- Email: [email protected]
Dedifferentiated chondrosarcoma of the maxilla. Report of a rare tumor in an extremely rare localization
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case Report
Dedifferentiated chondrosarcoma of the maxilla. Report of a rare tumor in an extremely rare localization Alireza Khooeia, Shahriar Mohammadreza Sharifianb, Mahdi Karimi Shahric, ⁎ Shirin Taraz Taraz Jamshidia, , Nooshin Sedaghat Sharifid a
Department of Pathology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Department of Otorhinolaryngology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran c Department of Pathology, Faculty of Medicine, Gonabad University of Medical Sciences, Gonabad, Iran d Mashhad University of Medical Sciences, Mashhad, Iran b
ARTICLE INFO
ABSTRACT
Keywords: Dedifferentiation Chondrosarcoma Maxilla Immunohistochemistry Recurrence
Dedifferentiated chondrosarcoma is a rare primary bone neoplasm and it is extremely rare in paranasal sinuses and skull bones in particular. Herein we describe a case of dedifferentiated chondrosarcoma of the maxilla extending to the orbit in a 51 year-old man.
1. Introduction Dedifferentiated chondrosarcoma is a rare neoplasm that mainly arises in the long bones and pelvis. It is referred to the juxtaposition of a poorly differentiated highly malignant sarcoma component to an otherwise typical low grade Chondrosarcoma [1]. The development of this dedifferentiated component is accompanied by an aggressive clinical course and poor prognosis. The overall outcome is mainly dependent to the early diagnosis and wide surgical resection with clear margins [2]. Therefore recognition of the dedifferentiation in the early stages of the disease is of a great value. The presentation of dedifferentiated chondrosarcoma in the skull bones is extremely rare and to our knowledge only five cases of maxillary bone involvement have been reported to date [3–5], including two cases of radiation-induced dedifferentiated chondrosarcoma of orbit and maxillary sinus [6]. Herein we present another case of maxillary dedifferentiated chondrosarcoma. 2. Case report A 51 year old man was admitted to our center with a large bulging of 8 cm diameter in his right cheek which had compressed and displaced the ocular globe laterally and posteriorly. The chief complains included pain, dysphagia, odynophagia and weight loss. Examination of
⁎
the mouth revealed a big mass with white necrotic surface in the right palate extending to the palatopharyngeal fold. The mass was also visible in the right nose deviating the nasal septum to the left. Computed tomography (CT) scan demonstrated a destructive lobulated soft tissue mass in the right maxilla and nasal cavity eroding the remaining ethmoidal air cells and even sphenoid sinuses and protruding to the floor and posterior regions of the right orbit. There were small areas compatible with chondroid tissues and foci of ossification in the tumor as well. Magnetic resonance imaging (MRI) showed close correlation to the CT scan findings (Fig. 1a–d). Brain MRI and chest X ray did not show any sign of metastasis. The patient had been first presented in another center two years ago with a hard palate mass originating from the lower part of the maxillary sinus which had been diagnosed as grade I chondrosarcoma. Since then and before this last recrudescence four recurrences had been taken place in the same localization with later involvement of the inferior concha, retropharyngeal and lateral pharyngeal tissues, temporal and ethmoidal bones all diagnosed as maxillary low grade chondrosarcoma without any dedifferentiated component. He had also gone through a course of chemotherapy and radiotherapy before the current presentation. The patient underwent a wide surgical resection including right sided enucleation. The resected specimen included the right periorbital skin and soft tissue, right globe and the bulk of the tumor that was a 8*7*6.5 cm,
Corresponding author at: Department of Pathology, Imam Reza Hospital, Ibn-e sina avenue, Mashhad, Iran. E-mail address: [email protected] (S.T. Taraz Jamshidi).
https://doi.org/10.1016/j.ajoms.2019.09.002 Received 7 February 2019; Received in revised form 22 August 2019; Accepted 10 September 2019 2212-5558/ © 2019 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd All rights reserved.
Please cite this article as: Alireza Khooei, et al., Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology, https://doi.org/10.1016/j.ajoms.2019.09.002
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
A. Khooei, et al.
Fig. 1. a : Axial CT scan with IV contrast showing huge solid mass with necrosis and calcification. b-d: Axial and coronal MRI without contrast demonstrating the tumor with foci of signal void (calcification) with extension to the frontal sinus, nasal cavities and the right orbit.
