Aggressive Diffuse Intraosseous Hemangioma: Case Report

Abstract

Vertebral hemangioma is a benign vascular tumor that is usually asymptomatic and is discovered incidentally on imaging. When symptomatic, the most frequent presentation occursinthe formofvague back painofinsidiousonset and,inrare cases, maybeassociated with root or spinal compression, causing sensory and motor deficits. The authors report the case of a 33-year-old man, previously healthy, with a diagnosis of thoracic spine hemangio-ma at multiple levels, in the sternum, in the scapula and in the costal arches; all lesions were symptomatic,and surgicalinterventionwas required; oneof thelesionsatthe thoracicspine level evolved with spinal compression and acute neurological deficit, requiring urgent surgical intervention. Intraosseoushemangiomas represent<1%ofall bonetumors, having few reports of multifocal presentation in the axial and appendicular skeleton. In the literature review, no other case of aggressive multifocal intraosseous hemangioma with this presentation was found, including associated neurological symptoms in the same case.

Keywords
bone diseases; drug therapy; hemangioma; spinal diseases

Resumo

O hemangioma vertebral, um tumor vascular benigno, geralmente é assintomático e descoberto incidentalmente em exames de imagem. Quando sintomático, a apresentação mais frequente ocorre sob a forma de dorsalgia vaga de início insidioso e, em raros casos, pode estar associadoa compressão radicularoumedular, causando déficit sensitivo emotor. Osautores relatamocasodeumhomemde33anos, previamentehígido, com diagnósticos de hemangioma na coluna torácica em múltiplos níveis, no esterno, na escápula e nos arcos costais; todas as lesões eram sintomáticas e houve necessidade de intervenção cirúrgica, sendo que uma das lesões ao nível da coluna torácica evoluiu com compressão medular e déficit neurológico agudo, com necessidade de intervenção cirúrgica de urgência. Os hemangiomas intraósseos representam<1% detodosostumores ósseos,eaapresentação multifocal no esqueleto axial e apendicular apresenta poucos relatos. Na revisão bibliográfica, não foi encontrado outro caso dehemangioma intraósseo multifocal agressivo com tal apresentação, inclusive com sintomas neurológicos associados em um mesmo caso.

Palavras-chave
doenças ósseas; tratamento farmacológico; hemangioma; doenças da coluna vertebral

Introduction

Hemangioma is a benign lesion in the group of vascular lesions, consisting of neoformed blood vessels. Some of these lesions are malformations, while others, due to the growth with neoplasia characteristics, are considered true benign tumors.¹ The most common locations of hemangiomas are the skull, where they can produce the classical image in "sun rays", and the axial skeleton, usually without any clinical symptomatology.¹ Most often, hemangiomas do not require treatment, but rather periodic follow-up.

The mean age of the patients is 40 years old, being more frequent in women, in the proportion of 3:2.¹,² In plain radiography, hemangiomas are characterized by parallel vertical trabeculates in vertebral bodies. Computed tomography (CT) reveals the presence of thick trabeculae and radiotransparent areas, and magnetic resonance imaging (MRI) is highly sensitive and specific, presenting hyperin-tense signs on T1 and T2.¹

We describe a rare case of aggressive hemangiomatosis in a young patient in order to demonstrate its clinical presentation, imaging, as well as the evolution of the patient with the treatment performed. Finally, we alert to the diagnosis of hemangioma as a differential diagnosis in multiple and also aggressive bone lesions.

Case Report

Male patient, 33 years old, metallurgical, previously healthy, with a history of chronic back pain without irradiation, previous thoracic spine MRI with a finding suggestive of hemangiomainT2, T3, T4,T9andL1(►Fig. 1). It evolvedwith worsening of back pain and bilateral irradiation to the lower limbs. The patient underwent a new MRI after worsening of symptoms, about 1 year after the first examination, and an increase in the previous injury in T3 was visualized with invasion of the medullary canal, causing spinal cord compression (►Fig. 2); the patient was then forwarded to our service.

Fig. 1
Sagittal section column magnetic resonance imaging demonstrating hemangiomas at the T2,T3, T4, T9 and L1 levels.

