European Journal of Radiology 44 (2002) 24 – 27 www.elsevier.com/locate/ejrad
Quiz case Sinan C ¸ akırer * Department of Radiology, Istanbul S¸is¸li Etfal Hospital, 80220 Istanbul, Turkey Received 24 September 2001; received in revised form 6 November 2001; accepted 7 November 2001
1. Introduction A 14-year-old male patient presented to the department of otorhinolaryngology with a dull pain over his left cheek for 2 months. The physical examination revealed a soft tissue mass, that was tender by palpation, of left superior dental arch, and most probably originating from left maxillary sinus. There was no history of trauma or significant paranasal sinus infections. Laboratory studies, including complete blood count, biochemical studies of serum calcium, phosphate, alkaline phosphatase, were all within the normal limits. A computed tomography (CT) scan of the paranasal sinuses was performed with 3-mm continuous slices on axial and coronal planes. A magnetic resonance imaging (MRI) scan was performed for the paranasal sinuses with a 1.5 T MR scanner, using spin – echo T1 (TR 600 TE 30), fast spin – echo T2 (TR 3000 TE 90), and post-gadolinium injection spin – echo T1 (TR 600, TE 30) weighted sequences on three orthogonal planes. CT and MRI scans revealed a mass of left maxillary sinus that caused expansile and erosive changes of the maxillary bone and sinus wall. The surgical resection
of the mass resulted with neither residual mass nor recurrence within the following 4 months.
2. Imaging findings CT scan showed a well-circumscribed mass with a homogeneous soft tissue density (51 HU), that was located within the left maxillary sinus, extending towards alveolar recess of the sinus. The mass caused expansion and erosion of the inferior and lateral sinus walls, and erosion of the neighboring alveolar process of maxillary bone. It caused a swelling of left superior dental arch into the buccal cavity (Fig. 1a,b). MRI scan of the mass revealed that it was an encapsulated mass, isointense to muscles on T1-weighted images, and hyperintense to muscles on T2-weighted images. The mass contained hypointense linear areas, prominent on both T1-weighted and T2-weighted images, that were consistent with fibrous septa. The mass also contained some small cystic components, which were hypointense on T1-weighted, and hyperintense on T2-weighted images. Following intravenous gadolinium injection, the mass enhanced homogeneously except the fibrous septa and small cystic areas (Fig. 2a–c).
* Present address: 67 Ada, Kardelen 4/2, Daire 37, 81120 Atas¸ehir, Istanbul, Turkey. Tel./fax: + 90-216-4553-522. E-mail address:
[email protected] (S. C ¸ akırer).
0720-048X/02/$ - see front matter © 2002 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 7 2 0 - 0 4 8 X ( 0 1 ) 0 0 4 7 0 - 3
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Fig. 1. (a) Coronal CT image on bone window reveals a soft tissue mass which is located in the left maxillary sinus, inferiorly extending towards alveolar recess of the sinus. The mass expands the lateral and inferior sinus walls, and erodes and causes destruction of the sinus walls and alveolar process (arrow) of the maxillary bone also. (b). Axial CT image on bone window reveals the soft tissue mass of left maxillary sinus, which extends to the alveolar recess of maxillary sinus, and causes erosion and destruction of alveolar process of the bone (arrows).
Fig. 2. (a) Axial spin – echo T1-weighted MR image reveals the soft tissue mass of left maxillary sinus, eroding and disrupting the lateral sinus wall isointense to muscle, and it contains anterior small cystic component (white arrow), and posterior fibrous septa (black arrow). (b) Coronal fast spin – echo T2-weighted MR image reveals the soft tissue of heterogeneous intermediate intensity (hyperintense to muscle), contains hypointense fibrous septa (black arrows), and some small cystic components with hyperintense signal characteristics (white arrow). (c) Axial spin – echo T1-weighted MR image following IV gadolinium injection reveals the soft tissue mass of left maxillary sinus with homogeneous contrast enhancement except hypointense fibrous septa (black arrow) and cystic components (white arrow).
Fig. 3. Histopathological examination of the surgical specimen with hematoxylin– eosin staining showed multiple clustered multinucleated giant cells (arrowheads) with plenty of mononuclear fibroblastic cells, associated with cords of fibrous tissue (arrows).
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3. Histopathology and diagnosis Histopathological examination of the surgical specimen with hematoxylin– eosin staining showed multiple clustered multinucleated giant cells with plenty of mononuclear fibroblastic cells, associated with cords of fibrous tissue (Fig. 3). Final diagnosis depending on the imaging findings and histopathological examination was giant cell reparative granuloma.
