CASE REPORT
Jonathan Irish, MD, FRCSC, Section Editor
Enchondroma of the nasal septum due to Ollier disease: A case report and review of the literature Christian M. C. Jacobi, MD,1,3 Egodage Samitha Hiranya, MD,2 Annietta Gay, MD,1 David Holzmann, MD,1 Spyridon Kollias, MD,2 Michael B. Soyka, MD1* 1
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Zurich, Zurich, Switzerland, 2Department of Neuroradiology, University Hospital Zurich, Zurich, Switzerland, 3Medical School of the Technical University Munich, Munich, Germany.
Accepted 28 May 2014 Published online 28 July 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/hed.23783
ABSTRACT: Background. Morbus Ollier is characterized by the presence of multiple enchondromas (ie, benign intraosseous cartilaginous lesions). Although their manifestation in the limb bones is well described, only a few cases with ear, nose, and throat (ENT) involvement, primarily arising from the skull, have been reported. The malignant transformation toward slowly growing low-grade chondrosarcomas is the most severe form of progression. Methods. We report a unique case of a 54-year-old patient with Ollier disease with an extensive nasal enchondroma apparently eroding the middle nasal concha and expanding to the lateral nasal wall that raised suspicion of malignant transformation.
INTRODUCTION Morbus Ollier is a rare, nonhereditary bone disorder with the prevalence of 1 in 100,000, defined by the presence of multiple enchondromas.1,2 Enchondromas are benign cartilaginous tumors that typically arise in the first decade of life as intraosseous cartilaginous lesions and predominantly occur unilaterally in the metaphyses and diaphyses of tubular bones.1,3 They usually affect the long bones of the hand, the humerus, and the tibia, followed by flat bones, such as the pelvis. In intracranial and extracranial involvement, the most frequently encountered area is the base of the skull.4 The association of multiple unilateral enchondromas and cutaneous hemangiomas is known as Maffucci syndrome and differs from Ollier disease, particularly in their higher potential of malignant transformation.3 Depending on their size and localization, enchondromas can cause a variety of symptoms, such as skeletal deformities, a discrepancy of the length of the limb, and pathologic fractures; the diagnosis is based on a combination of clinical, radiological, and pathological features.1,3,5 Malignant transformation toward secondary chondrosarcomas is the most severe form of enchondroma progression. As the incidence for secondary malignancy in the limb bones ranges from 5% to 50% in the literature, the extent of radi-
*Corresponding author: M. Soyka, Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zurich, Zurich, 8091, Switzerland. E-mail: [email protected]
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Results. Radiological and histological features of enchondromas can be controversial and seem to have limited sensitivity to exclude low-grade malignancy. The clinical symptoms play a decisive role in differentiation between enchondromas and low-grade chondrosarcomas. Conclusion. Surgery remains the only effective solution in removing an enchondroma and preventing the tendency toward malignant transforC 2014 Wiley Periodicals, Inc. Head Neck 37: E30–E33, 2015 mation. V
KEY WORDS: Ollier disease, enchondroma, multiple enchondromatosis, nasal septum, chondrosarcoma
ological and histological examination as well as the type of treatment is decided on an individual basis.1,2,6 Because there is no causal treatment to cure enchondromatosis, surgical resection remains the only available therapy if complications, such as growth defects, fractures, or malignant transformation, occur.2 Although enchondromatosis and the propensity for malignant transformation is well described for tubular bones of the limbs and the pelvis, only a few cases of head and neck chondrosarcoma associated with Ollier disease have been described. Most of these have developed in the skull base with intracranial or extracranial extension, but without affecting the nasal septum.2–5,7,8 Ear, nose, and throat (ENT) manifestation of enchondromas, particularly Ollier-related enchondromas of the nasal septum, seem to be extremely rare and, therefore, little is known about the biological behavior of this disease and its diagnostic features.7
CASE REPORT A 54-year-old woman was referred to our department because of a mass on her left nasal cavity in October 2012. In 1960, she was diagnosed with a left-sided Ollier disease enchondroma on her left femur. Since the onset of the disease, both the leg and the arm had been affected multiple times without any malignant transformation. As the patient was recently suffering from intermittent vertigo and mild left-sided nasal obstruction, a neurologist performed a CT scan of the brain. An incidental radiologic finding showed an expansive mass measuring 15 3 28 3 31 mm and extending to the left nasal cavity with
OLLIER
DISEASE PRESENT IN THE NASAL CAVITY
FIGURE 1. (A) and (B) Extension of the mass with cortical destruction in the axial and coronary CT, respectively. The left middle nasal concha seems to be eroded by the mass (arrow).
