Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 3  |  Page : 277-280

Intramuscular vascular malformation in triceps: A case report with literature review


1 Department of Orthopaedics, Maharishi Markandeshwar University of Health Sciences and Medical College, Solan, Himachal Pradesh, India
2 Department of Radiodiagnosis, AIIMS, Bathinda, Punjab, India

Date of Submission19-Sep-2019
Date of Acceptance11-Nov-2019
Date of Web Publication12-Sep-2020

Correspondence Address:
Harmeet Kaur
Department of Radiodiagnosis, AIIMS, Bathinda, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_80_19

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  Abstract 


Vascular anomalies comprise a widely heterogeneous group of tumors and malformations. The tendency of radiologists and pathologists to report the intramuscular vascular anomalies as hemangiomas may mislead the treating surgeon which affects the diagnosis and management plan. The International Society for the Study of Vascular Anomalies classification has helped clear the confusion created by interchangeable terminology like hemangioma for such lesions. The vascular tumors may involute with time and deserve a conservative trial, whereas the symptomatic vascular malformations will eventually need surgical intervention. Mass lesions with normal skin without any discoloration, venous engorgement, or inflammatory signs can be easily passed for a lipoma. The high index of suspicion and availability of ultrasound and color Doppler help in the early detection of vascular anomalies. In the case presented herein, the vascular malformation symptomatic for about two decades was excised successfully with a plane of dissection through surrounding normal triceps muscle. This case highlights that the correct classification of vascular anomalies cannot be overemphasized because it guides the management plan according to the type of lesion and helps in setting up a genuine database for scientific research.

Keywords: Case report, hemangioma, intramuscular, triceps, vascular anomaly, vascular malformation


How to cite this article:
Tiwari P, Kaur H, Jha V, Bansal K. Intramuscular vascular malformation in triceps: A case report with literature review. Indian J Vasc Endovasc Surg 2020;7:277-80

How to cite this URL:
Tiwari P, Kaur H, Jha V, Bansal K. Intramuscular vascular malformation in triceps: A case report with literature review. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Oct 25];7:277-80. Available from: https://www.indjvascsurg.org/text.asp?2020/7/3/277/294915




  Introduction Top


Vascular anomalies comprise a widely heterogeneous group of vascular tumors and malformations.[1] The diagnosis and management of these lesions present diagnostic and therapeutic challenges to surgeons, radiologists, and histopathologists.[2]

The term “hemangioma” is commonly misused to describe any type of vascular abnormality, including vascular malformation, in both medical and surgical fields.[3] The International Society for the Study of Vascular Anomalies (ISSVA) classification [Table 1] for vascular anomalies[4] (approved at the 20th ISSVA Workshop, Melbourne, April 2014) has helped clear the confusion and better planning for the management of such lesions is possible.
Table 1: International Society for the Study of Vascular Anomalies Classification[4] for vascular anomalies

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The demographics available are usually from the old papers and studies based on the outdated and imprecise nomenclature.[5],[6],[7],[8]

The malformations are most frequently located in the thigh (36%), followed by the calf (17%).[6] Only a few case reports have been published of vascular malformation in the triceps muscle with radiological and histopathological correlation.

We present herein a rare case of vascular malformation in the triceps muscle of the left arm with histopathological findings.


  Case Report Top


A 33-year-old, moderately built, female patient of an Indian subcontinent origin and homemaker by occupation presented with a 20-year history of swelling and intermittent pain in the distal arm left side on extensor aspect with an increase in intensity of pain for the past 1 year. There was no history of significant trauma involving her left elbow. The pain increased with exercise. No relevant family history could be elicited. Physical examination showed a painful mass in the left distal triceps region. No bruit could be heard on auscultation of the swelling. There was no local heat or redness on the overlying skin. The range of motion (ROM) of the left elbow was normal.

In previous years, the patient had visited many surgeons. Then, the swelling was diagnosed with a lipoma, and hence, conservative management prescribed.

In our institute, color Doppler findings revealed an isoecheic to heterogeneously hypoechoic mass lesion in the triceps muscle with some intralesional vascularity at places which cast doubt on the most probable diagnosis of intramuscular lipoma. On the basis of sonographic findings, differentials to be considered were lipoma, hemangioma, and neurogenic tumor. Hence, magnetic resonance imaging (MRI) of the suspected lesion was advised.

