Eur Arch Otorhinolaryngol (2008) 265:587–591 DOI 10.1007/s00405-007-0460-4
C A S E RE P O RT
Temporal bone central giant-cell granuloma presenting as a serous otitis media Milan Rudic · Alexis Bozorg Grayeli · Dominique Cazals-Hatem · Françoise Cyna-Gorse · Didier Bouccara · Olivier Sterkers
Received: 25 January 2007 / Accepted: 30 August 2007 / Published online: 15 November 2007 © Springer-Verlag 2007
Abstract Central giant cell granuloma is a benign intraosseous lesion that most commonly occurs in the facial bones. Its location in the temporal bone is extremely rare and only 20 cases have been reported in the literature. We report a case of an adult female patient presenting with a right serous otitis media and mastoiditis associated with a mixed hearing loss during 6 months. CT-scan and MRI revealed a temporal bone tumor involving the mastoid, and surrounding the right temporo-mandibular joint. Tumor was totally removed after a canal-wall-down mastoidectomy and middle ear exclusion. Pathology revealed a central giant cell granuloma. Seven months following the surgery there was no evidence of recurrence. Central giant cell granuloma is a rare temporal bone lesion, with non speciWc
M. Rudic · A. B. Grayeli (&) · D. Bouccara · O. Sterkers Department of Otorhinolaryngology Head and Neck Surgery, AP-HP, Hospital Beaujon, 100, Boulevard. du General Leclerc, 92118 Clichy Cedex, France e-mail:
[email protected] M. Rudic · A. B. Grayeli · D. Bouccara · O. Sterkers Inserm UNIT-M 867, Faculté Xavier Bichat, Université Paris 7, Paris, France D. Cazals-Hatem Department of Pathology, AP-HP, Hospital Beaujon, Clichy, France F. Cyna-Gorse Department of Radiology, AP-HP, Hospital Beaujon, Clichy, France Present Address: M. Rudic Department of Oto-rhino-laryngology Head and Neck Surgery, General Hospital Zadar, Zadar, Croatia
clinical and imaging signs but characteristic pathological features. Today, a total surgical removal and regular MRI follow-up is the best management option. Keywords Hearing loss · Serous otitis media · Giant cell granuloma · Temporal bone · Benign tumor
Introduction Central giant cell granuloma is a non-neoplastic Wbrous lesion usually involving the facial bones, and predominantly the mandible and the maxilla [1]. Other cranial bones are rarely aVected [1]. The Wrst description of the temporal bone central giant cell granuloma was reported by Hirsch and Katz [2] in 1974. Histologicaly, central giant cell granuloma must be distinguished from other giant cell lesions such as cherubism, brown tumors of hyperparathyroidism, aneurismal bone cysts and especially giant cell tumors [1]. Several etiological hypothesis are advanced (trauma induced intraosseous hemorrhage, infection, developmental anomalies, and hormonal inXuences) but the exact pathogenesis remains unclear [3–7]. Clinical symptoms are non speciWc and vary in intensity from a slowly growing asymptomatic swelling to a more aggressive form with pain and local destruction [5]. Temporal bone involvement may cause wide local spreading of the lesion to the cranial base and intracranial cavity [3]. This rare lesion with serious and incapacitating symptoms should be correctly diagnosed before an adapted treatment and follow-up. We present the case of an extensive temporal bone central giant cell granuloma revealed by a serous otitis media with its clinical, imaging and pathological Wndings in comparison to the literature.
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Case report A 40-year-old female presented with a right progressive hearing loss during 6 months, associated with a weight loss and poor general health condition. No vertigo, tinnitus or headache were reported. No other concomitant disease and no previous head trauma was noted. Otoscopic examination revealed normal external auditory canal with a right serous otitis media. Other physical Wndings were normal. Audiometry showed a right mixed sensorineural and conductive hearing loss, and the right tympanometry showed a B Xat pattern (Fig. 1). Videonystagmography with caloric tests showed normal vestibular function. CT-scan revealed an osteolytic lesion Wlling the right mastoid cavity, surrounding the superior portion of the right temporo mandibular joint and involving the anterior part of the tympanic cavity and eustachian tube. The lesion extended to the middle
Fig. 1 Audiometry at diagnosis. Audiometry evidenced a right mixed conductive and sensorineural hearing loss. Tympanometry showed a B Xat pattern on the right
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cranial fossa but without any meningeal involvement (Fig. 2a, b). MRI showed a 37 mm large expansive osseous lesion of the mastoid extending to the apex bone with low T1 and T2 signal and without enhancement after gadolinium (data not shown). The lesion was best visualized on FLAIR sequences with high heterogeneous signal due to granulomatous tissue associated with secretions (Fig. 3). The external auditory meatus, the inner ear structures and the facial canal were not involved. The imaging conWrmed the serous otitis media secondary to the eustachian tube obstruction. Blood tests (inXammatory markers, calcium, phosphorus, alkaline phosphatase and parathyroid hormone) were within normal values (data not shown). A surgical removal of the lesion was decided. A canal-wall-up mastoidectomy with middle ear exclusion was performed. Mastoid cavity was Wlled with a seromucous liquid and an osteolytic granulomatous lesion spreading towards the root of the zygomatic bone and to the tip of the mastoid. The lesion was partially removed. Histopathological analysis revealed a complete osteolysis by a highly cellular process comprising two cellular components: mononuclear cells with a spindle shaped Wbroblastic aspect (Fig. 4a), and osteoclastic giant cells, irregularly dispersed in an inXammatory background (Fig. 4b). Abundant hemosiderin deposits were observed after Perls’ staining (Fig. 4c). No necrosis or malignancy criteria were found. The diagnosis was a central giant cell granuloma. A complete excision
