Chondromyxoid Fibroma of the Skull Base Invading the Occipitocervical Junction: Report of a Unique Case and Discussion

Crocker, Matthew; Corns, Robert; Bódi, István; Zrinzo, Antoine; Gleeson, Michael; Thomas, Nick

doi:10.1055/s-0029-1225534

Cited by 15 publications

(27 citation statements)

References 17 publications

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“…The peak of incidence is in the second and third decade of life. 2,4,7,9,10 This lesion is more often found in the metaphyses of long bones and the region of the knee. 1,2 It is very rarely seen in the skull base.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 It is very rarely seen in the skull base. [1][2][3][4][5][6][7]9,10 However, there are reports of CMF in the sella turcica region, 4 the ethmoid sinus, 9 the craniocervical junction, 7 and the temporal bone. 2 Only six cases 2,[11][12][13][14][15] originating from the mastoid portion of the temporal bone have ever been reported in the literature.…”

Section: Discussionmentioning

confidence: 99%

“…1 For lesions involving the skull base, headache, palpable mass, and CN deficits such as diplopia, exophthalmos, anosmia, and dysphagia are very common. 1,7,10,11 When the lesion affects the temporal bone, hearing loss is the most common symptom, 17 and CN VII deficit is less common. 2 CT scans typically reveal an osteolytic and lobulated lesion surrounded by sclerotic bone, presenting a fine calcification of the neoplastic matrix in 25 to 75% of cases.…”

Section: Discussionmentioning

confidence: 99%

“…5,6 We report a case of CMF originating between the stylomastoid foramen and the facial nerve canal, posteriorly to the carotid canal and laterally to the jugular foramen, an exceptionally rare location for an extremely rare lesion. 2,7 We also review all the relevant literature to outline the clinical, radiologic, and surgical landmarks for the proper management of this rare condition.…”

Section: Introductionmentioning

confidence: 99%

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Chondromyxoid Fibroma of the Skull Base: Our Experience with an Elusive Disease

D’Andrea¹,

Pesce²,

Trasimeni

et al. 2017

J Neurol Surg A Cent Eur Neurosurg

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Chondromyxoid fibroma (CMF) is an extremely rare lesion of the skull base. This histologic type typically predilects metaphysis of the long bones. It is locally invasive/infiltrative, and this tendency is more concerning in the skull base, where a radical resection is often technically impossible because of the presence of vital neurovascular structures. We present a case of a 19-year-old woman who presented with a sudden onset of right facial weakness, progressively worsening to a severe disfiguring motor weakness. Gadolinium-enhanced brain magnetic resonance imaging showed an osteolytic lesion located in the right mastoid involving the stylomastoid foramen and the right seventh cranial nerve. A partial mastoidectomy was performed, with an excellent rate of tumor resection and complete local control of the disease at follow-up. The analysis of current literature indicates that a radiologic diagnosis is rarely strictly convincing of CMF. Histologic diagnosis is often difficult due to the lack of a specific immunohistochemical pattern of chondrosarcoma. Surgery is currently recognized as the mainstay to manage this lesion, although a trend toward adjuvant radiation therapy (RT) currently is seen. Although a tendency of local recurrence is well recognized in the literature, the very slow and indolent behavior of this lesion plus the trend to enhance local control of the disease with high-dose RT pushed us to a reappraise the role of radical skull base surgeries burdened by the risks of major complications, cosmetic deformities, and additional neurologic deficits.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Chondromyxoid Fibroma of the Skull Base: Our Experience with an Elusive Disease

D’Andrea¹,

Pesce²,

Trasimeni

et al. 2017

J Neurol Surg A Cent Eur Neurosurg

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“…7,13 A CMF tumor in the upper extremity is rare, but it has been described in the scapula, [14][15][16] rib, 17 sternum, 18 pelvis, 19 mandible, 20 and occiput. 21 A CMF of the scapula occurs in only 0.8% to 3% of all CMF cases. 14,22 A possibly related condition reported as ''snapping scapula'' results from a space-occupying lesion, typically on the ventral surface of the scapular fossa and proximal to the serratus anterior muscle, 23 although it has been observed in the elbow, pelvic ischial tuberosity, deltoid muscle, foot, ocular orbit, stomach, and inguinal region.…”

Section: Uniquenessmentioning

confidence: 98%