Abstract:Multiple myeloma is a malignant plasma cell dyscrasia that may invade the orbits in extramedullary presentations. Common manifestations of orbital involvement include unilateral proptosis, injection, chemosis, diplopia, and occasionally pain. Additionally, the soft-tissue tumors associated with multiple myeloma typically cause bony destruction of adjacent structures. However, in certain patients, bilateral proptosis and a lack of bony destruction may occur. In these instances, it is important for physicians to… Show more
“…Orbital plasmacytoma can be the presenting symptom of undiagnosed MM, or of uncontrolled and recurrent disease, such as in this case. The vast majority of these patients have unilateral involvement, with proptosis being the most common finding [ 4 ]. This case is an atypical presentation due to the bilateral involvement (12% of cases).…”
Section: Discussionmentioning
confidence: 99%
“…Plasmacytomas are associated with MM, a plasma cell neoplasm that is most often confined to the bone marrow and presents with symptoms such as bone pain, infection, hypercalcemia, anemia, and renal involvement [ 4 ]. Extramedullary plasmacytomas have been previously reported to be present at diagnosis in about 7% of MM cases [ 5 ].…”
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.
“…Orbital plasmacytoma can be the presenting symptom of undiagnosed MM, or of uncontrolled and recurrent disease, such as in this case. The vast majority of these patients have unilateral involvement, with proptosis being the most common finding [ 4 ]. This case is an atypical presentation due to the bilateral involvement (12% of cases).…”
Section: Discussionmentioning
confidence: 99%
“…Plasmacytomas are associated with MM, a plasma cell neoplasm that is most often confined to the bone marrow and presents with symptoms such as bone pain, infection, hypercalcemia, anemia, and renal involvement [ 4 ]. Extramedullary plasmacytomas have been previously reported to be present at diagnosis in about 7% of MM cases [ 5 ].…”
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.
“…Orbital involvement is most common in patients with active MM disease, invasion of the orbit may be one of the extramedullary lesions, or may be the first manifestation of disease recurrence[ 14 , 15 ]. Of seven patients in this study, two patients presented with ocular involvement as the primary symptom (28.6%), and the remaining 5 patients developed periocular EMD after the diagnosis of MM was established (71.4%).…”
BACKGROUND
Multiple myeloma (MM) complicated with extramedullary disease (EMD) has a poor prognosis and is a limiting factor in the treatment of MM, and no standard treatment is recommended in international guidelines. Few studies have reported MM with periorbital EMD.
CASE SUMMARY
In this paper, the clinical characteristics and survival of seven patients with multiple myeloma and orbital are described and analyzed. The common ocular symptoms were blurred vision, proptosis and/or eye movement disorders, IgG type MM may be a risk factor for orbital involvement. Of them, six patients were treated with bortezomib-based regimens. The median overall survival (OS) and progression free survival for the entire cohort were 48 and 33 mo, respectively, which was much worse than the OS reported for MM patients without orbital EMD
.
CONCLUSION
Orbital MM may have significantly shortened survival for the entire cohort, so multidisciplinary collaboration is emphasized and recommended in the diagnosis and treatment of these difficult cases.
“…Hastalığın kesin etiyolojisi belli olmamakla beraber plazma hücrelerinin proliferasyonu ve diferansiyasyonunun bazı sitokinlerle ilişkili olduğu düşünülmektedir. Bunlar arasında en önemlisi interlökin-6' dır (4,5). Aktif hastalığı bulunan olgularda bu sitokin yüksek bulunmaktadır ve bu durumun kötü prognoz ile ilişkili olduğu düşünülmektedir (5).…”
Section: Discussionunclassified
“…Bunlar arasında en önemlisi interlökin-6' dır (4,5). Aktif hastalığı bulunan olgularda bu sitokin yüksek bulunmaktadır ve bu durumun kötü prognoz ile ilişkili olduğu düşünülmektedir (5).…”
ÖzEkstramedüller plazmasitom nadir görülem plazma hücreli bir neoplazidir. Sıklıkla üst solunum yollarını tutar, göz tutulumu ise son derece nadirdir. Bu bildiride orbital kaynaklı bir ekstramedüller plazmasitom olgusu sunduk. 55 yaşında erkek hasta, sol gözde şişlik nedeniyle yapılan tetkiklerinde orbital yerleşimli 5×6 cm boyutlarında kitle tespit ediliyor. Kitlenin histopatolojik incelemesinde amiloid arasında bol miktarda plazma hücreleri izlenmiş olup kappa-lamda ile monoklonalite görülmüştür. Olgu plasma hücreli neoplazi olarak rapor edilmiş olup tetkiklerinde başka bir odak tesbit edilmediğinden soliter plasmasitom olarak kabul edilmiştir. Ekstramedüller plazmasitom orbital yerleşimli kitlelerde akılda tutulmalıdır. Prognozu farklılık gösterdiği için tanısında sistemik bir plazma hücre diskrazisinin varlığı mutlaka sorgulanmalıdır.
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