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Lipoblastoma: a rare cervical mass in childhood
International Journal of Pediatric Otorhinolaryngology 61 (2001) 265– 268 www.elsevier.com/locate/ijporl
Case report
Lipoblastoma: a rare cervical mass in childhood Umit Nusret Basaran a,*, Mustafa Inan b, Selcuk Bilgi c, Mehmet Pul a a
Department of Pediatric Surgery, Medical Faculty, Trakya Uni6ersity, Edirne, Turkey b Department of Pediatric Surgery, Edirne State Hospital, Edirne, Turkey c Department of Pathology, Trakya Uni6ersity, Medical Faculty, Edirne, Turkey
Received 19 May 2001; received in revised form 17 August 2001; accepted 17 August 2001
Abstract A 4-year-old girl had a left side cervical swelling for three months. The mass was free from surrounding tissues and excised completely. Histopathologic diagnosis of the specimen was lipoblastoma. Cervical lipoblastoma is a rapidly growing neoplasm. Only two cases of cervical lipoblastomas caused airway obstruction have been reported. Recurrence is rare in cases of lipoblastoma. Complete excision of the tumor is the treatment of choice. Seventeen cases of cervical lipoblastoma have been reported until now in the literature, but we think that lipoblastoma is more frequent than reported. Lipoblastoma must be remembered as a rare cause of cervical masses in childhood. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Lipoblastoma; Neck; Childhood
1. Introduction
2. Case report
Lipoblastoma is a rare benign mesenchymal tumor of embryonic fat cells that occurring in infants and children [1 – 8]. Lipoblastomas typically arise from the limbs and trunk [1 – 4], cases involving the head and neck are rare [1 – 3,5 – 9]. We would like to report on a case of lipoblastoma of the left side of the neck in a 4-year-old girl.
A 4-year-old girl had left side cervical swelling for three months. The swelling was painless and progressively increasing in size. On physical examination, 4× 2 cm soft and subcutaneous mass in left lateral cervical region was felt. The mass was free from the surrounding muscle and other tissues and the overlying skin was normal (Fig. 1). Ultrasound examination showed a homogeneous and thyroid like echoic mass. The laboratory tests and thyroid scanning were within normal limits. Our clinical impression was of lipoma. At the operation, we made a lateral cervical incision. There was no invasion of any of the neck struc-
* Corresponding author P.K. 97, 2201 Edirne. Tel.: + 90284-235-9343; fax: + 90-212-662-1242. E-mail address: [email protected] (U.N. Basaran).
0165-5876/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 1 ) 0 0 5 8 2 - 1
266
U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
tures and the subcutaneous mass was completely excised. The specimen consisted of a well-circumscribed, soft, yellowish-white and homogenous mass. Histopathologic examination showed wellencapsulated tumor. There were lipoblasts in lobulated pattern and separated by loose fibrous connective-tissue septa. Some of the cell contained large cytoplasmic vacuoles. The histopathologic diagnosis was lipoblastoma (Fig. 2). The postoperative course was uneventful and no recurrence was observed 5 years after the operation.
3. Discussion The tumors of embryonal fat tissue are very rare [1–8]. Two types are recognized; localized
Fig. 1. Photography of the patient’s neck is showing swelling in the left lateral cervical region. The mass was mobile and overlying skin was normal.
and well circumscribed is termed ‘lipoblastoma’ and second, diffuse and multicentric lesion is termed ‘lipoblastomatosis’ [1–4,6 –8]. Most of the cases occur in children younger than 3 years [2–4]. Approximately two-third of the cases are localized in the extremities [2]. Cervical lipoblastoma is extremely rare. There were only nine cases of cervical lipoblastoma in the largest review of 84 cases of lipoblastoma in 1993 [2]. Subsequently four cases of cervical lipoblastoma have been reported in the literature [5–8]. In addition, Dilley et al. [4] reported 25 cases of lipoblastoma that four cases of them were localized in cervical region [M. Bandlt (2001) personal communication]. As a result, we could find 17 cervical cases of lipoblastoma until now in the literature. Cervical lipoblastoma is a rapidly growing neoplasm, but they are not usually disclosed other complaint [5–7]. Only two cases causing airway obstruction were reported [5,9]. True clinical impression is difficult [4]. Imaging techniques are not adequate for diagnosis [10]. Radiologically, lipoblastoma presents as a nonspecific soft-tissue-density mass. Conventional Xrays may show transparent soft tissue density in pertinent cases. On ultrasound, lipoblastoma shows also high, low or isoechoic pattern. This pattern is usually heterogeneous. Computed tomography frequently confirms the fat density of the tumor separated by thick septae. The magnetic resonance imaging characteristics of the lipoblastomas are correlated with histopathologic stricture of specimen. In magnetic resonance imaging, there are usually intermediate or lower signal intensity in T1-weighted images than mature lipocyts and the high signal intensity in T2weighted images are due to the rich myxoid stroma combined with numerous lipoblasts [10 – 13]. If the mature fatty tissue forms a great portion of the mass like lipoma, T1-weighted image may be high signal intensity [10]. The hypointense septae visible in T1 and T2-weighted images are due to connective tissue [12]. In lipoblastomatosis, the mass is poorly defined from adherent tissue by magnetic resonance imaging [10]. However the magnetic resonance imaging characteristic of this neoplasm is nonspecific [12]. But computed tomography and magnetic resonance imaging are
U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
267
Fig. 2. Photomicrography of the excised specimen showing is lipoblasts contain small or large vacuoles and some of them contain multiple vacuoles in myxoid matrix (H&E, original magnification× 200).