MRI findings [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d displayed a well-defined oval-shaped intramuscular mass in the triceps muscle with surrounding frond-like margins. The mass appeared hyperintense on T2 and proton density fat saurated (PDFS) images and iso to hypointense on T1-weighted images. No suppression was seen on fat-suppressed images which ruled out lipoma. The mass involved lateral head of triceps, also reaching up to its distal aspect with sparing of its insertion at olecranon process. The rest of the scanned muscles, neurovascular bundles, and bones were unremarkable.
Figure 1: (a) Magnetic resonance imaging PDFS axial image showed a heterogeneously hyperintense mass with surrounding frond-like margins in the triceps muscle. (b) T2-weighted axial magnetic resonance image showed a heterogeneously hyperintense mass in the lateral head of the triceps muscle. (c) T1 magnetic resonance coronal image showed an isointense mass in the lateral head of the triceps muscle just adjacent to humerus. (d) Preoperative magnetic resonance imaging measurements for planning: PDFS magnetic resonance sagittal image showed a heterogeneously hyperintense mass in the triceps muscle with its distal extension seen about 2 cm above the insertion of the triceps muscle laterally

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Finally, the diagnosis of this patient was an intramuscular vascular anomaly of triceps.

Fine-needle aspiration cytology was attempted to rule out malignant transformation which is rare but theoretically possible. However, it could not reveal any additional meaningful information and yielded only blood.

As enough conservative clinical trial of about two decades had already been exhausted, and hence surgical excision of the mass lesion was planned. Moreover, the localized and well-circumscribed nature of the lesion favored the surgical excision over other treatment modalities such as embolization, sclerotherapy, and corticosteroids.

Keeping in mind the high recurrence rate of 18%–61%,[9] the mass was excised with a plane of dissection through surrounding normal triceps muscle. The location and size of lesion precluded the use of tourniquet. The ulnar nerve was not identified as the mass was lateral. Dissection was done from proximal to distal after identifying the uppermost margin of lesion [Figure 2]. No hemorrhage was encountered intraoperatively, and complete hemostasis was achieved. The tendinous insertion of triceps was then repaired meticulously using ethibond sutures to secure the early physiotherapy and ROM exercises.
Figure 2: Intraoperative photograph of vascular mass with a margin of normal muscle

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Postoperatively, the limb was protected by a removable back splint in slight extension till the removal of skin sutures at 2 weeks. The wound healed uneventfully. Physiotherapy and ROM exercises were started on day 5 and gradually increased in a stepwise manner till the achievement of normal ROM. On pathological examination [Figure 3] and [Figure 4]a, [Figure 4]b, [Figure 4]c, it was confirmed to be a vascular malformation.
Figure 3: Pathology specimen of the excised mass

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Figure 4: (a) Large and congested capillary channels invading skeletal muscle. (b) Section examined shows the skeletal muscle at focal places infiltrated by inflammatory cells comprising lymphocytes and polymorphs. (c) Section showing variable-sized congested blood vessels with one of the vessels showing thrombus formation, along with a fragment of the skeletal muscle. No endothelial proliferation was seen

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It has been more than 1 year postoperative, and there is no recurrence till date.


  Discussion Top


In patients with chronic limb pain and mass lesions with normal skin without any discoloration, venous engorgement or inflammatory signs the lesion can be easily passed for a lipoma as it was in our case. Being intramuscular, the lesions may take time to be visible as a lump under the skin and the size may be underestimated.

The high index of suspicion and availability of ultrasound and color Doppler help in the early detection.

Accurate delineation of the lesion, its extent, high flow or low flow, and site and number of draining/feeder vessels help in proper planning and execution of surgery.

Most of the case reports published have quoted the classic papers by Mulliken et al.[3] and Hassanein et al.[10] to bring to the attention the inappropriate use of term hemangioma. However, the irony is that most of them themselves do not classify the vascular anomalies as per the guidelines laid down by Mulliken et al.[3] and modified by ISSVA and have erroneously labeled the case reports as intramuscular “hemangiomas.”

Vascular tumors grow by cellular hyperplasia, while a vascular malformation represents a localized defect in vascular morphogenesis. Based on their biological and radiographic behavior, the malformations can be further characterized as slow-flow or fast-flow lesions.

