|Year : 2019 | Volume
| Issue : 3 | Page : 211-214
Vascular leiomyoma of the leg
B Nishan, Vivekanand, M Vishnu, Sumanth Raj, K Sivakrishna, Hudgi Vishal, Vaibhav Lende, C PS Sravan, Hemant Chaudhari, S Roshan Rodney
Department of Vascular Surgery, Jain Institute of Vascular Sciences, A Unit of Bhagwan Mahaveer Jain Hospital, Bengaluru, Karnataka, India
|Date of Web Publication||29-Aug-2019|
Dr. B Nishan
Department of Vascular Surgery, Jain Institute of Vascular Sciences, A Unit of Bhagwan Mahaveer Jain Hospital, Bengaluru, Karnataka
Source of Support: None, Conflict of Interest: None
A 48-year-old women with a subcutaneous vascular leiomyoma of the right leg, presented with pain in the right mid-leg medial aspect. Clinical symptoms, radiological images, and histopathological features are reported. Vascular leiomyoma should be included in the differential diagnosis of painful lower-extremity subcutaneous lesions.
Keywords: Histopathological features, painful lower-extremity subcutaneous lesions, vascular leiomyoma
|How to cite this article:|
Nishan B, Vivekanand, Vishnu M, Raj S, Sivakrishna K, Vishal H, Lende V, Sravan C P, Chaudhari H, Rodney S R. Vascular leiomyoma of the leg. Indian J Vasc Endovasc Surg 2019;6:211-4
|How to cite this URL:|
Nishan B, Vivekanand, Vishnu M, Raj S, Sivakrishna K, Vishal H, Lende V, Sravan C P, Chaudhari H, Rodney S R. Vascular leiomyoma of the leg. Indian J Vasc Endovasc Surg [serial online] 2019 [cited 2020 Feb 25];6:211-4. Available from: http://www.indjvascsurg.org/text.asp?2019/6/3/211/265788
| Introduction|| |
Leiomyoma is a benign, slow-growing tumor derived from smooth muscle cells and is predominantly found in uterus, esophagus, gastrointestinal system, pleura, and lower extremities where smooth muscles are located.,
Vascular or angioleiomyoma, is a rare mesodermal, benign tumor arising from smooth muscle cells of the arterial or venous walls. This type of tumor can arise from any part of the body and can be found in the dermis, subcutaneous fat, or the deep fascia. It commonly occurs in the extremities, most common in lower limbs (50%–70%). Clinically, it often presents as a small (<20 mm), slow-growing, firm, mobile mass associated with pain in approximately 60%.,
Vascular leiomyomas represent around 5% of soft-tissue benign tumors. It is more common in the third and sixth decades of life, more in females than that in males. The etiology of vascular leiomyoma includes traumatic venous congestion, pregnancy, and infection. Hormonal alterations and arteriovenous malformations have also been postulated.
Clinical diagnosis is very difficult due to their rare occurrence. Differential diagnosis for painful subcutaneous extremity lesions includes glomus tumor, hemangioma, angiolipoma, ganglion, and traumatic neuroma.
Ultrasound is the usual initial evaluation of soft-tissue masses and the treatment is usually surgical excision. Diagnosis is confirmed by histopathological examination. Due to the biological characteristics of leiomyoma, the recurrence rate is low after surgical excision.
This report presents a female patient with vascular leiomyoma of the right mid-leg. Written informed consent was obtained from the patient for publication of this case report and accompanying images.
| Case Report|| |
A 48-year-old women presented in June 2018 with complaints of pain in the right leg, medial aspect for 2 years, episodic pain after prolonged walking or intense activity. She had no other symptoms or comorbidities. Systemic examination was normal. There was a 0.5 cm × 0.5 cm palpable, tender, freely mobile subcutaneous nodule on the mid, medial aspect of the right leg. There was no evidence of arterial or venous insufficiency in both legs.
Right lower-limb venous duplex scan revealed cystic, noncompressible lesion in the medial aspect of the right leg in the subcutaneous plane measuring 5.4 mm × 4.5 mm with overlying probe tenderness. The lesion showed swirling pattern of flow and suspected to be partially thrombosed pseudoaneurysm arising from the cutaneous artery and no evidence of venous disease. Magnetic resonance (MR) angiography [Figure 1] of the right leg revealed a small hyperintense area within the medial superficial subcutaneous compartment. It was seen arising from the distal branch of the superficial vein and opacified during the venous phase. It measured about 6.5 mm (SI) and 5.5 mm (AP) suggestive of small venous aneurysm, with no evidence of adjacent vascular malformation or prominent arterial feeders or early draining veins. It was clinically diagnosed with the glomus tumor of the leg.
|Figure 1: Magnetic resonance angiogram of the right leg-green spot highlighting the lesion|
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The patient underwent surgical excision [Figure 2] under local anesthesia. A horizontal linear incision is made over the palpable lesion. The lesion was localized and excised and specimen [Figure 3] sent for histopathology.
The histopathology [Figure 4] reported a whitish nodule, measuring 8.0 mm in the greatest dimension, well encapsulated. It was composed of fascicles of spindle-shaped cells with fibrillary eosinophilic cytoplasm, with the lesion showing many thick-walled vessels and inferred to be vascular leiomyoma.
The patient experienced complete relief of pain after surgery. During follow-up, the patient was pain free with complete resolution of symptoms.
| Discussion|| |
Vascular leiomyoma is a benign solitary tumor of smooth muscle cells of the tunica media of the vessels. Stout published the first comprehensive review of this rare lesion in 1937.