very good tools for demonstrating anatomical detail of the lipoblastoma which is essential for successful surgical mass excision [10,14]. Complete excision of the tumor is the treatment of choice. Recurrence is rare in cases of lipoblastoma but lipoblastomatosis can recur in the range of 10– 25%, and requiring multiple excisions [4,7]. In the last years, two large single institutional retrospective review of lipoblastoma were reported by Collins et al [3] in 1997 (24 cases) and Dilley et al [4] in 2001 (25 cases). Therefore, we suspect that lipoblastoma is more frequent than previously known. If there is a rapidly growing cervical mass which is in fatty tissue density in a child, one must suspect of lipoblastoma.
References [1] G. Stringel, B. Shandling, K. Mancer, S.H. Ein, Lipoblastoma in infants and children, J. Pediatr. Surg. 17 (1982) 227 – 280. [2] A.I. Serdar, M.E. Senocak, S. Gogus, A. Buyukpamukcu, A case of benign intrascrotal lipoblastoma clinically mimicking testicular torsion and review of literature, J. Pediatr. Surg. 28 (1993) 259 – 261. [3] M.H. Collins, J. Chatten, Lipoblastoma/Lipoblastomatosis: A clinicopathologic study of 25 Tumors, Am J. Surg. Pathol. 21 (1997) 1131 –1137. [4] A.V. Dilley, D.L. Patel, M.J. Hicks, M.L. Brandt, Lipoblastoma: Pathophysiology and surgical management, J. Pediatr. Surg. 36 (2001) 229 – 231. [5] L.S. Rasmussen, J. Kirkegaard, M. Kaasb(l, Intermittent airway obstruction in a child caused by a cervical lipoblastoma, Acta Anaesthesiol. Scand. 41 (1997) 945 – 946.
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U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
[6] M.K. Farrugia, C. Fearne, Benign lipoblastoma arising in the neck, Pediatr. Surg. Int. 13 (1998) 213 – 214. [7] S.K. Ratan, A. Gambhir, S. Mullick, J. Ratan, Lipoblastoma of the neck, Indian J. Pediatr. 67 (2000) 301 – 303. [8] Y.-S. Chun, W.-K. Kim, K.-W. Park, S.-C. Lee, S.-E. Jung, Lipoblastoma, J. Pediatr. Surg. 36 (2001) 905 –907. [9] N. Gammelgaard, K. Jørgensen, C. Lund, Benign lipoblastoma in the neck causing respiratory insufficiency, Laryngoscope 93 (1983) 935 –937. [10] T. Reiseter, T. Nordshus, A. Borthne, B. Roald, P. Naess, O. Schistad, Lipoblastoma: MRI appearances of a rare pediatric soft tissue tumour, Pediatr. Radiol. 29 (1999) 542 – 545.
[11] C.H. Park, K.-I. Kim, Y.T. Lim, S.W. Chung, C.H. Lee, Ruptured giant intrathoracic lipoblastoma in a 4-mounthold infant: CT and MR finding, Pediatr. Radiol. 30 (2000) 38 – 40. [12] L. Letourneau, M. Dufour, J. Deschenes, Shoulder lipoblastoma: magnetic resonance imaging characteristics, Can. Assoc. Radiol. J. 44 (1993) 211 – 214. [13] E. Schultz, R. Rosenblatt, S. Mitsudo, G. Weinberg, Detection of a deep lipoblastoma by MRI and ultrasound, Pediatr. Radiol. 23 (1993) 409 – 410. [14] W.C. Black, J.W. Burke, P.S. Feldman, C.M. JohnsonIII, S. Swanson, CT appearance of cervical lipoblastoma, J. Comput. Assist. Tomogr. 10 (1986) 696 – 698.
