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Intraosseous angioma: a rare cause of a rib mass in childhood
Journal of Pediatric Surgery (2012) 47, E5–E7
www.elsevier.com/locate/jpedsurg
Intraosseous angioma: a rare cause of a rib mass in childhood Francesca Biscaro a , Marta Pillon b,⁎, Marta Bordignon a , Paolo Mesirca a , Lara Alessandrini c , Giovanni Cecchetto d , Francesco Spigariol e a
Pediatric Unit, Montebelluna's Hospital, Treviso, Italy Pediatric Hematology Oncology, Department of Pediatric, University of Padova, Padova, Italy c Department of Medicine (DIMED), Surgical Pathology Unit, University Hospital of Padova, Padova, Italy d Pediatric Surgery, Department of Pediatric, University of Padova, Padova, Italy e Radiology Unit, Montebelluna's Hospital, Treviso, Italy b
Received 14 December 2011; revised 9 May 2012; accepted 22 May 2012
Key words: Bone angioma; Rib tumors; Bone erosion; Childhood
Abstract Rib tumors are uncommon and represent 5% to 10% of all bony tumors. Regarding the benign rib lesions, costal angioma is very rare in childhood. We report a case of a rare angioma of a rib complicated by bone erosion in a young boy. © 2012 Elsevier Inc. All rights reserved.
Rib tumors are uncommon and represent 5% to 10% of all bone tumors. Previous reports have suggested that malignant rib tumors are significantly more common than benign ones [1,2]. The differential diagnosis can sometime be difficult. Imaging and histologic examination are mandatory to establish the correct treatment and follow-up. This report describes a young boy with a rare angioma of a rib complicated by bone erosion successfully treated with surgery.
1. Case report A 13-year-old boy was admitted to our clinic with a persistent and painful swelling of the left thorax, with no ⁎ Corresponding author. Pediatric Hematology Oncology, Department of Pediatrics, University of Padova, Via Giustiniani 3, 35128, Padova, Italy. Tel.: + 39 049 8213579; fax: +39 049 8213510. E-mail address: [email protected] (M. Pillon). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.05.033
history of chest trauma. One month earlier, he had fever and cough for 10 days followed by pain on the left side of the thorax, resistant to nonsteroidal anti-inflammatory therapy. On physical examination, a painful, fixed mass without clear boundaries at the level of the ninth rib on the left chest was noted. Laboratory tests were normal: in particular, inflammatory and infective investigations, lactate dehydrogenase (LDH), and muscular enzymes as well as the Mantoux test were negative. Ultrasonography showed edema and swelling in the tissues surrounding the ninth rib and nearby cartilage; however, no underlying bone lesions could be detected. The chest radiograph was negative for lung and chest wall masses. After 2 weeks, a second ultrasonography showed a nonhomogeneous swelling of the soft tissues surrounding the costochondral junction (5 × 3 × 1 cm diameter) with a cortical superficial discontinuity at the terminal part of the same rib. Three-dimensional computed tomography demonstrated gaps from bone loss (6-mm diameter), with partial disruption of the cortical profile, at the anterior osseous end
E6
F. Biscaro et al.
Fig. 1 Chest contrast-enhanced magnetic resonance imaging shows gaps from bone loss (6-mm diameter) of the ninth left rib, with partial disruption of the cortical profile. An increased contrast enhancement in the soft tissues surrounding the bone, with alteration of the corresponding costochondral junction of the same level, is clearly highlighted.
of the noticeable swelling of the rib. Chest contrast-enhanced magnetic resonance imaging (Fig. 1) confirmed the computed tomographic images showing an increased contrast enhancement in the soft tissues surrounding the bone, with alteration of the correspondent costochondral junction at the same level. Skeletal scintigraphy revealed increased isotopic uptake at the level of the anterior end of the 9th left rib, with normal uptake at the level of other osteocartilagenous junctions. Two months after the onset of the symptoms, a surgical procedure was planned. Through an incision over the palpable mass, a wide excision (approximately 8 cm in length) of the enlarged rib en bloc was performed, including the fibromuscular tissue around it. The mass was not identifiable during the operation, and the bone margins of the resection were macroscopically clear: their distance from the palpable mass was approximately 1.5 cm. Reconstruction of the thoracic wall was achieved directly, without the need for a patch. The postoperative period was uneventful, and the patient was discharged 5 days later. Histologic examination revealed a benign vascular proliferation in the bone
marrow. Medullary spaces were extensively replaced by venules and capillary-sized vessels (Fig. 2A) lined by a single layer of cytologically bland endothelial cells intermingled with cartilage nests and newly formed bony trabeculae (Fig. 2B). No signs of cytologic atypia were found. Surgical excision margins were negative. The overall morphological appearance was consistent with an intraosseous angioma, complicated by the presence of cartilage and bone proliferation. The outcome was excellent, and no signs of relapse were noted at 24 months of follow-up.