multiple cortical destruction and central mineralization of the matrix. The medial wall of the maxillary sinus was slightly bulged and the middle nasal concha seemed to be invasively eroded, which led to a high suspicion of malignancy (Figure 1). An MRI of the brain revealed 2 gadolinium enhancing areas, one with a homogenous peripheral enhancement (2 cm in diameter), and a small inhomogeneous region (1 cm in diameter) (Figure 2). The follow-up time from the first CT to final surgery was almost 10 months, in which no significant morphological alterations regarding size, calcifi-
cation, and enhancement could be detected, except for a right-sided deviation of the caudal septum (Figure 2D). The patient never felt any severe pain; however, a slight pressure over the left maxillary sinus was occasionally noted, but without any other symptoms, such as pathologic rhinorrhea. In April 2013, the patient finally consented to surgical resection, as a malignancy could no longer be excluded. Although it was resected and mobilized en bloc together with the middle concha, it had to be excised in several parts because of its large size (Figures 3, 4). The
FIGURE 2. (A–D) Axial, coronary, and sagittal T1-weighted MRI of the nasal cavity. The arrow shows the morphologic correlate of the right-sided septal deviation.
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FIGURE 3. (A) and (B) Intraoperative endoscopy of the right (A) and left (B) anterior nasal cavity, respectively. NS, nasal septum; EN, enchondroma; MT, middle turbinate; IT, inferior turbinate; LNW, lateral nasal wall.
subsequent histology revealed a benign enchondroma without atypical cells and low cellularity.
DISCUSSION We herein present the case of a 54-year-old patient with Ollier disease with an unusual ENT manifestation of enchondromatosis in the nasal cavity that led to a challenging clinical management because of atypical radiological and morphological features, suggestive of malignancy. Although several Ollier-related enchondromas of the skull have been reported, enchondromas of the nasal septum remain particularly rare. When they occur in the head and neck, they usually arise from cartilaginous rests in the skull base, especially around the basal synchondroses.5 As the rates for malignancy vary from 5% to 50%, their potential for malignant transformation is of importance in their clinical management.1,2,6,9 According to Verdegaal et al,2 58 of 144 (40%) patients with Ollier disease developed chondrosarcomas in dependency of their location; patients with enchondromas of the small bones of the limbs had the lowest risk to develop malignancy (14%), whereas enchondromas affecting the long axial bones showed the highest risk (44% to 55%). It is difficult to estimate the rate of malignancy in enchondromatosis of the nasal septum, because only 1 case of Ollier disease of the nasal septum without malignant transformation and 2 Maffucci syndrome-related cases with partial transformation have been described in the literature. Furthermore, only very few Ollier-related enchondromas and 7 secondary chondrosarcomas involving the sinuses, the skull base, and the parapharyngeal space are comparable to our case.3,5,7,8 In 1992, Stevenson7 first described the case of an 18year-old woman who suffered from an enchondroma assoE32
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MARCH 2015
ciated with Ollier disease expanding from the nasal septum. Compared to our case, the CT scan showed a much smaller lesion without infiltrative aspect. Although the histological examination revealed high cartilaginous cellularity and pleomorphic nuclei suggestive of a low-grade chondrosarcoma, it was suggested to be a simple enchondroma in the context of Ollier disease as enchondromas of other sites displayed similar histopathological features.7
FIGURE 4. Endonasal result after total resection. LMT, left middle turbinate; LIT, left inferior turbinate; LIS, lamina intersphenoidale; LNW, lateral nasal wall.