Case report
Lipoblastoma: a rare cervical mass in childhood Umit Nusret Basaran a,*, Mustafa Inan b, Selcuk Bilgi c, Mehmet Pul a a
Department of Pediatric Surgery, Medical Faculty, Trakya Uni6ersity, Edirne, Turkey b Department of Pediatric Surgery, Edirne State Hospital, Edirne, Turkey c Department of Pathology, Trakya Uni6ersity, Medical Faculty, Edirne, Turkey
Received 19 May 2001; received in revised form 17 August 2001; accepted 17 August 2001
Abstract A 4-year-old girl had a left side cervical swelling for three months. The mass was free from surrounding tissues and excised completely. Histopathologic diagnosis of the specimen was lipoblastoma. Cervical lipoblastoma is a rapidly growing neoplasm. Only two cases of cervical lipoblastomas caused airway obstruction have been reported. Recurrence is rare in cases of lipoblastoma. Complete excision of the tumor is the treatment of choice. Seventeen cases of cervical lipoblastoma have been reported until now in the literature, but we think that lipoblastoma is more frequent than reported. Lipoblastoma must be remembered as a rare cause of cervical masses in childhood. © 2001 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Lipoblastoma; Neck; Childhood
1. Introduction
2. Case report
Lipoblastoma is a rare benign mesenchymal tumor of embryonic fat cells that occurring in infants and children [1 – 8]. Lipoblastomas typically arise from the limbs and trunk [1 – 4], cases involving the head and neck are rare [1 – 3,5 – 9]. We would like to report on a case of lipoblastoma of the left side of the neck in a 4-year-old girl.
A 4-year-old girl had left side cervical swelling for three months. The swelling was painless and progressively increasing in size. On physical examination, 4× 2 cm soft and subcutaneous mass in left lateral cervical region was felt. The mass was free from the surrounding muscle and other tissues and the overlying skin was normal (Fig. 1). Ultrasound examination showed a homogeneous and thyroid like echoic mass. The laboratory tests and thyroid scanning were within normal limits. Our clinical impression was of lipoma. At the operation, we made a lateral cervical incision. There was no invasion of any of the neck struc-
* Corresponding author P.K. 97, 2201 Edirne. Tel.: + 90284-235-9343; fax: + 90-212-662-1242. E-mail address: [email protected] (U.N. Basaran).
0165-5876/01/$ - see front matter © 2001 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 0 1 ) 0 0 5 8 2 - 1
266
U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
tures and the subcutaneous mass was completely excised. The specimen consisted of a well-circumscribed, soft, yellowish-white and homogenous mass. Histopathologic examination showed wellencapsulated tumor. There were lipoblasts in lobulated pattern and separated by loose fibrous connective-tissue septa. Some of the cell contained large cytoplasmic vacuoles. The histopathologic diagnosis was lipoblastoma (Fig. 2). The postoperative course was uneventful and no recurrence was observed 5 years after the operation.
3. Discussion The tumors of embryonal fat tissue are very rare [1–8]. Two types are recognized; localized
Fig. 1. Photography of the patient’s neck is showing swelling in the left lateral cervical region. The mass was mobile and overlying skin was normal.
and well circumscribed is termed ‘lipoblastoma’ and second, diffuse and multicentric lesion is termed ‘lipoblastomatosis’ [1–4,6 –8]. Most of the cases occur in children younger than 3 years [2–4]. Approximately two-third of the cases are localized in the extremities [2]. Cervical lipoblastoma is extremely rare. There were only nine cases of cervical lipoblastoma in the largest review of 84 cases of lipoblastoma in 1993 [2]. Subsequently four cases of cervical lipoblastoma have been reported in the literature [5–8]. In addition, Dilley et al. [4] reported 25 cases of lipoblastoma that four cases of them were localized in cervical region [M. Bandlt (2001) personal communication]. As a result, we could find 17 cervical cases of lipoblastoma until now in the literature. Cervical lipoblastoma is a rapidly growing neoplasm, but they are not usually disclosed other complaint [5–7]. Only two cases causing airway obstruction were reported [5,9]. True clinical impression is difficult [4]. Imaging techniques are not adequate for diagnosis [10]. Radiologically, lipoblastoma presents as a nonspecific soft-tissue-density mass. Conventional Xrays may show transparent soft tissue density in pertinent cases. On ultrasound, lipoblastoma shows also high, low or isoechoic pattern. This pattern is usually heterogeneous. Computed tomography frequently confirms the fat density of the tumor separated by thick septae. The magnetic resonance imaging characteristics of the lipoblastomas are correlated with histopathologic stricture of specimen. In magnetic resonance imaging, there are usually intermediate or lower signal intensity in T1-weighted images than mature lipocyts and the high signal intensity in T2weighted images are due to the rich myxoid stroma combined with numerous lipoblasts [10 – 13]. If the mature fatty tissue forms a great portion of the mass like lipoma, T1-weighted image may be high signal intensity [10]. The hypointense septae visible in T1 and T2-weighted images are due to connective tissue [12]. In lipoblastomatosis, the mass is poorly defined from adherent tissue by magnetic resonance imaging [10]. However the magnetic resonance imaging characteristic of this neoplasm is nonspecific [12]. But computed tomography and magnetic resonance imaging are
U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
267
Fig. 2. Photomicrography of the excised specimen showing is lipoblasts contain small or large vacuoles and some of them contain multiple vacuoles in myxoid matrix (H&E, original magnification× 200).
