|Year : 2021 | Volume
| Issue : 2 | Page : 179-184
Angioleiomyoma of uterus and cervix: A rare report of two cases
Ankit Seth, Anjali Mathur
Department of Pathology, Kasturba Hospital, New Delhi, India
|Date of Submission||20-Jul-2019|
|Date of Decision||22-Feb-2021|
|Date of Acceptance||30-May-2021|
|Date of Web Publication||27-Jul-2021|
Department of Pathology, Kasturba Hospital, New Delhi - 110 002
Source of Support: None, Conflict of Interest: None
| Abstract|| |
We report two rare cases of genital angioleiomyomas (ALs), one each of uterus and cervix. The uterine AL showed a very rare presentation of endometrial polyp, while the cervical AL presented as an intramural cervical growth. We have also reviewed the literature and enlisted all uterine and cervical ALs reported till now.
Keywords: Angioleiomyoma, cervical, uterine
|How to cite this article:|
Seth A, Mathur A. Angioleiomyoma of uterus and cervix: A rare report of two cases. J Mid-life Health 2021;12:179-84
| Introduction|| |
Angioleiomyoma (AL) is a common tumor of extremities and the head-and-neck region but only rarely reported in uterus, cervix, ovary, and broad ligament. It is supposedly a morphological variant of leiomyoma, the most common tumor of the uterus. Uterine AL is quite a rare entity, and as per our search, only 38 case reports have been described in the literature so far [Table 1]. Out of them, only a very few have been described as a polypoid uterine angioleiomomatous lesion,, which makes our case (Case 1) rarer.
Another case we report is a cervical AL, of which we could find only three case reports reported in the literature so far [Table 2].,, None of them showed a lipomatous component in contrast to our case (Case 2) where it is present, making it the first cervical AL of its kind.
| Case Reports|| |
A multiparous patient of known hyperthyroidism presented with discharge per vaginum, heavy vaginal bleeding during periods, and dysmenorrhea for 2 years. Per speculum examination revealed an unhealthy cervix with the presence of discharge and bleeding. Furthermore, a polyp at external os measuring 3 cm × 3 cm was noticed. The rest of the clinical examination was within the normal limits. Her Pap examination showed endocervical squamous metaplasia. Her routine hematological, biochemical, urine, and X-ray chest findings were unremarkable. Venereal Disease Research Laboratory test was negative. Human immunodeficiency virus and Hepatitis C virus antibodies were nonreactive. Ultrasound examination of the abdomen showed an endometrial polyp arising from the anterior uterine wall with increased vascularity and loss of posterior wall differentiation. Bilateral adenexae were normal. Endometrial biopsy done 2 months ago showed proliferative endometrium. The patient had a history of pulmonary tuberculosis 22 years ago which was treated satisfactorily by antituberculous treatment. Hysteroscopic polypectomy was done and polyp sent for the histopathological examination.
A 47-year-old known diabetic woman presented with heavy cyclical vaginal bleeding for 3 months. Her abdominal examination was unremarkable. Per speculum examination revealed ectropion of the lower lip of cervix with erosion. Ultrasound examination of the abdomen showed a nabothian cyst on the anterior lip of cervix. A round irregular marginated echogenic mass (? polyp) measuring 22 mm × 20 mm was seen on cervix. Hematological, biochemical, and urine examinations were unremarkable. A clinical diagnosis of dysfunctional uterine bleeding with cervical polyp was made. Total abdominal hysterectomy was done and specimen sent for the histopathological examination.
Clinical history and findings of cases are summarized in [Table 3].
