Abraham Zephania Saitabau, Ngimba Caroline Philip, Malango Atuganile Edward, Mrema Alita, Saleh Mwajabu Athman, Vuhahula Edda
Department of Surgery, School of Medicine and Dentistry, University of Dodoma, Dodoma, Tanzania.
Department of Anatomical Pathology, Muhimbili National Hospital, Dar es Salaam, Tanzania.
Int J Surg Case Rep. 2022 Aug;97:107462. doi: 10.1016/j.ijscr.2022.107462. Epub 2022 Jul 28.
HPV-related carcinoma with adenoid cystic carcinoma-like features currently known as HPV-related multiphenotypic sinonasal carcinoma (HMSC) is a rare distinct head and neck high-risk HPV-related carcinoma. The high-risk HPV subtypes implicated are particularly type 33,35 and 56. So far this is the first reported rare case of a variant of sinonasal carcinoma in Tanzania.
We present a 59-year old female who presented with a history of right-sided nasal obstruction and intermittent epistaxis for about one year and later on had ipsilateral proptosis. A provisional diagnosis of advanced right-sided malignant sinonasal tumor was made. Trans nasal biopsy revealed HMSC.
The patient had a right sided fixed neck mass that measured about 7.5 × 8.2 cm. Magnetic resonance imaging (MRI) of the paranasal sinuses revealed a T1 weighted contrasted images that showed a huge extensive enhancing mass (estimated to measure 4.5 × 4.4) involving the nasal cavity, bilateral ethmoid sinuses, frontal and sphenoid sinuses and also the tumor exhibit intracranial extension (anterior cranial fossa) and tend to compress medial walls of both orbits though more marked on the right side. Histopathology and immunohistochemistry confirmed HPV-related multiphenotypic sinonasal carcinoma. The disease was staged to be T4bN3aM0 and the patient was referred for palliative chemoradiation.
Although HMSC presents at advanced stage in close to half of cases and has a high-grade histological appearance it paradoxically exhibits a relatively indolent manner with frequent local recurrences. Prompt histopathological diagnosis is important to prevent metastases and HMSC-related deaths.
具有腺样囊性癌样特征的人乳头瘤病毒（HPV）相关癌，目前被称为HPV相关多表型鼻窦癌（HMSC），是一种罕见的、独特的头颈部高危HPV相关癌。涉及的高危HPV亚型主要是33、35和56型。到目前为止，这是坦桑尼亚首次报道的鼻窦癌变异型罕见病例。
我们报告一名59岁女性，有右侧鼻塞和间歇性鼻出血病史约一年，后来出现同侧眼球突出。初步诊断为晚期右侧鼻窦恶性肿瘤。经鼻活检显示为HMSC。
患者右侧颈部有一个固定肿块，大小约为7.5×8.2厘米。鼻窦磁共振成像（MRI）的T1加权对比图像显示，有一个巨大的广泛强化肿块（估计大小为4.5×4.4），累及鼻腔、双侧筛窦、额窦和蝶窦，肿瘤还向颅内延伸（前颅窝），并倾向于压迫双侧眼眶内侧壁，右侧更为明显。组织病理学和免疫组织化学证实为HPV相关多表型鼻窦癌。该疾病分期为T4bN3aM0，患者被转诊接受姑息性放化疗。
尽管近一半的HMSC病例在晚期出现，且组织学表现为高级别，但矛盾的是，它表现出相对惰性的行为，局部复发频繁。及时的组织病理学诊断对于预防转移和HMSC相关死亡很重要。
