE
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