Hepatic angiomyolipoma (HAML) is a rare tumor comprising adipose tissue, smooth muscle cells, and blood vessels. On the other hand, inflammatory breast cancer (IBC) is a rare and severe form of breast cancer that progresses quickly and presents as breast inflammation. It is incredibly unusual for HAML and IBC to coexist in the same patient. In the present study, we describe a case of a 63-year-old Yemeni female patient diagnosed with locally advanced left breast cancer presented with pain at the left breast and axilla. A computed tomography (CT) scan for staging showed an incidental large hepatic mass, which was eventually discovered to be HAML. The patient underwent a modified radical mastectomy after completing her neoadjuvant treatment and later underwent parenchyma-sparing liver resection of that lesion; follow-up has continued till now. The diagnosis of HAML in the presence of IBC can pose challenges due to overlapping clinical and radiological features. Treatment decisions for patients with coexisting HAML and IBC require a multidisciplinary approach; surgical resection, embolization, targeted therapies, and systemic chemotherapy may be considered based on the extent of the disease and individual patient factors. Lastly, a brief review of the related literature was also carried out.

Birt-Hogg-Dubé syndrome (BHDS) is a rare hereditary autosomal dominant condition characterized by benign cutaneous lesions, lung cysts, and increased risk of spontaneous pneumothorax and renal cancer. We report a case of a young Indian boy with bilateral pneumothorax as the first symptom of BHDS. Detailed history examination and investigation showed multiple facial lesions; his computerized tomography was suggestive of renal angiomyolipoma, hepatic angiomyolipoma, pulmonary cyst with pneumothorax, and small bilateral subependymal soft tissue density lesion with calcification in the brain, all of which were collectively suggestive of BHDS. Identification of the above commonly presented clinical features as a syndrome is important for even a primary care physician so as to ensure the timely management and if required referral to a higher center.

Angiomyolipoma, a perivascular epithelioid cell tumour, is easily identifiable as a benign tumour in the kidneys. However, when occurring in extrarenal sites it can mimic malignancy (Cancer Cytopathol. 2017;125:257). Pathologists must be aware of the classical morphological features of this lesion, its pitfalls in extrarenal sites, and the need for immunohistochemistry in order to establish the correct diagnosis (World J Gastroenterol. 2000;6:608). We report a case of angiomyolipoma with extramedullary hematopoiesis presenting as a large hepatic mass, diagnosed by cytology through endoscopic ultrasound guided fine needle aspiration. Our case exemplifies the classic cytological findings that are important in the differentiation between hepatic angiomyolipoma (HAML) and differentials in this organ such as hepatocellular carcinoma (HCC) and focal nodular hyperplasia. A brief literature review and comparison of significant features between HAML and HCC are presented.

Indocyanine green fluorescence imaging (ICG-FI)-a sensitive tool for detecting tumor localization in laparoscopic surgery-produces false positive results for benign liver tumors. This report is the first case of hepatic angiomyolipoma (HAML) treated laparoscopically with ICG-FI. We present the case of a 31-year-old woman with a liver tumor that was a 13-mm mass in the anterior superior segment. Though a benign tumor was suspected, malignant potential could not be ruled out. Therefore, minimally invasive laparoscopic resection using ICG-FI was planned. ICG, intravenously injected preoperatively, revealed the tumor\'s existence. Pure laparoscopic hepatectomy with ICG-FI was performed for excisional biopsy, during which the tumor was resected with adequate surgical margins, followed by histological confirmation of HAML. In conclusion, it is suggested that laparoscopic resection with ICG-FI is an effective minimal invasive surgery for tumors that are difficult to detect, such as HAML, leading to a safe surgical margin.

BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare liver tumor, and hepatectomy is the only effective treatment. Due to the difficulty of correct diagnosis of HAML before surgery by image studies, more than 36.6% of reported HAMLs are misdiagnosed as other malignant liver tumors before surgery. As there are only few reported cases in which HAMLs were removed using laparoscopic hepatectomy, the effectiveness of laparoscopic hepatectomy for such HAMLs in which are diagnosed as other malignant liver tumor before surgery has not been reported. Case presentation Case 1: a 58-year-old female with a history of treatment for autoimmune hepatitis was preoperatively diagnosed with hepatocellular carcinoma (size: 20 mm) in segment 7 (S7) of the liver. The tumor was removed by laparoscopic partial resection and was diagnosed as a HAML through a pathological examination. The patient\'s postoperative course was good, and she was recurrence-free at 37 months after the hepatectomy. Case 2: a 29-year-old female with a history of surgery for a right mature cystic teratoma was referred to our department to receive treatment for a growing 20-mm liver tumor with some calcification, which arose in S3 of the liver. A metastatic liver tumor derived from the mature cystic teratoma was suspected, and laparoscopic left lateral sectionectomy was performed. The liver tumor was diagnosed as a HAML after a pathological examination. The patient\'s postoperative course was unremarkable, and more than 54 months have passed since the hepatectomy without any recurrence.
CONCLUSIONS: Two cases in which HAMLs were preoperatively diagnosed as other malignant liver tumor were successfully removed by laparoscopic hepatectomy with a correct postoperative diagnosis. Laparoscopic hepatectomy for the present 2 cases of HAML seemed to be effective for providing a correct diagnosis after the curative removement of liver tumor with a smaller invasion compared to open hepatectomy, and for denying risk of dissemination of the malignant tumor by needle biopsy that had to be considered before ruling out malignant tumor.

Hepatic angiomyolipoma (HAML) is a rare mesenchymal neoplasm that belongs to the perivascular epithelioid tumor family. Though it is characteristically, a triphasic tumor composed of smooth muscle, blood vessels, and adipocytes, the smooth muscle cells are often epithelioid and can represent the near-entirety of the tumor. A HAML composed predominantly of epithelioid smooth muscle cells occurring in the liver presents significant diagnostic challenges as many liver tumors are composed of large epithelioid cells. Furthermore, even if the tumor is not composed predominantly of epithelioid smooth muscle cells, this may be the only component present in a fine-needle aspiration (FNA) or core needle biopsy. A 38-year-old female with a 3 month history of abdominal pain, nausea, and diarrhea was found to have a 12 cm right hepatic lobe mass. FNA biopsy revealed a moderately cellular specimen composed of plump epithelioid cells with indistinct cell borders, low N:C ratio, round to oval nuclei, fine chromatin, occasional nucleoli, and abundant vacuolated to fibrillary cytoplasm. Rare intranuclear inclusions and occasional foamy macrophages were noted. Concurrent core biopsy revealed large polygonal cells with eccentric nuclei and clear, vacuolated to granular, eosinophilic cytoplasm that stained strongly for HMB45, confirming the diagnosis of HAML. Because HAML is a rare tumor, this diagnosis can be easily overlooked; cognizance of the typical cytologic, histologic, and immunophenotypic findings is crucial to establishing a diagnosis.

Hepatic angiomyolipoma (HAML) comprises epithelioid angiomyolipoma (EAML) and classic hepatic angiomyolipoma (CAML). The imaging appearance of HAML varies widely, and EAML is more easily misdiagnosed as hepatocellular carcinoma (HCC) than as CAML. The clinical and contrast-enhanced ultrasound (CEUS) features of CAML, EAML and HCC with negative alpha-fetoprotein protein expression (HCC[AFP-]) were retrospectively reviewed. The hyper-vascular type was more commonly found in CAML and EAML lesions than in HCC lesions. Most lesions were hyper-enhanced in the arterial phase. CAMLs showed prolonged hyper-enhancement or iso-enhancement during the portal and late phases on CEUS, making them easily distinguishable from HCC(AFP-). Some EAML lesions (41.7%) were hypo-echoic, similar to HCC(AFP-). However, the hypo-enhancement of EAML lesions occurred later than that of HCC(AFP-) lesions. Thus, our findings may be useful in distinguishing among these lesions to improve diagnostic accuracy.

