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Solitary fibrous tumor of the head and neck in a child: Case report and review of the literature
J Ped Surg Case Reports 1 (2013) 194e196
Contents lists available at SciVerse ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Solitary fibrous tumor of the head and neck in a child: Case report and review of the literature Ogasawara Noriko a, *, Kikuchi Keisuke b, Yoshioka Iwao c, Yamashita Keiji a, Himi Tetsuo a a
Department of Otorhinolaryngology, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido 060-8556, Japan Department of Pathology, Obihiro Kousei Hospital, West 6, South 8-1, Obihiro, Hokkaido 080-0016, Japan c Department of Otorhinolaryngology, Obihiro Kousei Hospital, West 6, South 8-1, Obihiro, Hokkaido 080-0016, Japan b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 28 February 2013 Received in revised form 11 June 2013 Accepted 11 June 2013 Available online 10 July 2013
We report the first case of a benign extrapleural solitary fibrous tumor (SFT) that occurred in the left side of the neck of a 2-year-old boy. Complete surgical resection was performed for this tumor. Immunohistochemistry was useful to diagnose the benign solitary fibrous tumor. Ó 2013 Elsevier Inc. All rights reserved.
Key words: Extrapleural solitary fibrous tumor CD34 Head and neck
SFT was first described in 1931 by Klemperer and Rabin as a distinct mesothelial tumor arising from the pleura [1]. It is a usually encapsulated lesion considered to be part of hemangiopericytoma spectrum. SFT, referred to by many other names such as fibrous mesothelioma, subpleural fibroma and localized fibrous tumors of the pleura, is usually benign but malignant variants are frequently found, and metastasis can occur long after the first treatment [2]. Solitary fibrous tumor is the currently preferred term in pathology. While SFT is most common in the pleura, it may occur in any site, with about 6% developing in the head and neck [3]. Commonly, SFTs occur in adults and are extremely rare in children under 10 years old, with only 12 cases of SFTs in the head and neck reported in children [2]. The characteristic histologic appearance with CD34 immunoreactivity supports the diagnosis [2]. Here we report a rare case of a resectable SFT in the left side of the neck of a 2-year-old boy. 1. Case report In February 2010 the parents of a 2-year-old male noticed swelling in the left side of his neck and brought him to our hospital. Computed tomography (CT) and ultrasonography revealed a solid
* Corresponding author. Tel.: þ81 11 611 2111x3491; fax: þ81 11 615 5405. E-mail address: [email protected] (O. Noriko). 2213-5766/$ e see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2013.06.001
smooth mass and low echoic area in his neck described as a nerve sheath tumor. Fine needle aspiration was performed under ultrasonography and the cytomorphologic features had no malignancy and did not have the features of nerve sheath tumors. Magnetic resonance imaging (MRI) revealed a 30 mm 35 mm well-circumscribed T2 hypointense and T1 hypointense mass in the left side of his neck. In MRI findings, the tumor did not show connections to any nerves. He was placed under medical observation because of the possibility of a neurological deficit after a curative operation. However, 10 months later, the tumor in his neck appeared to have grown based on MRI findings (Fig. 1) and he still had no neurological deficit. His parents expressed their desire for a curative operation considering the possibility of malignant disease. We then obtained their informed consent for resection of the tumor. In January 2011, an operation was performed. The tumor was located away from any cervical nerves and had no adhesions. We could resect the encapsulated tumor en bloc. The postoperative clinical course was uneventful, with no neurological deficit. Histopathologic analysis revealed a hypocellular fibrous tumor with a patternless architecture and branching hemangiopericytoma-like vessels. The tumor cells were spindle-shaped without significant nuclear atypia. The mitotic count was 1/10HPF or less. No coagulative necrosis was observed. Immunohistochemically, the tumor cells were strongly immunoreactive for cluster of differentiation (CD)34, vimentin, and weakly positive for CD99 (MIC2).
O. Noriko et al. / J Ped Surg Case Reports 1 (2013) 194e196
195
Table 1 Reports of solitary fibrous tumors in children. Patient number
Age (years)
Sex
Site
1 2 3 4 5 6 7 8 9 10 11 12 Present case
13 11 18 14 14 2 18 15 18 11 14 9 2
M M M F F F F M M F F M M
Epiglottis Meninges Meninges Meninges Orbit Scalp Orbit Orbit Orbit Parotid Orbit Orbit Neck
F, female; M, male.
Fig. 1. A coronal T2W1 MRI image shows a solid low intensity mass in the left side of the neck. The mass size was 41 mm 40 mm.