Fig. 2. Transition area between differentiated (right) and dedifferentiated (left) components of chondrosarcoma.
lobulated, fleshy mass with a necrotic surface located in the inferior part of the specimen. On sectioning it was a variegated yellowish white to tan mass with areas of whorling pattern and small residual chondroid foci. Microscopic examination revealed a highly cellular severely atypical spindle cell sarcoma in most parts of the tumor adjacent to some well-differentiated hyaline cartilage lobules with mild to moderate cytonuclear atypia mainly in the deep portions (Fig. 2, 3a, b). The anaplastic component showed various patterns of growth while some areas had fascicular appearance, obvious herringbone features and hemangipericytoma like pattern were visible in other areas (Fig. 3c, d). There were also a few scattered foci of osteoid formation (Fig. 3e).Numerous typical and atypical mitotic figures were readily identifiable. The tumor had invaded the dermis and the nearby fibromuscular tissues while the eyeball did not show tumoral involvement. Immunohistochemical studies showed reactivity for vimentin on both chondromatous and nonchondromatous components while
cytokeratin and desmin had negative reactions. S 100 protein was positive in some scattered spindle cells in the high grade portions in addition to the chondroid tissues. Both components stained focally with P53 marker (Fig. 3f). 3. Discussion Dedifferentiated chondrosarcoma was first described by Dahlin and Beabout in 1971 as a combination of well differentiated chondrosarcoma of conventional type usually in the central position and zones of anaplastic fibrosarcoma or osteosarcoma at the periphery [1]. It represents approximately 11% of all chondrosarcomas arising more commonly in the femur and pelvis of the adult patients with an average age between 50 to 60 years [7,8]. Occurrence of chondrosarcoma in the bones of nose or paranasal sinuses is very unusual. Our case presented as a hard palate mass originating from the inferior wall of the right
2
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
A. Khooei, et al.
Fig. 3. a: Grade I chondrosarcoma component with lobulated pattern. b: The predominant feature of the tumor showing a highly atypical spindle cell sarcoma with high mitotic activity. c: Foci with hemangiopericytoma pattern. d: Areas with herringbone pattern of fibrsarcoma. e: Osteoid formation in the osteosarcoma like foci of the tumor. f: Immunohistochemical study with peroxidase showing focal and scattered positive reactions with P53 (right) and S-100 protein (left) in the high grade component of the tumor.
with few scattered foci of osteoid production. According to its morphologic appearance the nonchondroid portion of the dedifferentiated chondrosarcoma could be immunoreactive for a variety of markers such as alpha 1-Antitrypsin alpha 1Antichimotrypsin, lysozyme, smooth muscle myosin, myglobin and desmin, while S-100 protein reactivity have rarely been observed in some cells of the anaplastic component in addition to the chondroid cells [11]. However the diagnosis is mainly made morphologically and the role of the immunohistochemistry study is to help identifying the nature of the less differentiated parts. In the present case vimentin showed diffuse positive immunoreactivity while some cells of the high grade noncartilaginous component stained with S100 protein as well. Cytokeratin and desmin showed negative reactions. There are cytogenetic evidences showing the two components of the dedifferentiated chondrosarcomas arise from a common primitive mesenchymal clone indicating the term "dedifferentiated" may be not a precise name [9]. There is a loss in P16 expression as well as overexpression of cyclin D1, RB and p53 in dedifferentiated component [12]. At the molecular level mutation of P53 and H-ras mutations may be responsible for the anaplastic transformation [13]. We studied P53 mutation immunohistochemically which showed focal and scattered positive reactions in the anaplastic component. While local control of the tumor by wide surgical resection is the treatment of choice there is a high rate of distant metastasis [2]. The overall prognosis is uniformly poor with a 5 year survival below 25% and there is no convincing evidence of considerable advantages from adjuvant chemotherapy though the available data are very limited [2,14]. In the current case all previous operations and chemoradiotherapy had failed to control the disease. We did not discover distant metastasis in our imaging studies. Dedifferentiation occurred at the third recurrence. The patient was managed by surgical excision including right side enucleation and he is receiving palliative therapy at present.
