Diagnosis and management of solitary extramedullary. Plasmacytomas in four cases of multiple solitary plasmacytoma comprised multiple extramedullary plasmacytomas, whereas in two cases a bone. Pdf diagnosis, treatment, and response assessment in solitary. Localized variant can occur in bone or in soft tissues extramedullary from 1. Plasmacytoma genetic and rare diseases information. The patient presented with a 4year history of continuous pain in the right chest area and moderate fever lasting 7 days. Extramedullary plasmacytoma carries a better prognosis than a solitary plasmacytoma of the bone. A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue extramedullary. They may involve any bone, but they have a predisposition for the red marrowcontaining axial skeleton. Since the previous guideline several new articles on prognostic factors, treatment and outcome in patients with solitary plasmacytoma of bone have been published. Solitary plasmacytoma comprises 24% of cases of plasma cell myeloma. In this article, the authors presented a 40yearold man who had a painless protruding mass over the midline of the posterior head region. They are solitary lesions, and are most often located in the head and neck region, mainly in the upper aerodigestive tract, but may also occur in the gastrointestinal tract, urinary bladder, central.
Diagnosis, treatment, and response assessment in solitary. In terms of clinical course, multiple solitary plasma cytomas can arise from a solitary plasmacytoma of bone or result from recurrent multiple myeloma 2e3 years. Solitary bone plasmacytoma radiology reference article. Radiation therapy for solitary plasmacytoma and multiple myeloma. Solitary plasmacytoma of the bone, especially of a single rib, is a rare disease. Solitary plasmacytoma sp is a plasma cell disorder characterized by localized accumulation of neoplastic monoclonal plasma cells in bone, or in soft tissues with no skeletal component, without any evidence of systemic involvement as demonstrated by the lack of clonal plasma cells in the bone marrow and absence of features of endorgan damage. Overall, plasmacytomas are more commonly seen in african americans than asians. Plasmacytoma is a plasma cell dyscrasia in which a plasma cell tumour grows within soft tissue or within the axial skeleton the international myeloma working group lists three types. However, 70 percent of people with solitary plasmacytoma eventually develop multiple myeloma. In radiation therapy, there is focusing of radiation on the solitary plasmacytoma to destroy the abnormal cancerous cells. Solitary plasmacytoma of the bone spb is a malignant proliferation of plasma cells that occurs within the axial skeleton and is characterized by destruction of the bone. Adjuvant chemotherapy has been administered with inconclusive results.
It is an infrequent form of plasma cell neoplasm and represents 5% to 10% of all plasma cell neoplasms according to the literature 15. Learn about myeloma plasmacytoma solitary myeloma and how it differs from multiple myeloma. The case of a 56yrold man is presented, who had a massive tumor of the right pulmonary apex. We reported a rare case of hepatic solitary plasmacytoma that successfully responded to fractionated stereotactic radiotherapy. They are found in the bone marrow of the axial skeleton or long bones of limbs. Solitary plasmacytoma of bone spb, also called osseous plasmacytoma is a localized tumor in the bone comprised of a single clone of plasma cells in the absence of other features of mm ie, anemia, hypercalcemia, renal insufficiency, or multiple lytic bone lesions. Although some studies have found that adjuvant therapy may prevent or delay progression to myeloma, most have noted no benefit with the early administration of chemotherapy. Solitary plasmacytomas represent approximately 5% of all patients with myeloma. Clinical features and prognostic factors in solitary. Each year, 5% of patients diagnosed with solitary plasmacytoma of bone progress to multiple solitary plasmacytomas.
