Extrarenal angiomyolipoma in uterine cervix: rare presentation in unusual site

Abstract

Angiomyolipoma is a benign neoplasm composed of variable admixture of blood vessels, smooth muscle cells and adipose tissue. Cervical angiomyolipoma are extremely rare and to the best of our knowledge only five cases of angiomyolipoma in cervix have been reported in the literature till date. Authors are presenting a case of angiomyolipoma arising from the uterine cervix. 43 years old female presented with mass descending per vagina for 6 months. This case had no association with tuberous sclerosis. Microscopic examination showed an ill-defined polypoidal, non-encapsulated lesion covered by keratinized stratified epithelium. The lesion is made up of three components, predominantly by fascicles of spindle shaped cells, varying sized blood vessels and multiple foci of mature adipocytes with no evidence of atypia or increased mitotic activity. Smooth muscle component showed strong immunoreactivity to SMA and absence of elastic fibres in the blood vessels were confirmed by histochemistry. Non-vascular smooth muscle cells were negative for HMB-45 in contrast to renal and other extra-renal angiomyolipoma in which HMB-45 immunoreactivity in seen in these cells. To conclude, the differential diagnosis of lower abdominal mass and dysfunctional uterine bleeding should include the angiomyolipoma, even though the uterine cervix is an extremely rare location where they occur.

Cervical Angiomyolipoma Coexisting with Endometrial Carcinoma in the Absence of Tuberous Sclerosis: A Rare Case Report

“…AML is extremely rare in the female genital tract. 3,5,6 AML in female genital tract usually arises in women over 40 years of age. 6 It is not included in the WHO classification of tumors of the female genital tract.…”

“…1 It is a benign mesenchymal neoplasm that is composed of variable admixture of blood vessels, smooth muscle, and adipose tissue. [2][3][4][5][6] Most common location of this tumor is kidney, in which case, it is associated with tuberous sclerosis. 2,[4][5][6][7][8][9][10] Extrarenal AML has been reported in liver, retroperitoneum, mediastinum, lung, nasal cavity, oral cavity, ear lobe, salivary glands, pharynx, heart, large intestine, bone, soft tissue, skin, thoracic spine, spermatic cord, penis, thigh, foot and infrequently in female genital tract.…”

“…2,[4][5][6][7][8][9][10] Extrarenal AML has been reported in liver, retroperitoneum, mediastinum, lung, nasal cavity, oral cavity, ear lobe, salivary glands, pharynx, heart, large intestine, bone, soft tissue, skin, thoracic spine, spermatic cord, penis, thigh, foot and infrequently in female genital tract. [2][3][4][5][6]8,11 In female genital tract, body of uterus is the most common site of AML and other sites are cervix, fallopian tube, ovary, broad ligament, parametrium, vaginal wall, and vulva [2][3][4][5][7][8][9][10][11][12][13] AML in cervix is extremely rare and only six cases have been reported so far (►Table 1). 4,13 AML belongs to the family of tumors called perivascular epithelioid cell tumors.…”

“…4,5,11 Microscopically, it presents as unencapsulated, polypoidal tumor lined by stratified squamous epithelium. 3 It is composed of three components: the smooth muscle fascicles, thick walled hyalinized blood vessels, and mature adipose tissue. [2][3][4][5][6]11 Verhoeff Van Gieson demonstrates the lack of elastic fibers in blood vessels in this tumor.…”

“…3 It is composed of three components: the smooth muscle fascicles, thick walled hyalinized blood vessels, and mature adipose tissue. [2][3][4][5][6]11 Verhoeff Van Gieson demonstrates the lack of elastic fibers in blood vessels in this tumor. 3 We report the first case of AML of cervix that presented along with adenocarcinoma of endometrium in the absence of tuberous sclerosis.…”