- Email: [email protected]
Chondromyxoid fibroma of the inferior turbinate: A case report
Otolaryngology Case Reports 11 (2019) 100118
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.elsevier.com/locate/xocr
Chondromyxoid fibroma of the inferior turbinate: A case report a,∗
a
b
c
Jie Wang , Jing Zhu , Ming-xia Huang , An Lu a b c
T
Department of Otolaryngology-Head and Neck Surgery, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China Department of Medical Imaging, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China Department of Pathology, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China
Keywords: Chondromyxoid fibroma Inferior turbinate Head and neck neoplasm
ABSTRACT
Chondromyxoid fibroma (CMF) is a rare bone tumor, accounting for less than 0.5% of all bone tumors. This is a case report of a CMF in the inferior turbinate of a 2 years old girl with nearly 1.5 years history of congestion. Endoscope found a tumor completely obstructing nasal cavities from inferior turbinate. The patient underwent a partial turbinectomy and CMF was shown in Pathology. No recurrence or new lesion was observed during the 4 years of follow up. There is no need to remove too much of the nasal sinus bone to avoid facial deformities.
1. Introduction Chondromyxoid fibroma (CMF) is a rare cartilaginous tumor that usually arises from the lower extremity long bone metaphyses, constituting around 0.5% of all bone tumors [1]. It was first described by Jaffe and Lichtenstein in 1948 as a tumor commonly mistaken for chondrosarcoma [2]. CMF is characterized by incomplete cartilaginous differentiation with chondroid, fibrous, and myxoid areas in varying proportions [3]. The majority of cases occur in the second and third decades of life, with approximately 75% of cases occurring before the age of 30, and there are no significant sex predilections [4]. Its usual presentation is in the proximal tibia and the distal femur [5,6]. Approximately 5.4% of all CMF presents in the skull and facial bones [7]. Normally, these tumors predominantly present in the mandible and maxilla [8]. To date, only 1 case of CMF arising from the inferior turbinate have been reported [9]. 2. Case report A patient, who is a two years old girl, had endured persistent nasal congestion for 1.5 years, with the simultaneous phenomenon such as snoring with no nasal bleeding and tearing in the left eye when breathing. Due to nasal congestion, she went to another hospital, but had no examination or medicine, then her symptoms continued to enhance. Moreover, her parents are good in health, and especially her mother was healthy during pregnancy. The patient was born through cesarean delivery at 42weeks gestation with no identifiable perinatal
problems. On endoscopic examination, the tumor were completely obstructing nasal cavities, and the inferior turbinate could not be delineated, with nasal septum deviation (Fig. 1). CT scans showed soft tissue mass with clear border in the left nasal cavity shifted the nasal septum to the right under pressure. The bone cortex of the medial wall of the ipsilateral maxillary sinus became thinner, with the sinus cavity smaller and effusion found (Fig. 2). MRI showed a 2.8 × 2.4 × 3.5 cm well-defined soft tissue mass with clear border in the left nasal cavity, the nasal septum pressed to the right, and the sinus effusion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images (Fig. 3). Under general anesthesia, 2% lidocaine and 1:1000 epinephrine were used. During surgery, it's found that the tumor came from the inferior turbinate, so a partial turbinectomy was performed with a plasma knife on the left side. The inner part of the mass consisted of gelatinous material (Fig. 4). Histologic sections showed the tumor was lobulated and the lobular central cells are sparse. The center of the lobule is a light blue mucoid matrix with few cell components. There are abundant cells around the lobule, including multinucleated giant cells, fibroblasts and chondroblasts (Fig. 5). The patient had no signs of recurrent disease. Due to nasosinusitis, nose endoscope and CT examination were done 4 years after tumor operation, and the patient had no signs of recurrent (Fig. 6). 3. Discussion There have been 21 reported cases of sinonasal CMF presenting in
Abbreviations:CMF, Chondromyxoid fibroma ∗ Corresponding author. Department of Otolaryngology-Head and Neck Surgery, The Affiliated Children Hospital of Xi'an Jiao Tong University. 17 Western Ju-yuan Road, Xi'an, Shaanxi Province, 710032, China. E-mail address: [email protected] (J. Wang). https://doi.org/10.1016/j.xocr.2019.100118 Received 13 November 2018; Received in revised form 2 March 2019; Accepted 16 April 2019 Available online 19 April 2019 2468-5488/ © 2019 Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
the nasal bones and paranasal sinuses [9,10]. Symptoms of CMF vary depending on the location and the size of the tumor, which may include diplopia, facial pain, exophthalmos, neuralgia, dysarthria, epistaxis, nasal congestion, headache, bony swelling, or persistent chronic rhinosinusitis symptoms despite appropriate medical management [11]. CMF should be distinguished from highly vascular tumors and chondrosarcoma in the head and neck. The typical MRI presentation of CMF shows low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images, The signal is often heterogeneous in relation to the different tissue components [12]. Under microscope, typical CMF shows clear border or confertus lobular lesions. Loose mucous matrix and stellate long-thin fusiform cells are found in the center of the lobular lesions, of which cells at the margin are dense. Multinuclear osteoclast-like giant cells, hemosiderin, and chondrocytes are found between the lobular lesions [13], which is separated by thin-layer fibrous vascular bundles. Frank hyaline cartilage center is rarely seen, and only 19% of patients with CMF have hyaline cartilage [14]. It is reported that the recurrence rate is 11.5% and malignant transformation rate is 0.7% [7]. If the tumor is not completely excised,
Fig. 1. The tumor were completely obstructing nasal cavities, and the inferior turbinate could not be delineated, with nasal septum deviation.