Solitary plasmacytomas are uncommon plasma cell disorders, which may present as a single bone lesion pbone or extramedullary plasmacytoma pem. Pdf solitary plasmacytoma is a rare disease characterized by a. To discuss the clinical characteristics, therapeutic methods and factors affecting prognosis of sp in the cervical spine summary of background data. A type of cancer from plasma cells where the plasma cells multiply uncontrollably. Solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located either extramedullary or intraosseous. Currently, radiation therapy remains the primary choice of treatment for spb, as plasmacytoma is a radio. This may possibly be combined with a stem cell transplant. Solitary bone plasmacytoma sbp progresses to multiple myeloma at a rate of 6584% at 10 years and 65100% at 15 years. In general, the risk of progression to myeloma is higher in patients with a solitary bone plasmacytoma sbp compared to those with. Percutaneous transthoracic lung biopsy demonstrated a tumorcell population consisting of mature plasma cells, proplasmacytes, and rare plasmablasts. Solitary bone plasmacytomas is an uncommon plasma cell tumor which is localized to bone. They then need additional treatment, such as chemotherapy.
A 64yearold white french man had monoclonal gammopathy of the. Original article the outcome and analysis of different. Treatment of multiple solitary plasmacytomas with cytokine. We report four cases of solitary plasmacytoma of the bone and an extramedullary plasmacytoma of the paranasal sinuses and soft palate. The treatment is generally given over several days to reduce sideeffects. A multiplicity of multiple solitary plasmacytoma lesions was noted only after radiologic evaluation in five of the six cases. Malignant bone tumours of the spine are extremely rare pdf solitary plasmacytoma is a rare disease characterized by a localized proliferation of neoplastic monoclonal plasma cells, without evidence of. In approximately 20% of the cases involvement of other bones like rib, sternum, scapula, or clavicle have also been reported 10.
Solitary plasmacytoma of the skull, a single malignant monoclonal plasma cell proliferation without systemic involvement, is rare and often misdiagnosed by radiological examinations only. A consecutive series of 19 cases of solitary plasmacytoma sp of cervical spine that underwent surgical treatment and one case that underwent pure radiotherapy were observed from 1995 to 2006 objective. Diagnosis and management of solitary plasmacytoma of bone. Solitary plasmacytoma symptoms, diagnosis, treatments and. Pdf solitary plasmacytoma is an infrequent form of plasma cell dyscrasia that presents as a single mass of monoclonal plasma cells, located. To the best of our knowledge, very few cases have been reported without systemic disease.
Solitary bone plasmacytoma is one of the two subgroups of plasmacytoma, the. On an annual basis, about 1,000 new cases of solitary plasmacytoma are reported. Solitary or extramedullary plasmacytoma memorial sloan. Primary solitary plasmacytoma of the liver successful.
Solitary plasmacytoma of the spine associated with. Spb generally occurs in the pelvis, femur, humerus, and rib. Extramedullary plasmacytomas happen when a plasmacytoma develops in the lungs, throat or other organs. Radiotherapy involves focusing radiation similar to xrays on the plasmacytoma to kill the abnormal cells. Tumour sizes were larger in patients with bone involvement p 0 003. Radiation therapy for treating solitary plasmacytoma. Find out about solitary myeloma symptoms, diagnosis and treatment. Most people with extramedullary plasmacytomas can be cured after the treatment of radiation therapy. Solitary plasmacytoma of the bone can sometimes be cured with radiation therapy or surgery to destroy or remove the tumor. Guidelines on the diagnosis and management of solitary.
The clinical course, the response to treatment and the propensity to. The solitary plasmacytoma sp is characterized by a localized accumulation of neoplastic monoclonal plasma cells without a proof of a systemic plasma cell proliferative disorder. The treatment that is used most commonly for both types of plasmacytoma is radiotherapy. We present the case of a 44yearold han chinese man who was diagnosed with a solitary plasmacytoma of the bone located in the right sixth rib. Ct scan of the plasmacytoma should be obtained prior to radiation therapy. The treatment used most commonly for both types of plasmacytoma is radiotherapy. Local radiotherapy is the treatment of choice for solitary bone plasmacytome sbp.
