Hemangioendothelioma: a rare tumor of the mediastinum

Abstracts

Apresentou-se para atendimento um homem de 30 anos, branco, natural e procedente de São Paulo, com quadro de dor em hemitórax esquerdo, na região anterior e lateral, constante e de leve intensidade havia três meses, associado a dispnéia aos grandes esforços havia um mês. Durante a investigação foi visualizada em radiograma, tomografia e ressonância magnética de tórax grande tumoração em mediastino anterior e médio, com possível invasão dos vasos da base. Ele foi submetido à mediastinotomia paraesternal esquerda com biópsia da massa mediastinal, a qual complicou por sangramento intenso. Optou-se pela esternotomia mediana total e toracotomia ântero-lateral esquerda de urgência, com controle do sangramento e ressecção completa do tumor. Houve boa evolução, com alta hospitalar no nono dia pós-operatório. O exame anatomopatológico mostrou tratar-se de hemangioendotelioma de mediastino.

Hemangioendotelioma; Neoplasias do mediastino

CASE REPORT

Hemangioendothelioma: a rare tumor of the mediastinum ^*

Marcelo Loze de Queiroz; Petrucio Arantes Sarmento de Souza; Carlos Jogi Imaeda^(TE-SBCT); Vicente Forte ^(TE-SBCT)

^{Correspondence}

ABSTRACT

A 30-year-old Caucasian male from São Paulo was admited to the hospital. He had been complaining about constant, moderate pain in the anterior and lateral left hemi-thoracic region for the last three mouths as well as associatede great effort dyspnea over the last mounth. Investigation with chest X-rays, CT scans and MRI revealed an large vessel invasion. The patient was submitted to a left side parasternal madiastinostomy and a biopsy of the mediastinal mass which was complicated by severe bleeding. An immediate median full sternotomy was elected in addition to a left anterior-lateral thoracotomy for total tumor resection and control of the bleeding. Evolution was good, with hospital discharge on the ninth postoperative day. The anatomical-pathological essay disclosed a hemangioendothelioma of the mediastinum.

Key words: Hemangioendothelioma/diagnosis. Mediastinal neoplasms.

Introduction

Hemangioendothelioma is a tumor that is vascular in origin and presents in varying degrees of differentiation, ranging from the benign (hemangioma) to the malignant (angiosarcoma).^(1,2) Hemangioendotheliomas can be found in various organs, such as the lungs, liver, spleen or bones, although they are rarely seen in the mediastinum. As of 1999, only 19 such cases had been reported.⁽³⁾ That group of 19 patients consisted of both males and females, and the age range was from 0 to 66, with a mean age of 28. Patients with this type of mediastinal mass may be asymptomatic or may present with nonspecific symptoms.^(3,4) Because of its low incidence, hemangioendothelioma is seldom considered in the differential diagnosis of mediastinal masses.

Case report

A 30-year-old male ex-smoker (10 pack years) presented with pain, characterized as a light burning sensation in the left hemithorax, that had been constant over the last 3 months. The patient also reported dyspnea on exertion over the last month. Physical examination revealed pain upon palpation of the left anterior chest wall at the level of the third, fourth, and fifth intercostal spaces. A chest X-ray revealed a large mass in the left hemithorax, with precise lateral borders and medial blurring of the cardiac silhouette (Figure 1). Computed tomography and magnetic resonance imaging revealed a mass in the anterior mediastinum, with heterogeneous density, extending from 2 cm below the furcula over the left anterior chest wall, with vascularization (Figures 2 and 3). A biopsy of the tumor mass through parasternal mediastinostomy on the left side was indicated. Due to profuse bleeding at the biopsy site, a total sternotomy and an anterolateral thoracotomy on the left side were promptly performed, revealing an encapsulated tumor attached to the chest wall, pericardium, left brachiocephalic vein and left phrenic nerve. A complete tumor resection was performed, and the bleeding was brought under control. The postoperative evolution was favorable and uneventful, and the patient was discharged on the ninth postoperative day.

