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E-JCRT CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 649

Uterine angioleiomyoma: A rare variant of uterine leiomyoma - A case report and literature review


Department of Pathology and Laboratory Medicine, Pushpanjali Crosslay Hospital, Ghaziabad, New Delhi, NCR, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Dinesh Pradhan
Department of Pathology and Laboratory Medicine, Pushpanjali Crosslay Hospital, Ghaziabad, New Delhi, NCR
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.138127

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 > Abstract 

Uterine angioleiomyoma (AL) is an extremely rare variant of leiomyoma and only 15 cases have been reported till date. Herein we present a case of AL of the uterus in a 39-year-old multiparous female with polymenorrhagia and pain abdomen. A pelvic ultrasonogram showed a large heterogeneously hypoechoic intramural nodule in the posterior myometrium. The patient underwent a total abdominal hysterectomy. Histological examination of the nodule revealed a moderately cellular spindle cell tumor composed of interlacing fascicles of spindle to plump cells swirling around the thick walled vessels. No hypercellularity, pleomorphism, mitotic figures, or necrosis was identified. The spindle to plump cells showed strong and diffuse immunoreactivity for smooth muscle actin, desmin and progesterone receptor, focal and weak positivity for CD10 and estrogen receptor and were negative for CD34 and HMB-45. The Ki-67 labeling index was low (1%). A diagnosis of AL was offered. The patient is on follow up for over 10 months and is asymptomatic.

Keywords: Angioleiomyoma, leiomyoma, uterus


How to cite this article:
Diwaker P, Pradhan D, Garg G, Bisaria D, Gogoi K, Mohanty SK. Uterine angioleiomyoma: A rare variant of uterine leiomyoma - A case report and literature review. J Can Res Ther 2015;11:649

How to cite this URL:
Diwaker P, Pradhan D, Garg G, Bisaria D, Gogoi K, Mohanty SK. Uterine angioleiomyoma: A rare variant of uterine leiomyoma - A case report and literature review. J Can Res Ther [serial online] 2015 [cited 2019 Nov 11];11:649. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/649/138127


 > Introduction Top


Angioleiomyoma (AL) is a rare variant of leiomyoma originating from smooth muscle cells and contains thick walled vessels. [1] It usually occurs in the subcutaneous tissue, most often in the lower extremities, [1] rarely it can be located in the head and neck region, [2] in the submandibular gland [3] and retroperitonium. [4] This uncommon entity has been rarely described in the female urogenital tract. Only 15 cases of this entity have been described in the uterine corpus [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] and we are reporting the 16 th case of uterine AL. Cervix, [18] ovary [19] and the broad ligaments are the other very rare sites of involvement by AL. We came across a case of uterine AL in a 39-year-old female which was diagnosed after histopathologic examination.


 > Case report Top


The present case report is about a 39-year-old multiparous female presented to gynecology out-patient department with polymenorrhagia and pain in the lower abdomen. The general and systemic examination of the patient was within the normal limits. The per speculum examination revealed moderate cervical erosion on the posterior lip. Chest X-ray and all the laboratory tests were within the normal limits. The ultrasonogram of the pelvis showed a large heterogeneously hypoechoic lesion in the posterior myometrium, which measured 4.5 × 3.5 × 3 cm [Figure 1a]The cervical smear was negative for intraepithelial lesion or malignancy. The cervical biopsy showed chronic erosive cervicitis with focal squamous metaplasia. The patient underwent a total abdominal hysterectomy and the specimen was submitted for histopathologic examination.
Figure 1a: Ultrasonographyof the pelvis showing large heterogeneously, hypoechoic lesion measuring 4.5 cm × 3.5 cm × 3 cm seen in posterior myometrium (left lateral aspect) indenting and displacing endometrial echo. No definite hyperechoic foci to suggest calcification seen within the lesion

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Pathological examination

On gross examination, the uterine corpus measured 8 × 7 × 4.5 cm. On bivalving the uterus, a tan-gray lobulated intramural nodule was noted in the posterior myometrium. The nodule measured 4.5 × 3.5 × 3 cm; the cut surfaces of the nodule were whorled, tan-grey to tan-white with foci of congestion; no area of softening was present [Figure 1b]
Figure 1b: Macroscopic image of uterine angioleiomyoma: The cut sections of the tan-gray lobulated intramural nodule were whorled, tan-grey to tan-white with foci of congestion; no area of softening was present

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Histologic examination of the nodule showed a moderately cellular spindle cell tumor composed of interlacing fascicles of spindle to plump cells with interspersed abundant thick walled vessels. The spindle cells were seen swirling around the vessels [Figure 2a] and [Figure 2b]. No hypercellularity, pleomorphism, mitotic figures, or necrosis was identified.
Figure 2a: Microscopic image of uterine angioleiomyoma: The intramural nodule showed a moderately cellular spindle cell tumor composed of interlacing fascicles of spindle to plump cells with interspersed abundant thick muscular walled vessels (H and E, ×10)

