Temporal bone squamous cell carcinoma: Aggressive behavior coursing with cerebellar invasion and hydrocephalus

Abstract

Background: Temporal bone squamous cell carcinoma (TBSCC) is a very rare condition. The prognosis is dismal for advanced tumors. Due to its rarity, information in the literature is scarce. Here, we report a unique case of TBSCC with cerebellar invasion and hydrocephalus.

Case description: A 46-year-old reported right-sided hearing loss and a painful right retroauricular mass for 4 months. Magnetic resonance imaging revealed a 8.7 × 7.6 × 6.4 cm mass invading the right temporal and occipital bones. After a biopsy and 3 surgical procedures over 6 months, the diagnosis of TBSCC was obtained. Due to invasion of the cerebellar tissue and obstructive hydrocephalus, a ventriculoperitoneal shunt was performed. The patient was referred for adjuvant radiotherapy. However, palliative care was initiated due to tumor progression.

Conclusion: We report a case of advanced TBSCC with poor prognosis despite surgical treatment and radiotherapy. More data are necessary to provide new and better treatment to these patients.

Keywords: Cerebellum; Hydrocephalus; Squamous cell carcinoma; Temporal bone.

Figure 1:

The patient exhibited multiple subepidermal keratin cysts that appeared as small, firm white papules in varying numbers, consistent with milia.

Figure 2:

First magnetic resonance imaging of the brain showing a huge lesion in the temporal bone. (a) Axial T1-weighted with contrast exhibiting temporal bone mass with peripheral contrast enhancement – right side. (b) Axial T2-weighted image shows hyperintense signal abnormality in the mastoid part of the temporal bone. The occipital bone was invaded. (c) Contrast-enhanced axial T1-weighted showing a large temporal bone tumor on the right side. (d) The mass was predominantly hyperintense on the T2-weighted image.

Figure 3:

Contrast-enhanced axial T1-weighted brain magnetic resonance imaging shows temporal bone tumor invading the cerebellar tissue with dislocation of the IV ventricle.

Figure 4:

Microsurgical resection of the tumor invading the cerebellar tissue. (a) The tumor was identified after the dural opening. (b) Circumferential dissection between the tumor and normal cerebellum parenchyma. (c) Tumor removal using a microdissector.

Figure 5:

Temporal bone tumor samples: (a) Hematoxylin and Eosin (H&E) stain (×200). Squamous epithelium with some maturation, keratin sheets with parakeratosis, sometimes forming horny pearls. (b) H&E stain (×400). Foci of preserved squamous epithelium showing areas with enlarged nuclei, but no signs of stromal invasion. (c) H&E stain (×100). Sample from the cerebellar region. Squamous epithelium presenting poorly cohesive cells with moderate to marked pleomorphism, superficially infiltrating the cerebellar cortex. (d) H&E stain (×400). Sample from the dural region. Squamous epithelium with an enlarged and irregular nucleus, and frequent atypical mitotic figures infiltrating fibroconnective tissue.

Figure 6:

Comparison between the first brain magnetic resonance imaging (MRI) (fluid-attenuated inversion recovery [FLAIR] sequence) and head computed tomography (CT) after symptoms of hydrocephalus. (a) Preoperative MRI-FLAIR shows a normal lateral ventricle without radiological signs of hydrocephalus. Normal anatomical variation is observed: Cavum septum pellucidum. (b) Head CT showing enlargement of the lateral ventricles and transependymal edema: Low-density change on CT around the margins of the ventricles.

Figure 7:

Final appearance of the wound after four attempts at tumor resection and adjuvant radiotherapy. The surgical wound is open and the patient was referred for palliative care.