scholarly journals Hepatic epithelioid angiomyolipoma: a case report

Author(s):  
Ana Logrado ◽  
Raquel Pereira ◽  
Júlio Constantino ◽  
Milene Sá ◽  
Jorge Pereira ◽  
...  

Angiomyolipoma (AML) is a rare benign solid tumor, of mesenchymal origin. Angiomyolipomas can be subdivided, according to their predominant component. The predominance of epithelioid cells characterizes the epithelioid variant (EAML). These express a more aggressive clinical behaviour with a greater potential for malignant transformation. In the absence of specific radiological features, the diagnosis of hepatic EAML depends on the pathological and immunohistochemical study. We present the case of an 80 years old female with a computed tomography (CT) scan showing a hepatic nodule at the transition of segments 5 and 8, and a left kidney nodule previously biopsied and confirmed to be a renal angiomyolipoma. A liver nodule biopsy was performed, whose histology revealed a probable gastrointestinal stromal tumor (GIST). The patient underwent a 5/8 subsegmentectomy and pathology report revealed epithelioid angiomyolipoma. After discussion of the case in the multidisciplinary tumor board, it was decided to perform a left nephrectomy, whose pathology revealed leiomyomatous angiomyolipoma. No further treatment and surveillance on outpatient clinic were decided. Hepatic EAML is a rare lesion that, although mostly benign, can exhibit malignant behavior with distant metastasis and local invasion. Its identification is of paramount importance. The definitive diagnosis is only possible through histological and immunohistochemical analysis. Additional studies are needed in order to establish diagnostic criteria and predictive characteristics of malignancy. 

2015 ◽  
Vol 9 (5-6) ◽  
pp. 337 ◽  
Author(s):  
Jing Xie ◽  
Jin Wen ◽  
Ya-lan Bi ◽  
Han-zhong Li

Primitive neuroectodermal tumour (PNET) is a highly aggressive neoplasm that develops classically in the central nervous system. PNET of the kidney (rPNET) is extremely rare. Recently, a 23-yearold woman complained of left flank pain and intermittent hematuria for 3 months and was admitted to our hospital. A computed tomography (CT) scan and magnetic resonance imaging demonstrated a 5.1 × 4.4-cm heterogenous mass with unconspicuous reinforcement in the upper pole of the left kidney. F18-FDG positron emission tomography CT (PET-CT) revealed the mass as a benign lesion with internal extensive bleeding. Renal angiomyolipoma with minimal fat was diagnosed. Three months later, a CT scan showed that the mass shrank to 3.1 × 2.6 cm and nephron-sparing surgery of the left kidney was performed at the patient’s request. However, histologic features and immunohistochemical analysis confirmed the diagnosis of rPNET. Five cycles of combined chemotherapy were executed. At the 11-month follow-up, the patient showed no evidence of metastasis or recurrence.


2020 ◽  
Vol 27 (1) ◽  
pp. 33-38
Author(s):  
Aurelija Liulytė ◽  
Algirdas Žalimas ◽  
Raimundas Meškauskas ◽  
Jurgita Ušinskienė ◽  
Feliksas Jankevičius

Background. Renal epithelioid angiomyolipoma is a rare tumour which involves kidneys in most cases. It is known for its aggressive behaviour as a significant number of cases have been associated with metastatic epithelioid angiomyolipoma. Usually, radical treatment with systemic therapy is recommended. Only a small number of cases of epithelioid angiomyolipomas have been reported with the standard treatment being radical or partial nephrectomy. We present a case report showing that partial nephrectomy can be a successful treatment option for renal epithelioid angiomyolipoma. This is the first case of this nature in Lithuania. Case presentation. In this case, a 40-year-old male with epithelioid angiomyolipoma of the left kidney is presented. In 2012, a cystic left renal mass 40 × 41 mm in size was diagnosed incidentally while performing ultrasound and later confirmed by MRI. Due to the size of the tumour and the possibility of renal cell carcinoma, surgery was scheduled. Left partial nephrectomy was performed successfully. Final pathology report came back with the diagnosis of renal epithelioid angiomyolipoma. The patient had yearly follow-up for six years by CT scan, and neither recurrence nor progression were observed. Conclusion. Early detection and diagnosis are crucial for treatment as the tumour tends to have malignancy potential. With early diagnosis, partial nephrectomy can be performed with yearly follow-up and no systemic treatment required.