Fig. 2. The tissue mass with clear border in the left nasal cavity shifted the nasal septum to the right under pressure. The bone cortex of the medial wall of the ipsilateral maxillary sinus became thinner, the sinus cavity became smaller, with the sinus cavity smaller and effusion found.
Fig. 3. MRI showed a 2.8 × 2.4 × 3.5 cm well-defined soft tissue mass with clear border in the left nasal cavity, the nasal septum pressed to the right, and the sinus effusion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
2
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
there is a potential to have local recurrence. Some doctors choose extended resection to prevent recurrence. The radiation therapy [15,16] and cryotherapy [17] are used in the recurrences or primary tumors that are surgically difficult to reach. In the case of this report, CMF is located on the face, and no recurrence was found during the 4 years follow-up. 4. Conclusion CMF is a rare, benign neoplasm occasionally occurring in the head and neck. There is no need to remove too much of the nasal sinus bone to avoid facial deformities. Funding This study was supported by the National Natural Science Foundation of China (No. 81670925), Shaanxi Health Research Fund (No. 2018D006), Xi'an Health and Family Planning Commission Fund (No. J201902034).
Fig. 4. During surgery, it's found that the tumor came from the inferior turbinate, and the inner part of the mass consisted of gelatinous material.
Fig. 5. The tumor is lobulated and the lobular central cells are sparse. The center of the lobule is a light blue mucoid matrix with few cell components. There are abundant cells around the lobule, including multinucleated giant cells, fibroblasts and chondroblasts.
Fig. 6. Endoscope showed nasal mucosa is congested and edema, and purulent secretions are seen, but no new organisms are found. CT showed the tumor resection complete.
3
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
Conflict of interest disclosures
[7] Wu CT, Inwards CY, O'Laughlin S, Rock MG, Beabout JM, et al. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol 1998;29(5):438–46. [8] Hammad HM, Hammond HL, Kueago ZB, Frank JA. Chondromyxoid fibroma of the jaws. Case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85(3):293–300. [9] Yoo YT, Park JH, Sunwoo WS, Rhee CS. A huge chondromyxoid fibroma of the nasal cavity in a newborn baby. Auris Nasus Larynx 2012;39(4):422–4. [10] Mcclurg SW, Marino Leon MD, Teknos TN, OH Lwenofu. Chondromyxoid fibroma of the nasal septum: case report and review of literature. Head Neck J Sci Spec Head Neck 2013;35(1):E1–5. [11] Wang C, Morrow T, Friedman P, Lara JF. Chondromyxoid fibroma of the nasal septum: a case report emphasizing clinical correlation. Am J Rhinol 2000;14(1):45–9. [12] Mitchell M, Sartoris DJ, Resnick D. Magnetic resonance imaging of chondromyxoid fibroma of the third metatarsal. J Foot Surg 1990;29(6):613. [13] Fomete B, Adeosun OO, DI Awelimobor, Olayemi L. Chondromyxoid fibroma of the mandible: case report and review of the literature. Ann Maxillofac Surg 2014-0523;4(1):78–80. 2014. 4(1). [14] Sudhakara M, Bavle RM, Srinath N, Paremala K. Chondromyxoid fibroma of zygoma: a rare case report. J Oral Maxillofac Pathol Jomfp 2014;18(1):93–6. [15] Baujat B, Attal P, Racy E, Quilard J, Parker F, et al. Chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses: report of one case and a review of the literature. Am J Otolaryngol 2001;22(2):150–3. [16] Keel SB, Bhan AK, Liebsch NJ, Rosenberg AE. Chondromyxoid fibroma of the skull base: a tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. Am J Surg Pathol 1997;21(5):577. [17] Zhu Zhi-chao, Yi-fei Yang, Xu Yang, Liu Yan, Cheng Yi-nan, et al. Treatment of cryotherapy and orthotopic transplantation following chondromyxoid fibroma of zygomatic bone: a case report. Medicine 2018;97(31):e11707.
