Renal Angiomyolipoma: A Clinicopathological study in a Tertiary Care Centre

Annie Mary Prashanthy, T (2020) Renal Angiomyolipoma: A Clinicopathological study in a Tertiary Care Centre. Masters thesis, Christian Medical College, Vellore.

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Abstract

INTRODUCTION: Angiomyolipoma (AML) is a benign mesenchymal tumour with an incidence of less than 1% of surgically excised renal tumours. AML is composed of variable proportions of dysmorphic blood vessels (angio), epithelioid smooth muscle (myo), spindle cells and mature adipose tissue(lipo).Though these tumours are mostly seen in kidney, they are also seen in spleen, uterus, liver and fallopian tube. The term “angiomyolipoma” was first coined by Morgan in 1951 to describe benign renal tumor composed of blood vessels, muscle, and fat. Angiomyolipoma belongs to the family of lesions called (PEComas) perivascular epithelioid cell tumours, recognised by World Health Organization (WHO) in 2002. These PEComas includes angiomyolipoma(AML), lymphangioleiomyomatosis (LAM) and other perivascular epithelioid cell tumours occurring in soft tissue, cutaneous and visceral sites in the body. OBJECTIVES: 1. To evaluate the prevalence and clinicopathological characteristics of renal angiomyolipoma. 2. To evaluate the association of renal angiomyolipoma with renal or other malignancies and with tuberous sclerosis. METHODS: This retrospective descriptive study was carried out in the Department of Pathology, Christian Medical College, and Vellore during the period 2017-2019 on diagnosed cases of renal angiomyolipoma from 2001 to 2018. Demographic and clinical details were obtained from the electronic medical records. The histopathological and immunohistochemical details of all cases were reviewed either from archives or by examining the slides. Data analysis was done using Statistical Package for Social Services (SPSS) software Version 16.0. The association between the categorical variables was done using Chi-square statistics/Fisher exact test where a P value of <0.05 was considered statistically significant. RESULTS: Angiomyolipoma had a female preponderance (F: M = 2.2:1) with median age at diagnosis of 41±11.7 years. Fifty one cases (58%) s were symptomatic. Seventy nine (90%) were classic AML, while 9 (10%) were epithelioid AML. Ten AMLs (11%) were associated with Tuberous sclerosis (TS). Compared to sporadic cases those associated with TS differed in tumour size (P = 0.029), were more often bilateral (P=0.000) and multiple (P=0.003). This relatively large series of renal AMLs from a tertiary care centre in India highlights the clinicopathological features of this rare tumor. CONCLUSION: • Prevalence of renal AML was 0.1% of all surgically resected renal tumours in this series. • There was a female preponderance, M:F (1:2.2). • Renal AMLs occur predominantly sporadically (89%) with few associated with tuberous sclerosis (11%). • Renal AML had a median age at diagnosis of 41 years (range 14-68 years). • Sporadic cases had a mean age at diagnosis of 37.4years (+/-11.01) a decade younger than AML cases associated with tuberous sclerosis. • More than 50% of cases were symptomatic with flank pain being most common clinical presentation. • Renal AML cases had associated co- morbidities like hypertension and diabetes. • There was no significant relationship between size of tumour and occurrence of symptoms. • Fat poor AMLs can mimic radiologically malignant renal neoplasms like renal cell carcinoma (RCC) radiologically. • The most common site of occurrence was lower pole followed by upper pole. • The most common side involved was right side. • Compared to sporadic cases those associated with tuberous sclerosis differed in tumour size, and were more often bilateral and multiple. • Multicentricity and lymph node involvement did not indicate malignant behavior. • Renal AMLs were predominantly well circumscribed benign tumours with yellow brown to dark brown cut surface. • Classic variant was more common than the epithelioid variant (10%). • Epithelioid variants are more often associated with intratumoral hemorrhage. • Immunohistochemical profile showed co expression of smooth-muscle actin and HMB45.

Item Type: Thesis (Masters)
Additional Information: 201713351
Uncontrolled Keywords: Renal Angiomyolipoma, Tertiary Care Centre, Clinicopathological study.
Subjects: MEDICAL > Pathology
Depositing User: Subramani R
Date Deposited: 29 Jan 2021 17:51
Last Modified: 29 Jan 2021 17:51
URI: http://repository-tnmgrmu.ac.in/id/eprint/13429

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