OLLIER
Another case of a 20-year-old woman was reported in 1991 with an extensive enchondroma arising in the ethmoidal cells and eroding the cribriform plate.8 Histologically, it was first diagnosed as a low-grade chondrosarcoma because of nuclear polymorphism and hyperchromasia as well as the presence of binucleate cells. As the cartilaginous lesion was the initial manifestation of the later diagnosed Morbus Ollier, Pfleiderer et al8 revised their decision of malignant transformation in the context of Ollier disease. Atypical cells are always suspicious for early malignant transformation, such as those presented in 2 additional Ollier-related cases with a possible malignant enchondroma and a low-grade chondrosarcoma arising from the sphenoid and the sellar region.3,5 In the context of Maffucci syndrome, 2 more relevant cases of nasal septal affection are described in the literature. Hyde et al10 presented a nasal chondrosarcoma in 1995, and Dellagi et al11 discussed a case of a solitary nasal enchondroma in 1985 that was first diagnosed as a myxoma and later as the onset of Morbus Ollier, because multiple skeletal enchondromas occurred 10 years later. The concomitant finding of an angioma in the medical history made the authors reassess and conclude on the diagnosis of Maffucci syndrome rather than Ollier disease.10,11 A significant number of studies deal with malignant forms of these tumors found in the pelvis and limbs, whereas the propensity for malignant transformation of Ollier disease involving the nasal septum seems to be slightly less frequent after reviewing these cases.3,5,7,8 In general, only additional histology can definitely exclude malignancy. However, the histological specificity to distinguish between enchondromatosis and low-grade chondrosarcoma, based purely on morphology, is believed to be poor.12,13 Our patient was observed for almost 10 months without any clinical deterioration, which points toward a nonmalignant behavior despite the suspicious radiological findings. The enchondroma displayed cortical destruction, which is deemed to be a sign for malignancy as these features are usually not seen with enchondromas.4,14 Verdegaal et al2 showed that areas of cortical destruction and soft tissue extension together in Ollier-related enchondromas of the limbs increase the chance of a chondrosarcoma by a factor of 2.3. Furthermore, the heterogeneously enhancing area present in the enchondroma also raised suspicion, as Rogers et al5 showed that a heterogeneously enhancing Ollierrelated enchondroma of the sphenoid bone indicates a suspicious histology for early malignant transformation, consistent with a low-grade chondrosarcoma. Taori et al15 also described a non-Ollier-related chondrosarcoma of the nasal septum that appeared as a “heterogeneously enhancing low-attenuation mass.” Combining these findings with the fact that the enchondroma in our case seemed to erode the middle nasal concha in an infiltrative manner, radiological findings led to high suspicions for malignant transformation. To effectively treat enchondromas as well as grade 1 chondrosarcomas, total en bloc resection should be performed with a possible adjuvant radiation in case of chondrosarcomas.16 The ensuing surgery in our case revealed that the middle concha was pushed laterally and was sub-
DISEASE PRESENT IN THE NASAL CAVITY
sequently atrophied by the pressure of the enchondroma rather than being eroded. After the retrospective integration of these radiological and clinical findings with the definite benign histological result, it is possible that the cortical destruction could also have been caused by the anatomy of the thin cortical bone of the nasal septum. The fact that the tumor only bulged into the wall of the maxillary sinus could have provided a hint that the concha was not truly infiltrated. In addition, the constant size with an absence of pain over almost 10 months would favor a benign tendency.
CONCLUSIONS This case report emphasizes the challenging clinical management of patients with an ENT manifestation of Ollier disease and reveals new dimensions in radiologic aspects of a nasal enchondroma. Radiological and histological features can be highly controversial and have limited sensitivity to exclude low-grade malignant transformation. Clinical symptoms, such as increasing size and onset of pain, play an important role in the difficult diagnostic differentiation between enchondromas and low-grade chondrosarcomas. Surgery remains the only effective solution to remove the enchondroma and prevent malignant transformation.
