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Pathology Oncology Research : POR 2022The renal angiomyolipoma (AML) is a benign tumor characteristically composed of fat, smooth muscle tissue, and vessels. We collected AMLs from our nephrectomy database,...
The renal angiomyolipoma (AML) is a benign tumor characteristically composed of fat, smooth muscle tissue, and vessels. We collected AMLs from our nephrectomy database, reclassified them according to their histological appearance, recorded the demographic, clinical, and pathological parameters, and compared them with oncocytoma (RO) and renal cell carcinoma (RCC). Immunohistochemistry was ordered in 41 cases. In 2224 nephrectomies, we found 52 AMLs with a 53 mm median size. The mean age was 52.76. Forty-eight tumors were sporadic, while four were hereditary. The revision resulted in 31 classic, 13 leiomyoma-like, five lipoma-like, two epithelioid, and one AML with epithelial cysts. SMA was diffusely positive, except for the epithelioid type, while MelanA harbored stronger expression than HMB45. AML was more frequent in females and appeared ten and 7 years earlier than RO and RCC, respectively. The follow-up time was 7.42 years, and neither tumor-related death nor relapse occurred. AML is rare in nephrectomies and develops primarily in females in their 50s with an average size of 50-60 mm at the surgery. The histological appearance in order of frequency is classic, leiomyoma-like, lipoma-like, epithelioid, and cystic. The MelanA, HMB45, and SMA immunohistochemistry can support the light-microscopic findings.
Topics: Female; Humans; Middle Aged; Kidney Neoplasms; Angiomyolipoma; Carcinoma, Renal Cell; MART-1 Antigen; Hamartoma; Lipoma; Antibodies, Monoclonal; Kidney; Leiomyoma
PubMed: 36699622
DOI: 10.3389/pore.2022.1610831 -
BMC Ophthalmology Jan 2016Angiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas... (Review)
Review
BACKGROUND
Angiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). PEComas share overlapping histopathological features with epithelioid cells along a perivascular distribution and characteristic immunohistochemistry with coexpression of myoid and melanocytic markers (HMB-45 /or Melan-A). We report the first case of primary orbital angiomyolipoma with negative melanocytic marker.
CASE PRESENTATION
An 80-year-old Asian woman had a 2-year history of progressive swelling in the left upper eyelid. External examination revealed 3 cm of relative proptosis of the left eye and a palpable mass in the left superonasal orbit. Computed tomographic scan demonstrated a circumscribed, heterogeneous orbital mass. Excision biopsy was done and the histological finding demonstrated the orbital mass was composed of mature adipocytes, intermingled with spindle or oval-shaped cells, and accompanied by thick-walled blood vessels. Immunohistochemically, tumor cells were positive for CD34 and HHF-35, but negative for cytokeratin, HMB-45 and Melan-A. The diagnosis of angiomyolipoma was made. No recurrence was noted at 2-year follow-up.
CONCLUSION
In our case, the HMB-45 negativity may be explained by the rarity of the epithelioid cells, and the HMB-45 positivity is often weaker or absent in spindle cells. Angiomyolipoma, although rare, should be added to the differential diagnosis of space-occupying orbital lesion.
Topics: Aged, 80 and over; Angiomyolipoma; Antigens, CD34; Biomarkers, Tumor; Biopsy; Humans; Immunohistochemistry; MART-1 Antigen; Melanoma-Specific Antigens; Orbital Neoplasms; Tomography, X-Ray Computed; gp100 Melanoma Antigen
PubMed: 26754205
DOI: 10.1186/s12886-016-0185-5 -
Journal of Clinical Pathology Sep 2001Fatty tumours of the adrenal gland are uncommon and their features have received little attention in the literature. The aim of this study is to analyse the features of... (Review)
Review
AIMS
Fatty tumours of the adrenal gland are uncommon and their features have received little attention in the literature. The aim of this study is to analyse the features of adrenal lipomatous tumours.
METHODS
The histological features of primary adrenal tumours reported over a 30 year period (1970 to 1999) in Queen Mary Hospital, Hong Kong were reviewed and the clinicopathological features of adrenal lipomatous tumours were analysed.
RESULTS
Adrenal lipomatous tumours were noted in 20 patients (12 men, eight women), and they accounted for 4.8% of the primary adrenal tumours reported. The adrenal fatty tumours comprised 11 myelolipomas, three lipomas, three teratomas, two angiomyolipomas, and one liposarcoma. Calcification or bone was noted in one third (seven of 20) of the adrenal tumours. In some fatty tumours (myelolipoma and angiomyolipoma), the fatty component may be inconspicuous. This is the first report in the English literature of angiomyolipoma and liposarcoma of the adrenal gland.
