XML Template (2015) [22.5.2015–5:07pm] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/150087/APPFile/SG-AANJ150087.3d

(AAN)

[1–4] [PREPRINTER stage]

Case Study

About a challenging mediastinal tumor Mona Mlika1, Emna Braham1, Rim Hamrouni1, Hazem Zribi2 and Faouzi El Mezni1

Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492315589868 aan.sagepub.com

Abstract Mediastinal hemangiomas are rare, accounting for 0.5% of all mediastinal tumors. These tumors are challenging because of the lack of specific clinical and radiologic signs. We report a 10-year experience of 5 mediastinal hemangiomas in a single institution, with neurologic signs related to neuroforaminal extension in 2 cases. Surgical treatment was performed in all 5 patients, without complications after a follow-up period varying from 9 months to 2 years.

Keywords Hemangioma, mediastinal neoplasms, thoracic surgery, video-assisted

Introduction Mediastinal hemangioma is a rare tumor causing diagnostic and therapeutic challenges. Although the diagnosis is based on microscopic examination, some radiologic features may highlight the diagnosis and encourage surgeons consider it. A few cases of mediastinal hemangioma, mainly located in the anterior mediastinum, have been reported.1

Case reports Our study deals with 3 women and 2 men, with a mean age of 60 years (range 24–79 years). Most patients were symptomatic, mainly with respiratory signs (chest pain and dyspnea), neurological signs (spastic paraplegia), and urinary problems (Table 1). Chest radiography showed mediastinal enlargement in all cases. A transthoracic ultrasound examination in one patient (case no. 1) and showed an intrapericardiac multicystic mass (Figure 1a). Angiography was also performed in case no. 1 and demonstrated a pericardial mass with a pericardial effusion (Figure 1b). Computed tomography (CT) was performed in 3 patients (nos. 3, 4, 5) and revealed a mediastinal mass in all cases, which was adherent to the right atrial cavity in case no. 3, with extension to the sixth dorsal vertebra in case no. 4, and extension to the third dorsal vertebra and costovertebral foramina in case no. 5 (Figure 1d). Chest magnetic resonance imaging in one case (no. 4) showed a

posterior mediastinal tumor with neuroforaminal extension to the fourth and fifth right paravertebral spaces (Figure 1c). A diagnosis of neurinoma was made in 2 cases based on radiological findings. Surgical resection was performed in all patients, through a median sternotomy in one, and through a right posterolateral thoracotomy with laminectomy in 2. Video-assisted thoracic surgery was performed initially in one case; it showed a hypervascular mass adhering intimately to the superior vena cava, and the operation was converted to a median sternotomy. An anterior and posterior approach was used in one case (no. 5). Surgical resection was complete in 4 cases and incomplete in case no. 1 because of connection of the tumor to the cardiac cavities and the increased risk of bleeding. Microscopic examinations were performed on the surgical specimens (from open biopsy in case no. 1). Gross examination showed a multicystic mass in almost all cases (Figure 2a). Microscopic features were similar in all specimens and consisted of a benign vascular tumor with vascular channels of the cavernous and/or capillary type. Cavernous hemangioma was described in 2 patients 1

Department of Pathology, Abderrahman Mami Hospital, Tunis, Tunisia Department of Thoracic Surgery, Abderrahman Mami Hospital, Tunis, Tunisia 2

Corresponding author: Mona Mlika, Department of Pathology, Abderrahman Mami Hospital, University of Medicine Tunis El Manar, Tunis 2037, Tunisia. Email: [email protected]

Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on June 16, 2015

XML Template (2015) [22.5.2015–5:07pm] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/150087/APPFile/SG-AANJ150087.3d

(AAN)

[1–4] [PREPRINTER stage]

GGS (2 years)

GGS (1 year) Mixed hemangioma

GGS (9 months)

Cavernous hemangioma Capillary hemangioma

Mixed hemangioma Cavernous hemangioma

Diabetes mellitus 65 M

Difficulty walking

58 F

Chest pain, dysphagia, dyspnea

79 F

Atrial arrhythmia

24 76 F M

Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on June 16, 2015

DGS: degeneration of the general state; GGS: good general state; VATS: video-assisted thoracic surgery.

Posterior mediastinal tumor with neuroforaminal extension Posterior mediastinal mass

Mass in mid mediastinum

Median sternotomy Posterolateral thoracotomy þ laminectomy VATS þ median sternotomy Posterolateral thoracotomy þ laminectomy Combined anterior and posterior approach Pericardial multicystic mass Dumbbell-shaped mass þ neuroforaminal extension

Surgery Radiologic findings History Symptoms Age (years) Sex

Table 1. Clinical, radiologic, histologic and follow-up data.