REFERENCES 1. Silve, C, J€ uppner, H. Ollier disease. Orphanet J Rare Dis 2006;1:37. 2. Verdegaal SH, Bovee JV, Pansuriya TC, et al. Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients. Oncologist 2011;16:1771–1779. 3. No€el G, Feuvret L, Calugaru V, et al. Chondrosarcomas of the base of the skull in Ollier’s disease or Maffucci’s syndrome–three case reports and review of the literature. Acta Oncol 2004;43:705–710. 4. D’Angelo L, Massimi L, Narducci A, Di Rocco C. Ollier disease. Childs Nerv Syst 2009;25:647–653. 5. Rogers DJ, Boseley ME, Stephan MJ, Browd S, Semerad DC. Enchondroma of the skull base secondary to generalized enchondromatosis: a case report and review of the literature. Ear Nose Throat J 2011;90:535–537. 6. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier’s disease. Cancer 1987;59:1376–1385. 7. Stevenson DS. Nasal obstruction due to enchondromas in Ollier’s disease. J R Soc Med 1992;85:703–704. 8. Pfleiderer AG, Thomson P, Milroy CM. View from beneath: pathology in focus. ENT presentation of Ollier’s disease. J Laryngol Otol 1991;105: 148–150. 9. Nengsu A, Bernat I, Brocheriou–Spelle I, et al. Parapharyngeal chondrosarcoma and Ollier’s disease: case report and review of the literature [in French]. Ann Otolaryngol Chir Cervicofac 2002;119:287–292. 10. Hyde GE, Yarington, CT Jr, Chu FW. Head and neck manifestations of Maffucci’s syndrome: chondrosarcoma of the nasal septum. Am J Otolaryngol 1995;16:272–275. 11. Dellagi K, Micheau C, Estelin R, Genin J. Nasal chondroma associated with Ollier’s disease. Apropos of a case observed at the Gustave–Roussy Institute [in French]. Ann Otolaryngol Chir Cervicofac 1985;102:537–541. 12. Geirnaerdt MJ, Hermans J, Bloem JL, et al. Usefulness of radiography in differentiating enchondroma from central grade 1 chondrosarcoma. AJR Am J Roentgenol 1997;169:1097–1104. 13. Muramatsu K, Kawakami Y, Tani Y, Taguchi T. Malignant transformation of multiple enchondromas in the hand: case report. J Hand Surg Am 2011; 36:304–307. 14. Murphey MD, Flemming DJ, Boyea SR, Bojescul JA, Sweet DE, Temple HT. Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics 1998;18:1213–1237; quiz 1244– 1245. 15. Taori K, Chandanshive S, Attarde V, Patil P, Rangankar V, Londhe S. Nasal chondrosarcoma in an infant: radiologic and histologic correlation. AJNR Am J Neuroradiol 2006;27:1394–1395. 16. van der Geest IC, de Valk MH, de Rooy JW, Pruszczynski M, Veth RP, Schreuder HW. Oncological and functional results of cryosurgical therapy of enchondromas and chondrosarcomas grade 1. J Surg Oncol 2008;98: 421–426.
HEAD & NECK—DOI 10.1002/HED
MARCH 2015
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Jonathan Irish, MD, FRCSC, Section Editor
Enchondroma of the nasal septum due to Ollier disease: A case report and review of the literature Christian M. C. Jacobi, MD,1,3 Egodage Samitha Hiranya, MD,2 Annietta Gay, MD,1 David Holzmann, MD,1 Spyridon Kollias, MD,2 Michael B. Soyka, MD1* 1
Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Zurich, Zurich, Switzerland, 2Department of Neuroradiology, University Hospital Zurich, Zurich, Switzerland, 3Medical School of the Technical University Munich, Munich, Germany.
Accepted 28 May 2014 Published online 28 July 2014 in Wiley Online Library (wileyonlinelibrary.com). DOI 10.1002/hed.23783
ABSTRACT: Background. Morbus Ollier is characterized by the presence of multiple enchondromas (ie, benign intraosseous cartilaginous lesions). Although their manifestation in the limb bones is well described, only a few cases with ear, nose, and throat (ENT) involvement, primarily arising from the skull, have been reported. The malignant transformation toward slowly growing low-grade chondrosarcomas is the most severe form of progression. Methods. We report a unique case of a 54-year-old patient with Ollier disease with an extensive nasal enchondroma apparently eroding the middle nasal concha and expanding to the lateral nasal wall that raised suspicion of malignant transformation.