CONCLUSIONS
Different types of fatty tumours were noted in the adrenal gland. A high index of suspicion should be maintained with an aim of surgical treatment for selected patients with large and symptomatic adrenal lipomatous lesions. Histological confirmation is needed for diagnosis.
Topics: Adolescent; Adrenal Gland Neoplasms; Adult; Aged; Aged, 80 and over; Angiomyolipoma; Female; Humans; Lipoma; Liposarcoma; Male; Middle Aged; Myelolipoma; Neoplasms, Adipose Tissue; Teratoma
PubMed: 11533079
DOI: 10.1136/jcp.54.9.707 -
Prague Medical Report 2022Angiomyolipomas (AMLs) are mesenchymal tumours derived from perivascular epithelioid cells. Although AMLs are generally known as benign and extremely rare epithelioid... (Review)
Review
Angiomyolipomas (AMLs) are mesenchymal tumours derived from perivascular epithelioid cells. Although AMLs are generally known as benign and extremely rare epithelioid variants of AML, they may be potentially aggressive. Here we present an adrenal epithelioid AML and the literature review. A 64-year-old female patient was diagnosed with a left adrenal mass detected incidentally on ultrasonography. Preoperative abdominal CT (computed tomography) showed a 95×68 mm heterogeneous contrast enhancement mass lesion in the left adrenal gland. The lesion was hormone inactive in the endocrinological evaluation, and left laparoscopic adrenalectomy was performed. The patient was discharged on the 2nd postoperative day. Pathology was reported as epithelioid subtype AML. The patient has no local recurrence or metastasis in the 18-month follow-up period and imaging. Adrenal epithelioid AML is an extremely rare and potentially aggressive variant. According to the literature, open or laparoscopic adrenalectomy seems to be suitable option for disease management.
Topics: Adrenal Glands; Adrenalectomy; Angiomyolipoma; Female; Hormones; Humans; Leukemia, Myeloid, Acute; Middle Aged
PubMed: 36107449
DOI: 10.14712/23362936.2022.19 -
The American Journal of Case Reports Mar 2022BACKGROUND The co-occurrence of renal oncocytoma and angiomyolipoma is exceedingly rare. To date, 17 such cases have been reported in the literature. This report...
BACKGROUND The co-occurrence of renal oncocytoma and angiomyolipoma is exceedingly rare. To date, 17 such cases have been reported in the literature. This report describes a unique case of that association that presented as a single renal mass on imaging. CASE REPORT A 75-year-old woman presented with epigastric discomfort. A CT scan of the abdomen revealed a 6.6×5.7×4.7 cm enhancing right renal mass. Gross examination revealed a nodular, well-circumscribed, tan-brown mass located in the lower pole of the kidney that was abutting the renal capsule. Interestingly, superior to this mass, there was an adjacent, pale tan-white, firm, well-circumscribed nodule in the mid-pole, which was not detected on the CT scan and grossly extended to 1.1 cm of the overlying renal capsule. Histologically, the larger tumor showed characteristic features of oncocytoma. The smaller tumor had an admixture of mature adipose tissue, smooth muscle, and vessels, consistent with a renal angiomyolipoma. CONCLUSIONS We present a new case of synchronous renal angiomyolipoma and oncocytoma, which were uniquely adjacent and coexisted with minimal intermingling renal parenchyma. Other "eosinophilic renal tumors" are significant differential diagnosis considerations. Due to the close proximity of these lesions, this association can present clinically and radiologically as a single renal mass. Careful examination of the nephrectomy specimen is essential for the proper detection of small-sized tumors.
Topics: Adenoma, Oxyphilic; Aged; Angiomyolipoma; Female; Humans; Kidney; Kidney Neoplasms; Tomography, X-Ray Computed
PubMed: 35228507
DOI: 10.12659/AJCR.935407 -
BMC Pulmonary Medicine Aug 2016Lymphangioleiomyomatosis (LAM) and angiomyolipoma are two different, but related rare diseases. To the best of our knowledge, pulmonary LAM and pulmonary angiomyolipoma...
BACKGROUND
Lymphangioleiomyomatosis (LAM) and angiomyolipoma are two different, but related rare diseases. To the best of our knowledge, pulmonary LAM and pulmonary angiomyolipoma have not previously been observed in the same patient.
