鼻窦腺样囊性癌:一种休眠但好斗的肿瘤- 1例报告

Chenchulakshmi Vasudevan, P. Apoorva, Adarsh C. Sanikop
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摘要

腺样囊性癌,也被称为筛状瘤或柱状瘤,由于其细胞排列或起源于组织类型,是罕见的,并表现出神经周围侵袭性作为一个病理特征,使治疗具有挑战性。已知该肿瘤起源于唾液腺,很少见于鼻腔或鼻窦。我们在此报告一个罕见的ACC发生在其通常前提之外的病例。男性患者,32岁,表现为单侧鼻塞和鼻出血,检查发现右鼻腔的生长特别起源于鼻底粘膜下,向后延伸至鼻窦,未累及鼻甲或鼻中隔。行DNE活检及CECT PNS进一步评估,肿块诊断为III期鼻窦腺样囊性癌。这种肿瘤起源于小唾液腺,据报道,它在鼻子和鼻窦的表现非常稀少。这可能是它在日常实践中被忽视的原因,因为它的临床特征与鼻和副鼻窦的几种炎症性和肿瘤性疾病相似。组织病理学筛状和管状亚型比实状亚型侵袭性小,并决定预后。这是一种六七十岁的疾病,生长缓慢,局部侵袭,在像我们这样的年轻病人身上发现它非常罕见。ACC虽然不常见,但不能漏诊,必须与普通鼻肿块鉴别。耳鼻喉科医生需要与病理学家和肿瘤学家合作,以多方面的方法准确诊断和治疗这种好战的肿瘤。完全手术切除和术后放疗是最被接受的治疗方案
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A dormant yet belligerent tumour, the sinonasal adenoid cystic carcinoma- A case report
Adenoid cystic carcinoma, also known as cribriform tumour or Cylindroma owing to the cellular arrangement or type of tissue that it originates from, is rare and shows perineural invasiveness as a pathognomic feature making treatment challenging. The tumour is known to arise in the salivary glands and seldom seen in the nasal cavity or paranasal sinuses. We here present a rare case of ACC occurring beyond its usual premise.A male patient aged 32 presented with unilateral nasal obstruction and epistaxis and on examination a growth in the right nasal cavity was noted to peculiarly arise from beneath the mucosa of the nasal floor, extending posteriorly up to the choana, not involving the turbinates or septum. DNE with biopsy was done and CECT PNS was done for further evaluation and the mass was diagnosed to be stage III sinonasal adenoid cystic carcinoma.This tumour originates from minor salivary glands and its presentation in the nose and paranasal sinuses has been reported to be very sparse. This is perhaps a reason for it to be missed out in daily practice due to clinical features being similar to several inflammatory and neoplastic diseases of the nose and paranasal sinuses. Histopathological cribriform and tubular subtypes are less aggressive than solid form and that determine the prognosis. It is a disease of 6th and 7th decade, slow growing and locally invasive very unusual to find it in a young patient such as ours.Though uncommon the ACC mustn’t go undiagnosed and must be differentiated from commoner nasal masses. Otorhinolaryngologists need to work in collaboration with pathologists and oncologists to accurately diagnose and treat this belligerent tumour in a multifaceted approach. Complete surgical excision with post-operative radiotherapy is the most accepted treatment plan
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