Anatomopathological examination revealed hemangioendothelioma in the mediastinum. Macroscopic examination of the material revealed a globular tumor with distinct borders, measuring 12 cm along its longest axis and surrounded by a fibrous capsule. Upon dissection, the tumor presented a firm consistency and comprised areas that were brownish or purple, interspersed with friable necrotic areas (Figure 4). Under microscopy, samples submitted to hematoxylin-eosin (HE) staining revealed neoplasia, characterized by the proliferation of highly stained, irregular cells with discrete nuclear atypia. These cells delimited vascular spaces that were also irregular in both shape and size. The neoplasia also presented a great deal of necrosis caused by ischemia, with only a few islands of viable cells remaining. These few viable cells were submitted to immunohistochemical study, which revealed high CD34 positivity, confirming the endothelial origin of the tumor (Figure 5).

Discussion

Hemangioendothelioma is a tumor that is vascular in origin and comprises three different subtypes: epithelioid hemangiothelioma,⁽⁵⁾ endovascular papillary angioendothelioma (Dabska tumor)⁽⁶⁾ and spindle cell hemangioendothelioma. It can arise in various organs, such as the lungs, liver, spleen or bones.⁽⁵⁾ In 1990, Toursarkissian et al.⁽³⁾ reviewed 19 cases of hemangioendothelioma in the mediastinum.

Hemangioendotheliomas in the mediastinum can present symptoms similar to those provoked by other types of mediastinal masses (dyspnea, stridor, cough, chest pain, dysphagia, etc.) or can be asymptomatic.^(3,4)

Some mediastinal masses present typical radiographic findings and can be easily ruled out. Radiographs can also be used to establish the extent of the lesion and aid in determining the most appropriate surgical strategy.

Enzinger and Weiss,⁽⁶⁾ in a review of soft tissue hemangioendothelioma, reported that, over a 48-month period, there was local recurrence in 13% of cases, metastasis in 31% and mortality in 19%.

In predicting malignant behavior, the most significant histological parameters are the number of mitoses per field, the presence of necrosis and bone metaplasia, and the increase in cellular pleomorphism.

Since bleeding may occur, biopsies are not recommended. The aim of treatment should be the complete surgical resection of the lesion. For cases in which there is a higher probability of malignancy, complementary therapies such as radiotherapy and chemotherapy may be applied.⁽⁶⁾

References

Submitted: 17 March 2003.

Accepted, after revision: 27 November 2003.

• 1. Pearson FG, Cooper JD, Deslauries J, Ginsberg RJ, Hiebert CA, Patterson GA, Urschel HC Jr. Unusual mediastinal tumors. In: Fang HK, Sundaresan S, editors. Thoracic surgery. 2nd ed. New York: Churchil Livingstone; 2002.
• 2. Rubinowitz NA, Moreira AL, Naidich DP. Mediastinal hemangioendothelioma: radiologic-pathologic correlation. J Comp Assist Tomogr 2000;24:721-3.
• 3. Toursarkissian B, O'Connor WN, Dillon ML. Mediastinal epithelioid hemangioendothelioma. Ann Thorac Surg 1990;49:680-5.
• 4. Suster S, Moran CA, Koss MN. Epithelioid hemangioendothelioma of the anterior mediastinum. Clinicopathologic, immunohistochemical, and ultrastructural analysis of 12 cases. Am J Surg Pathol 1994;18:871-81.
• 5. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma a tumor often mistaken for a carcinoma. Cancer 1982;50:970-81.
• 6. Sharon W, Weiss JR, Md Goldblum, Franz M. Hemangioendothelioma: vascular tumor of intermediate malignancy. In: Sharon W, Weiss JR, Goldblum, FM, editors. Enzinger and Weiss's soft tissue tumors. 4th ed. St Louis: C. V. Mosby; 2001. p.891-916.

Correspondence

Marcelo Loze de Queiroz

Rua. Dr. Altino Arantes, 895 Apto 131, Vila Clementino

CEP 04042-034 São Paulo, SP

Phone: [phone]

*

Study carried out in the Serviço de Cirurgia Torácica e Pneumologia do Hospital Beneficiência Portuguesa de São Paulo

Publication Dates

• Publication in this collection
  08 June 2004
• Date of issue
  Apr 2004

History

• Accepted
  27 Nov 2003
• Received
  17 Mar 2003