Intracranially Extended Sinonasal Undifferentiated Carcinoma: A Case Report and Literature Review

Abstract

BACKGROUND Sinonasal undifferentiated carcinomas (SNUC) are highly malignant and rare lesions. Therapeutic efforts often provide frustrating results. Their course is characterized by indolent progression, until it culminates in extensive local infiltration of adjacent anatomical structures or cervical lymphadenopathy in approximately one-third of patients upon admission. It most frequently affects males, with a sex ratio of 3: 1. The age at manifestation tends to be about 40-50 years. CASE REPORT We report the case of a 41-year-old man with intracranial expansion of SNUC. Two previous sinus surgeries were performed endoscopically because the lesion at that moment was exclusively located endonasally. Within the last few months, he had been having persistent headaches. Magnetic resonance imaging (MRI) revealed an anterior cranial fossa lesion. Therefore, he underwent a bifrontal craniotomy and excision of the space-occupying lesion (SOL). The osseous defect of the skull base was covered with a titanium mesh. Finally, we performed a duraplasty using a pericranial flap and fat tissue taken from his abdomen. Postoperatively, his wound was dehisced. We proceeded then to a frontal craniectomy with surgical debridement, subgaleal empyem and epidural abscess removal, and copious irrigation with oxygen peroxide. Enterococcus spp. were isolated from pus cultures. Despite receiving bacteria-focused antibiotics, he unfortunately developed sepsis and died. The histopathologic findings revealed a SNUC, which is the criterion standard for diagnosis. CONCLUSIONS Multimodal treatment offers the best prognosis to patients with SNUC. Combined operations by otolaryngologists and neurosurgeons provide the necessary radicality. There is high risk of wound healing disorders, especially when local irradiation had been administered.

Figure 1.

(A) CT scan of head with contrast medium axial slice: Infiltration of left frontal lobe. (B) Coronal slice: Impaired endonasal structures due to previous surgeries. Intracranially extended SNUC-perilesional edema. Red arrow: Lesion arising from ethmoidal cells and infiltrating dura matter.

Figure 2.

(A) T1-weighted sequence: Contrast medium enhancing lesion arising from ethmoidal cells. Red arrow indicates the lesion. (B) T1-weighted sequence: Intracranial expansion of the lesion adjacent to inner orbital wall and left orbital gyri. Red arrow indicates the lesion. (C) T2-weighted sequence: Isointense tumor abutting ethmoidal bone. Red arrow indicates the tumor. (D) T2-weighted sequence: Infiltration of left orbital gyri; arachnoidal spaces are not depicted due to parenchyma’s edema. Red arrow indicates the tumor. (E) FLAIR sequence: Vertical extension of vasogenic edema up to the ipsilateral frontal ventricular horn. Red arrow indicates the edema.

Figure 3.

CT scan of head, coronal slice: Postoperative control-tumor resection and titanium mesh for skull base reconstruction. Red arrow: Titanium mesh covering upper nasal and (partially) orbital wall defect.

Figure 4.

CT scan with contrast medium: Axial slice-subgaleal empyema and epidural abscess-no parenchymal infectious collection. Red stars: Subcutaneous fluid collection with heterogeneous contrast enhancement.

Figure 5.

(A) H-E x100 Cuboidal cells with increased nuclear-cytoplasmic ratio. (B) H-E ×400A Round and oval nuclei with nucleoli and moderate to increased pleomorphism and atypia. (C1) H-E ×100 densely arranged neoplasmatic cell with extended necrosis. (C2) GFAP ×400 glial cells lie at the margin of tumor specimen. (D1). CK7 ×100 cells positive in CK7 stain. (D2) CK 8/18 ×200 cells positive in CK8/18 stain.