Epithelial membrane antigen (EMA), S-100, a-smooth muscle actin, desmin, anti-muscle actin antigen (HHF-35), B-cell lymphoma 2 (bcl-2) and b-catenin were negative (Fig. 2A and B). The diagnosis was extrapleural benign SFT. The surgical margin was negative. The patient was discharged 7 days after the operation and no additional treatments were performed. No sign of recurrence or metastasis has been detected during 10 months after the operation. However long-term follow-up will be necessary to determine whether the operation was truly curative. 2. Discussion SFT is a rare spindle-cell tumor first reported by Klemperer in 1931 as originating from the pleura [1]. Extrapleural SFTs have been increasingly reported in recent years, although occurrence in the pediatric neck is extremely rare. In 1991 Witkin first described SFT in the head and neck, mainly in the nasal cavity [4]. Histologically, SFT has been described as a spindle cell tumor with a patternless architecture, hemangiopericytoma-like branching vascular pattern and strong CD34 immunoreactivity [3].
CD34 monoclonal antibodies recognize a single-chain transmembrane glycoprotein that is selectively expressed on human hemopoietic progenitor cells and vascular endothelium [5]. CD34 immunoreactivity is consistently found in SFTs, and this marker has potential use as an adjuvant in the diagnosis of this entity [3]. Thus, in extrapleural lesions, more detailed investigation of CD34 immunoreactivity might be necessary. Only twelve previous cases in pediatric patients have been reported in the English literature [6,7] (Table 1). Although most previously reported patients were adults, SFTs of extrapleural origin have been diagnosed with increasing frequency in recent years as the result of improved methods of pathologic examination. Under the World Health Organization (WHO) classification of tumors of soft tissue and bone in 2013, hemangiopericytomas (HPC) and SFTs of the soft tissues are regarded as features of the same entity in the soft tissue fascicle [8]. SFT is preferred by most pathologists as a better term than “hemangiopericytoma.” HPC left only a name including SFTs. Furthermore, HPC of the head and neck in children is extremely rare [9]. We could not obtain any case report about HPC of the neck in children under 10 years old. Complete surgical resection is the only method of curative management. SFTs are usually benign; however, they can recur and metastasize after surgical resection, thus making long-time followup of all cases strongly advisable [2,3].
3. Conclusion We reported a rare case of an SFT in the left side of the neck of a 2-year-old boy. CD34 immunoreactivity was useful to exclude other soft tissue tumors.
Fig. 2. A: Pathological findings of tumor (hematoxylin-eosin staining 40). B: Immunohistochemical analysis of tumor (200). Spindle cells strongly express CD34.
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O. Noriko et al. / J Ped Surg Case Reports 1 (2013) 194e196
Conflict of interest None. Sources of funding None. Acknowledgment We are grateful to Makiko Ito, Mika Sato, Mayumi Kitaura, and Kyoko Matsuura for their continuous support. References [1] Klemperer P, Rabin CB. Primary neoplasms of the pleura. Arch Pathol (Chic) 1931;11:385e412. [2] Witkin GB, Rosai J. Solitary fibrous tumor of the upper respiratory tract. A report of six cases. Am J Surg Pathol 1991;15:842e8.
[3] Westra WH, Gerald WL, Rosai J. Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 1994;18: 992e8. [4] Gold JS, Antonescu CR, Hajdu C, Ferrone CR, Hussain M, Lewis JJ, et al. Clinicopathological correlates of solitary fibrous tumors. Cancer 2002;94: 1057e68. [5] Sato J, Asakura K, Yokoyama Y, Satoh M. Solitary fibrous tumor of the parotid gland extending to the parapharyngeal space. Eur Arch Otorhinolaryngol 1998; 255:18e21. [6] Rakheja D, Wilson KS, Meehan JJ, Schultz RA, Maale GE, Timmons CF. Extrapleural benign solitary fibrous tumor in the shoulder of a 9-year-old girl: case report and review of the literature. Pediatr Dev Pathol 2004;7: 653e60. [7] Cox DP, Daniels T, Jordan RC. Solitary fibrous tumor of the head and neck. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110: 79e84. [8] Fletcher CDM, Bridge JA, Hogendoorn P, editors. WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC; 2013. lu E, Cansiz H, Mercan H, Dervis¸og lu S. Head and neck heman[9] Aciog giopericytomas: diagnostic contradictions. J Craniofac Surg 2009;20: 930e5.