Solitary lung plasmacytoma is a rare form of plasma cell tumors. The most common of these is spb, accounting for 35% of all plasma cell. Solitary plasmacytoma of the liver is a very rare and aggressive form of plasma cell dyscrasia. It is most commonly found in the thoracic vertebrae but can also be seen in following order in other vertebrae lumbar, sacral, and cervical vertebrae 9. Solitary bone and extramedullary plasmacytoma hematology. Surgical treatment for a giant solitary plasmacytoma with.
Treat as plasmacytoma, see page 2 yes no 1 see physical activity, nutrition, and tobacco cessation algorithms. Radiation therapy is the commonly used treatment for both the types of solitary plasmacytoma, as solitary plasmacytomas are localized and there is only one tumor present. In terms of clinical course, multiple solitary plasmacytomas can arise from a solitary plasmacytoma of bone or result from recurrent multiple myeloma 23 years after local radiotherapy or surgical resection. Radiation therapy for solitary plasmacytoma and multiple. A solitary plasmacytoma is a discreet, single mass of abnormal cancerous plasma cells which can occur in bone or in soft tissue. Radiotherapy is the common modality of treatment with, or without, adjuvant chemotherapy. Of the extramedullary plasmacytomas, 80% occur in the head and neck, usually in the upper respiratory tract. The prognosis and treatment of solitary plasmacytomas is very different to multiple myeloma. Solitary extramedullary plasmacytomas sep, solitary extraosseous plasmacytoma are plasma cell tumors that arise outside of the bone marrow. Softtissue or nonosseous extramedullary plasmacytoma emp solitary bone plasmacytoma sbp multifocal form of multiple myeloma multiple myeloma plasmablastic sarcoma t. A solitary plasmacytoma is a single cancer site that may involve the bone osseous or other tissues extraosseous or extramedullary. Solitary plasmacytoma occurring in bone definition solitary plasmacytoma s are single localized tumors consisting of monoclonal plasma cell s with no clinical features of plasma cell myeloma pcm see 97323 and no physical or radiological evidence of additional plasma cell tumor s. Ask your doctor to recommend what other types of doctors, physicians, medical specialists, or other medical professionals should be part of the team for your medical issues. A brain magnetic resonance imaging mri revealed an enhanced mass.
What is the prognosis of solitary bone plasmacytoma sbp. Solitary plasmacytoma, extramedullary plasmacytoma, myeloma, plasma cell dyscrasia, petct, mri, radiotherapy. This section presents information about some of the possible medical professionals that might be involved with solitary plasmacytoma. For spinal lesions, the margins should include at least 1 uninvolved vertebra. They are solitary lesions, and are most often located in the head and neck region, mainly in the upper aerodigestive tract, but may also occur in the gastrointestinal tract, urinary bladder, central nervous system, thyroid, breast, testes, parotid gland, lymph nodes, and skin. Doctors and medical specialists for solitary plasmacytoma. If the cancer is located in only one part of the body it is called a solitary plasmacytoma and if there are multiple sites it is called a multiple myeloma. Solitary and extramedullary plasmacytoma page 1 of 4. Solitary plasmacytoma in mandible with 7 year follow up data. Among all plasmacytoma cases, solitary bone plasmacytomas account for about 5%. Diagnosis of a solitary plasmacytoma requires a very thorough evaluation. The diagnosis and treatment of other plasma cell disorders eg, solitary extramedullary plasmacytoma, mm, primary al amyloidosis, monoclonal gammopathy of undetermined significance are discussed separately.
Standard treatment for solitary plasmacytoma is irradiation to the entire lesion with appropriate margins. Multiple myeloma is a type of blood cancer of the plasma cells which are found in the bone marrow. Treatment approaches and outcomes in plasmacytomas. Consider clinical trials as treatment options for eligible patients. Solitary and extramedullary plasmacytoma md anderson. Some patients diagnosed with a solitary plasmacytoma will go on to develop myeloma. Additional information is now available on the role of serum free light chains and pet scanning. Solitary plasmacytoma multiple myeloma texas oncology. Solitary plasmacytoma and extramedullary plasmacytoma of.