very good tools for demonstrating anatomical detail of the lipoblastoma which is essential for successful surgical mass excision [10,14]. Complete excision of the tumor is the treatment of choice. Recurrence is rare in cases of lipoblastoma but lipoblastomatosis can recur in the range of 10– 25%, and requiring multiple excisions [4,7]. In the last years, two large single institutional retrospective review of lipoblastoma were reported by Collins et al [3] in 1997 (24 cases) and Dilley et al [4] in 2001 (25 cases). Therefore, we suspect that lipoblastoma is more frequent than previously known. If there is a rapidly growing cervical mass which is in fatty tissue density in a child, one must suspect of lipoblastoma.
References [1] G. Stringel, B. Shandling, K. Mancer, S.H. Ein, Lipoblastoma in infants and children, J. Pediatr. Surg. 17 (1982) 227 – 280. [2] A.I. Serdar, M.E. Senocak, S. Gogus, A. Buyukpamukcu, A case of benign intrascrotal lipoblastoma clinically mimicking testicular torsion and review of literature, J. Pediatr. Surg. 28 (1993) 259 – 261. [3] M.H. Collins, J. Chatten, Lipoblastoma/Lipoblastomatosis: A clinicopathologic study of 25 Tumors, Am J. Surg. Pathol. 21 (1997) 1131 –1137. [4] A.V. Dilley, D.L. Patel, M.J. Hicks, M.L. Brandt, Lipoblastoma: Pathophysiology and surgical management, J. Pediatr. Surg. 36 (2001) 229 – 231. [5] L.S. Rasmussen, J. Kirkegaard, M. Kaasb(l, Intermittent airway obstruction in a child caused by a cervical lipoblastoma, Acta Anaesthesiol. Scand. 41 (1997) 945 – 946.
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U.N. Basaran et al. / Int. J. Pediatr. Otorhinolaryngol. 61 (2001) 265–268
[6] M.K. Farrugia, C. Fearne, Benign lipoblastoma arising in the neck, Pediatr. Surg. Int. 13 (1998) 213 – 214. [7] S.K. Ratan, A. Gambhir, S. Mullick, J. Ratan, Lipoblastoma of the neck, Indian J. Pediatr. 67 (2000) 301 – 303. [8] Y.-S. Chun, W.-K. Kim, K.-W. Park, S.-C. Lee, S.-E. Jung, Lipoblastoma, J. Pediatr. Surg. 36 (2001) 905 –907. [9] N. Gammelgaard, K. Jørgensen, C. Lund, Benign lipoblastoma in the neck causing respiratory insufficiency, Laryngoscope 93 (1983) 935 –937. [10] T. Reiseter, T. Nordshus, A. Borthne, B. Roald, P. Naess, O. Schistad, Lipoblastoma: MRI appearances of a rare pediatric soft tissue tumour, Pediatr. Radiol. 29 (1999) 542 – 545.
[11] C.H. Park, K.-I. Kim, Y.T. Lim, S.W. Chung, C.H. Lee, Ruptured giant intrathoracic lipoblastoma in a 4-mounthold infant: CT and MR finding, Pediatr. Radiol. 30 (2000) 38 – 40. [12] L. Letourneau, M. Dufour, J. Deschenes, Shoulder lipoblastoma: magnetic resonance imaging characteristics, Can. Assoc. Radiol. J. 44 (1993) 211 – 214. [13] E. Schultz, R. Rosenblatt, S. Mitsudo, G. Weinberg, Detection of a deep lipoblastoma by MRI and ultrasound, Pediatr. Radiol. 23 (1993) 409 – 410. [14] W.C. Black, J.W. Burke, P.S. Feldman, C.M. JohnsonIII, S. Swanson, CT appearance of cervical lipoblastoma, J. Comput. Assist. Tomogr. 10 (1986) 696 – 698.