Mediastinal hemangioma is a rare tumor representing 0.5% of all mediastinal tumors.2 The anterior compartment is the most frequent site but the tumor may also be observed in the middle or posterior mediastinum.2 In our study, 3 tumors were located in the posterior mediastinum, one in the middle mediastinum, and one in the anterior mediastinum. Hemangiomas can be seen at any age but the cavernous type is mostly seen in children and adolescents. One third to one half of patients are symptomatic.2 Physical symptoms are nonspecific, consisting mainly of dyspnea and chest pain as a result of infiltration of adjacent organs.2 In our study, two patients presented with neurologic signs related to neuroforaminal extension of the tumor. One patient was asymptomatic and the tumor was discovered incidentally. It is often difficult to diagnose hemangioma preoperatively because the lesion manifests as a nonspecific mediastinal mass.3 Two of our patients were suspected to have a neurinoma preoperatively. This was due to localization in the posterior mediastinum, radiologic features, and to a past history of neurinoma in one patient. Radiological findings, especially CT and magnetic resonance imaging, are helpful in suggesting the diagnosis.3 Mediastinal hemangiomas commonly appear as heterogeneous lesions on non-enhanced CT. A gradual increase and persistent enhancement has been seen on contrast-enhanced CT scans.3 Phleboliths are present in up to 10% of cases. The presence of calcification poses a challenge in the diagnosis of mediastinal lymphoma, teratoma, and hemangioma.3 Surgical resection is the mainstay of treatment. Total resection is recommended when possible. Only one patient had a subtotal resection because of connection of the tumor to the cardiac cavities. Surprisingly, this patient has had no postoperative complications. This may be due to the histologic subtype of this hemangioma, which was mainly the capillary type. In fact, cavernous hemangiomas are most often reported to invade the adjacent organs, resulting in incomplete resection and postoperative bleeding. A posterolateral thoracotomy has become the standard approach for resection; it was performed in 3 patients in our study and was

Pathology

Discussion

Neurinoma resected 25 years earlier

Follow-up

and was composed mainly of thin dilated vessels. Capillary hemangioma was described in one patient, which was characterized by many small vessels like capillaries. Mixed hemangioma was noted in 2 patients, associated with features of capillary and cavernous hemangioma (Figure 2b). No patient had complications after a follow-up period ranging from 9 months to 2 years. The clinical characteristics of the patients are summarized in Table 1.

GGS (2 years) GGS (1 year)

Asian Cardiovascular & Thoracic Annals 0(0)

Chest pain, DGS Spastic paraplegia, urinary problems

2

XML Template (2015) [22.5.2015–5:07pm] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/150087/APPFile/SG-AANJ150087.3d

(AAN)

[1–4] [PREPRINTER stage]

Mlika et al.

3

Figure 1. (a) Transthoracic ultrasonography showing a multicystic mass (arrow) in patient no. 1. (b) Angiography showing the connection of a pericardiac multicystic mass to the interventricular artery. (c) Chest radiography showing a dumbbell-shaped mass. (d) Computed tomography showing a posterior mediastinal multicystic mass extending to the vertebra.

Figure 2. (a) Gross aspects of a mediastinal hemangioma consisting of a 6.5-cm multicystic mass. (b) Microscopy of a mixed hemangioma: the cavernous structures are dilated with a thin wall (arrow), capillary structures are small and are observed surrounding the cavernous structures. Hematoxylin and eosin stain, original magnification 200.

followed by a laminectomy in 2 of them. In cases of neuroforaminal extension, surgical procedures using a combined anterior and posterior approach are recommended. In fact, thoracotomy before laminectomy is optimal for resection because it enables subsequent laminectomy procedures.3 Video-assisted thoracic surgery had also been used for excision of mediastinal

hemangiomas located in the anterior, middle, or posterior mediastinum.3 The conversion rate is less than 10%. In our study, this approach was attempted in one patient with a tumor in the middle mediastinum, but conversion was decided because of connection of the tumor to the superior vena cava. There are reports of bevacizumab treatment in a case of incomplete

Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on June 16, 2015

XML Template (2015) [22.5.2015–5:07pm] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/150087/APPFile/SG-AANJ150087.3d

(AAN)

[1–4] [PREPRINTER stage]

4

Asian Cardiovascular & Thoracic Annals 0(0)

excision, and propranolol in children with laryngotracheal hemangiomas, but medical treatment seems to be ineffective.4 The definitive diagnosis is based on microscopic examination which necessitates thorough sampling of the tumor. Transthoracic puncture has no role because it shows only the hemorrhagic content of the lesion, without any specificity. Gross features consist of a soft purplish encapsulated mass. Microscopically, hemangiomas consist of benign vascular elements with various amounts of interposed stromal elements.2 They are divided into 3 types: capillary, cavernous, and venous, depending on the size of the vascular structures. Funding This research received no specific grant from any funding agency in the public, commerical, or not-for-profit sectors.

Conflict of interest statement None declared.

References 1. Ohebsion J, O’Connor WN, Attili AK and Diaz-Guzman E. A 30-year-old man with facial flushing and a mediastinal mass. Chest 2010; 138: 746–749. 2. Yoshino N, Okada D, Ujiie H, Akiyama H, Nishimura Y, Koizumi K and Shimizu K. Venous Hemangioma of the Posterior Mediastinum. Ann Thorac Cardiovasc Surg 2011; 28: 247–250. 3. Odaka M, Nakada T, Asano H, et al. Thoracoscopic resection of a mediastinal venous hemangioma: report of a case. Surg Today 2011; 41: 1455–1457. 4. Chen X, Lodge AJ, Dibernardo LR and Milano CA. Surgical treatment of a cavernous haemangioma of the heart. Eur J Cardiothorac Surg 2011; 41: 1182–1183.

Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on June 16, 2015

About a challenging mediastinal tumor.

Mediastinal hemangiomas are rare, accounting for 0.5% of all mediastinal tumors. These tumors are challenging because of the lack of specific clinical...
401KB Sizes 3 Downloads 8 Views