Department of Neurology, BP-Beneficência Portuguesa Hospital of São Paulo, São Paulo, Brazil.
Department of Interventional Neuroradiology, BP-Beneficência Portuguesa Hospital of São Paulo, São Paulo, Brazil.
Spinal Cord Ser Cases. 2022 Jul 1;8(1):64. doi: 10.1038/s41394-022-00532-8.
The sinonasal carcinoma are rare tumors of the head and neck. The undifferentiated sinonasal carcinoma subtypes are constantly being explored and new mutations, with different prognosis markers and biological behaviors are being described. The SMARCB1 negative sinonasal carcinoma subtypes have been recently described with few reports of leptomeningeal and spinal cord invasion.
This study presents the case of a 59-year-old woman, with no previous disease, presenting initially with epistaxis that evolved to cranial nerve deficits and a left eye complete oftalmoplegia. After diagnostic investigation, she had a diagnosis of a left ethmoid sinus sinonasal carcinoma. Following resection of the tumor, she evolved with a right foot drop that eventually has been linked to diffuse spinal cord impairment. The histopathological diagnosis confirmed a SMARCB1 negative sinonasal carcinoma. Due to the diffuse metastasis, she underwent palliative care and died eight months after the surgery.
Spinal cord metastasis may manifest with different clinical signs. Our case shows a rare manifestation of SMARCB1-deficient sinonasal carcinoma, a new subtype of sinonasal carcinoma, summarizing the importance of a high grade of suspicion of spinal cord invasion on these patients. SMARCB1 sinonasal carcinomas are rare new tumors of the head and neck, whose biological behaviors are yet to be explored. To the best of our knowledge, this is one of the few case reports describing simultaneous spread of this tumor to the central nervous system and spinal cord.
鼻窦癌是头颈部罕见的肿瘤。未分化型鼻窦癌亚型不断被探索,具有不同预后标志物和生物学行为的新突变也在不断被描述。SMARCB1 阴性鼻窦癌亚型最近被描述,其脑膜和脊髓侵犯的报道较少。
本研究报告了一例 59 岁女性患者,无既往病史,最初表现为鼻出血,随后出现颅神经功能障碍和左眼完全眼肌麻痹。经过诊断性检查,诊断为左侧筛窦鼻窦癌。肿瘤切除后,她出现右脚下垂,最终与弥漫性脊髓损伤有关。组织病理学诊断证实为 SMARCB1 阴性鼻窦癌。由于广泛转移,她接受了姑息治疗,并在手术后 8 个月死亡。
脊髓转移可能表现出不同的临床症状。我们的病例显示了一种罕见的 SMARCB1 缺陷型鼻窦癌表现,这是一种新的鼻窦癌亚型,强调了对这些患者脊髓侵犯的高度怀疑的重要性。SMARCB1 鼻窦癌是头颈部罕见的新肿瘤,其生物学行为仍有待探索。据我们所知,这是少数几例同时发生该肿瘤向中枢神经系统和脊髓扩散的病例报告之一。