maxilla in a 51-year old male with secondary involvement of the adjacent nasal cavity and paranasal sinuses soft tissues and the orbit while the dedifferentiated portion was detected only in the last recurrence. To the best of our knowledge, five cases of dedifferentiated chondrosarcoma with maxillary involvement have been reported in the literature to date, including two radiation induced ones (Table 1) [3–6]. Similar to other bone tumors, pain and swelling are the most common symptoms and many cases are diagnosed following a pathologic fracture [9]. In the reported maxillary dedifferentiated chondrosarcomas pain facial swelling epistaxis and proptosis have been the initial symptoms [3–5]. Our patient presented with a hard palate mass following by several recurrences during a nearly 2-year duration suffering from pain, weight loss and finally a remarkable mass in his cheek and infraorbital area with secondary deformity of his globe. The typical findings in imaging studies that may provide a clue to diagnosis include a tumor with bimorphic pattern as a lytic area adjacent to a mineralizes mass on roentgenography or juxtaposing of a cartilaginous mass with a destructive unmineralized soft tissue component [7]. CT scan and MRI studies of the current case also showed a destructive soft tissue mass with calcified foci compatible with chondorid components. Microscopically the cartilaginous part may appear benign or malignant mostly of grade I or grade II. The dedifferentiated areas may be discovered in the primary tumor however more frequently it is detected in the recurrences. The histological features of the latter component could be of malignant fibrohistiocytoma, fibrosarcoma, osteosarcoma, rhabdomyosarcoma, or sarcoma with the appearance of conventional giant cell tumor [10]. The cartilaginous component of the present case had the appearance of both grade I and grade II chondrosarcoma. We also reviewed all the previous slides and pathology reports of the patient that confirmed grade I and grade II conventional chondrosarcoma. The dedifferentiated portion which was found only in the last recurrence had the histological features of a high grade pleomorphic sarcoma
3
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
Declaration of Competing Interest None. Acknowledgement None. References
Left Maxillary sinus and nasal cavity maxilla
7 -
no -
Caldwell Luc Radical surgery with maxillofacial prosthetic rehabilitation
[1] Dahlin DC, Beabout JW. Dedifferentiation of low‐grade chondrosarcomas. Cancer 1971;28(2):461–6. [2] Grimer RJ, Gosheger G, Taminiau A, Biau D, Matejovsky Z, Kollender Y, et al. Dedifferentiated chondrosarcoma: prognostic factors and outcome from a European group. Eur J Cancer (Oxf Eng: 1990) 2007;43(September (14)):2060–5. PubMed PMID: 17720491. Epub 2007/08/28. eng. [3] Munshi A, Atri SK, Pandey KC, Sharma MC. Dedifferentiated chondrosarcoma of the maxilla. J Cancer Res Ther 2007;3(January–March (1)):53–5. PubMed PMID: 17998723. Epub 2007/11/14. eng. [4] Paume P, Grippari JL, Payement G, Dandrau JP, Cariou JL, Bellavoir A. Dedifferentiated chondrosarcoma with maxillary involvement. An anatomo-clinical study, surgical treatment and prosthetic rehabilitation. Apropos of a case. Rev Stomatol Chir Maxillofac 1993;94(2):104–9. PubMed PMID: 8389488. Epub 1993/ 01/01. Chondrosarcome dedifferencie a localisation maxillaire. Etude anatomoclinique, traitement chirurgical et rehabilitation prothetique. A propos d’un cas. fre. [5] Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LDR. Primary chondrosarcoma of the head and neck in