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Figure 2b: Microscopic image of uterine angioleiomyoma: The tumor cells were seen swirling around the thick walled blood vessels. No pleomorphism or mitotic figures was identified (H and E, ×40)

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On immunohistochemistry, the spindle cells showed positivity for smooth muscle actin (SMA) [Figure 3]a, desmin [Figure 3]b and progesterone receptor [Figure 3]d. Faint focal positivity was seen in spindle cells for CD10 [Figure 3]c and estrogen receptor. CD34 was positive in the vascular endothelium and negative in the spindle cells. HMB-45 was negative. The Ki-67 labeling index was less than 1%. CD10 negativity and histologic features ruled out an endometrial stromal nodule. Similarly HMB-45 negativity argued against a perivascular epithelioid cell tumor (PECOMA). A diagnosis of AL was offered. The patient was asymptomatic on follow up for over 10 months.
Figure 3: Immunohistochemical profile of angioleiomyoma: (a) Smooth muscle actin positive (Immunoperoxidase stain for smooth muscle actin, ×40); (b) desmin positive (immunoperoxidase stain for desmin, ×40); (c) CD10 focal, weak positive (Immunoperoxidase stain for CD10, ×40); (d) progesterone receptor positive (original immunoperoxidase stain for progesterone receptor, ×20)

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 > Discussion Top


Leiomyomas are the most common uterine neoplasms. There are different variants of leiomyomas viz. cellular, epithelioid, myxoid, lipoleiomyoma etc., and amongst these variants AL is extremely rare, as till date only fifteen cases of AL of the uterine corpus have been described in the English literature. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] Uterine AL usually occurs in the middle aged females. The age range in the cases described is from 30 years to 69 years. Most commonly the patients present with menorrhagia, pain abdomen and/or abdominal mass. Handler et al. have described a case of consumptive coagulopathy secondary to a large degenerated AL. [7] Rarely the patient may present with Pseudo-Meig syndrome and raised CA125 levels, thereby mimicking an ovarian neoplasm. [8]

AL commonly present as a well-circumscribed mass arising from the uterine corpus. Few cases with multiple uterine AL have also been reported. [5],[13] They range from 4 cm to 26 cm in the maximum dimension. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] These can be submucosal, intramural and/or subserosal. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] Those arising in the posterior myometrium in a subserosal location may present as a pelvic mass and may be difficult to differentiate from primary ovarian neoplasm preoperatively. [5] On sectioning, the cut surfaces of AL are tan-white, whorled with hemorrhagic areas, or may exhibit a variegated appearance with pink-brown and gray areas. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] Some are multiloculated with blood filled cysts. Histologically, AL is composed of interlacing fascicles of spindle cells with interspersed abundant thick walled blood vessels. The monotonous and spindled smooth muscle cells swirl around the blood vessels. This is in contrast to the usual leiomyomas in which the density of the vascular network is similar to or less than the normal myometrium. [20] Moreover, the blood vessels in the usual leiomyoma are predominantly capillaries along with a few arterioles and small arteries in contrast to the thick walled muscular vessels as seen in AL. Most of these lesions lack mitotic figures, pleomorphism, or necrosis. Mitosis up to 2 mitoses per 10 high power fields was seen in one reported case. [10] This emphasizes the importance of extensive sampling in cases where there is nuclear atypia or mitosis in order to exclude a leiomyosarcoma. Other changes that have been described in this tumor are myxoid change, edema, hyalinization of the stroma and fibrin deposition in the vessel wall. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] Even though, fibrin thrombi within the vessels are characteristically present in AL of the skin, only two cases of uterine AL revealed organizing thrombi. [7] AL may undergo degenerative changes with large cavernous deformation of the vascular spaces. On immunohistochemistry, the spindle cells show diffuse and strong positivity for SMA, desmin and caldesmon and a low Ki-67 labeling index.

The microscopic differential diagnoses include endometrial stromal nodule, angiomyofibroblastoma and PECOMA. Though each one of these has characteristic morphologic features, at times overlapping histologic features may warrant the use of immunohistochemistry to arrive at the correct diagnosis [Table 1]. If extensive nuclear atypia is present in the tumor, extensive sampling should be done to look for increased or atypical mitosis, border of the tumor and necrosis in order to exclude a leiomyosarcoma.
Table 1: Immunohistochemical markers to differentiate angioleiomyoma from its morphologic mimickers (endometrial stromal nodule, angiomyofibroblastoma and perivascular epithelioid cell tumor)

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As AL is a benign tumor, complete excision of the lesion remains the main stay of the treatment. Total hysterectomy with or without salpingo-oophrectomy is the treatment of choice and was done in all the reported cases except one where the patient underwent myomectomy. [10] All patients reported had an uneventful postoperative course with clinical improvement in the symptoms. No recurrence was reported in any case. Thus, if completely excised these tumors have an excellent prognosis. However, the role of myomectomy alone or uterus preserving surgery has not been studied extensively and will need further research.