Fig. 2 Pre operative CT scan. Preoperative CT-scan showed an osteolytic lesion of the right petrous bone, involving the anterior part of the tympanic cavity and Eustachian tube (*) on the axial view (a) and spreading towards the superior portion of the right temporo mandibular joint (arrow) on the coronal view (b)
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Fig. 3 Pre operative MRI. MRI with FLAIR sequences and axial views showed high and heterogeneous signal in the mastoid cavity in favor of granulomatous tissue associated with middle ear secretions (high signal). Middle ear cavity (arrow) also showed a high signal due to serous otitis media
was decided. The second intervention was carried out one month after the Wrst. A canal-wall-down mastoidectomy and a middle ear exclusion were performed under facial nerve monitoring. The lesion was totally removed in a piece-meal manner and the cavity was Wlled with abdominal fat grafts. The postoperative period was uneventful and 7 months after surgery imaging did not show recurrence (data not shown).
Discussion Localization of the central giant cell granuloma in the temporal bone is extremely rare and only 20 cases have been reported in the literature [3]. The etiology of the central giant cell granuloma is unclear. Although in our case no previous head trauma was reported, bone trauma followed by intraosseous hemorrhage with a reactive granulomatous process is advocated as the main etiological factor [3, 4]. Clinical symptoms depend on the aVected region. Initially a localized pain followed Wrstly by a diVuse, and then localized swelling is present [8]. Signs of elevated intracranial pressure, and cerebral compression in intracranial extensions, orbital symptoms in aVected sphenoid bone and nasal symptoms in ethmoidal lesions are reported [8]. As in
our case, the involvement of the temporal bone causes hearing loss, but vertigo, tinnitus, and facial paresis are also possible [8]. The reported radiographic features correspond to the Wndings in our case. CT-scan shows a bony destruction without distinctive radiological signs, and MRI demonstrates low T1 signal, high T2 signal and variable contrast enhancement [4]. Hence, the diagnosis is conWrmed by pathology. Histologically, this tumor consists of mononuclear, spindleshaped, stromal cells along with the giant multinucleated cells that tend to cluster, along with the new bone formation [4]. Central giant cell granuloma must be distinguished from other giant cell lesion with similar histopathological Wndings such as cherubism, brown tumor of hyperparathyroidism, aneurismal bone cyst, and giant cell tumor [4]. Cherubism is an autosomal dominant disorder, with similar histological but diVerent clinical and radiological Wndings [5]. Aneurismal bone cyst is a non-neoplastic rare condition distinguished from the central giant cell granuloma by a typical radiological image, which is cystic blow out of the bone [6]. Brown tumors in hyperparathyroidism can be easily excluded by blood tests showing normal calcium, phosphorus, alkaline phosphatase and parathyroid hormone plasma levels as in the present case. Giant cell tumor is a benign, locally aggressive neoplasm, composed of sheets of
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Treatment of choice in central giant cell granuloma is complete surgical removal [6, 11]. But due to multiple tumor extensions in the temporal bone and the risk of incomplete excision, a regular follow-up is mandatory. Radiotherapy is not of great value in this benign lesion, and it has been associated with a higher risk of sarcomatous transformation of the bone [7, 11–13]. Recently, the eYciency of medical treatment with calcitonin has been reported in one case. This possibility is promising especially in cranial base locations where surgery has a risk of cranial nerve palsy [14].
Conclusion Central giant cell granuloma is a benign intraosseous, nonneoplastic lesion with distinct clinicopathological features. Its location in the temporal bone revealed by serous otitis media and a mixed hearing loss is rare. Today, the best proposed therapy is a complete microsurgical excision and a postoperative follow-up by MRI.
References
Fig. 4 Pathological examination of the tumor. a The lesion was osteolytic, densely cellular, composed of Wbroblastic cells loosely arranged in a Wbrous and vascularized stroma (Hematein and eosin, £100). b Clusters of giant osteoclastic cells are noted around foci of hemorrhage, mixed with mononucleated cells (Hematein and eosin, £200). c Perls’ stain shows constant hemosiderin deposits in the stroma (£300)
neoplastic ovoid mononuclear cells interspersed with uniformly distributed large, osteoclastic giant cells [9]. This tumor typically aVects the ends of long bones (femur, tibia, radius, and humerus) or Xat bones (sacrum) as well as hands and feet. Its location in the head and neck region is exceptional although there are several cases reporting its localization in the larynx especially in thyroid cartilage [10].
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