INTRODUCTION Morbus Ollier is a rare, nonhereditary bone disorder with the prevalence of 1 in 100,000, defined by the presence of multiple enchondromas.1,2 Enchondromas are benign cartilaginous tumors that typically arise in the first decade of life as intraosseous cartilaginous lesions and predominantly occur unilaterally in the metaphyses and diaphyses of tubular bones.1,3 They usually affect the long bones of the hand, the humerus, and the tibia, followed by flat bones, such as the pelvis. In intracranial and extracranial involvement, the most frequently encountered area is the base of the skull.4 The association of multiple unilateral enchondromas and cutaneous hemangiomas is known as Maffucci syndrome and differs from Ollier disease, particularly in their higher potential of malignant transformation.3 Depending on their size and localization, enchondromas can cause a variety of symptoms, such as skeletal deformities, a discrepancy of the length of the limb, and pathologic fractures; the diagnosis is based on a combination of clinical, radiological, and pathological features.1,3,5 Malignant transformation toward secondary chondrosarcomas is the most severe form of enchondroma progression. As the incidence for secondary malignancy in the limb bones ranges from 5% to 50% in the literature, the extent of radi-
*Corresponding author: M. Soyka, Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Zurich, Zurich, 8091, Switzerland. E-mail: [email protected]
E30
HEAD & NECK—DOI 10.1002/HED
MARCH 2015
Results. Radiological and histological features of enchondromas can be controversial and seem to have limited sensitivity to exclude low-grade malignancy. The clinical symptoms play a decisive role in differentiation between enchondromas and low-grade chondrosarcomas. Conclusion. Surgery remains the only effective solution in removing an enchondroma and preventing the tendency toward malignant transforC 2014 Wiley Periodicals, Inc. Head Neck 37: E30–E33, 2015 mation. V
KEY WORDS: Ollier disease, enchondroma, multiple enchondromatosis, nasal septum, chondrosarcoma
ological and histological examination as well as the type of treatment is decided on an individual basis.1,2,6 Because there is no causal treatment to cure enchondromatosis, surgical resection remains the only available therapy if complications, such as growth defects, fractures, or malignant transformation, occur.2 Although enchondromatosis and the propensity for malignant transformation is well described for tubular bones of the limbs and the pelvis, only a few cases of head and neck chondrosarcoma associated with Ollier disease have been described. Most of these have developed in the skull base with intracranial or extracranial extension, but without affecting the nasal septum.2–5,7,8 Ear, nose, and throat (ENT) manifestation of enchondromas, particularly Ollier-related enchondromas of the nasal septum, seem to be extremely rare and, therefore, little is known about the biological behavior of this disease and its diagnostic features.7
CASE REPORT A 54-year-old woman was referred to our department because of a mass on her left nasal cavity in October 2012. In 1960, she was diagnosed with a left-sided Ollier disease enchondroma on her left femur. Since the onset of the disease, both the leg and the arm had been affected multiple times without any malignant transformation. As the patient was recently suffering from intermittent vertigo and mild left-sided nasal obstruction, a neurologist performed a CT scan of the brain. An incidental radiologic finding showed an expansive mass measuring 15 3 28 3 31 mm and extending to the left nasal cavity with
OLLIER
DISEASE PRESENT IN THE NASAL CAVITY
FIGURE 1. (A) and (B) Extension of the mass with cortical destruction in the axial and coronary CT, respectively. The left middle nasal concha seems to be eroded by the mass (arrow).
multiple cortical destruction and central mineralization of the matrix. The medial wall of the maxillary sinus was slightly bulged and the middle nasal concha seemed to be invasively eroded, which led to a high suspicion of malignancy (Figure 1). An MRI of the brain revealed 2 gadolinium enhancing areas, one with a homogenous peripheral enhancement (2 cm in diameter), and a small inhomogeneous region (1 cm in diameter) (Figure 2). The follow-up time from the first CT to final surgery was almost 10 months, in which no significant morphological alterations regarding size, calcifi-
cation, and enhancement could be detected, except for a right-sided deviation of the caudal septum (Figure 2D). The patient never felt any severe pain; however, a slight pressure over the left maxillary sinus was occasionally noted, but without any other symptoms, such as pathologic rhinorrhea. In April 2013, the patient finally consented to surgical resection, as a malignancy could no longer be excluded. Although it was resected and mobilized en bloc together with the middle concha, it had to be excised in several parts because of its large size (Figures 3, 4). The
FIGURE 2. (A–D) Axial, coronary, and sagittal T1-weighted MRI of the nasal cavity. The arrow shows the morphologic correlate of the right-sided septal deviation.
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FIGURE 3. (A) and (B) Intraoperative endoscopy of the right (A) and left (B) anterior nasal cavity, respectively. NS, nasal septum; EN, enchondroma; MT, middle turbinate; IT, inferior turbinate; LNW, lateral nasal wall.
subsequent histology revealed a benign enchondroma without atypical cells and low cellularity.
