Merzouqi B, Halily S, Oukessou Y, Regragui M, Abada R, Mahtar M
Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Casablanca University Hospital, Casablanca, Morocco.
Department of Oto-Rhino-Laryngology, Head and Neck Surgery, Casablanca University Hospital, Casablanca, Morocco.
Int J Surg Case Rep. 2021 Feb;79:193-197. doi: 10.1016/j.ijscr.2021.01.033. Epub 2021 Jan 15.
Plasmacytoma is a rare clonal neoplastic disorder of bone marrow that originates from plasma cells. It usually presents as a multiple myeloma (MM). Less than 5% of patients present with either a single bone lesion as a solitary bone plasmacytoma (SBP) or, even more rarely, as a soft tissue mass of monoclonal plasma cells representing a solitary extra medullary plasmacytoma (SEP).
We report a case of a 59-year-old man presenting with a mass of the soft palate evolving for a year. Physical examination showed an extension to the nasal cavity. Biopsy with immunohistochemical study demonstrated sheets of mononucleated plasmacytoid cells diffusely expressing CD138. The plasma cells showed monoclonal light chain Kappa. Further investigations did not show any other locations including bone and bone marrow. Thus, diagnosis of solitary extramedullary plasmacytoma of the soft palate was established. The patient was treated with chemotherapy with total remission on his one year follow-up.
SEP may arise in any organ, either as a primary tumor or as part of a MM. Almost 90% of SEP arise in the head and neck, especially in the upper respiratory tract. Primary treatment for most patients is radiotherapy, but surgery may also be required, and multidisciplinary decision between surgeon, hematologist and radiotherapist is crucial for planning optimum care.
SEP is an extremely rare condition which requires diagnostic and therapeutic management in the same level of MM. Prognosis is better than the two other forms (MM and SBP).
浆细胞瘤是一种罕见的起源于浆细胞的骨髓克隆性肿瘤性疾病。它通常表现为多发性骨髓瘤(MM)。不到5%的患者表现为单个骨病变,即孤立性骨浆细胞瘤(SBP),或更罕见的情况,表现为单克隆浆细胞的软组织肿块,即孤立性髓外浆细胞瘤(SEP)。
我们报告一例59岁男性,软腭肿物已发展一年。体格检查显示肿物已延伸至鼻腔。活检及免疫组化研究显示成片的单核浆细胞样细胞弥漫性表达CD138。浆细胞显示单克隆轻链κ。进一步检查未发现包括骨骼和骨髓在内的任何其他部位有病变。因此,确诊为软腭孤立性髓外浆细胞瘤。患者接受化疗,随访一年时完全缓解。
SEP可发生于任何器官,可为原发性肿瘤或作为MM的一部分。几乎90%的SEP发生于头颈部,尤其是上呼吸道。大多数患者的主要治疗方法是放疗,但也可能需要手术,外科医生、血液科医生和放疗科医生之间的多学科决策对于规划最佳治疗至关重要。
SEP是一种极其罕见的疾病,需要与MM相同水平的诊断和治疗管理。其预后优于其他两种类型(MM和SBP)。