Contents lists available at SciVerse ScienceDirect
Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com
Solitary fibrous tumor of the head and neck in a child: Case report and review of the literature Ogasawara Noriko a, *, Kikuchi Keisuke b, Yoshioka Iwao c, Yamashita Keiji a, Himi Tetsuo a a
Department of Otorhinolaryngology, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido 060-8556, Japan Department of Pathology, Obihiro Kousei Hospital, West 6, South 8-1, Obihiro, Hokkaido 080-0016, Japan c Department of Otorhinolaryngology, Obihiro Kousei Hospital, West 6, South 8-1, Obihiro, Hokkaido 080-0016, Japan b
a r t i c l e i n f o
a b s t r a c t
Article history: Received 28 February 2013 Received in revised form 11 June 2013 Accepted 11 June 2013 Available online 10 July 2013
We report the first case of a benign extrapleural solitary fibrous tumor (SFT) that occurred in the left side of the neck of a 2-year-old boy. Complete surgical resection was performed for this tumor. Immunohistochemistry was useful to diagnose the benign solitary fibrous tumor. Ó 2013 Elsevier Inc. All rights reserved.
Key words: Extrapleural solitary fibrous tumor CD34 Head and neck
SFT was first described in 1931 by Klemperer and Rabin as a distinct mesothelial tumor arising from the pleura [1]. It is a usually encapsulated lesion considered to be part of hemangiopericytoma spectrum. SFT, referred to by many other names such as fibrous mesothelioma, subpleural fibroma and localized fibrous tumors of the pleura, is usually benign but malignant variants are frequently found, and metastasis can occur long after the first treatment [2]. Solitary fibrous tumor is the currently preferred term in pathology. While SFT is most common in the pleura, it may occur in any site, with about 6% developing in the head and neck [3]. Commonly, SFTs occur in adults and are extremely rare in children under 10 years old, with only 12 cases of SFTs in the head and neck reported in children [2]. The characteristic histologic appearance with CD34 immunoreactivity supports the diagnosis [2]. Here we report a rare case of a resectable SFT in the left side of the neck of a 2-year-old boy. 1. Case report In February 2010 the parents of a 2-year-old male noticed swelling in the left side of his neck and brought him to our hospital. Computed tomography (CT) and ultrasonography revealed a solid
* Corresponding author. Tel.: þ81 11 611 2111x3491; fax: þ81 11 615 5405. E-mail address: [email protected] (O. Noriko). 2213-5766/$ e see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.epsc.2013.06.001
smooth mass and low echoic area in his neck described as a nerve sheath tumor. Fine needle aspiration was performed under ultrasonography and the cytomorphologic features had no malignancy and did not have the features of nerve sheath tumors. Magnetic resonance imaging (MRI) revealed a 30 mm 35 mm well-circumscribed T2 hypointense and T1 hypointense mass in the left side of his neck. In MRI findings, the tumor did not show connections to any nerves. He was placed under medical observation because of the possibility of a neurological deficit after a curative operation. However, 10 months later, the tumor in his neck appeared to have grown based on MRI findings (Fig. 1) and he still had no neurological deficit. His parents expressed their desire for a curative operation considering the possibility of malignant disease. We then obtained their informed consent for resection of the tumor. In January 2011, an operation was performed. The tumor was located away from any cervical nerves and had no adhesions. We could resect the encapsulated tumor en bloc. The postoperative clinical course was uneventful, with no neurological deficit. Histopathologic analysis revealed a hypocellular fibrous tumor with a patternless architecture and branching hemangiopericytoma-like vessels. The tumor cells were spindle-shaped without significant nuclear atypia. The mitotic count was 1/10HPF or less. No coagulative necrosis was observed. Immunohistochemically, the tumor cells were strongly immunoreactive for cluster of differentiation (CD)34, vimentin, and weakly positive for CD99 (MIC2).
O. Noriko et al. / J Ped Surg Case Reports 1 (2013) 194e196
195
Table 1 Reports of solitary fibrous tumors in children. Patient number
Age (years)
Sex
Site
1 2 3 4 5 6 7 8 9 10 11 12 Present case
13 11 18 14 14 2 18 15 18 11 14 9 2
M M M F F F F M M F F M M
Epiglottis Meninges Meninges Meninges Orbit Scalp Orbit Orbit Orbit Parotid Orbit Orbit Neck
F, female; M, male.
Fig. 1. A coronal T2W1 MRI image shows a solid low intensity mass in the left side of the neck. The mass size was 41 mm 40 mm.