DISCUSSION We herein present the case of a 54-year-old patient with Ollier disease with an unusual ENT manifestation of enchondromatosis in the nasal cavity that led to a challenging clinical management because of atypical radiological and morphological features, suggestive of malignancy. Although several Ollier-related enchondromas of the skull have been reported, enchondromas of the nasal septum remain particularly rare. When they occur in the head and neck, they usually arise from cartilaginous rests in the skull base, especially around the basal synchondroses.5 As the rates for malignancy vary from 5% to 50%, their potential for malignant transformation is of importance in their clinical management.1,2,6,9 According to Verdegaal et al,2 58 of 144 (40%) patients with Ollier disease developed chondrosarcomas in dependency of their location; patients with enchondromas of the small bones of the limbs had the lowest risk to develop malignancy (14%), whereas enchondromas affecting the long axial bones showed the highest risk (44% to 55%). It is difficult to estimate the rate of malignancy in enchondromatosis of the nasal septum, because only 1 case of Ollier disease of the nasal septum without malignant transformation and 2 Maffucci syndrome-related cases with partial transformation have been described in the literature. Furthermore, only very few Ollier-related enchondromas and 7 secondary chondrosarcomas involving the sinuses, the skull base, and the parapharyngeal space are comparable to our case.3,5,7,8 In 1992, Stevenson7 first described the case of an 18year-old woman who suffered from an enchondroma assoE32
HEAD & NECK—DOI 10.1002/HED
MARCH 2015
ciated with Ollier disease expanding from the nasal septum. Compared to our case, the CT scan showed a much smaller lesion without infiltrative aspect. Although the histological examination revealed high cartilaginous cellularity and pleomorphic nuclei suggestive of a low-grade chondrosarcoma, it was suggested to be a simple enchondroma in the context of Ollier disease as enchondromas of other sites displayed similar histopathological features.7
FIGURE 4. Endonasal result after total resection. LMT, left middle turbinate; LIT, left inferior turbinate; LIS, lamina intersphenoidale; LNW, lateral nasal wall.
OLLIER
Another case of a 20-year-old woman was reported in 1991 with an extensive enchondroma arising in the ethmoidal cells and eroding the cribriform plate.8 Histologically, it was first diagnosed as a low-grade chondrosarcoma because of nuclear polymorphism and hyperchromasia as well as the presence of binucleate cells. As the cartilaginous lesion was the initial manifestation of the later diagnosed Morbus Ollier, Pfleiderer et al8 revised their decision of malignant transformation in the context of Ollier disease. Atypical cells are always suspicious for early malignant transformation, such as those presented in 2 additional Ollier-related cases with a possible malignant enchondroma and a low-grade chondrosarcoma arising from the sphenoid and the sellar region.3,5 In the context of Maffucci syndrome, 2 more relevant cases of nasal septal affection are described in the literature. Hyde et al10 presented a nasal chondrosarcoma in 1995, and Dellagi et al11 discussed a case of a solitary nasal enchondroma in 1985 that was first diagnosed as a myxoma and later as the onset of Morbus Ollier, because multiple skeletal enchondromas occurred 10 years later. The concomitant finding of an angioma in the medical history made the authors reassess and conclude on the diagnosis of Maffucci syndrome rather than Ollier disease.10,11 A significant number of studies deal with malignant forms of these tumors found in the pelvis and limbs, whereas the propensity for malignant transformation of Ollier disease involving the nasal septum seems to be slightly less frequent after reviewing these cases.3,5,7,8 In general, only additional histology can definitely exclude malignancy. However, the histological specificity to distinguish between enchondromatosis and low-grade chondrosarcoma, based purely on morphology, is believed to be poor.12,13 Our patient was observed for almost 10 months without any clinical deterioration, which points toward a nonmalignant behavior despite the suspicious radiological findings. The enchondroma displayed cortical destruction, which is deemed to be a sign for malignancy as these features are usually not seen with enchondromas.4,14 Verdegaal et al2 showed that areas of cortical destruction and soft tissue extension together in Ollier-related enchondromas of the limbs increase the chance of a chondrosarcoma by a factor of 2.3. Furthermore, the heterogeneously enhancing area present in the enchondroma also raised suspicion, as Rogers et al5 showed that a heterogeneously enhancing Ollierrelated enchondroma of the sphenoid bone indicates a suspicious histology for early malignant transformation, consistent with a low-grade chondrosarcoma. Taori et al15 also described a non-Ollier-related chondrosarcoma of the nasal septum that appeared as a “heterogeneously enhancing low-attenuation mass.” Combining these findings with the fact that the enchondroma in our case seemed to erode the middle nasal concha in an infiltrative manner, radiological findings led to high suspicions for malignant transformation. To effectively treat enchondromas as well as grade 1 chondrosarcomas, total en bloc resection should be performed with a possible adjuvant radiation in case of chondrosarcomas.16 The ensuing surgery in our case revealed that the middle concha was pushed laterally and was sub-
DISEASE PRESENT IN THE NASAL CAVITY
sequently atrophied by the pressure of the enchondroma rather than being eroded. After the retrospective integration of these radiological and clinical findings with the definite benign histological result, it is possible that the cortical destruction could also have been caused by the anatomy of the thin cortical bone of the nasal septum. The fact that the tumor only bulged into the wall of the maxillary sinus could have provided a hint that the concha was not truly infiltrated. In addition, the constant size with an absence of pain over almost 10 months would favor a benign tendency.
