Pyon Rachel E, Wang Grace C, Chu Yan, Tulpule Sunil
Department of Internal Medicine, Saint Louis University School of Medicine, Saint Louis, USA.
Department of Hospital Medicine, John Cochran VA Medical Center, Saint Louis, USA.
Cureus. 2022 Jun 23;14(6):e26269. doi: 10.7759/cureus.26269. eCollection 2022 Jun.
Orbital plasmacytomas are uncommon soft-tissue plasma cell neoplasms that are associated with a monoclonal or myeloma protein. There are four types of plasma cell neoplasms: multiple myeloma (MM), monoclonal gammopathy of undetermined significance (MGUS), amyloidosis, and plasmacytoma. Plasmacytomas may be classified as medullary, occurring only within the bone, or extramedullary, occurring in soft tissues. Orbital plasmacytomas are extramedullary manifestations associated with MM and they may present with signs and symptoms such as unilateral proptosis, conjunctival injection, ocular pain, diplopia, and vision changes. The diagnosis of orbital plasmacytomas is based on tissue biopsy and histologic and immunohistochemical confirmation of a homogenous infiltrate of monoclonal plasma cells. In this report, we present a case of a 60-year-old female patient with a prior diagnosis of MM and new-onset bilateral orbital plasmacytomas following an autologous peripheral blood stem cell transplant; her condition improved significantly following treatment with dexamethasone, cisplatin, doxorubicin, cyclophosphamide, and etoposide along with palliative radiation therapy (RT) of 2000 cGy in 10 fractions to the orbits. Unfortunately, three months later, she had progression of extramedullary disease with parotid gland involvement. She had multiple complicated hospitalizations and eventually expired. As patients with orbital plasmacytomas classically have lower remission and survival rates compared to those with extramedullary plasmacytomas involving other locations, they must be considered high-risk patients who require a multidisciplinary approach for early diagnosis and timely treatment in order to prevent disease progression and to alleviate symptoms related to the disease.
眼眶浆细胞瘤是一种罕见的软组织浆细胞肿瘤，与单克隆或骨髓瘤蛋白相关。浆细胞肿瘤有四种类型：多发性骨髓瘤（MM）、意义未明的单克隆丙种球蛋白病（MGUS）、淀粉样变性和浆细胞瘤。浆细胞瘤可分为仅发生于骨内的髓质型或发生于软组织的髓外型。眼眶浆细胞瘤是与MM相关的髓外表现，可出现单侧眼球突出、结膜充血、眼痛、复视和视力改变等体征和症状。眼眶浆细胞瘤的诊断基于组织活检以及单克隆浆细胞均匀浸润的组织学和免疫组化证实。在本报告中，我们介绍了一例60岁女性患者，此前诊断为MM，在自体外周血干细胞移植后新发双侧眼眶浆细胞瘤；经地塞米松、顺铂、阿霉素、环磷酰胺和依托泊苷治疗，以及眼眶姑息性放射治疗（RT）2000 cGy分10次进行后，她的病情显著改善。不幸的是，三个月后，她出现了累及腮腺的髓外疾病进展。她多次住院治疗，最终死亡。由于眼眶浆细胞瘤患者的缓解率和生存率通常低于累及其他部位的髓外浆细胞瘤患者，因此必须将他们视为高危患者，需要多学科方法进行早期诊断和及时治疗，以防止疾病进展并缓解与疾病相关的症状。