Angioleiomyomas account for 5% of all benign neoplasms of soft tissues. Minor trauma, venous stasis and hormonal changes, especially that of estrogen, have been proposed as etiological features. The presence of chronic inflammatory cell infiltrates in some lesions supports the venous stasis theory. A few karyotypic abnormalities 6p21-23, 13q, and 21q21 rearrangements and translocation t(X; 10) (q22;q23) have also been described in the literature.
The peak incidence is between the third and sixth decades of life, and it occurs more frequently in females than males, with ratio ranging from 1.6 to 2.3:1.
Vascular leiomyoma can occur anywhere in the body and can be found in the dermis, subcutaneous fat, and fascia. The lesion occurs usually in the extremities, with 60% in the lower extremity below the thigh. In the upper extremity, the distal sites predominate, while occurrence in the trunk, head, oral cavity, and perioral region, especially lip and palate, is rare.,
The typical lesion is a solitary, small, slow-growing, firm, and mobile subcutaneous nodule. Majority of them measure less than 20 mm. Pain is the most striking clinical feature of angioleiomyoma (58% in Hachisuga et al. series and 62% of patients from Mayo clinic). Pain is often paroxysmal and is provoked by exposure to cold and wind. It is thought to be due to active contraction of smooth muscle resulting in local ischemia. Rest appears to be a relieving factor. Examination may show compressibility, with pain on compression.,
Painful subcutaneous extremity lesions such as glomus tumor, hemangioma, angiolipoma, ganglion cysts, and traumatic neuroma should be considered for differential diagnosis.,
X-ray findings are usually normal, but rarely, dystrophic calcification may be seen. On ultrasound examination, angioleiomyoma shows well-differentiated margins and a homogenous structure, suggesting benign nature of the lesion. Colored Doppler examination shows high resistance in intratumor arteries, suggesting the presence of muscular arteries. MR imaging (MRI) features and T2-weighted MR images show mixed areas that are hyperintense and hypointense to skeletal muscle. Hyperintense areas on T2-weighted MR images show strong enhancement after intravenous contrast injection. Isointense areas on T2-weighted MR images did not show enhancement after intravenous contrast. Hyperintense areas on T2-weighted MR images correspond to the smooth muscle bundle cells and isointense areas on T2-weighted MR images correlate to the tough fibrous tissue or intravascular thrombi. MR images cannot differentiate between histological subtypes. For an extremity mass with mixed areas of hyper- and isointensity to skeletal muscle on T2-weighted MR images and with a hypointense rim, a diagnosis of vascular leiomyoma should be considered. MRI is very useful for distinguishing the benign and malignant soft-tissue tumors and describing the anatomic borders and vascularity preoperatively.,
Histopathological identification with staining techniques, such as Masson's trichrome, hematoxylin-eosin, alcian blue, van-Gieson and Periodic Acid-Schiff can be of great value for the diagnosis of the leiomyoma. Furthermore, immunohistochemical stains for vimentin, desmin, and smooth muscle actin are adjunct to histopathological identification of leiomyoma. Histological examination demonstrates smooth muscle bundles and the vascular channels surrounded by a thin capsule. Myxomatous and hyaline degeneration might be seen due to circulatory disturbance. Organizing thrombus, mature fat cells, and lymphocytic infiltrate may be found in some tumors.
In 1995, Ezinger distinguished clinically and pathologically into three main groups of leiomyomas:
- Superficial leiomyomas: including piloleiomyoma (most frequent type originating from arrector pili muscles, which can be isolated or multiple), genital leiomyoma (arise from the smooth muscles of the vulva, labia majora, scrotum, penis, and mammary areola)
- Vascular leiomyoma or angioleiomyomas: mainly localized in the subcutaneous region
- Deep leiomyomas: localized in the intrafascial-intramuscular region and larger than the superficial leiomyomas, in which calcium deposits often develop.
Four histological subtypes of angioleiomyomas have been described:
- Capillary or solid angioleiomyomas which have a rich smooth muscle cell stratification which surround and hold a few thin vascular channels
- Venous angioleiomyomas which are characterized by more numerous and thicker vascular channels than are found in capillary angioleiomyoma
- Cavernous angioleiomyoma which has widened vascular channels which are surrounded by a thin layer of smooth muscle cells
- Combined capillary and venous angioleiomyoma.
Complications, such as secondary calcification, myxoid degeneration, hyalinization, and malignant transformation, have been reported. The dystrophic calcification has been reported to occur due to minor pressure, an injury, a previous inflammatory process, or de novo.
However, because of the lack of specificity of clinical and sonographic findings for the differentiation of soft-tissue masses, biopsy remains the reference standard if a clinical uncertainty exists. The therapeutic approach depends on the size of the lesion and severity of the symptoms. Surgical excision is the treatment of choice for solitary vascular leiomyoma. Complete excision rarely leads to recurrence. A drug treatment with nifidipine, gabapentin, and antidepressants can be used in cases where tumors are numerous and painful.,
| Conclusion|| |
Although angioleiomyoma is an infrequent soft-tissue tumor, it has a typical nonspecific presentation. It has to be considered in the differential diagnosis of subcutaneous swellings, especially in the extremities. Excision of the lesion leads to complete resolution of symptoms, and histopathology gives a definitive diagnosis.
Increased awareness of this tumor and judicious use of ultrasound and/or MRI should help in most cases for diagnosis. It causes minimal morbidity and excision is usually curative.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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