Vilarello Brandon J, Jacobson Patricia T, Gudis David A, Overdevest Jonathan B
Vagelos College of Physicians and Surgeons, Columbia University, New York, USA.
Department of Otolaryngology-Head and Neck Surgery, Columbia University Irving Medical Center, New York, USA.
Cureus. 2024 Apr 12;16(4):e58132. doi: 10.7759/cureus.58132. eCollection 2024 Apr.
B-cell lymphoblastic lymphoma (B-LBL) is an abnormal proliferation of lymphocyte precursor cells located primarily outside of the bone marrow and peripheral blood, typically in the mediastinum or other lymph nodes. It is often a disease of childhood that presents with lymphadenopathy, fatigue, pallor, bone pain, and weight loss with laboratory findings of anemia and thrombocytopenia. Initial presentations prompted by head and neck manifestations are exceedingly rare. A five-year-old girl with no significant past medical history presented with right facial swelling and mild proptosis on ophthalmologic evaluation. She was referred to a tertiary care facility by her local otolaryngologist for further management after computed tomographic imaging revealed right maxillary sinus opacification and erosion of the anterior maxillary bone. Her symptoms were initially responsive to prednisone and amoxicillin-clavulanate, and only right unilateral nasal discharge persisted with a near-complete resolution of other sinonasal symptoms. Notably, laboratory values, including complete blood count, were within normal limits. Given concern for the etiology of the bony erosion, the patient presented for a second opinion, where imaging and biopsy resulted in flow cytometry findings consistent with B-ALL/LBL. After a bone marrow biopsy, the ultimate diagnosis was Murphy's stage III B-cell lymphoblastic lymphoma. Malignant neoplasms of the sinonasal region are rare in children, where primary sinonasal B-LBL is a unique occurrence. Clinical features of sinonasal B-LBL in the paranasal sinuses may masquerade as pathologies such as acute sinusitis, orbital cellulitis, and benign tumors or polyps that can lead to a confounding diagnosis. In this case presentation, an initial response to steroids and antibiotics should not provide false reassurance when other features and signs, such as maxillary bone erosion, may suggest the presence of malignancy.
B 细胞淋巴母细胞淋巴瘤（B-LBL）是淋巴细胞前体细胞的异常增殖，主要发生于骨髓和外周血之外，通常位于纵隔或其他淋巴结。它常为儿童疾病，表现为淋巴结病、疲劳、面色苍白、骨痛和体重减轻，实验室检查可见贫血和血小板减少。由头颈部表现引发的初始症状极为罕见。一名既往无重大病史的 5 岁女孩出现右面部肿胀，眼科检查时有轻度眼球突出。计算机断层扫描成像显示右上颌窦混浊及上颌骨前部侵蚀后，她被当地耳鼻喉科医生转诊至三级医疗机构进行进一步治疗。她的症状最初对泼尼松和阿莫西林 - 克拉维酸有反应，仅右单侧鼻分泌物持续存在，其他鼻窦症状几乎完全缓解。值得注意的是，包括全血细胞计数在内的实验室检查值均在正常范围内。鉴于对骨侵蚀病因的担忧，患者寻求第二种意见，在此处成像和活检的流式细胞术结果与 B-ALL/LBL 一致。骨髓活检后，最终诊断为墨菲 III 期 B 细胞淋巴母细胞淋巴瘤。鼻窦区域的恶性肿瘤在儿童中很少见，原发性鼻窦 B-LBL 是一种独特的情况。鼻窦 B-LBL 在鼻窦的临床特征可能会伪装成诸如急性鼻窦炎、眼眶蜂窝织炎以及良性肿瘤或息肉等病变，从而导致诊断混淆。在这个病例中，当其他特征和体征，如上颌骨侵蚀，可能提示存在恶性肿瘤时，对类固醇和抗生素的初始反应不应让人产生错误的安心感。