Gross specimen of Case 1 showed a polyp measuring 6 cm × 3 cm × 2 cm. Its cut surface was grayish brown and solid. Gross specimen of Case 2 showed uterus and cervix measuring 10 cm × 3 cm × 2 cm. Cut section of the uterus was unremarkable; endometrium measured 1.2 cm. Cervix was elongated measuring 3 cm length. Cut surface of cervix showed a well-defined grayish-white round growth on the lower end measuring 3.2 cm in diameter. Microscopic examinations of polyp (Case 1) and lower cervical end growth (Case 2) showed almost similar picture, comprising spindle-shaped cells, arranged in fascicles or evenly distributed in compact form. Cells had spindle-shaped nucleus with no atypia or mitosis. In between were areas having thick-walled arterioles, sheathed by spindle cell layers and showing areas of vessel wall hyalinization. Hemorrhagic areas as well as few fibrin clots were also seen in and around vessels. Occasional fat cells with areas of hyalinization were noticed in the smooth muscle component areas in the cervical lesion [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d, [Figure 1]e, [Figure 1]f. Microscopic examinations of the uterus and cervix of Case 2 were unremarkable.
|Figure 1: Uterine angioleiomyoma with perivascular swirling of smooth muscles and adjacent normal uterine tissue (arrow head) (H and E, ×50, ×100, ×100, ×400, a-d); cervical angioleiomyoma with occasional clusters of fat cells (arrows) and adjacent normal cervical tissue (arrow head) (H and E, ×50, e-f)|
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Immunohistochemically, both the cases were diffusely positive for smooth muscle antigen (SMA) in fascicles encircling blood vessels as well as in the smooth muscle component in the background and CD 34 positivity in endothelial lining of blood vessels. Estrogen receptor staining was weekly positive to negative while progesterone receptor staining was negative. CD 10, S-100, Sudan Black, and HMB-45 were negative in all cases [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d, [Figure 2]d, [Figure 2]f.
|Figure 2: Immunohistochemistry of uterine angioleiomyoma showing strong smooth muscle antigen positivity in perivascular smooth muscle fascicles (×100, a); negative reaction with CD 10 (×100, b); positive CD 34 staining in vascular endothelium (×100, c); negative Estrogen receptor staining (×100, d); focally positive progesterone receptor staining (×100, e); negative staining with reticulin stain (×100, f)|
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| Discussion|| |
ALs are benign tumors which are considered a distinct variant of leiomyomas. They are predominantly present in subcutis of lower extremities and rarely in the head and neck region and trunk.  Very rarely, they occur in the female reproductive tract, mostly in middle aged women and usually present with abnormal uterine bleeding and pain in the abdomen. They may also present with atypical symptomatology such as acute abdomen, massive bleeding per vaginum, Pseudo-Meigs Syndrome, puberty menorrhagia, coagulopathies, or catastrophic events such as rupture of the uterus.
Pathogenesis of AL is unclear. Few authors have found karyotypic abnormalities in such patients. Histogenetically, most authors believe these tumors to be hamartomatous, akin to renal angiomyolipomas. This view is substantiated by the presence of mature fat cells in some of the cases, as in our case (Case 2). Abnormal uterine bleeding often results from dysregulated growth factors and their receptors, which affect vascular morphology and regulate angiogenesis, while pain may be caused by ischemia or vascular contraction.
Microscopically, three histological types are distinguished in uterine or cervical AL, capillary or solid, cavernous, and venous. Tumors of the solid type are composed of abundant small-sized vascular channels in the background of compact and intersecting smooth muscle bundles which also sheath these channels. Tumors of the cavernous type have dilated vascular channels with smaller amounts of smooth muscle tissue in their wall, merging imperceptibly with the surrounding smooth muscle bundles. Tumors of the venous type consist of vascular channels with thick muscular walls of venous type with not so compact smooth muscle background. Hyaline and myxoid degeneration may be present, as seen in our cases also. Both of our cases are of venous variant.
The differential diagnosis of uterine AL depends upon the histopathological picture. In our case 1, it was confused with endometrial stromal tumor (EST) because of strikingly similar arrangement of blood vessels. However, on closer scrutiny, other features of EST such as resemblance with endometrial stroma, hyalinization, infiltrative margins, and individual cell encircling by reticulin-positive cells were missing. CD-10 negativity along with diffuse SMA positivity confirmed the diagnosis of AL, as CD-10 positivity is usually strong in EST but negative or weak in AL, as in our case. Another differential diagnosis is perivascular epithelioid cell tumor (PEcoma), distinguished by its clear/eosinophilic cytoplasm and HMB 45 positivity, which is negative in AL. Vascular leiomyoma can be reasonably distinguished by its myometrium-like capillaries and few arterioles unlike thick sheathed blood vessels of AL. Moreover, sheer number and density of AL are very different from that of vascular leiomyomas. Fibrin deposition in the vessel walls is also an important feature of AL (as seen in our cases too) but unusual in vascular leiomyomas.
