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We report a case of an 11-year-old girl who presented with a slowly enlarging mass in the right posterolateral chest wall. Computed tomography showed a soft-tissue mass 8.5 × 7.5 × 5.5 cm in size, arising from the right posterolateral 9th, 10th, and 11th intercostal spaces. Magnetic resonance imaging confirmed a vascular mass. The patient underwent complete resection of the tumor, together with the right 8th, 9th, 10th, 11th, and 12th ribs and their intercostal muscles. Reconstruction of the chest wall was performed with methyl methacrylate and Marlex mesh. Histopathologic examination of the tumor confirmed an intercostal cavernous hemangioma. At last examination, 6 months after the operation, the child was doing well, with no evidence of recurrence.
Chest wall tumors account for fewer than 1% of all tumors and arise mainly in bone and cartilage. Vascular chest wall tumors are so rare that little information regarding their incidence and prevalence is available in the literature.1 Moreover, because intercostal hemangiomas comprise only about 0.01% of all benign hemangiomas, they are unexpected and seldom are distinguished preoperatively from other chest wall tumors, such as lipomas, neurogenic tumors, chondromas, fibromas, and invasive tumors found in association with lung cancer.2 To the best of our knowledge, this is the 1st report in the world literature1–3 of arteriovenous hemangioma arising from the intercostal muscles of a child.
In May 2008, an 11-year-old girl was admitted to our clinic for treatment of a right posterolateral chest wall mass. There was no history of chest trauma. To the best of the parents' knowledge, the lesion had been present since the child was 2 years of age and had gradually increased in size. No family history of other such tumors could be recollected. The laboratory data were normal. The lesion had been diagnosed as an intercostal hemangioma 2 years before in a local hospital, where a surgeon had attempted excision, only to discontinue the procedure upon discovering the vascular nature of the tumor. Diagnosis was made by incisional biopsy.
On palpation, the lesion had a smooth surface. It measured approximately 8.5 × 7.5 cm and was situated on the right posterolateral chest wall at the level of ribs 8 through 12. There was a scar on the mass. Auscultation revealed a continuous thrill over the deep-seated hemangioma.
Plain chest radiography showed a smooth-walled 8-cm mass in the right posterolateral field. A computed tomographic (CT) scan revealed a soft-tissue mass 8.5 × 7.5 × 5.5 cm in size arising from right posterolateral intercostal spaces 9 through 11 and extending into the extrapleural space corresponding to ribs 8 through 12; there was minimal cortical thickening at the last 2 ribs (Fig. 1). No destruction of bone was noted in the CT scan. Magnetic resonance imaging (MRI) revealed a homogeneous mass 8.5 × 7.5 × 4.5 cm in size; the signal intensity was high in the T1- and T2-weighted images and was a little higher than that of adjacent muscles in the inferolateral right chest wall (Fig. 2).
At thoracotomy, we found a well-demarcated mass in the right 9th, 10th, and 11th intercostal spaces; the 9th, 10th, and 11th ribs were surrounded by this vascular mass. We performed a complete resection of the tumor with clean margins: this involved resecting ribs 8 through 12 and the intercostal muscles. The hemangioma involved the adjacent spinal muscles but did not involve the spine or intrathoracic contents. The hemangioma did not rupture, and there was no blood loss. Neither a blood transfusion nor a “cell saver” machine was needed.
The surgical specimen measured approximately 10 × 9 cm, which included a hemorrhagic, highly vascular, 8.5 × 7.5 cm mass that extended from the intercostal muscles to the extrapleural space (Fig. 3A). Microscopically, the mass consisted of a homogeneous conglomerate of thin-walled blood vessels with dilated channels. Neither cellular atypia nor mitotic figures were apparent in the endothelial cells, nor were tumor cells seen at the surgical margins. The histopathologic diagnosis of intercostal cavernous hemangioma was established. The hemangioma did not involve the skin, and the chest wall was reconstructed with methyl methacrylate and Marlex mesh (Gallini S.p.A.; Mantova, Italy) (Fig. 3B). The patient was discharged on postoperative day 5, after an uneventful postoperative course. There was no evidence of recurrence at the 6-month follow-up examination.
Vascular tumors arising in the chest wall are very rare. Although various factors such as chest trauma, repetitive bruising, intrinsic hormonal imbalance, and chronic liver disease can play a role in the development of intramuscular hemangioma, the disease is considered to be congenital. Many causal mechanisms have been postulated, including the partial persistence of the fetal capillary bed and multicentric hamartomatous proliferation of vessels of the dermal subpapillary plexus.2,4 In our patient, there was no history of trauma or chronic liver disease.
Hemangiomas are usually slow-growth tumors. In our patient, the mass was first noticed at the age of 2 years and had been monitored until the patient's referral to us.
Soft-tissue hemangiomas are usually seen first upon conventional radiography. In our patient, the lateral chest radiograph revealed the shadow of a soft-tissue mass. Computed tomography of the chest can aid in the evaluation of the hemangioma's effect on adjacent structures, because the characteristic fatty appearance of the tumor is evident upon CT, as was seen in our patient. Computed tomography can also reveal phleboliths, which sometimes accompany hemangiomas. Upon MRI, soft-tissue hemangiomas have a characteristic appearance, with features that vary in accordance with the proportion of adipose and vascular tissue: fat is manifested as high signal intensity on T1-weighted MRI, whereas vascularity is manifested as high signal intensity on T2-weighted images.5,6 In our patient, T2-weighted MRI showed a predominantly hyperintense mass in the chest wall. Magnetic resonance imaging is also useful in evaluating the presence and extent of intraspinal involvement of posterior mediastinal tumors. In our patient, the hemangioma did not extend into the spinal canal.
Prompt treatment after diagnosis is important because arteriovenous hemangiomas are prone to bleed spontaneously or after minor traumatic injury. Treatment is difficult and often dangerous—particularly when huge tumors are adjacent to main vessels. Ligation or embolization of the feeding arteries is palliative and ultimately ineffective, because the condition is subject to recurrence: the arteriovenous communications are small and numerous.7,8 Some investigators,7–9 however, have suggested selective embolization of feeding vessels as an alternative to surgical treatment, especially for children. Preoperative embolization of hemangiomas facilitates meticulous dissection. It also might prevent cardiac failure, reduce the size of the tumor before surgery, and minimize the risk of intraoperative complications like excessive bleeding. If embolization is performed, the tumor should be excised immediately thereafter, to prevent recruitment of a collateral blood supply. Even after embolization, tumors may retain their hypervascularity, requiring careful dissection during complete surgical excision. Materials used in therapeutic embolization include coils, ethanol, sodium tetradecyl sulfate, cyanoacrylate, polyvinyl alcohol, microspheres, and gelatin sponge (Gelfoam®, Pharmacia & Upjohn Co., a division of Pfizer, Inc.; New York, NY), among others.9,10
The standard treatment is complete excision with clean surgical margins. Chest wall reconstruction should be considered if more than 2 ribs are resected. Partial extirpation usually causes rapid deterioration, disturbed wound healing, and secondary bleeding.11 Complete excision is mandatory to achieve a cure.7,8,11 We performed chest wall reconstruction because of the diameter of the affected area: not only the chest wall but 5 ribs had been resected. We encountered no difficulty in excising the tumor, for it was well demarcated.
Address for reprints: Refik Ulku, MD, Department of Thoracic Surgery, Dicle University School of Medicine, 21280 Diyarbakir, Turkey