 > Conclusion Top


AL is a unique variant of uterine smooth muscle neoplasm that exhibit characteristic morphology and immunophenotypic features. AL has no specific imaging findings to differentiate it from other smooth muscle neoplasm and hence pre-operative differential diagnosis from other tumors is extremely difficult. Therefore, it is important for the clinician and surgical pathologist to recognize this rare benign entity and differentiate it from its mimickers including endometrial stromal tumor and leiomyosarcoma by thorough sampling and when required utilizing a proper immunohistochemistry panel.

 
 > References Top

1.
Weiss SW, Goldblum JR. Benign tumors of smooth muscle. In: Weiss SW, Goldblum JR, editors. Enzinger and Weiss′s Soft Tissue Tumors. 4 th ed. St. Louis: Mosby; 2001. p. 699-700.  Back to cited text no. 1
    
2.
Wang CP, Chang YL, Sheen TS. Vascular leiomyoma of the head and neck. Laryngoscope 2004;114:661-5.  Back to cited text no. 2
    
3.
Ide F, Mishima K, Saito I. Angiomyoma in the submandibular gland: A rare location for a ubiquitous tumour. J Laryngol Otol 2003;117:1001-2.  Back to cited text no. 3
    
4.
Paal E, Miettinen M. Retroperitoneal leiomyomas: A clinicopathologic and immunohistochemical study of 56 cases with a comparison to retroperitoneal leiomyosarcomas. Am J Surg Pathol 2001;25:1355-63.  Back to cited text no. 4
    
5.
Hakverdi S, Dolapçioðlu K, Güngören A, Yaldiz M, Hakverdi AU. Multiple uterine angioleiomyomas mimicking an ovarian neoplasm: A case report. Eur J Gynaecol Oncol 2009;30:592-4.  Back to cited text no. 5
    
6.
Koshy AK, Chaturvedula L, Habeebullah S, Basu D. Angiomyoma of the uterus: Case report and review of literature. Gynecol Surg 2008;5:169-71.  Back to cited text no. 6
    
7.
Handler M, Rezai F, Fless KG, Litinski M, Yodice PC. Uterine angioleiomyoma complicated by consumptive coagulopathy. Gynecol Oncol Case Rep 2012;2:89-91.  Back to cited text no. 7
    
8.
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.  Back to cited text no. 8
    
9.
Sahu L, Tempe A, Agrawal A. Angioleiomyoma of uterus. J Obstet Gynaecol 2012;32:713-4.  Back to cited text no. 9
    
10.
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.  Back to cited text no. 10
    
11.
Sakai Y. Epithelioid vascular leiomyoma of the uterus mimicking glomangiomyoma. Arch Gynecol Obstet 2007;275:59-61.  Back to cited text no. 11
    
12.
Culhaci N, Ozkara E, Yüksel H, Ozsunar Y, Unal E. Spontaneously ruptured uterine angioleiomyoma. Pathol Oncol Res 2006;12:50-1.  Back to cited text no. 12
    
13.
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.  Back to cited text no. 13
    
14.
Agorastos T, Dinas K, Patsiaoura K. Cystic degenerated angioleiomyoma mimicking ovarian pathology. Acta Obstet Gynecol Scand 2001;80:863-5.  Back to cited text no. 14
    
15.
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.  Back to cited text no. 15
    
16.
Jameson CF. Angiomyoma of the uterus in a patient with tuberous sclerosis. Histopathology 1990;16:202-3.  Back to cited text no. 16
    
17.
Konichezky M, Reif R, Bukovsky I. Benign angiomyoma of the uterus with unusual macroscopic appearance. Int J Gynaecol Obstet 1980;18:4-6.  Back to cited text no. 17
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18.
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.  Back to cited text no. 18
    
19.
Bouraoui S, El Amine El Hadj O, Rekik W, Goutallier-Ben Fadhel C, Kébir FZ, Lahmar A, et al. First case of angioleiomyoma originating from the ovary of an adult woman. Gynecol Obstet Invest 2010;70:8-10.  Back to cited text no. 19
    
20.
Walocha JA, Litwin JA, Miodoñski AJ. Vascular system of intramural leiomyomata revealed by corrosion casting and scanning electron microscopy. Hum Reprod 2003;18:1088-93.  Back to cited text no. 20
    


    Figures

  [Figure 1a], [Figure 1b], [Figure 2a], [Figure 2b], [Figure 3]
 
 
    Tables

  [Table 1]



 

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