CONCLUSIONS This case report emphasizes the challenging clinical management of patients with an ENT manifestation of Ollier disease and reveals new dimensions in radiologic aspects of a nasal enchondroma. Radiological and histological features can be highly controversial and have limited sensitivity to exclude low-grade malignant transformation. Clinical symptoms, such as increasing size and onset of pain, play an important role in the difficult diagnostic differentiation between enchondromas and low-grade chondrosarcomas. Surgery remains the only effective solution to remove the enchondroma and prevent malignant transformation.
REFERENCES 1. Silve, C, J€ uppner, H. Ollier disease. Orphanet J Rare Dis 2006;1:37. 2. Verdegaal SH, Bovee JV, Pansuriya TC, et al. Incidence, predictive factors, and prognosis of chondrosarcoma in patients with Ollier disease and Maffucci syndrome: an international multicenter study of 161 patients. Oncologist 2011;16:1771–1779. 3. No€el G, Feuvret L, Calugaru V, et al. Chondrosarcomas of the base of the skull in Ollier’s disease or Maffucci’s syndrome–three case reports and review of the literature. Acta Oncol 2004;43:705–710. 4. D’Angelo L, Massimi L, Narducci A, Di Rocco C. Ollier disease. Childs Nerv Syst 2009;25:647–653. 5. Rogers DJ, Boseley ME, Stephan MJ, Browd S, Semerad DC. Enchondroma of the skull base secondary to generalized enchondromatosis: a case report and review of the literature. Ear Nose Throat J 2011;90:535–537. 6. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier’s disease. Cancer 1987;59:1376–1385. 7. Stevenson DS. Nasal obstruction due to enchondromas in Ollier’s disease. J R Soc Med 1992;85:703–704. 8. Pfleiderer AG, Thomson P, Milroy CM. View from beneath: pathology in focus. ENT presentation of Ollier’s disease. J Laryngol Otol 1991;105: 148–150. 9. Nengsu A, Bernat I, Brocheriou–Spelle I, et al. Parapharyngeal chondrosarcoma and Ollier’s disease: case report and review of the literature [in French]. Ann Otolaryngol Chir Cervicofac 2002;119:287–292. 10. Hyde GE, Yarington, CT Jr, Chu FW. Head and neck manifestations of Maffucci’s syndrome: chondrosarcoma of the nasal septum. Am J Otolaryngol 1995;16:272–275. 11. Dellagi K, Micheau C, Estelin R, Genin J. Nasal chondroma associated with Ollier’s disease. Apropos of a case observed at the Gustave–Roussy Institute [in French]. Ann Otolaryngol Chir Cervicofac 1985;102:537–541. 12. Geirnaerdt MJ, Hermans J, Bloem JL, et al. Usefulness of radiography in differentiating enchondroma from central grade 1 chondrosarcoma. AJR Am J Roentgenol 1997;169:1097–1104. 13. Muramatsu K, Kawakami Y, Tani Y, Taguchi T. Malignant transformation of multiple enchondromas in the hand: case report. J Hand Surg Am 2011; 36:304–307. 14. Murphey MD, Flemming DJ, Boyea SR, Bojescul JA, Sweet DE, Temple HT. Enchondroma versus chondrosarcoma in the appendicular skeleton: differentiating features. Radiographics 1998;18:1213–1237; quiz 1244– 1245. 15. Taori K, Chandanshive S, Attarde V, Patil P, Rangankar V, Londhe S. Nasal chondrosarcoma in an infant: radiologic and histologic correlation. AJNR Am J Neuroradiol 2006;27:1394–1395. 16. van der Geest IC, de Valk MH, de Rooy JW, Pruszczynski M, Veth RP, Schreuder HW. Oncological and functional results of cryosurgical therapy of enchondromas and chondrosarcomas grade 1. J Surg Oncol 2008;98: 421–426.
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MARCH 2015
E33
