Intramuscular deep forearm hemangioma aggravated by a traumatic event: A case report

Abstract

Introduction

Hemangioma is a unique benign vascular tumor. Intramuscular seated lesions tend to cause pain, swelling, and disability in adulthood, especially after a traumatic aggravating event.

Presentation of case

A young, healthy female suffered a previous trauma to her left forearm. Persistent pain developed after it with intermediate swelling that turned out to be a deep-seated hemangioma, which was treated successfully with excision.

Discussion

Though it is often a benign-acting congenital lesion, with several treatment methods, early diagnosis of hemangioma is a dilemma. Expelling other similar malignant lesions is essential. Surgical intervention could be necessary and effective in specific indications.

Conclusion

Adequately planned surgery can successfully manage traumatic aggravated intramuscular hemangioma.

Highlights

• •Interesting case of misdiagnosed lesion that was wrongly treated as a traumatic insult
• •Various investigations that were enough to make adequate decisions
• •Favorable outcome after 1 year follow-up

1. Introduction

Musculoskeletal soft tissue tumors have distinct features from other skeletal tumors that can be concise in painless, slowly enlarging mass. With rare exceptions, those features often lead to late missed or wrong diagnoses [1].

Hemangioma of soft tissue does not necessarily follow the path of soft tissue tumors. Still, it is not easily diagnosed. It is a neoplasm of vascular origin. It could be superficial or deep-capillary or cavernous [2]. Half the cases exist at birth but go symptomatic in adulthood, often isolated and rarely intramuscular, and a fifth of the cases are associated with traumatic events [[3], [4], [5]].

The lesion itself is painful with variable intensity. It can also cause secondary aching due to enlargement and pressure on surrounding structures. It usually has cutaneous manifestations that lead to cosmetic impairment, and the size of the swelling could vary with activity [6].

Intramuscular hemangioma could stay asymptomatic till the second or third decade of life when it goes through a suddenly escalated phase of enlargement, which usually raises suspicion toward more severe conditions like sarcomas [6,7].

This article presents a case of forearm-deep hemangioma with a spectrum of symptoms and manifestations over seven years.

This case report has been reported by SCARE 2020 standards [8].

2. Presentation of case

A 19-year-old healthy female attended our clinic 18 months ago complaining of tender swelling in the lateral volar aspect of her left forearm and inability to form a firm grip and lift heavy things. She mentioned falling on her left hand seven years ago and was taken to the emergency room. No fractures nor dislocations were noticed, and she was treated with analgesics. However, the pain persisted, and her doctor assured her that it would go away eventually, as no significant injuries existed, but it did not. It became intermittent with different intensities aggravated by manual labor and hard work, and intermittent mild swelling developed and progressed over the seven years. It was rarely disabling pain, but it was enough to seek a medical consultant. She had seen several doctors with no success in a precise diagnosis or a relieving treatment.

She had no comorbidities, medical or surgical history, full-term birth, normal growth, and no history of cancer or congenital malformations in the family.

On initial examination, mild swelling on the volar aspect of the left forearm, no redness, no local heat, no cutaneous abnormalities, diffuse tenderness in the forearm, full elbow range of motion but limited pronation, and wrist movements were normal. Still, extreme flexion was tender. She could not form a fist due to lacking full-digit flexion. Her grip was markedly weaker compared to the contralateral side. The rest of her musculoskeletal examination was remarkably normal. Her Disabilities of the Arm, Shoulder, and Hand (DASH) score was 42.5 [9].

We asked for plain radiographs, which showed no abnormalities, so we asked for an MRI, which showed a large, well-defined heterogenous mass measuring 85 × 33 × 22 mm. The forearm mass showed a bright signal on T2 and STIR-weighted images and a mixed signal on T1. The mass lay on the radius but spared the bone. The study suggested a vascular tumor or a bleeding neoplasm; further investigations were recommended (Fig. 1).

Fig. 1.

Subsequential MRI images showing a heterogenous mass lying in the deep volar compartment of the forearm.

a: axial T1, b: axial T2, c: coronal T2, d: sagittal T2, e: axial STIR.

A vascular consultant was done based on examination and Doppler sonography; arteriovenous malformations were excluded, and a benign vascular hemangioma was suggested. After that, a chest, abdomen, and pelvis CT scan was obtained, which was all normal other than reactive inflamed lymph nodes in both axillae, the largest measuring 12 mm with fatty hilum.

Still, hemangioma was the most probable diagnosis, although it is a deep-seated lesion that measures more than 5 cm, the benign course, the contained appearance on images, and the absence of distant metastases suggested benign neoplasm, so an excisional biopsy was made under general anesthesia, applying a tourniquet to the upper arm. After disinfecting and draping, a Henry volar 10 cm incision was made mid-forearm, blunt dissection was done, and we went in the interval between the radial artery and flexor carpi radialis while protecting the superficial radial nerve. In the mass of the flexor digitorum profundus - FDP - a distinct tissue was noticed, matching the anatomical location of the tumor on MRI, so excision with one cm margins was done (Fig. 2), the tourniquet was inflated, hemostasis was done, and closure on layers,

Fig. 2.

Subsequent images showing a: neurovascular bundle, b: superficial radial nerve, c, d, e, and f: clinical images of excising the lesion.

The patient was discharged home the next day on paracetamol - Tylenol - three times a day. Regular check-up visits were done every two weeks for two months, then every three months for a year. The wound healed fine, but the scar developed into a keloid, and the patient reported pain relief in the second week. The histological study of the mass confirmed the diagnosis of cavernous hemangioma (Fig. 3).

Fig. 3.

Microscopic examination reveals the proliferation of cavernous vessels lined by benign endothelium.