pediatric patients. Cancer 2000;88(9):2181–8. [6] Davies BW, Prescott CR, Said SA, Campana J, Attie-Castro FA, Velasco ECAA, et al. Radiation-induced dedifferentiated chondrosarcoma with orbital invasion. Ophthal Plast Reconstr Surg 2014;30(May–June (3)):205–8. PubMed PMID: 24807535. Epub 2014/05/09. eng. [7] Littrell LA, Wenger DE, Wold LE, Bertoni F, Unni KK, White LM, et al. Radiographic, CT, and MR imaging features of dedifferentiated chondrosarcomas: a retrospective review of 174 de novo cases. RadioGraphics 2004;24(5):1397–409. PubMed PMID: 15371616. [8] Liu C, Xi Y, Li M, Jiao Q, Zhang H, Yang Q, et al. Dedifferentiated chondrosarcoma: radiological features, prognostic factors and survival statistics in 23 patients. PLoS One 2017;12(3). e0173665. [9] Aigner T, Unni KK. Is dedifferentiated chondrosarcoma a’ de-differentiated’ chondrosarcoma? J Pathol 1999;189(December (4)):445–7. PubMed PMID: 10629541. Epub 2000/01/12. eng. [10] Staals EL, Bacchini P, Bertoni F. Dedifferentiated central chondrosarcoma. Cancer 2006;106(12):2682–91. [11] Siegal G, Mills S. Dedifferentiated chondrosarcoma of bone. An immunohistochemical and lectin histochemicai study. Virchows Arch 1987;411(1):23. [12] Franchi A, Baroni G, Sardi I, Giunti L, Capanna R, Campanacci D. Dedifferentiated peripheral chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of four cases. Virchows Arch 2012;460(March (3)):335–42. [13] Sakamoto A, Oda Y, Adachi T, Oshiro Y, Tamiya S, Tanaka K, et al. H-ras oncogene mutation in dedifferentiated chondrosarcoma: polymerase chain reaction-restriction fragment length polymorphism analysis. Mod Pathol 2001;14(April (4)):343–9. PubMed PMID: 11301351. Epub 2001/04/13. eng. [14] Staals EL, Bacchini P, Bertoni. Dedifferentiated central chondrosarcoma. Cancer 2006;106(June (12)):2682–91. PubMed PMID: 16691621. Epub 2006/05/13. eng.
17 19 4 5
male female
20 3
male
2000 1993
2007
No recurrence six months after the treatment. alive, no evidence of disease after 6 years 12 × 9 × 5
no
radical maxillectomy
2014 2014 passed away from disease, 2 years later passed away from disease subtotal maxillectomy and sphenoidectomy wide excision yes yes 6.5 × 5.2 -
Left Maxillary sinus and nasal cavity ethmoid, sphenoid, and maxillary sinuses right maxillary sinus 36 43 1 2
female male
location
A written informed consent was obtained.
patient’s age(years)
gender
Ethical approval
Case no.
Table 1 Reported cases of dedifferentiated chondrosarcoma in maxillary region.
tumor size (cm)
Radiation history
operation
Fallow up
Publication year
A. Khooei, et al.
4
Contents lists available at ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case Report
Dedifferentiated chondrosarcoma of the maxilla. Report of a rare tumor in an extremely rare localization Alireza Khooeia, Shahriar Mohammadreza Sharifianb, Mahdi Karimi Shahric, ⁎ Shirin Taraz Taraz Jamshidia, , Nooshin Sedaghat Sharifid a
Department of Pathology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran Department of Otorhinolaryngology, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran c Department of Pathology, Faculty of Medicine, Gonabad University of Medical Sciences, Gonabad, Iran d Mashhad University of Medical Sciences, Mashhad, Iran b
ARTICLE INFO
ABSTRACT
Keywords: Dedifferentiation Chondrosarcoma Maxilla Immunohistochemistry Recurrence
Dedifferentiated chondrosarcoma is a rare primary bone neoplasm and it is extremely rare in paranasal sinuses and skull bones in particular. Herein we describe a case of dedifferentiated chondrosarcoma of the maxilla extending to the orbit in a 51 year-old man.