CASE PRESENTATION
A 38-year-old woman presented with a dry cough and left flank pain. She had a right nephrectomy for renal angiomyolipoma 17 years ago. A magnetic resonance imaging scan demonstrated a round mass in the left kidney. A chest computed tomography scan demonstrated scattered small thin-walled cysts and multifocal round nodules in both lungs. A lung biopsy via video-assisted thoracoscopic surgery revealed that the cysts and nodules were manifestations of LAM and angiomyolipomas, respectively. After sirolimus therapy, the renal angiomyolipoma and metastasized pulmonary angiomyolipomas shrank, but pulmonary cysts were unchanged.
CONCLUSIONS
LAM and angiomyolipoma are significantly associated, and may coexist in the lungs in rare cases. Sirolimus is effective for both renal angiomyolipoma and metastasized pulmonary angiomyolipomas.
Topics: Adult; Angiomyolipoma; Female; Humans; Kidney Neoplasms; Lung; Lung Neoplasms; Lymphangioleiomyomatosis; Magnetic Resonance Imaging; Sirolimus; Thoracic Surgery, Video-Assisted; Tomography, X-Ray Computed
PubMed: 27527652
DOI: 10.1186/s12890-016-0286-1 -
Journal of Ultrasound Jun 2018The purpose of this study was to evaluate the rate of nondiagnostic ultrasound-guided renal mass biopsies (RMBs) at our institution and to determine what patient,...
PURPOSE
The purpose of this study was to evaluate the rate of nondiagnostic ultrasound-guided renal mass biopsies (RMBs) at our institution and to determine what patient, procedural, and focal renal mass (FRM) factors were associated with nondiagnostic ultrasound-guided RMBs.
METHODS
Eighty-two ultrasound-guided renal mass biopsies performed between January 2014 and October 2016 were included in our study. Biopsy outcomes (diagnostic vs. nondiagnostic) and patient, procedural, and FRM characteristics were retrospectively reviewed and recorded. Univariate statistical analyses were performed to identify biopsy characteristics that were indicative of nondiagnostic biopsy.
RESULTS
Ultrasound-guided RMBs were diagnostic in 70 out of 82 cases (85%) and non-diagnostic in 12 cases (15%). Among the diagnostic biopsies, 54 (77%) were malignant cases, 94% of which were renal cell carcinoma (RCC). Of the 12 nondiagnostic cases, the final diagnosis was RCC in 4 cases and angiomyolipoma in one case; seven of the nondiagnostic cases were lost to follow-up. A weak association (p = 0.04) was found between the number of needle passes and the biopsy outcome. None of the remaining collected RMB characteristics showed a significant correlation with a diagnostic or nondiagnostic RMB. Six patients (7%) experienced complications.
CONCLUSION
Ultrasound-guided renal mass biopsy is a safe and effective method for the diagnosis of renal masses with a low rate of nondiagnostic outcomes. A nondiagnostic biopsy should not be treated as a surrogate for a diagnosis since a significant number of patients with nondiagnostic biopsies have subsequently been shown to have renal malignancies. Repeat biopsy should be considered in such cases.
Topics: Angiomyolipoma; Carcinoma, Renal Cell; Humans; Image-Guided Biopsy; Kidney; Kidney Neoplasms; Postoperative Complications; Retrospective Studies; Ultrasonography, Interventional
PubMed: 29696566
DOI: 10.1007/s40477-018-0299-0 -
Archives of Pathology & Laboratory... May 2011This review examines the histopathologic, immunohistochemical, ultrastructural, and molecular biologic features of epithelioid angiomyolipoma (EAML), with an emphasis on... (Review)
Review
This review examines the histopathologic, immunohistochemical, ultrastructural, and molecular biologic features of epithelioid angiomyolipoma (EAML), with an emphasis on the differential diagnosis of intra-abdominal EAML. Epithelioid angiomyolipoma is an uncommon mesenchymal tumor with malignant potential, frequently associated with tuberous sclerosis complex. Histologically, EAML is characterized by sheets or nests of large polygonal epithelioid cells with abundant eosinophilic or occasionally clear cytoplasm, often with prominent nucleoli, and EAML may include multinucleated and markedly pleomorphic forms. As these tumors share a distinctive perivascular epithelioid cell phenotype, they belong to the PEComa tumor family. Nearly all EAMLs show immunoreactivity for both melanocytic and myoid markers. Ultrastructurally, EAMLs show evidence of melanogenesis by the presence of premelanosomes. Epithelioid angiomyolipoma can pose significant diagnostic challenges as it mimics morphologically a variety of neoplasms including renal cell carcinoma, renal oncocytoma, adrenal cortical neoplasm, epithelioid smooth muscle tumor, epithelioid peripheral nerve sheath tumor, epithelioid gastrointestinal stromal tumor, epithelioid melanoma, hepatoblastoma, and hepatocellular carcinoma. The variation in immunophenotype in these tumors requires a prudent use of immunohistochemistry, which may occasionally need complementation by electron microscopy to establish the correct diagnosis.