No conflicts of interest exist for the authors in this study. Acknowledgements No acknowledgements. References [1] Siddiqui B, Faridi S, Faizan M, Sherwani RK, et al. Cytodiagnosis of ChondromyxoidFibroma of the metatarsal head: a case report. Iran J Plant Pathol 2016;11(3):272. [2] Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol 1948;45(4):541–51. [3] Armah HB, Mcgough RL, Goodman MA, Gollin SM, Surti U, et al. Chondromyxoid fibroma of rib with a novel chromosomal translocation: a report of four additional cases at unusual sites. Diagn Pathol 2007;2(1). p. 44-44. [4] Xu H, Qin Z, Shi Z. Chondromyxoid fibroma in the sella turcica region. J Clin Neurosci 2011;18(10):1419–21. [5] Damm DD, White Jr. DK, GR Drummond JF, Gonty AA. Chondromyxoid fibroma of the maxilla. Electron microscopic findings and review of the literature. Oral Surg Oral Med Oral Pathol 1985;59(2):176–83. [6] Nazeer T, Ro JY, Varma DG, Hermosa JRDL, Ayala AG. Chondromyxoid fibroma of paranasal sinuses: report of two cases presenting with nasal obstruction. Skeletal Radiol 1996;25(8):779–82.
4
Contents lists available at ScienceDirect
Otolaryngology Case Reports journal homepage: www.elsevier.com/locate/xocr
Chondromyxoid fibroma of the inferior turbinate: A case report a,∗
a
b
c
Jie Wang , Jing Zhu , Ming-xia Huang , An Lu a b c
T
Department of Otolaryngology-Head and Neck Surgery, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China Department of Medical Imaging, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China Department of Pathology, The Affiliated Children Hospital of Xi'an Jiao Tong University, Xi'an, Shaanxi, 710003, PR China
Keywords: Chondromyxoid fibroma Inferior turbinate Head and neck neoplasm
ABSTRACT
Chondromyxoid fibroma (CMF) is a rare bone tumor, accounting for less than 0.5% of all bone tumors. This is a case report of a CMF in the inferior turbinate of a 2 years old girl with nearly 1.5 years history of congestion. Endoscope found a tumor completely obstructing nasal cavities from inferior turbinate. The patient underwent a partial turbinectomy and CMF was shown in Pathology. No recurrence or new lesion was observed during the 4 years of follow up. There is no need to remove too much of the nasal sinus bone to avoid facial deformities.