1. Introduction Dedifferentiated chondrosarcoma is a rare neoplasm that mainly arises in the long bones and pelvis. It is referred to the juxtaposition of a poorly differentiated highly malignant sarcoma component to an otherwise typical low grade Chondrosarcoma [1]. The development of this dedifferentiated component is accompanied by an aggressive clinical course and poor prognosis. The overall outcome is mainly dependent to the early diagnosis and wide surgical resection with clear margins [2]. Therefore recognition of the dedifferentiation in the early stages of the disease is of a great value. The presentation of dedifferentiated chondrosarcoma in the skull bones is extremely rare and to our knowledge only five cases of maxillary bone involvement have been reported to date [3–5], including two cases of radiation-induced dedifferentiated chondrosarcoma of orbit and maxillary sinus [6]. Herein we present another case of maxillary dedifferentiated chondrosarcoma. 2. Case report A 51 year old man was admitted to our center with a large bulging of 8 cm diameter in his right cheek which had compressed and displaced the ocular globe laterally and posteriorly. The chief complains included pain, dysphagia, odynophagia and weight loss. Examination of
⁎
the mouth revealed a big mass with white necrotic surface in the right palate extending to the palatopharyngeal fold. The mass was also visible in the right nose deviating the nasal septum to the left. Computed tomography (CT) scan demonstrated a destructive lobulated soft tissue mass in the right maxilla and nasal cavity eroding the remaining ethmoidal air cells and even sphenoid sinuses and protruding to the floor and posterior regions of the right orbit. There were small areas compatible with chondroid tissues and foci of ossification in the tumor as well. Magnetic resonance imaging (MRI) showed close correlation to the CT scan findings (Fig. 1a–d). Brain MRI and chest X ray did not show any sign of metastasis. The patient had been first presented in another center two years ago with a hard palate mass originating from the lower part of the maxillary sinus which had been diagnosed as grade I chondrosarcoma. Since then and before this last recrudescence four recurrences had been taken place in the same localization with later involvement of the inferior concha, retropharyngeal and lateral pharyngeal tissues, temporal and ethmoidal bones all diagnosed as maxillary low grade chondrosarcoma without any dedifferentiated component. He had also gone through a course of chemotherapy and radiotherapy before the current presentation. The patient underwent a wide surgical resection including right sided enucleation. The resected specimen included the right periorbital skin and soft tissue, right globe and the bulk of the tumor that was a 8*7*6.5 cm,
Corresponding author at: Department of Pathology, Imam Reza Hospital, Ibn-e sina avenue, Mashhad, Iran. E-mail address: [email protected] (S.T. Taraz Jamshidi).
https://doi.org/10.1016/j.ajoms.2019.09.002 Received 7 February 2019; Received in revised form 22 August 2019; Accepted 10 September 2019 2212-5558/ © 2019 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd All rights reserved.
Please cite this article as: Alireza Khooei, et al., Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology, https://doi.org/10.1016/j.ajoms.2019.09.002
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
A. Khooei, et al.
Fig. 1. a : Axial CT scan with IV contrast showing huge solid mass with necrosis and calcification. b-d: Axial and coronal MRI without contrast demonstrating the tumor with foci of signal void (calcification) with extension to the frontal sinus, nasal cavities and the right orbit.
Fig. 2. Transition area between differentiated (right) and dedifferentiated (left) components of chondrosarcoma.
lobulated, fleshy mass with a necrotic surface located in the inferior part of the specimen. On sectioning it was a variegated yellowish white to tan mass with areas of whorling pattern and small residual chondroid foci. Microscopic examination revealed a highly cellular severely atypical spindle cell sarcoma in most parts of the tumor adjacent to some well-differentiated hyaline cartilage lobules with mild to moderate cytonuclear atypia mainly in the deep portions (Fig. 2, 3a, b). The anaplastic component showed various patterns of growth while some areas had fascicular appearance, obvious herringbone features and hemangipericytoma like pattern were visible in other areas (Fig. 3c, d). There were also a few scattered foci of osteoid formation (Fig. 3e).Numerous typical and atypical mitotic figures were readily identifiable. The tumor had invaded the dermis and the nearby fibromuscular tissues while the eyeball did not show tumoral involvement. Immunohistochemical studies showed reactivity for vimentin on both chondromatous and nonchondromatous components while
cytokeratin and desmin had negative reactions. S 100 protein was positive in some scattered spindle cells in the high grade portions in addition to the chondroid tissues. Both components stained focally with P53 marker (Fig. 3f). 3. Discussion Dedifferentiated chondrosarcoma was first described by Dahlin and Beabout in 1971 as a combination of well differentiated chondrosarcoma of conventional type usually in the central position and zones of anaplastic fibrosarcoma or osteosarcoma at the periphery [1]. It represents approximately 11% of all chondrosarcomas arising more commonly in the femur and pelvis of the adult patients with an average age between 50 to 60 years [7,8]. Occurrence of chondrosarcoma in the bones of nose or paranasal sinuses is very unusual. Our case presented as a hard palate mass originating from the inferior wall of the right
2
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
A. Khooei, et al.
Fig. 3. a: Grade I chondrosarcoma component with lobulated pattern. b: The predominant feature of the tumor showing a highly atypical spindle cell sarcoma with high mitotic activity. c: Foci with hemangiopericytoma pattern. d: Areas with herringbone pattern of fibrsarcoma. e: Osteoid formation in the osteosarcoma like foci of the tumor. f: Immunohistochemical study with peroxidase showing focal and scattered positive reactions with P53 (right) and S-100 protein (left) in the high grade component of the tumor.
