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Pre- and postoperative MRI scans of Case 1. (a) Preoperative MRI T2 sequence showing the tumour measuring 25 x 20 mm at the jugular foramen with infralabyrinthine extension causing erosion of the posterior semicircular canal. Note clear distinction between the tumour and cerebellum posteriorly. (b) Preoperative MRI T1 with gadolinium enhancement. (c) Intraoperative picture after extended mastoidectomy and neck dissection. The next step was anterior rerouting of the facial nerve, which gives required access to the jugular foramen. (d) Postoperative MRI T1 with gadolinium enhancement after 3 years of follow-up. Total tumour clearance and no evidences of recurrence could be confirmed.
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An MRI scan revealed a tumour measuring 25 x 20 mm at the right jugular foramen with extension into middle ear and mastoid, causing involvement of the mastoid segment of the facial nerve and erosion of the posterior semicircular canal (Figure 1).
Histopathological features of LGFMS. (a) Fibrous stroma containing myxoid parts and whorling spindle cells (H&E x 100) (b) Tumour cells show diffuse reactivity for MUC4. (c) LGFMS with alternating fibrous and myxoid areas (H&E x 100). (d) Tumour cells show strong cytoplasmic staining for MUC4.
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The definite diagnosis of LGFMS was established based on postoperative histopathology and immunohistochemistry (Figures 2(c) and 2(d)).
PMC6644311_02
Histopathological features of LGFMS. (a) Fibrous stroma containing myxoid parts and whorling spindle cells (H&E x 100) (b) Tumour cells show diffuse reactivity for MUC4. (c) LGFMS with alternating fibrous and myxoid areas (H&E x 100). (d) Tumour cells show strong cytoplasmic staining for MUC4.
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The definite diagnosis of LGFMS was established based on postoperative histopathology and immunohistochemistry (Figures 2(c) and 2(d)).
Pre- and postoperative CT and MRI scans of Case 2. (a) Preoperative CT scan showing calcifications within the tumour. (b) Preoperative MRI showing a large arachnoid cyst posteriorly to the tumour at the right cerebellopontine angle. Note remarkable compression of the cerebellum and brainstem caused by the cyst. (c, d) Preoperative MRI scans showing tumour masses in the right parapharyngeal space and at the projection of the temporal bone, respectively. (e, f) Postoperative MRI scans showing fat tissue at the right parapharyngeal space and at the projection of the temporal bone, respectively.
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MRI showed a large arachnoid cyst at the left cerebellopontine angle markedly displacing the brainstem (Figure 3(b)). The MRI scan also revealed two expansive lesions, one at the projection of the left temporal bone measuring 4.9 x 3.0 x 4.4 cm and another in the left parapharyngeal space measuring 4.2 x 2.5 x 3.7 cm (Figures 3(c) and 3(d)). Postoperative MRI and CT scans showed almost total tumour clearance except the small residual tumour at the level of the left jugular foramen (Figures 3(e) and 3(f)).