Epithelial membrane antigen (EMA), S-100, a-smooth muscle actin, desmin, anti-muscle actin antigen (HHF-35), B-cell lymphoma 2 (bcl-2) and b-catenin were negative (Fig. 2A and B). The diagnosis was extrapleural benign SFT. The surgical margin was negative. The patient was discharged 7 days after the operation and no additional treatments were performed. No sign of recurrence or metastasis has been detected during 10 months after the operation. However long-term follow-up will be necessary to determine whether the operation was truly curative. 2. Discussion SFT is a rare spindle-cell tumor first reported by Klemperer in 1931 as originating from the pleura [1]. Extrapleural SFTs have been increasingly reported in recent years, although occurrence in the pediatric neck is extremely rare. In 1991 Witkin first described SFT in the head and neck, mainly in the nasal cavity [4]. Histologically, SFT has been described as a spindle cell tumor with a patternless architecture, hemangiopericytoma-like branching vascular pattern and strong CD34 immunoreactivity [3].
CD34 monoclonal antibodies recognize a single-chain transmembrane glycoprotein that is selectively expressed on human hemopoietic progenitor cells and vascular endothelium [5]. CD34 immunoreactivity is consistently found in SFTs, and this marker has potential use as an adjuvant in the diagnosis of this entity [3]. Thus, in extrapleural lesions, more detailed investigation of CD34 immunoreactivity might be necessary. Only twelve previous cases in pediatric patients have been reported in the English literature [6,7] (Table 1). Although most previously reported patients were adults, SFTs of extrapleural origin have been diagnosed with increasing frequency in recent years as the result of improved methods of pathologic examination. Under the World Health Organization (WHO) classification of tumors of soft tissue and bone in 2013, hemangiopericytomas (HPC) and SFTs of the soft tissues are regarded as features of the same entity in the soft tissue fascicle [8]. SFT is preferred by most pathologists as a better term than “hemangiopericytoma.” HPC left only a name including SFTs. Furthermore, HPC of the head and neck in children is extremely rare [9]. We could not obtain any case report about HPC of the neck in children under 10 years old. Complete surgical resection is the only method of curative management. SFTs are usually benign; however, they can recur and metastasize after surgical resection, thus making long-time followup of all cases strongly advisable [2,3].
3. Conclusion We reported a rare case of an SFT in the left side of the neck of a 2-year-old boy. CD34 immunoreactivity was useful to exclude other soft tissue tumors.
Fig. 2. A: Pathological findings of tumor (hematoxylin-eosin staining 40). B: Immunohistochemical analysis of tumor (200). Spindle cells strongly express CD34.
196
O. Noriko et al. / J Ped Surg Case Reports 1 (2013) 194e196
Conflict of interest None. Sources of funding None. Acknowledgment We are grateful to Makiko Ito, Mika Sato, Mayumi Kitaura, and Kyoko Matsuura for their continuous support. References [1] Klemperer P, Rabin CB. Primary neoplasms of the pleura. Arch Pathol (Chic) 1931;11:385e412. [2] Witkin GB, Rosai J. Solitary fibrous tumor of the upper respiratory tract. A report of six cases. Am J Surg Pathol 1991;15:842e8.
[3] Westra WH, Gerald WL, Rosai J. Solitary fibrous tumor. Consistent CD34 immunoreactivity and occurrence in the orbit. Am J Surg Pathol 1994;18: 992e8. [4] Gold JS, Antonescu CR, Hajdu C, Ferrone CR, Hussain M, Lewis JJ, et al. Clinicopathological correlates of solitary fibrous tumors. Cancer 2002;94: 1057e68. [5] Sato J, Asakura K, Yokoyama Y, Satoh M. Solitary fibrous tumor of the parotid gland extending to the parapharyngeal space. Eur Arch Otorhinolaryngol 1998; 255:18e21. [6] Rakheja D, Wilson KS, Meehan JJ, Schultz RA, Maale GE, Timmons CF. Extrapleural benign solitary fibrous tumor in the shoulder of a 9-year-old girl: case report and review of the literature. Pediatr Dev Pathol 2004;7: 653e60. [7] Cox DP, Daniels T, Jordan RC. Solitary fibrous tumor of the head and neck. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110: 79e84. [8] Fletcher CDM, Bridge JA, Hogendoorn P, editors. WHO Classification of Tumours of Soft Tissue and Bone. Lyon: IARC; 2013. lu E, Cansiz H, Mercan H, Dervis¸og lu S. Head and neck heman[9] Aciog giopericytomas: diagnostic contradictions. J Craniofac Surg 2009;20: 930e5.