2. Discussion To our knowledge, this is the first case described in literature of intraosseous angioma of the rib in children. Reports about bone angioma in children are few, and there are no general guidelines for the clinical management of these lesions. The differential diagnosis of rib lesions
Fig. 2 Hematoxylin-eosin staining shows an extensive network of anastomosing dilated vessels (A, original magnification ×25), lined by a single endothelial layer without atypia, intermingled to newly formed bone trabeculae and cartilage nests (B, original magnification × 40).
Intraosseous angioma: a rare cause of rib mass in childhood includes both benign and malignant masses. Some rapidly growing sternal masses can be benign self-limited tumors and a wait-and-see approach is suggested [3]. In our case, pain and the gaps from bone loss justified a more aggressive approach to exclude a malignant origin, especially Ewing sarcoma of the rib that is common in adolescents and young adults [4,5]. Another very aggressive but rare lesion is the primitive neuroectodermal tumor (Askin tumor) that can destroy the rib in approximately 25% to 63% of the cases [6,7]. Regarding the benign rib lesions, cartilaginous tumors are the most commonly reported (50%), including chondroma and osteochondroma, which are most frequent. They are likely to become malignant (11%) after years of benign growth [8,9]. Among cartilaginous tumors, enchondromatosis is a group of rare disorders characterized by absence or presence of hemangiomas (Maffucci and Ollier disease) [10]. Langerhans cell histiocytosis is a rare but not uncommon tumor of the rib that sometimes can manifest as a painful soft tissue mass [4]. Other benign lesions of the rib are the fibrous tissue tumors, neurilemoma (schwannoma), and hemangioma [11,12]. As described in this report, a rare benign lesion such as bone angioma can also be a cause of bone erosions, and if preoperative investigations are uncertain, surgical excision to achieve a definitive diagnosis should be considered.
E7
References [1] Teitelbaum SL. Tumours of the ribs. Ann Thorac Surg Gynecol Obstet 1969;129:1059-73. [2] Schmidt FE, Trummer MJ. Primary tumours of the ribs. Ann Thorac Surg 1972;13:251-7. [3] te Winkel ML, Lequin MH, de Bruyn JR, et al. Self-limiting sternal tumours of childhood (SELSTOC). Pediatr Blood Cancer 2010;55:81-4. [4] Resnick D. Lipoidoses, histiocytoses, and hypoproteinemias. In: Resnick D, editor. Diagnosis of bone and joint disorders. 3rd ed. Philadelphia, Pa: Saunders; 1995. p. 2215-7. [5] Moser Jr RP, Davis MJ, Gilkey FW, et al. Primary Ewing sarcoma of rib. Radiographic 1990;10:899-914. [6] Schulman H, Newman-Heinman N, Kurtzbart E, et al. Thoracoabdominal peripheral primitive neuroecrodermal tumors in childhood: radiological features. Eur Radiol 2000;10:1649-52. [7] Glass RBF, Norton KI, Mitre SA, et al. Ribs: a spectrum of abnormalities. Radiographics 2002;22:87-104. [8] Sabanathan S, Salana F, Morgan WE, et al. Primary chest wall tumours. Ann Thorac Surg 1985;39:4-15. [9] Ryan MB, McMurtrey MJ, Roth JA. Current management of chest wall tumours. Surg Clin North Am 1989;69:1061-80. [10] Bovee JV, Van Roggen JF, Cleton-Jansen AM, et al. Malignant progression in multiple enchondromatosis (Ollier's disease): an autopsybased molecular genetic study. Hum Pathol 2000;31:1299-303. [11] Tew K, Constantine S, Lew WY. Intraosseous hemangioma of the rib mimicking an aggressive chest wall tumor. Diagn Interv Radiol 2011;17(2):118-21. [12] Ulku R, Onat S, Avci A, et al. Resection of intercostal hemangioma with involved chest wall and ribs. Tex Heart Inst J 2010;37(4):486-9.