with few scattered foci of osteoid production. According to its morphologic appearance the nonchondroid portion of the dedifferentiated chondrosarcoma could be immunoreactive for a variety of markers such as alpha 1-Antitrypsin alpha 1Antichimotrypsin, lysozyme, smooth muscle myosin, myglobin and desmin, while S-100 protein reactivity have rarely been observed in some cells of the anaplastic component in addition to the chondroid cells [11]. However the diagnosis is mainly made morphologically and the role of the immunohistochemistry study is to help identifying the nature of the less differentiated parts. In the present case vimentin showed diffuse positive immunoreactivity while some cells of the high grade noncartilaginous component stained with S100 protein as well. Cytokeratin and desmin showed negative reactions. There are cytogenetic evidences showing the two components of the dedifferentiated chondrosarcomas arise from a common primitive mesenchymal clone indicating the term "dedifferentiated" may be not a precise name [9]. There is a loss in P16 expression as well as overexpression of cyclin D1, RB and p53 in dedifferentiated component [12]. At the molecular level mutation of P53 and H-ras mutations may be responsible for the anaplastic transformation [13]. We studied P53 mutation immunohistochemically which showed focal and scattered positive reactions in the anaplastic component. While local control of the tumor by wide surgical resection is the treatment of choice there is a high rate of distant metastasis [2]. The overall prognosis is uniformly poor with a 5 year survival below 25% and there is no convincing evidence of considerable advantages from adjuvant chemotherapy though the available data are very limited [2,14]. In the current case all previous operations and chemoradiotherapy had failed to control the disease. We did not discover distant metastasis in our imaging studies. Dedifferentiation occurred at the third recurrence. The patient was managed by surgical excision including right side enucleation and he is receiving palliative therapy at present.
maxilla in a 51-year old male with secondary involvement of the adjacent nasal cavity and paranasal sinuses soft tissues and the orbit while the dedifferentiated portion was detected only in the last recurrence. To the best of our knowledge, five cases of dedifferentiated chondrosarcoma with maxillary involvement have been reported in the literature to date, including two radiation induced ones (Table 1) [3–6]. Similar to other bone tumors, pain and swelling are the most common symptoms and many cases are diagnosed following a pathologic fracture [9]. In the reported maxillary dedifferentiated chondrosarcomas pain facial swelling epistaxis and proptosis have been the initial symptoms [3–5]. Our patient presented with a hard palate mass following by several recurrences during a nearly 2-year duration suffering from pain, weight loss and finally a remarkable mass in his cheek and infraorbital area with secondary deformity of his globe. The typical findings in imaging studies that may provide a clue to diagnosis include a tumor with bimorphic pattern as a lytic area adjacent to a mineralizes mass on roentgenography or juxtaposing of a cartilaginous mass with a destructive unmineralized soft tissue component [7]. CT scan and MRI studies of the current case also showed a destructive soft tissue mass with calcified foci compatible with chondorid components. Microscopically the cartilaginous part may appear benign or malignant mostly of grade I or grade II. The dedifferentiated areas may be discovered in the primary tumor however more frequently it is detected in the recurrences. The histological features of the latter component could be of malignant fibrohistiocytoma, fibrosarcoma, osteosarcoma, rhabdomyosarcoma, or sarcoma with the appearance of conventional giant cell tumor [10]. The cartilaginous component of the present case had the appearance of both grade I and grade II chondrosarcoma. We also reviewed all the previous slides and pathology reports of the patient that confirmed grade I and grade II conventional chondrosarcoma. The dedifferentiated portion which was found only in the last recurrence had the histological features of a high grade pleomorphic sarcoma