Topics: Abdominal Neoplasms; Angiomyolipoma; Biomarkers, Tumor; Epithelioid Cells; Humans; Immunohistochemistry; Soft Tissue Neoplasms
PubMed: 21526965
DOI: 10.5858/2009-0637-RSR.1 -
Journal of the Formosan Medical... Jan 2019To analyze and present the demography, clinical behavior, especially the risk factors of tumor hemorrhage and management of sporadic angiomyolipoma (SAML), tuberous...
OBJECTIVES
To analyze and present the demography, clinical behavior, especially the risk factors of tumor hemorrhage and management of sporadic angiomyolipoma (SAML), tuberous sclerosis complex associated angiomyolipoma (TSCAML) and epithelioid angiomyolipoma (EAML) in our institution.
METHODS
A retrospective study of 587 patients who were diagnosed with renal angiomyolipoma in our institution between January 2000 and May 2015 was done. The AMLs were diagnosed by ultrasonography, CT, or MRI. EAML was confirmed by histopathology. Medical records and follow-up results were analyzed using the SPSS version 22 software.
RESULTS
Out of 587 cases of renal AMLs, 87.4% were SAMLs, 8.7% were TSCAMLs and 3.9% were EAMLs. Most of the AML patients were asymptomatic. The most common presenting symptoms included flank pain and abdominal pain. The median tumor size of SAML, TSCAML, EAML were 4.7, 2.7, 10.5 cm respectively. Approximately half of SAMLs were conservatively treated, almost all TSCAMLs were treated conservatively, while all EAMLs were surgically treated. The median tumor size of hemorrhagic SAML cases was 8 cm versus non-hemorrhagic cases of 4.1 cm. The optimal cut-off point on the ROC curve for predicting SAML tumor hemorrhage was 7.35 cm.
CONCLUSION
A larger tumor size, younger patient's age and higher BMI value correlated with a higher risk of tumor hemorrhage. For tumor sizes less than 7.35 cm, we recommend active surveillance or TAE for hemorrhage prevention. We also suggest that surgical management should be considered for patients with tumors larger than 7.35 cm, symptomatic and progressive AML, or suspicious EAML.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Angiomyolipoma; Child; Child, Preschool; Diagnosis, Differential; Embolization, Therapeutic; Female; Hemorrhage; Humans; Infant; Kidney; Kidney Neoplasms; Magnetic Resonance Imaging; Male; Middle Aged; ROC Curve; Retrospective Studies; Taiwan; Tomography, X-Ray Computed; Tuberous Sclerosis; Ultrasonography; Young Adult
PubMed: 29549981
DOI: 10.1016/j.jfma.2018.02.012 -
Indian Journal of Pathology &... 2023Renal tumors constitute approximately 3% of all malignancies in adults. They form a heterogenous group with variable morphological, immunohistochemical, and molecular... (Review)
Review
BACKGROUND
Renal tumors constitute approximately 3% of all malignancies in adults. They form a heterogenous group with variable morphological, immunohistochemical, and molecular features.
AIM
The objective of this study was to analyze the spectrum of adult renal tumors at a tertiary care center and study the demographic and histomorphological features.
MATERIALS AND METHODS
In this study, 55/87 nephrectomy specimens resected for adult renal tumors during a 1-year period were analyzed retrospectively.
RESULTS
There were 4 benign (7.2%) and 51 (92.7%) malignant tumors. There was a male preponderance with a male: female ratio of 3.42:1. The tumors were seen to occur equally in both kidneys. The most common tumor was clear cell renal cell carcinoma (RCC), the conventional type accounting for 65.5% of our study group. There were one each of multilocular cystic renal neoplasm of low malignant potential, papillary RCC, chromophobe RCC, Mit family RCC, oncocytoma and angiomyolipoma and two clear cell papillary RCC during this 1-year period. Uncommon tumors included neuroendocrine carcinoma (1), epithelioid angiomyolipoma (1), mixed epithelial stromal tumor (1), Ewings sarcoma (2), and glomangioma (1). Five cases of urothelial carcinoma of renal pelvis/ureter also were present.
CONCLUSION
This article gives an overview of the spectrum of adult renal tumors at a tertiary care center with an in-depth literature review providing recent advances in each category of tumors.
Topics: Adult; Male; Humans; Female; Carcinoma, Renal Cell; Angiomyolipoma; Retrospective Studies; Carcinoma, Transitional Cell; Urinary Bladder Neoplasms; Kidney Neoplasms
PubMed: 37077073
DOI: 10.4103/ijpm.ijpm_53_21