www.elsevier.com/locate/jpedsurg
Intraosseous angioma: a rare cause of a rib mass in childhood Francesca Biscaro a , Marta Pillon b,⁎, Marta Bordignon a , Paolo Mesirca a , Lara Alessandrini c , Giovanni Cecchetto d , Francesco Spigariol e a
Pediatric Unit, Montebelluna's Hospital, Treviso, Italy Pediatric Hematology Oncology, Department of Pediatric, University of Padova, Padova, Italy c Department of Medicine (DIMED), Surgical Pathology Unit, University Hospital of Padova, Padova, Italy d Pediatric Surgery, Department of Pediatric, University of Padova, Padova, Italy e Radiology Unit, Montebelluna's Hospital, Treviso, Italy b
Received 14 December 2011; revised 9 May 2012; accepted 22 May 2012
Key words: Bone angioma; Rib tumors; Bone erosion; Childhood
Abstract Rib tumors are uncommon and represent 5% to 10% of all bony tumors. Regarding the benign rib lesions, costal angioma is very rare in childhood. We report a case of a rare angioma of a rib complicated by bone erosion in a young boy. © 2012 Elsevier Inc. All rights reserved.
Rib tumors are uncommon and represent 5% to 10% of all bone tumors. Previous reports have suggested that malignant rib tumors are significantly more common than benign ones [1,2]. The differential diagnosis can sometime be difficult. Imaging and histologic examination are mandatory to establish the correct treatment and follow-up. This report describes a young boy with a rare angioma of a rib complicated by bone erosion successfully treated with surgery.
1. Case report A 13-year-old boy was admitted to our clinic with a persistent and painful swelling of the left thorax, with no ⁎ Corresponding author. Pediatric Hematology Oncology, Department of Pediatrics, University of Padova, Via Giustiniani 3, 35128, Padova, Italy. Tel.: + 39 049 8213579; fax: +39 049 8213510. E-mail address: [email protected] (M. Pillon). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpedsurg.2012.05.033
history of chest trauma. One month earlier, he had fever and cough for 10 days followed by pain on the left side of the thorax, resistant to nonsteroidal anti-inflammatory therapy. On physical examination, a painful, fixed mass without clear boundaries at the level of the ninth rib on the left chest was noted. Laboratory tests were normal: in particular, inflammatory and infective investigations, lactate dehydrogenase (LDH), and muscular enzymes as well as the Mantoux test were negative. Ultrasonography showed edema and swelling in the tissues surrounding the ninth rib and nearby cartilage; however, no underlying bone lesions could be detected. The chest radiograph was negative for lung and chest wall masses. After 2 weeks, a second ultrasonography showed a nonhomogeneous swelling of the soft tissues surrounding the costochondral junction (5 × 3 × 1 cm diameter) with a cortical superficial discontinuity at the terminal part of the same rib. Three-dimensional computed tomography demonstrated gaps from bone loss (6-mm diameter), with partial disruption of the cortical profile, at the anterior osseous end
E6
F. Biscaro et al.
Fig. 1 Chest contrast-enhanced magnetic resonance imaging shows gaps from bone loss (6-mm diameter) of the ninth left rib, with partial disruption of the cortical profile. An increased contrast enhancement in the soft tissues surrounding the bone, with alteration of the corresponding costochondral junction of the same level, is clearly highlighted.
of the noticeable swelling of the rib. Chest contrast-enhanced magnetic resonance imaging (Fig. 1) confirmed the computed tomographic images showing an increased contrast enhancement in the soft tissues surrounding the bone, with alteration of the correspondent costochondral junction at the same level. Skeletal scintigraphy revealed increased isotopic uptake at the level of the anterior end of the 9th left rib, with normal uptake at the level of other osteocartilagenous junctions. Two months after the onset of the symptoms, a surgical procedure was planned. Through an incision over the palpable mass, a wide excision (approximately 8 cm in length) of the enlarged rib en bloc was performed, including the fibromuscular tissue around it. The mass was not identifiable during the operation, and the bone margins of the resection were macroscopically clear: their distance from the palpable mass was approximately 1.5 cm. Reconstruction of the thoracic wall was achieved directly, without the need for a patch. The postoperative period was uneventful, and the patient was discharged 5 days later. Histologic examination revealed a benign vascular proliferation in the bone
marrow. Medullary spaces were extensively replaced by venules and capillary-sized vessels (Fig. 2A) lined by a single layer of cytologically bland endothelial cells intermingled with cartilage nests and newly formed bony trabeculae (Fig. 2B). No signs of cytologic atypia were found. Surgical excision margins were negative. The overall morphological appearance was consistent with an intraosseous angioma, complicated by the presence of cartilage and bone proliferation. The outcome was excellent, and no signs of relapse were noted at 24 months of follow-up.