2021 ◽  
pp. 1-8
Author(s):  
Wen Hao Justin Leong ◽  
Xia Huang Andrew Tan ◽  
Ennaliza Salazar

Hepatic angiomyolipomas (AML) are rare mesenchymal tumours of which the epithelioid type is a rare type with malignant potential. We report a case of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess. A 46-year-old man presented with a 5-day history of fever with epigastric pain and nausea. On the night of admission, his temperature spiked to 39°C, his blood pressure was 135/79, his heart rate 98, his liver function test revealed albumin 37 g/L, bilirubin 25 μmol/L, ALP 298 U/L, ALT 247 U/L, and AST 344 U/L. The clinical suspicion was hepatobiliary sepsis and intravenous ceftriaxone was commenced. CT of the abdomen showed an ill-defined hypodense focus in segment 4A/8 (4.5 × 3.5 cm) with a minimal fluid component implying a developing abscess or phlegmon. The images were reviewed by a radiologist and showed minimal fluid for percutaneous drainage. MRI of the liver was performed to further characterize the lesion and revealed a solid mass with nodular areas of arterial enhancement and washout, suspicious of neoplasm. Ultrasound-guided liver biopsy of the lesion was performed. Histology showed a histiocyte-rich epithelioid neoplasm consistent with the epithelioid variant of AML. Immunohistochemical staining was positive for human melanoma black 45, melan-A and cluster of differentiation 68. He successfully underwent liver resection of segment 4A/8 after 6 weeks of antibiotics. To our knowledge, this is the first reported case in the literature of primary hepatic epithelioid angiomyolipoma masquerading as liver abscess.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Mehmet Kalkan ◽  
Coşkun Şahin ◽  
Ömer Etlik ◽  
Ergun Uçmaklı

A 21-year-old female patient admitted to the emergency department complaining of left side pain. Hypovolemic shock, which was probably caused by retroperitoneal bleeding from left sided renal angiomyolipoma, was developed. Abdominal computed tomography showed multiple fat containing lesions in different, regions including right bladder wall, lower pole of left kidney, and right kidney. Some lesions compatible with tuberous sclerosis such as angiofibromas, Shagreen patches, myocardial, and brain hamartomas were also detected. Bladder wall mass showing intra- and extravesical extensions was seen at exploration. We removed the tumor completely preserving bladder trigone. Angiomyolipoma located at lower pole of left kidney was also removed. Diagnosis of bladder angiomyolipoma was confirmed by the immunohistochemical examination. Recurrent or residual mass was not detected at the three-months-follow-up. We report the first case of bladder angiomyolipoma confirmed by histopathologically as a tuberous sclerosis.


2015 ◽  
Vol 9 (1) ◽  
pp. 54-56 ◽  
Author(s):  
Anton Maré ◽  
Shehan Wickramasinghe ◽  
Victor Ilie ◽  
Maurice Mulcahy

Epithelioid angiomyolipoma is a rare mesenchymal derived neoplasm of the kidney. Thought to be a variant of classical angiomyolipoma, a benign tumour, its malignant potential has been highlighted by case reports of loco-regional and distant metastasis. Given the potentially adverse clinical course associated with epithelioid angiomyolipoma compared to classical angiomyolipoma, the distinction and comprehensive histological characterisation of this rare entity is essential.


2017 ◽  
Vol 30 (3) ◽  
pp. 162-163
Author(s):  
Agnieszka Fronczek ◽  
Dorota Lewkowicz ◽  
Jaroslaw Swatek ◽  
Katarzyna Cieszczyk ◽  
Janusz Ciechan ◽  
...  

AbstractAdenomatosis is a rare lesion of unknown etiology, defined as multiple (usually 5 or more) adenomas in one kidney. A case of renal adenomatosis in a 68-year-old woman treated previously for urolithiasis, who underwent nephrectomy because of the nonfunctional left kidney is reported. Apart from multiple adenomas, numerous hyperplastic lesions involving single tubules were present in the resected kidney. Both adenomas and hyperplastic lesions exhibited the expression of alpha-methylacyl-coenzyme A racemase (AMACR). Renal adenomatosis is worth special attention, since renal papillary adenomas are suggested as precursor lesions of papillary renal cell carcinoma that show similar AMACR expression.