3
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (xxxx) xxx–xxx
Declaration of Competing Interest None. Acknowledgement None. References
Left Maxillary sinus and nasal cavity maxilla
7 -
no -
Caldwell Luc Radical surgery with maxillofacial prosthetic rehabilitation
[1] Dahlin DC, Beabout JW. Dedifferentiation of low‐grade chondrosarcomas. Cancer 1971;28(2):461–6. [2] Grimer RJ, Gosheger G, Taminiau A, Biau D, Matejovsky Z, Kollender Y, et al. Dedifferentiated chondrosarcoma: prognostic factors and outcome from a European group. Eur J Cancer (Oxf Eng: 1990) 2007;43(September (14)):2060–5. PubMed PMID: 17720491. Epub 2007/08/28. eng. [3] Munshi A, Atri SK, Pandey KC, Sharma MC. Dedifferentiated chondrosarcoma of the maxilla. J Cancer Res Ther 2007;3(January–March (1)):53–5. PubMed PMID: 17998723. Epub 2007/11/14. eng. [4] Paume P, Grippari JL, Payement G, Dandrau JP, Cariou JL, Bellavoir A. Dedifferentiated chondrosarcoma with maxillary involvement. An anatomo-clinical study, surgical treatment and prosthetic rehabilitation. Apropos of a case. Rev Stomatol Chir Maxillofac 1993;94(2):104–9. PubMed PMID: 8389488. Epub 1993/ 01/01. Chondrosarcome dedifferencie a localisation maxillaire. Etude anatomoclinique, traitement chirurgical et rehabilitation prothetique. A propos d’un cas. fre. [5] Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LDR. Primary chondrosarcoma of the head and neck in pediatric patients. Cancer 2000;88(9):2181–8. [6] Davies BW, Prescott CR, Said SA, Campana J, Attie-Castro FA, Velasco ECAA, et al. Radiation-induced dedifferentiated chondrosarcoma with orbital invasion. Ophthal Plast Reconstr Surg 2014;30(May–June (3)):205–8. PubMed PMID: 24807535. Epub 2014/05/09. eng. [7] Littrell LA, Wenger DE, Wold LE, Bertoni F, Unni KK, White LM, et al. Radiographic, CT, and MR imaging features of dedifferentiated chondrosarcomas: a retrospective review of 174 de novo cases. RadioGraphics 2004;24(5):1397–409. PubMed PMID: 15371616. [8] Liu C, Xi Y, Li M, Jiao Q, Zhang H, Yang Q, et al. Dedifferentiated chondrosarcoma: radiological features, prognostic factors and survival statistics in 23 patients. PLoS One 2017;12(3). e0173665. [9] Aigner T, Unni KK. Is dedifferentiated chondrosarcoma a’ de-differentiated’ chondrosarcoma? J Pathol 1999;189(December (4)):445–7. PubMed PMID: 10629541. Epub 2000/01/12. eng. [10] Staals EL, Bacchini P, Bertoni F. Dedifferentiated central chondrosarcoma. Cancer 2006;106(12):2682–91. [11] Siegal G, Mills S. Dedifferentiated chondrosarcoma of bone. An immunohistochemical and lectin histochemicai study. Virchows Arch 1987;411(1):23. [12] Franchi A, Baroni G, Sardi I, Giunti L, Capanna R, Campanacci D. Dedifferentiated peripheral chondrosarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of four cases. Virchows Arch 2012;460(March (3)):335–42. [13] Sakamoto A, Oda Y, Adachi T, Oshiro Y, Tamiya S, Tanaka K, et al. H-ras oncogene mutation in dedifferentiated chondrosarcoma: polymerase chain reaction-restriction fragment length polymorphism analysis. Mod Pathol 2001;14(April (4)):343–9. PubMed PMID: 11301351. Epub 2001/04/13. eng. [14] Staals EL, Bacchini P, Bertoni. Dedifferentiated central chondrosarcoma. Cancer 2006;106(June (12)):2682–91. PubMed PMID: 16691621. Epub 2006/05/13. eng.
17 19 4 5
male female
20 3
male
2000 1993
2007
No recurrence six months after the treatment. alive, no evidence of disease after 6 years 12 × 9 × 5
no
radical maxillectomy
2014 2014 passed away from disease, 2 years later passed away from disease subtotal maxillectomy and sphenoidectomy wide excision yes yes 6.5 × 5.2 -
Left Maxillary sinus and nasal cavity ethmoid, sphenoid, and maxillary sinuses right maxillary sinus 36 43 1 2
female male
location
A written informed consent was obtained.
patient’s age(years)
gender
Ethical approval
Case no.
Table 1 Reported cases of dedifferentiated chondrosarcoma in maxillary region.
tumor size (cm)
Radiation history
operation
Fallow up
Publication year
A. Khooei, et al.
4