The main reason for this continuous mislabeling in spite of the milestone classification by Mulliken et al.[3] about 40 years ago in 1982 is a lack of popularity or awareness among pathologists and radiologists of this classification system and its latest modification by the ISSVA in May 2018.

The use of interchangeable terminology has an undesirable impact on the mind of treating surgeon which affects the diagnosis and management plan of the vascular anomalies.

Physicians have recommended radical surgery to many patients. Conversely, many children who might benefit from other therapeutic approaches have been treated with watchful waiting. Many patients with vascular malformations have been misinformed that their lesions are hemangiomas and will eventually resolve.[11]

A radiologist should not hesitate to use color Doppler while doing ultrasonography (USG) of suspected lipomas mass lesions. The lesions must be described as vascular tumor versus malformation and slow flow versus high flow on USG/MRI rather than term hemangioma.

Similiarly, the pathologist should report based on endothelial proliferation and clinicoradiological correlation and discard old confusing classification and terminology.

The correct classification of vascular anomalies cannot be overemphasized because it guides the management plan according to the type of lesion. Besides that, it helps in setting up a genuine database with more authentic demographic indicators for scientific research regarding the incidence, prevalence rates, prognosis of the lesion, and studying the newer treatment modalities and their success rate.

Dissemination of knowledge and sharing of personal experience about the management of vascular anomalies may lead to decrease in the number of unnecessary referrals to higher centers by surgeons who have enough skill to handle such cases but lesser exposure.


  Conclusion Top


There is a generalized tendency to report vascular malformations as hemangiomas in diagnostic investigations and published case reports too which leads to the setting of inaccurate precedence and inappropriate management. Clinicians should keep this in mind and not confuse between hemangioma and malformations. The latest classification by ISSVA should be followed for study purposes and as a treatment guide.

Only a subtle focus of vascularity seen on Doppler in a supposed intramuscular lipoma should prompt the radiologist to investigate on the lines of a vascular anomaly even in the absence of classic book picture.

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 understands that name and initial will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Acknowledgment

We acknowledge the help and support of Dr. Neetu Bala, Resident, Department of Pathology, Maharishi Markandeshwar University of Health Sciences and Medical College, Kumarhatti, Solan, Himachal Pradesh, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chang MW. Updated classification of hemangiomas and other vascular anomalies. Lymphat Res Biol 2003;1:259-65.  Back to cited text no. 1
    
2.
Hand JL, Frieden IJ. Vascular birthmarks of infancy: Resolving nosologic confusion. Am J Med Genet 2002;108:257-64.  Back to cited text no. 2
    
3.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-22.  Back to cited text no. 3
    
4.
Dasgupta SJ. Fishman ISSVA classification, Semin Pediatr Surg. 2014:23;158-61.  Back to cited text no. 4
    
5.
Watson WL, McCarthy WD. Blood and lymph vessel tumors. Surg Gynecol Obstet 1940;71:569.  Back to cited text no. 5
    
6.
Tang P, Hornicek FJ, Gebhardt MC, Cates J, Mankin HJ. Surgical treatment of hemangiomas of soft tissue. Clin Orthop Relat Res 2002;399:205-10.  Back to cited text no. 6
    
7.
Beham A, Fletcher CD. Intramuscular angioma: A clinicopathological analysis of 74 cases. Histopathology 1991;18:53-9.  Back to cited text no. 7
    
8.
Enzinger FM, Weiss SW. Benign tumors and tumorlike lesions of blood vessels. In: Soft Tissue Tumors. 2nd ed. St Louis, MO: Mosby; 1988. p. 512.  Back to cited text no. 8
    
9.
Bella GP, Manivel JC, Thompson RC Jr, Clohisy DR, Cheng EY. Intramuscular hemangioma: Recurrence risk related to surgical margins. Clin Orthop Relat Res 2007;459:186-91.  Back to cited text no. 9
    
10.
Hassanein AH, Mulliken JB, Fishman SJ, Greene AK. Evaluation of terminology for vascular anomalies in current literature. Plast Reconstr Surg 2011;127:347-51.  Back to cited text no. 10
    
11.
Donnelly LF, Adams DM, Bisset GS 3rd. Vascular malformations and hemangiomas: A practical approach in a multidisciplinary clinic. AJR Am J Roentgenol 2000;174:597-608.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

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