Medicina ◽  
2019 ◽  
Vol 55 (5) ◽  
pp. 170
Author(s):  
Marius Anglickis ◽  
Rokas Stulpinas ◽  
Giedrė Anglickienė ◽  
Justinas Gabrilevičius ◽  
Arūnas Jaškevičius

Testicular Sertoli cell tumors are extremely rare. Generally, they are benign neoplasms, which belong to a group called sex cord–stromal tumors. In this article, we present a case report of a Sertoli cell tumor, which was accidentally discovered during a urological consultation of a 42-year-old male. An ultrasound showed a 2.1 × 2.2 cm hypoechogenic, hypervascular tumor in the middle third of the left testicle. Serum tumor markers (α-fetoprotein, alkaline phosphatase, β-human chorionic gonadotropin, and lactic dehydrogenase) were all within the normal range. Rapid microscopic evaluation of fresh frozen sections during the operation was inconclusive, which led to a decision not to perform a radical orchiectomy immediately. On formalin-fixed paraffin-embedded (FFPE) sections, the tumor histology showed atypical patterns, and immunohistochemical analysis was performed in order to determine the type of neoplasm and differentiate it from other types of testicular tumors, so as to assign the further course of treatment. Radical inguinal orchiectomy was performed. The final pathology report showed a tumor with no predictive signs of aggressive behavior, which most closely resembled a Sertoli cell tumor.


2021 ◽  
Vol 39 (28_suppl) ◽  
pp. 107-107
Author(s):  
Omar Peña-Curiel ◽  
Orestes Valles-Guerra ◽  
Karen M Velazquez-Ayala ◽  
Griselda Peña-Iturbide ◽  
Sonia Maria Flores Moreno ◽  
...  

107 Background: UNEME-DEDICAM (UD) clinics are part of a national public health initiative to provide women prompt access to cervical and breast cancer (BC) screening and diagnosis. Furthermore, UD clinics play a central role in the coordination and prioritization of patient transfer to treatment-specialized institutions. To facilitate this process, we planned and implemented an interinstitutional virtual multidisciplinary tumor board (VMDT). Herein, we present our current experience. Methods: We planned and implemented our VMDT in September 2020. Weekly sessions were established for the multidisciplinary discussion of every newly diagnosed patient at UD with a complete radiology and pathology report. Communication was accomplished through an encrypted and secure internet connection using Microsoft Teams software. VMDT members included breast pathologist, breast imaging, radio oncologist, medical oncologist, and surgical oncologist. Treatment consensus were registered in a Microsoft Word template and integrated into the medical record for each patient. Importantly, the report also included date and time for the consultation at the referral institution. Results: Between September 2020 through May 2021, 74 BC patients were diagnosed at UD. Mean age at diagnosis was 52 years. Sixty-eight patients had invasive BC, of whom early stage (I and II) accounted for 67% of patients; locally advanced (III) for 29%, and advanced (IV) for 4%. Luminal A and B type accounted for 68%; HER2+ve for 25%; and triple negative for 7%. Mean time from biopsy to complete histopathology report (biopsy-report interval) was 2.5 weeks. The mean time from VMDT consensus to patient´s first consultation at referral center (VMDT-referral interval) was 2 weeks. The mean time from biopsy to patient´s first consultation at referral center (total interval) was 5.5 weeks. Conclusions: The VMDT is a plausible strategy to streamline the inter-institutional organization for the timely care of BC patients. UD clinics play a central role in the coordination of transfer of BC patients to tertiary care centers.


2014 ◽  
Vol 71 (4) ◽  
pp. 408-412 ◽  
Author(s):  
Sladjana Petrovic ◽  
Aleksandar Tasic ◽  
Dragan Mihailovic ◽  
Nikola Zivkovic ◽  
Marija Vitanovic ◽  
...  

Introduction. Angiomyolipomas (AML) are benign neoplasms composed of fat, smooth muscle and thick-walled blood vessels in varying proportions. These tumors have a significant female predominance. Case report. We reported a 61-year-old man with spontaneous rupture of AML. Computerized tomography revealed a change in morphology of both kidneys. Multiple lesions of fat density with dilated blood vessels were found in the left kidney. The right retroperitoneum was obliterated with a giant heterogeneous mass originating from the right kidney with a massive hemorrhage, active extravasations, compression of inferior the vena cava and intraperitoneal collections. After radical nephrectomy, histological examination revealed that the tumor was composed of relative proportions of fat, smooth muscle and blood vessels. We incidentally found small renal adenoma. Conclusion. The true nature of AML is unclear, but they are usually classified as hamartomas. Angiomyolipomas are generally benign lesions, although the epithelioid angiomyolipoma, a subtype that occurs in about 3% of cases, can behavior aggressively.


2013 ◽  
Vol 3 (3) ◽  
pp. 169-172
Author(s):  
Yoshinori Ikehata ◽  
Toshiaki Tanaka ◽  
Megumi Hirobe ◽  
Jiro Hashimoto ◽  
Hiroshi Kitamura ◽  
...  

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