Case Report: Angiomyolipoma of the cervix – report of a rare entity

Abstract

Angiomyolipoma (AML) is a mesenchymal neoplasm seen usually in the kidney. Few cases of extra renal AML have been documented in various organs including the female genital tract, where the uterus is the most common site. To the best of our knowledge, only 4 cases of AML in the cervix have been reported in the literature. Association of AML with tuberous sclerosis is well known. Presently AML is included in the spectrum of disease entities called PEComa. We report a case of AML without tuberous sclerosis arising from the uterine cervix, which has to be differentiated from lipoleiomyoma.

Extrarenal angiomyolipoma in uterine cervix: rare presentation in unusual site

“…Literature has shown that patients with tuberous sclerosis have 5%-50% risk to develop angiomyolipomas. 8 In the present case, the patient was not associated with tuberous sclerosis.…”

“…6,7 It is interesting to know that HMB-45 was negative for nonvascular smooth muscle cells in this case, in contrast to angiomyolipomas occurring in renal and other extrarenal sites where HMB-45 is positive in these cells. 8 According to Aung et al, 20% angiomyolipomas are negative for HMB-45. This should be kept in mind while using HMB-45 alone for diagnosing angiomyolipoma occurring in uterine.…”

“…Lipoleiomyomas show mature adipocytes along with muscle cells, but no prominent vascular elements as compared to angiomyolipoma, where there are prominent thick-walled blood vessels. 8 Degenerated myomas may also mimick angiomyolipomas but they can be distinguished from angiomyolipoma by echogenicity, without shadowing and irregular margins. 8 Angiomyolipoma are usually benign, but they may be associated with haemorrhage and even show invasion of the surrounding organs.…”

“…8 Degenerated myomas may also mimick angiomyolipomas but they can be distinguished from angiomyolipoma by echogenicity, without shadowing and irregular margins. 8 Angiomyolipoma are usually benign, but they may be associated with haemorrhage and even show invasion of the surrounding organs. Therefore, recently angiomyolipoma is considered as a slow growing malignant neoplasm with ability to metastasize.…”