A progressive physiotherapy program was initiated ten days after surgery. Full range of motion of the wrist and the digits were achieved in the second month and improved grip strength within six months. At one year, considerable grip power was obtained, and no tenderness, swelling, or reduced range of motion was noticed, and her DASH score became 8.3 (Fig. 4).

Fig. 4.

Clinical follow-up images six months after the surgical excision, shows the improved range of motion and the keloid.

3. Discussion

Hemangioma is the most common true vascular neoplasm and the most common diagnosis of benign soft tissue tumors in infants. It is an error in the formation of a new vascular segment, a hamartomatous proliferation of vascular endothelial cells. It is never reported to go through a malignant transformation or metastasize, but in case of inadequate excision, a high local recurrence rate is expected – up to 18 % [10,11] Hemangiomas are usually latent tumors. It usually goes through an early rapid proliferation but eventually becomes involute in early childhood [12,13]. It could occur in any tissue in the body but rarely ever take an aggressive, destructive way. It has been linked with trauma as a triggering factor, yet no precise causative mechanisms have been established [5,14,15]. Diagnosis can be challenging; clinically, it manifests as a painful mass that varies in size. It is easily distinguished from infection and abscesses but not so much from other soft tumors and malformations. On plain X-rays, it might show characteristic phleboliths inside the lesion, and the adjacent bone might be irritated [16,17]. A Doppler sonography helps show these calcifications and plays a role in excluding arteriovascular malformations [18]. A CAT scan is of little help [19]. Still, MRI with gadolinium is the gold standard as it shows a heterogenous lesion with multiple small blood vessels and a fatty infiltration - bag of worms sign [21] Angiography is rarely indicated. Still, it could have a diagnostic/therapeutic role in articulate cases [22].

The optimal treatment method is controversial; asymptomatic cases undergo observation and vascular stockings in case of a lower limp seated lesion. Steroids could be effective, orally administrated or locally in the form of an injection; sclerotherapy done by an interventional radiologist is also effective in certain cases. Surgery is reserved for patients who failed conservative modalities or in case of rapid growth. Wide surgical resection is the preferred technique but might not always be possible, and radiation is also anticipated in case of failure of the previous methods or nasty anatomical locations. [4,23,24]

Our case was misdiagnosed for seven years, and conservative treatment wasn't successful. Sunyarn Niempoog and Waroot Pholsawatchai reported a similar case in 2019. A 25-year-old male had a painful flexion contracture in his right hand developed slowly following a motorcycle accident, and it was successfully treated by surgically removing a 2 ∗ 2 cm intramuscular hemangioma in the belly of FDP [25].

Kamath et al. reported a case of a 14-year-old female who complained of painless swelling in the left forearm and flexion contracture of the contaminant ring finger. She was treated by surgical excision of the small tumor and anastomosis of the injured belly to the belly of the middle finger flexor. Complete relief of the symptoms was obtained [26].

Sunil reported a bizarre case of a Volkman-like contracture in a 12-year-old female who had sustained major trauma to the same forearm at the age of six. After that, a diffuse swelling progressed slowly along with the disabling painful contracture, and surgical intervention revealed a multiloculated mass in the flexors: excision and histopathological study consistent with an intramuscular capillary hemangioma. After a prolonged intensive physiotherapy program, normal functional recovery was obtained [27].

Vakilha M reported a case with a sizeable recurrent lesion in the forearm that had failed surgical resection twice and was eventually treated with radiation - 15 fractions of a total 300 cGy dose - with a marked lesion regression. [28]

A high level of suspicion is essential for diagnosis, first distinguishing the lesion from other soft tissue masses, especially sarcomas, second exclusion of other lesions that have the same profile of increased blood flow like angiomatosis, arteriovenous malformations, and vascular ectasia, if the decision is made for surgical intervention Then thorough planning of the approach and anatomical affected units is crucial for adequate resection that would decrease the risk of recurrence, and close follow up for recurrence is mandatory. In 89 cases of skeletal hemangiomas, Allen P and Enzinger F classified the lesions based on type - small-vessel type, large-vessel type, and mixed type - and the local recurrence rate over 12 years follow-up was respectively - 20 %, 9 %, 28 %.

Though hemangiomas are mostly congenital neoplasms, a traumatic event may disturb their latent attitude. The suggested mechanism debates that the increased blood flow following mechanical damage might be the stimulator of enlargement and symptoms [15,29], but the management is still the same. The absolute confirmation of the diagnoses is built on a histopathological study, as it should show hyperplastic endothelium instead of still one in vascular malformations and the absence of malignant cells seen in angiosarcoma [16,31].

When correctly indicated, comprehensively planned, and adequately executed, surgical excision could lead to a favorable outcome. Our patient returned to doing all kinds of recreational activities pain-free, and her DASH score improved after one year to a satisfactory result.

4. Conclusion

Intramuscular Hemangioma is a rare lesion that could cause pain and disability. After the failure of conservative treatment, surgical excision is an effective modality. However, adequate investigations and surgical planning are crucial in decreasing the risk of recurrence.

Consent

Written informed consent was obtained from the patient to publish this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Ethical approval

This study is exempt from ethical approval in our institution.

Funding

This research did not receive any specific grant from any funding agencies in the public, commercial, or not-for-profit sectors.

Guarantor

Hakam al-asaad M.D.

Research registration number

N/A.

CRediT authorship contribution statement

• Hussain Muhammed M.D: conceptualization, investigation, data curation, writing, editing, and reviewing.

• Doried al-diri M.D: investigation, data curation, writing, and editing.

• Hakam al Asaad M.D: conceptualization, writing, and reviewing.

• Sedra Abou Ali Mhana MD: data curation, writing, and editing.

• Jaber Ibrahim M.D PHD: conceptualization, investigation, data curation, writing, editing and reviewing.

Declaration of competing interest

The author has no conflicts to disclose.