Fig. 2
Magnetic resonance imaging, sagittal section (A,B and C) demonstrating an increase in the dimensions of the lesion at the T3 level with invasion of the medullary canal; axial section (D,E and F) at the level of the lesion evidencing medullary dysmorphism and compression of the medullary canal.

On examination, the patient presented paresthesia of the lowerlimbs, clonus and ataxic gait. Duetothe aggressiveness of the lesion and to the acute neurological symptoms, hospitalization and surgical intervention were indicated. Spinal decompression associated with T2-T5 segment ar-throdesis was performed. Hemangioma of the vertebral body was found by pathological examination. Twenty sessions of radiotherapy were performed after the surgery and a semiannual follow-up was maintained, with good evolution.

The other hemangiomas of the vertebral bodies described remained with conservative treatment, follow-up with imaging, since they showed no growth or symptoms (►Fig. 3).

Fig. 3
Sagittal (A) and axial (B) magnetic resonance imaging cutting demonstrating nodular hemangioma at the T9 level.

Two years later, the patient complained of significant pain in the right costal arches, with radiography without special characteristics. Bone scintigraphy was requested, which showed uptake in the 4^th and 6^th costal arches on the right. Complement with MRI and CT and resection of 4^th and 6^th costal arches on the right was performed and confirmed by anatomopatology (►Fig. 4A).

Fig. 4
Computed tomography reconstruction (A) with hemangioma in the 4^th and 6^th costal arches; computed tomography of the sternum (B) demonstrating the initial injury.

During the follow-up, 3 years after the intervention at the costal arches level, the patient presented with pain in the right shoulder, with investigation diagnosing a lesion in the right scapula (►Fig. 5); resection was indicated, and the pathological report resulted in another hemangioma.

Fig. 5
Axial (A), coronal (B) and sagittal (C) cuts in magnetic resonance imaging of the scapula with an expansive lesion.

In the same year, the patient complained of anterior chest pain, including seeking an emergency department due to the intensity of the symptoms. An MRI was performed, which presented a lesion on the sternum (►Fig. 4B). Resection was indicated, and the material was sent to pathological examination and confirmed a new hemangioma.

Discussion

The diffuse presentation of intrabone hemangioma is uncommon. When reported, it usually presents in a region, such as several vertebral bodies, but it is not usually distributed in different places of the body.¹,⁴

The most common locations of hemangiomas arethe skull and axial skeleton. In the spine, thoracic localization is more common in the middle and lower regions, followed by the lumbar region, occurring more rarely in the cervical region.¹,²

Treatment of intraosseous hemangioma depends on the symptoms in each affected region.⁴

Most vertebral hemangiomas are latent and do not require specific treatment; few cases evolve with symptoms and, when those present, they are usually limited to the presence of pain. However, in rare cases, they may be aggressive, with neurological deficit due to spinal cord compression.¹,²,⁵,⁶,⁷,⁸

The evolutionary form of the present case demonstrates aggressive and nonconcomitant diffuse lesions in a symptomatic male patient, with the appearance of new heman-giomas during follow-up, which were not subject to conservative treatment.

The therapeutic modalities are broad: radiation therapy, arterial embolization, ligation of nutrient vessels, decom-pressive surgery for spine and tumor resection.⁸,⁹ In cases of spinal cord compression, decompressive surgery followed or not by radiotherapy has been the treatment of choice.³,¹⁰ Treatment options can be used alone or associated,⁹ varying according to the symptoms, on the evolution of the case, and depending on the experience of the attending physician.¹

Vertebroplasty is also described as a therapeutic modality in cases without neurological deficit to improve pain symptoms, but with less long-term benefit in pain relief.¹⁰

Radiotherapy can be used to treat vertebral hemangiomas exclusively or associated with surgery.⁵

Conditions associated with back pain and without neurological deficits can be conducted through periodic observation and clinical treatment. In refractory cases, exclusive radiotherapy may be an option for pain control through vascular necrosis and/or an anti-inflammatory effect.⁵

• Study developed at the Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil.

• Financial Support
  This research did not receive any specific grant from funding agencies in the public, commercial, or not-forprofit sectors.

References

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Publication Dates

• Publication in this collection
  05 Feb 2024
• Date of issue
  2023

History

• Received
  15 Aug 2020
• Accepted
  02 Oct 2020
• Published
  19 Apr 2021