Mitosis is sometimes seen in AL but is usually <2 per 10 HPF. In cases with higher mitotic count, differential diagnosis of leiomyosarcoma should be considered if it coexists with necrosis, infiltration, and atypical nuclear features. Rarely, atypical ALs may also be present. Hence, the importance of thorough sampling of the specimen cannot be overemphasized.
In our experience, endometrial or cervical polyps are sometimes replete with proliferating vascular channels, with spindly compact stroma. Misdiagnosis of AL in such cases can be avoided by SMA staining which is positive in vascular walls but negative in stroma, unlike AL where both tissues are strongly positive.
AL of uterus can rarely be diagnosed in patients without histopathology although infrequently, an alert ultrasonologist can suspect it by increased vascularity in a lesion showing echogenicity different from typical leiomyomas, as in our Case 1. Sometimes, contrast-enhanced computed tomography may also indicate its possibility by revealing the presence of multivascular branches within the tumor mass and uterine artery hypertrophy.
Treatment is hysterectomy. Procedures such as myomectomy are known to have increased rate of incomplete removal and recurrence or even intra-operative failure of surgeon to reach plane of cleavage and uncontrolled hemorrhage. However, sometimes angiomyomectomy with free margins may be attempted in unmarried of childless women.
At present, the WHO does not recognize AL of uterus or cervix as a distinct tumor. In this context, we agree with McCluggage and Boyde who proposed that the WHO should include AL among benign variants of uterine leiomyomas.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We would like to thank Dr. Namrata, Head of Department, Hindurao Hospital for her support in immunohistochemistry.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Gupta M, Suryawanshi M, Kumar R, Peedicayil A. Angioleiomyoma of Uterus: A Clinicopathologic Study of 6 Cases. Int J Surg Pathol 2018;26:18-23.
McAdams CR, Athanasatos G, Jorizzo JR. Case report of a uterine angioleiomyoma. Ultrasound Q 2016;32:384-6.
Koleskas D, Karagiannis G, Beukenholdt RW. A case of a cervical angioleiomyoma presenting with menorrhagia and pelvic pain: A common presentation of a rare tumour. J Obstet Gynaecol 2009;29:161-3.
Al-Sannaa GA, Al-Manea M. Cervical angioleiomyoma. J Obstet Gynaecol 2011;31:555.
Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126-30.
Thomas S, Radhakrishnan L, Abraham L, Matthai A. Uterine Angioleiomyoma with Atypia, Raised CA-125 Levels, and Pseudo-Meigs Syndrome: An Alarming Presentation. Case Rep Pathol 2012;2012:519473.
Kamath MS, Acharya M, Kamath V, Aleyamma TK. Uterine angioleiomyoma with hypofibrinogenemia: A rare presentation. J Gynecol Surg 2014;30:50-2.
Culhaci N, Ozkara E, Yüksel H, Ozsunar Y, Unal E. Spontaneously ruptured uterine angioleiomyoma. Pathol Oncol Res 2006;12:50-1.
Hennig Y, Caselitz J, Stern C, Bartnitzke S, Bullerdiek J. Karyotype evolution in a case of uterine angioleiomyoma. Cancer Genet Cytogenet 1999;108:79-80.
Hsieh CH, Lui CC, Huang SC, Ou YC, ChangChien CC, Lan KC, et al.
Multiple uterine angioleiomyomas in a woman presenting with severe menorrhagia. Gynecol Oncol 2003;90:348-52.
McCluggage WG, Boyde A. Uterine angioleiomyomas: A report of 3 cases of a distinctive benign leiomyoma variant. Int J Surg Pathol 2007;15:262-5.
Sharma C, Sharma M, Chander B, Soni A, Soni PK. Angioleiomyoma uterus in an adolescent girl: A highly unusual presentation. J Pediatr Adolesc Gynecol 2014;27:e69-71.
[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]