Thompson Lester D R, Penner Carla, Ho Ngoc J, Foss Robert D, Miettinen Markku, Wieneke Jacqueline A, Moskaluk Christopher A, Stelow Edward B
Department of Pathology, Southern California Permanente Medical Group, Woodland Hills Medical Center, 5601 De Soto Avenue, Woodland Hills, CA, 91365, USA,
Head Neck Pathol. 2014 Mar;8(1):88-109. doi: 10.1007/s12105-013-0487-3. Epub 2013 Sep 15.
Primary sinonasal tract and nasopharyngeal adenoid cystic carcinomas (STACC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. Eighty-six cases of STACC included 45 females and 41 males, aged 12-91 years (mean 54.4 years). Patients presented most frequently with obstructive symptoms (n = 54), followed by epistaxis (n = 23), auditory symptoms (n = 12), nerve symptoms (n = 11), nasal discharge (n = 11), and/or visual symptoms (n = 10), present for a mean of 18.2 months. The tumors involved the nasal cavity alone (n = 25), nasopharynx alone (n = 13), maxillary sinus alone (n = 4), or a combination of the nasal cavity and paranasal sinuses (n = 44), with a mean size of 3.7 cm. Patients presented equally between low and high stage disease: stage I and II (n = 42) or stage III and IV (n = 44) disease. Histologically, the tumors were invasive (bone: n = 66; neural: n = 47; lymphovascular: n = 33), composed of a variety of growth patterns, including cribriform (n = 33), tubular (n = 16), and solid (n = 9), although frequently a combination of these patterns was seen within a single tumor. Pleomorphism was mild with an intermediate N:C ratio in cells containing hyperchromatic nuclei. Reduplicated basement membrane and glycosaminoglycan material was commonly seen. Necrosis (n = 16) and atypical mitotic figures (n = 11) were infrequently present. Pleomorphic adenoma was present in 9 cases; de-differentiation was seen in two patients. Immunohistochemical studies showed positive reactions for pan-cytokeratin, CK7, CK5/6, CAM5.2, and EMA, with myoepithelial reactivity with SMA, p63, calponin, S100 protein and SMMHC. CD117, CEA, GFAP and p16 were variably present. CK20 and HR HPV were negative. STACC needs to be considered in the differential diagnosis of most sinonasal malignancies, particularly poorly differentiated carcinoma, olfactory neuroblastoma and pleomorphic adenoma. Surgery (n = 82), often accompanied by radiation therapy (n = 36), was generally employed. A majority of patients developed a recurrence (n = 52) 2-144 months after initial presentation. Overall mean follow-up was 19.4 years (range 0.4-37.5 years): 46 patients died with disease (mean 6.4 years); 5 were alive with disease (mean 5.4 years), and 35 patients were either alive or had died of unrelated causes (mean 16.3 years). ACC of the SNT is uncommon. Recurrences are common. The following parameters, when present, suggest an increased incidence of either recurrence or dying with disease: mixed site of involvement, high stage disease (stage IV), skull base involvement, tumor recurrence, a solid histology, perineural invasion, bone invasion, and lymphovascular invasion.
原发性鼻窦和鼻咽腺样囊性癌（STACC）是罕见肿瘤，常被误诊，导致临床处理不当。86例STACC患者中，女性45例，男性41例，年龄12 - 91岁（平均54.4岁）。患者最常见的症状为阻塞性症状（n = 54），其次是鼻出血（n = 23）、听觉症状（n = 12）、神经症状（n = 11）、鼻分泌物增多（n = 11）和/或视觉症状（n = 10），症状平均持续18.2个月。肿瘤仅累及鼻腔（n = 25）、仅累及鼻咽（n = 13）、仅累及上颌窦（n = 4）或鼻腔和鼻窦联合受累（n = 44），平均大小为3.7 cm。疾病分期为低期和高期的患者数量相当：I期和II期（n = 42）或III期和IV期（n = 44）。组织学上，肿瘤具有侵袭性（骨侵袭：n = 66；神经侵袭：n = 47；淋巴管侵袭：n = 33），由多种生长模式组成，包括筛状（n = 33）、管状（n = 16）和实性（n = 9），尽管单个肿瘤内常可见这些模式的组合。多形性较轻，细胞核深染的细胞N:C比值中等。常见基底膜重复和糖胺聚糖物质。坏死（n = 16）和非典型有丝分裂象（n = 11）较少见。9例存在多形性腺瘤；2例患者出现去分化。免疫组化研究显示，泛细胞角蛋白、CK7、CK5/6、CAM5.2和EMA呈阳性反应，肌上皮对SMA、p63、钙调蛋白、S100蛋白和SMMHC有反应。CD117、CEA、GFAP和p16呈不同程度表达。CK20和HR HPV均为阴性。大多数鼻窦恶性肿瘤的鉴别诊断中都需要考虑STACC，尤其是低分化癌、嗅神经母细胞瘤和多形性腺瘤。通常采用手术治疗（n = 82），常联合放疗（n = 36）。大多数患者在初次就诊后2 - 144个月出现复发（n = 52）。总体平均随访19.4年（范围0.4 - 37.5年）：46例患者死于疾病（平均6.4年）；5例患者带瘤生存（平均5.4年），35例患者存活或死于非相关原因（平均16.3年）。鼻窦的腺样囊性癌并不常见。复发很常见。出现以下参数时，提示复发或死于疾病的发生率增加：多部位受累、高分期疾病（IV期）、颅底受累、肿瘤复发、实性组织学、神经周围侵袭、骨侵袭和淋巴管侵袭。