4. Discussion Giant cell reparative granuloma, that was first described by Jaffe in 1953, is a rare benign lesion usually located in the mandible and maxilla [1]. Giant cell reparative granuloma is not a true neoplastic process. The pathogenesis of the lesion is not well clarified, but is thought to represent a local hyperplastic reparative process, following a posttraumatic intraosseous hemorrhage or periosteal reaction [2– 5]. However, a definite history of trauma has not been verified in many cases of giant cell reparative granuloma as in our case. Infection and developmental causes are the other possible theories for the pathogenesis of giant cell reparative granuloma [2,5]. Giant cell reparative granuloma is a slow growing mass, which occurs most commonly in the second and third decades of life. It affects females more frequently than males. The clinical findings listed as pain, swelling over the lesion, headaches are not specific. The lesion is tender by palpation [3,5– 9]. Although giant cell reparative granulomas are most frequently detected in the mandible and maxilla, they can also be observed in the orbit, paranasal sinuses, cranial vault, cranial cavity, and very rarely small bones of the hands and feet [2,3,10– 19]. The most commonly reported radiological finding of giant cell reparative granuloma is lysis with expansile remodeling of the bone as in the case of our patient. The cortex around the lesion is thin with or without bone destruction. However these radiological appearances are indistinguishable from other radiolucent bony lesions such as odontogenic cyst, aneurysmal bone cyst, ameloblastoma, odontogenic myxoma, and odontogenic fibroma. CT appearance of giant cell reparative granuloma is not distinctive either. Cortical thinning and destruction, soft tissue extension, and lesion density similar to that of muscle are usual findings as observed in our case. Calcifications can be seen within the lesion. However we could not detect calcifications within the mass lesion of our case. Giant cell reparative granuloma shows contrast enhancement with intravenous iodinated contrast agents. MRI findings are variable in the reported cases. They have low to intermediate sig-
nal intensity on T1-weighted sequences, and intermediate to high signal intensity on T2-weighted sequences, with hypointense fibrous septations and usually intense contrast enhancement following intravenous gadolinium injection. Cystic areas are uncommon, and when they are present they typically constitute only small components within the lesion as in our case [2– 5,8,10,19,20]. The soft tissue mass of our patient revealed typical CT and MRI characteristics and contrast enhancement pattern as described above. Histopathologically giant cell reparative granulomas are generally considered benign, although some may exhibit aggressive behavior. They are characterized by a loose, vascular stroma composed of plump round and oval mononuclear fibroblastic cells and multinucleated giant cells surrounding foci of hemorrhage. Osteoid production along hemorrhagic foci can be observed [4,5,8,10]. Although our case revealed a characteristic histopathological appearance of giant cell reparative granuloma, it did not show any osteoid foci or hemorrhagic foci. The main differential diagnoses for giant cell reparative granuloma both radiologically and histopathologically are giant cell tumor and brown tumor of hyperparathyroidism. The lesions can be radiologically indistinguishable from giant cell reparative granuloma with their expansile and locally invasive characteristics. Giant cell tumors can be seen in the skull, usually in the mandible, facial bones, and paranasal sinuses, although they are more commonly seen in epiphyseal portions of long bones. Histopathologically giant cell tumor and giant cell reparative granuloma are similar to each other. However, although both show multinucleated giant cells in a connective tissue stroma, a cytoplasmic predominance in stromal cells, clustering giant cells, and abundant new bone formation in giant cell reparative granuloma is opposed to the nuclear predominance in stromal cells, more uniformly distributed giant cells, and no new bone formation in giant cell tumors. Clinical information is also useful in the differential diagnosis of these two lesions, giant cell reparative granuloma is seen in the first two decades of life whereas giant cell tumors are usually seen in the third to fourth decades of life [3,5,6,15,16,21]. Histopathological appearance of giant cell reparative granuloma is similar to that of brown tumor of hyperparathyroidism, but laboratory analysis in showing elevated serum calcium, alkaline phophatase, parathyroid hormone levels, and decreased serum phophate help us for differential diagnosis [3,5,21]. Differential diagnosis of an expansile paranasal sinus lesion should also include mucoceles, sinus polyposis, sinus infections, neoplasms such as squamous cell carcinomas and other sinonasal sarcomas, and other fibroosseous lesions such as fibrous dysplasia and ossifying fibroma. Imaging findings, clinical history of the
S. C ¸ akırer / European Journal of Radiology 44 (2002) 24–27
patient, and finally histopathological studies of the lesions are helpful for differential diagnosis of the lesions. Mucoceles are the most common expansile lesions of the paranasal sinuses, the signal intensities on MRI and the density values on CT are variable, but they lack central homogeneous enhancement with intravenous contrast injection on MRI and CT scans, which is characteristic for giant cell reparative granulomas [3–5,8,10,15,21,22]. The treatment of giant cell reparative granuloma is total surgical resection. Radiation therapy has been advocated in inoperable cases. Recurrence is seen in 10 – 15% of the cases. Spontaneous sarcomatous transformation or aggressive regional spread has not been detected with giant cell reparative granulomas. However, sarcomatous degeneration following radiation therapy has been reported [2,4,5,7]. In summary giant cell reparative granuloma is a rare, benign fibroosseous lesion typically presenting as an expansile soft tissue mass, more typically occurring in the mandible and maxilla, and rarely occuring in the paranasal sinuses. Giant cell reparative granuloma has distinctive radiological as well as histopathological appearance. The treatment is total surgical resection with a low rate of recurrence.
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