1. Introduction Chondromyxoid fibroma (CMF) is a rare cartilaginous tumor that usually arises from the lower extremity long bone metaphyses, constituting around 0.5% of all bone tumors [1]. It was first described by Jaffe and Lichtenstein in 1948 as a tumor commonly mistaken for chondrosarcoma [2]. CMF is characterized by incomplete cartilaginous differentiation with chondroid, fibrous, and myxoid areas in varying proportions [3]. The majority of cases occur in the second and third decades of life, with approximately 75% of cases occurring before the age of 30, and there are no significant sex predilections [4]. Its usual presentation is in the proximal tibia and the distal femur [5,6]. Approximately 5.4% of all CMF presents in the skull and facial bones [7]. Normally, these tumors predominantly present in the mandible and maxilla [8]. To date, only 1 case of CMF arising from the inferior turbinate have been reported [9]. 2. Case report A patient, who is a two years old girl, had endured persistent nasal congestion for 1.5 years, with the simultaneous phenomenon such as snoring with no nasal bleeding and tearing in the left eye when breathing. Due to nasal congestion, she went to another hospital, but had no examination or medicine, then her symptoms continued to enhance. Moreover, her parents are good in health, and especially her mother was healthy during pregnancy. The patient was born through cesarean delivery at 42weeks gestation with no identifiable perinatal
problems. On endoscopic examination, the tumor were completely obstructing nasal cavities, and the inferior turbinate could not be delineated, with nasal septum deviation (Fig. 1). CT scans showed soft tissue mass with clear border in the left nasal cavity shifted the nasal septum to the right under pressure. The bone cortex of the medial wall of the ipsilateral maxillary sinus became thinner, with the sinus cavity smaller and effusion found (Fig. 2). MRI showed a 2.8 × 2.4 × 3.5 cm well-defined soft tissue mass with clear border in the left nasal cavity, the nasal septum pressed to the right, and the sinus effusion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images (Fig. 3). Under general anesthesia, 2% lidocaine and 1:1000 epinephrine were used. During surgery, it's found that the tumor came from the inferior turbinate, so a partial turbinectomy was performed with a plasma knife on the left side. The inner part of the mass consisted of gelatinous material (Fig. 4). Histologic sections showed the tumor was lobulated and the lobular central cells are sparse. The center of the lobule is a light blue mucoid matrix with few cell components. There are abundant cells around the lobule, including multinucleated giant cells, fibroblasts and chondroblasts (Fig. 5). The patient had no signs of recurrent disease. Due to nasosinusitis, nose endoscope and CT examination were done 4 years after tumor operation, and the patient had no signs of recurrent (Fig. 6). 3. Discussion There have been 21 reported cases of sinonasal CMF presenting in
Abbreviations:CMF, Chondromyxoid fibroma ∗ Corresponding author. Department of Otolaryngology-Head and Neck Surgery, The Affiliated Children Hospital of Xi'an Jiao Tong University. 17 Western Ju-yuan Road, Xi'an, Shaanxi Province, 710032, China. E-mail address: [email protected] (J. Wang). https://doi.org/10.1016/j.xocr.2019.100118 Received 13 November 2018; Received in revised form 2 March 2019; Accepted 16 April 2019 Available online 19 April 2019 2468-5488/ © 2019 Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
the nasal bones and paranasal sinuses [9,10]. Symptoms of CMF vary depending on the location and the size of the tumor, which may include diplopia, facial pain, exophthalmos, neuralgia, dysarthria, epistaxis, nasal congestion, headache, bony swelling, or persistent chronic rhinosinusitis symptoms despite appropriate medical management [11]. CMF should be distinguished from highly vascular tumors and chondrosarcoma in the head and neck. The typical MRI presentation of CMF shows low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images, The signal is often heterogeneous in relation to the different tissue components [12]. Under microscope, typical CMF shows clear border or confertus lobular lesions. Loose mucous matrix and stellate long-thin fusiform cells are found in the center of the lobular lesions, of which cells at the margin are dense. Multinuclear osteoclast-like giant cells, hemosiderin, and chondrocytes are found between the lobular lesions [13], which is separated by thin-layer fibrous vascular bundles. Frank hyaline cartilage center is rarely seen, and only 19% of patients with CMF have hyaline cartilage [14]. It is reported that the recurrence rate is 11.5% and malignant transformation rate is 0.7% [7]. If the tumor is not completely excised,
Fig. 1. The tumor were completely obstructing nasal cavities, and the inferior turbinate could not be delineated, with nasal septum deviation.
Fig. 2. The tissue mass with clear border in the left nasal cavity shifted the nasal septum to the right under pressure. The bone cortex of the medial wall of the ipsilateral maxillary sinus became thinner, the sinus cavity became smaller, with the sinus cavity smaller and effusion found.
Fig. 3. MRI showed a 2.8 × 2.4 × 3.5 cm well-defined soft tissue mass with clear border in the left nasal cavity, the nasal septum pressed to the right, and the sinus effusion showed low signal intensity on T1-weighted images and high signal intensity on T2-weighted images.
2
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
there is a potential to have local recurrence. Some doctors choose extended resection to prevent recurrence. The radiation therapy [15,16] and cryotherapy [17] are used in the recurrences or primary tumors that are surgically difficult to reach. In the case of this report, CMF is located on the face, and no recurrence was found during the 4 years follow-up. 4. Conclusion CMF is a rare, benign neoplasm occasionally occurring in the head and neck. There is no need to remove too much of the nasal sinus bone to avoid facial deformities. Funding This study was supported by the National Natural Science Foundation of China (No. 81670925), Shaanxi Health Research Fund (No. 2018D006), Xi'an Health and Family Planning Commission Fund (No. J201902034).
