Atypical cartilaginous tumor/chondrosarcoma, grade 1, of the mastoid in three family members: A new entity

Abstract

We present a case series of a family with three members having cartilaginous tumors of the mastoid. All patients presented between the ages of 9 to 12 years with acute onset facial nerve paralysis. Histologic analysis of all tumors showed similar features, consistent with atypical cartilaginous tumors/chondrosarcoma, low-grade. Conventional cytogenetic analysis performed on one of the sons' tumor showed no evidence of chromosomal abnormality. High-resolution array comparative genomic hybridization performed on the same patient's blood also showed no unbalanced chromosomal abnormality. This is the first report of family members with this unusual combination of clinical, radiologic, and histologic finding. Laryngoscope, 126:E310-E313, 2016.

A rare case of chondrosarcoma arising in parotid with extension to lateral skull base

“…However, there was a lack of the characteristic whorls or soap-bubble like appearance seen on T2-weighted and T1 postcontrast9 in our case. Histologically, these tumours demonstrate a hyaline cartilage neoplasm with mild to moderate cellularity, areas of myxoid change and focal host bone entrapment 9…”

“…Radiographically, chondrosarcomas present with bony scalloping, contrast enhancement or matrix calcification on CT. On MRI, there is typically T1 hypointensity, T2 hyperintensity and postcontrast enhancement9 as was seen in our case. However, there was a lack of the characteristic whorls or soap-bubble like appearance seen on T2-weighted and T1 postcontrast9 in our case. Histologically, these tumours demonstrate a hyaline cartilage neoplasm with mild to moderate cellularity, areas of myxoid change and focal host bone entrapment 9…”

Chondrosarcoma Arising in the Mastoid Involving the Intratemporal Facial Nerve

“…Most intratemporal facial nerve tumors are schwannomas or hemangiomas, with malignant tumors being rare. A chondrosarcoma in the mastoid had previously been reported, but it was separate from the facial nerve and caused only facial nerve edema . This patient’s lesion masqueraded as a schwannoma owing to its intimate involvement with the facial nerve.…”

“…A chondrosarcoma in the mastoid had previously been reported, but it was separate from the fac ial ner ve and c aused only fac ial ner ve edema. 4 This patient's lesion masqueraded as a schwannoma owing to its intimate involvement with the facial nerve. G ive n t h e ex p a n s i o n o f t h e a re a s u r ro u n d i ng t h e facial nerve, the tumor's route of spread along the nerve, and replacement of a large segment of the nerve in the mastoid, it is possible that the tumor began in the fallopian canal.…”

An activating germline IDH1 variant associated with a tumor entity characterized by unilateral and bilateral chondrosarcoma of the mastoid

“…The family in this report has been previously described. 9 The 9-year-old male proband (individual II-1, Figure 1 A) presented with a history of bilateral conductive hearing loss and had developed right facial paresis. Computed tomography (CT) revealed a mass involving the mastoid ( Figure 1 B), and the proband underwent a right tympanomastoidectomy.…”