Albaldawy Mazin J, Ebaied Tamer, Bin Harmal Al Shamsi Humaid O, Nadaf Ashraf, Faruk Abbu Rahil U
Department of Oncology, Al Dhannah Hospital, Al Dhannah, ARE.
Department of ENT (Ear, Nose and Throat), Al Dhannah Hospital, Al Dhannah, ARE.
Cureus. 2024 Dec 21;16(12):e76161. doi: 10.7759/cureus.76161. eCollection 2024 Dec.
Pediatric-type follicular lymphoma (PTFL) is an extremely rare B-cell lymphoma that primarily affects children and young adults, typically in individuals under 25 years old, with a median age of 15 years. Here, we report a rare case of PTFL in a 27-year-old adult male who presented with a slow-growing mass near his left ear. Initial CT scans of the neck revealed two oval-shaped, smooth, well-defined, homogeneously enhancing soft tissue density lesions in the superficial lobe of the left parotid gland. A complete surgical excision was performed, and a postoperative ultrasound of the neck confirmed complete removal, with only hypoechoic changes observed in the parotid gland. Comprehensive immunohistochemical analysis showed CD20-positive B cells co-expressing germinal center markers CD10 and BCL6 but negative for BCL2 and cyclin D1. The Ki-67 proliferation index was notably elevated, indicating high cellular activity. Additional findings included MEF2B positivity, confirming the lymphoma diagnosis, and an immunoglobulin gene rearrangement, which demonstrated a monoclonal B-cell population consistent with a neoplastic process. CD21 staining further revealed distorted follicular dendritic cell networks and attenuated IgD-positive mantle. Fluorescence in situ hybridization (FISH) analysis showed no rearrangements in BCL2, IRF4, or BCL6, and any deletion in the 1p36 region (TNFRSF14 gene), ruling out other lymphoma types. Histologically, the nodules showed distorted secondary follicles with obscured germinal centers, confirming PTFL. At the three-month follow-up, the patient demonstrated satisfactory healing with no signs of recurrence. This case underscores the importance for oncologists to perform a thorough differential diagnosis of head and neck masses, as PTFL can present with characteristics similar to classical follicular lymphoma, IRF4-rearranged large B-cell lymphoma, pediatric nodal marginal zone lymphoma, and reactive follicular hyperplasia. A comprehensive diagnostic approach, including clinical, pathological, and immunohistochemical analyses, is essential for developing an accurate diagnosis and management plan for PTFL, especially in atypical adult presentations.
儿童型滤泡性淋巴瘤(PTFL)是一种极其罕见的B细胞淋巴瘤,主要影响儿童和年轻成人,通常为25岁以下个体,中位年龄为15岁。在此,我们报告一例罕见的PTFL病例,患者为一名27岁成年男性,左耳附近出现缓慢生长的肿块。颈部初始CT扫描显示左腮腺浅叶有两个椭圆形、边界光滑、清晰、均匀强化的软组织密度病变。进行了完整的手术切除,术后颈部超声证实已完全切除,仅腮腺出现低回声改变。综合免疫组化分析显示CD20阳性B细胞共表达生发中心标志物CD10和BCL6,但BCL2和细胞周期蛋白D1为阴性。Ki-67增殖指数显著升高,表明细胞活性高。其他发现包括MEF2B阳性,证实了淋巴瘤诊断,以及免疫球蛋白基因重排,显示与肿瘤过程一致的单克隆B细胞群体。CD21染色进一步显示滤泡树突状细胞网络扭曲和IgD阳性套区变薄。荧光原位杂交(FISH)分析显示BCL2、IRF4或BCL6无重排,1p36区域(TNFRSF14基因)无任何缺失,排除了其他淋巴瘤类型。组织学上,结节显示次级滤泡扭曲,生发中心模糊,确诊为PTFL。在三个月的随访中,患者愈合情况良好,无复发迹象。该病例强调了肿瘤学家对头颈部肿块进行全面鉴别诊断的重要性,因为PTFL可能表现出与经典滤泡性淋巴瘤、IRF4重排的大B细胞淋巴瘤、儿童淋巴结边缘区淋巴瘤和反应性滤泡增生相似的特征。对于PTFL,尤其是非典型成人病例,包括临床、病理和免疫组化分析在内的综合诊断方法对于制定准确的诊断和管理计划至关重要。