Fig. 4. During surgery, it's found that the tumor came from the inferior turbinate, and the inner part of the mass consisted of gelatinous material.
Fig. 5. The tumor is lobulated and the lobular central cells are sparse. The center of the lobule is a light blue mucoid matrix with few cell components. There are abundant cells around the lobule, including multinucleated giant cells, fibroblasts and chondroblasts.
Fig. 6. Endoscope showed nasal mucosa is congested and edema, and purulent secretions are seen, but no new organisms are found. CT showed the tumor resection complete.
3
Otolaryngology Case Reports 11 (2019) 100118
J. Wang, et al.
Conflict of interest disclosures
[7] Wu CT, Inwards CY, O'Laughlin S, Rock MG, Beabout JM, et al. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol 1998;29(5):438–46. [8] Hammad HM, Hammond HL, Kueago ZB, Frank JA. Chondromyxoid fibroma of the jaws. Case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85(3):293–300. [9] Yoo YT, Park JH, Sunwoo WS, Rhee CS. A huge chondromyxoid fibroma of the nasal cavity in a newborn baby. Auris Nasus Larynx 2012;39(4):422–4. [10] Mcclurg SW, Marino Leon MD, Teknos TN, OH Lwenofu. Chondromyxoid fibroma of the nasal septum: case report and review of literature. Head Neck J Sci Spec Head Neck 2013;35(1):E1–5. [11] Wang C, Morrow T, Friedman P, Lara JF. Chondromyxoid fibroma of the nasal septum: a case report emphasizing clinical correlation. Am J Rhinol 2000;14(1):45–9. [12] Mitchell M, Sartoris DJ, Resnick D. Magnetic resonance imaging of chondromyxoid fibroma of the third metatarsal. J Foot Surg 1990;29(6):613. [13] Fomete B, Adeosun OO, DI Awelimobor, Olayemi L. Chondromyxoid fibroma of the mandible: case report and review of the literature. Ann Maxillofac Surg 2014-0523;4(1):78–80. 2014. 4(1). [14] Sudhakara M, Bavle RM, Srinath N, Paremala K. Chondromyxoid fibroma of zygoma: a rare case report. J Oral Maxillofac Pathol Jomfp 2014;18(1):93–6. [15] Baujat B, Attal P, Racy E, Quilard J, Parker F, et al. Chondromyxoid fibroma of the nasal bone with extension into the frontal and ethmoidal sinuses: report of one case and a review of the literature. Am J Otolaryngol 2001;22(2):150–3. [16] Keel SB, Bhan AK, Liebsch NJ, Rosenberg AE. Chondromyxoid fibroma of the skull base: a tumor which may be confused with chordoma and chondrosarcoma. A report of three cases and review of the literature. Am J Surg Pathol 1997;21(5):577. [17] Zhu Zhi-chao, Yi-fei Yang, Xu Yang, Liu Yan, Cheng Yi-nan, et al. Treatment of cryotherapy and orthotopic transplantation following chondromyxoid fibroma of zygomatic bone: a case report. Medicine 2018;97(31):e11707.
No conflicts of interest exist for the authors in this study. Acknowledgements No acknowledgements. References [1] Siddiqui B, Faridi S, Faizan M, Sherwani RK, et al. Cytodiagnosis of ChondromyxoidFibroma of the metatarsal head: a case report. Iran J Plant Pathol 2016;11(3):272. [2] Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol 1948;45(4):541–51. [3] Armah HB, Mcgough RL, Goodman MA, Gollin SM, Surti U, et al. Chondromyxoid fibroma of rib with a novel chromosomal translocation: a report of four additional cases at unusual sites. Diagn Pathol 2007;2(1). p. 44-44. [4] Xu H, Qin Z, Shi Z. Chondromyxoid fibroma in the sella turcica region. J Clin Neurosci 2011;18(10):1419–21. [5] Damm DD, White Jr. DK, GR Drummond JF, Gonty AA. Chondromyxoid fibroma of the maxilla. Electron microscopic findings and review of the literature. Oral Surg Oral Med Oral Pathol 1985;59(2):176–83. [6] Nazeer T, Ro JY, Varma DG, Hermosa JRDL, Ayala AG. Chondromyxoid fibroma of paranasal sinuses: report of two cases presenting with nasal obstruction. Skeletal Radiol 1996;25(8):779–82.
4