El angiomiolipoma hepático es un tumor mesenquimatoso infrecuente perteneciente al grupo de los PEComas (Perivascular Epithelioid Cells), que cuenta con un potencial maligno indeterminado. Los síntomas clínicos son inespecíficos, siendo las características radiológicas principales la alta vascularización de la lesión y la presencia de tejido adiposo macroscópico. Presentamos un caso clínico de un angiomiolipoma hepático izquierdo diagnosticado por tomografía computada de manera incidental y asintomático en una paciente sin antecedentes de importancia que se sometió a hepatectomía lateral izquierda con resección completa del tumor, el cual fue negativo para malignidad y sin complicaciones.
Hepatic angiomyolipoma is a mesenchymal tumor known as PEComas (Perivascular Epithelioid Cells) with unknown malignancy pattern. We present a case report of left hepatic angiomyolipoma diagnoses by computed tomography in an asymptomatic patient with no previous medical diseases. The tumor was complete resected during a left lateral hepatectomy with no complications and final histologic report was negative to cancer.

OBJECTIVE: To identify specific ultrasonographic features that differentiate hepatic angiomyolipoma (HAML) from hepatocellular carcinoma (HCC).
METHODS: Twelve patients with HAML and 73 patients with HCC, whose diagnosis were pathologically confirmed at a single center in Japan between 2006 and 2016, were included in this study. The HAML and HCC cases were histologically evaluated and their histological growth patterns were compared. Using ultrasonographic data, we evaluated the imaging features representing the distinct histological differences. Ultrasonographic findings, reviewed by two examiners, were compared via interobserver variability analysis. This retrospective study was approved by the institutional ethics committee at our institute (No. 2017-1004).
RESULTS: The enrolled patients were carefully divided into two case sets: discovery case set (6 HAML patients and 37 HCC patients) and validation case set (6 HAML patients and 36 HCC patients). In the discovery case set, half of the HAML cases had intratumoral regions showing a reticular growth pattern. None of the HCC cases appeared as a region with the reticular growth pattern. The regions with the reticular growth pattern present as an intratumoral hyper echoic foci on ultrasound images. The presence of the intratumoral hyper echoic foci was significantly associated with HAML (P < .01). In the validation case set, the intratumoral hyper echoic foci predicted HAML at a specificity of 100% and a sensitivity of 50%.
CONCLUSIONS: Intratumoral hyper echoic foci, representing reticular growth pattern, can be a promising ultrasonographic finding to help differentiate HAML from HCC.

Hepatic angiomyolipoma (AML) is a rare stromal tumor composed of variable admixtures of thick-walled vessels, smooth muscles and adipose tissue. One of the specific radiological findings of hepatic AML is an early drainage vein noted via enhanced computed tomography (CT). We report a case of hepatic AML showing early drainage veins into both the hepatic and portal vein. The case involved a 46-year-old woman who was referred to our hospital because of a giant hepatic tumor. CT revealed well-enhanced 14 cm and 1 cm tumors in the left and right lobes, respectively. Magnetic resonance imaging demonstrated the existence of adipose tissues in the larger tumor. Hepatic arteriography revealed early drainage veins draining into both the hepatic and portal vein. Based on a diagnosis of hepatic AML, left hepatectomy and partial hepatectomy were performed. Pathology revealed both tumors as hepatic AML based on human melanoma black-45 immuno-positivity. Hepatic AML with early drainage veins into both the hepatic and portal vein is rare. The dilated and retrogressive vein drains the abundant arterial blood flow of the tumor. The finding of early drainage veins into not only the hepatic vein but also the portal vein should be helpful for diagnosing hepatic AMLs.