2. Discussion To our knowledge, this is the first case described in literature of intraosseous angioma of the rib in children. Reports about bone angioma in children are few, and there are no general guidelines for the clinical management of these lesions. The differential diagnosis of rib lesions
Fig. 2 Hematoxylin-eosin staining shows an extensive network of anastomosing dilated vessels (A, original magnification ×25), lined by a single endothelial layer without atypia, intermingled to newly formed bone trabeculae and cartilage nests (B, original magnification × 40).
Intraosseous angioma: a rare cause of rib mass in childhood includes both benign and malignant masses. Some rapidly growing sternal masses can be benign self-limited tumors and a wait-and-see approach is suggested [3]. In our case, pain and the gaps from bone loss justified a more aggressive approach to exclude a malignant origin, especially Ewing sarcoma of the rib that is common in adolescents and young adults [4,5]. Another very aggressive but rare lesion is the primitive neuroectodermal tumor (Askin tumor) that can destroy the rib in approximately 25% to 63% of the cases [6,7]. Regarding the benign rib lesions, cartilaginous tumors are the most commonly reported (50%), including chondroma and osteochondroma, which are most frequent. They are likely to become malignant (11%) after years of benign growth [8,9]. Among cartilaginous tumors, enchondromatosis is a group of rare disorders characterized by absence or presence of hemangiomas (Maffucci and Ollier disease) [10]. Langerhans cell histiocytosis is a rare but not uncommon tumor of the rib that sometimes can manifest as a painful soft tissue mass [4]. Other benign lesions of the rib are the fibrous tissue tumors, neurilemoma (schwannoma), and hemangioma [11,12]. As described in this report, a rare benign lesion such as bone angioma can also be a cause of bone erosions, and if preoperative investigations are uncertain, surgical excision to achieve a definitive diagnosis should be considered.
E7
References [1] Teitelbaum SL. Tumours of the ribs. Ann Thorac Surg Gynecol Obstet 1969;129:1059-73. [2] Schmidt FE, Trummer MJ. Primary tumours of the ribs. Ann Thorac Surg 1972;13:251-7. [3] te Winkel ML, Lequin MH, de Bruyn JR, et al. Self-limiting sternal tumours of childhood (SELSTOC). Pediatr Blood Cancer 2010;55:81-4. [4] Resnick D. Lipoidoses, histiocytoses, and hypoproteinemias. In: Resnick D, editor. Diagnosis of bone and joint disorders. 3rd ed. Philadelphia, Pa: Saunders; 1995. p. 2215-7. [5] Moser Jr RP, Davis MJ, Gilkey FW, et al. Primary Ewing sarcoma of rib. Radiographic 1990;10:899-914. [6] Schulman H, Newman-Heinman N, Kurtzbart E, et al. Thoracoabdominal peripheral primitive neuroecrodermal tumors in childhood: radiological features. Eur Radiol 2000;10:1649-52. [7] Glass RBF, Norton KI, Mitre SA, et al. Ribs: a spectrum of abnormalities. Radiographics 2002;22:87-104. [8] Sabanathan S, Salana F, Morgan WE, et al. Primary chest wall tumours. Ann Thorac Surg 1985;39:4-15. [9] Ryan MB, McMurtrey MJ, Roth JA. Current management of chest wall tumours. Surg Clin North Am 1989;69:1061-80. [10] Bovee JV, Van Roggen JF, Cleton-Jansen AM, et al. Malignant progression in multiple enchondromatosis (Ollier's disease): an autopsybased molecular genetic study. Hum Pathol 2000;31:1299-303. [11] Tew K, Constantine S, Lew WY. Intraosseous hemangioma of the rib mimicking an aggressive chest wall tumor. Diagn Interv Radiol 2011;17(2):118-21. [12] Ulku R, Onat S, Avci A, et al. Resection of intercostal hemangioma with involved chest wall and ribs. Tex Heart Inst J 2010;37(4):486-9.