What is the risk of recurrence of solitary plasmacytoma. Spinal cord compression was reversed in every patient. Solitary plasmacytoma management and outcomes irish. Herein, we describe a patient with extramedullary plasmacytoma of the thyroid gland. Discussion the patient reported herein had an igga secreting plasmacytoma of the skull associated with a peripheral neuropathy, lymphadenopathy, and throm bocytosis. Extramedullary plasmacytoma of the thyroid associated with a. Solitary plasmacytoma of the bone can sometimes be cured with radiation therapy or surgery to destroy or remove the tumor. Clinical features and prognostic factors in solitary. Some authors advocate the use of radiotherapy as a sole treatment due to the good response in most cases.
Solitary plasmacytoma of the bone appears more vividly in the axial skeleton 2560%, which has the red marrow and usually affects the thoracic vertebrae. This shows invasion of the bone or tissue by monoclonal identical plasma cells see cutaneous plasmacytoma pathology. However, they have made progress in understanding how certain changes in dna can make plasma cells become cancerous. Currently, radiation therapy remains the primary choice of treatment for spb, as plasmacytoma is a radiosensitive tumor. Solitary plasmacytomas most frequently occur in bone plasmacytoma of bone, but can also be found outside bone in soft tissues extramedullary plasmacytoma. Diagnosis, treatment, and response assessment in solitary plasmacytoma. A solitary plasmacytoma is a discreet, single mass of abnormal cancerous plasma cells which can occur in bone or in soft tissue. Solitary plasmacytoma of the skull revealed by a mononeuritis.
This algorithm has been developed for md anderson using a multidisciplinary approach considering circumstances particular to md anderson s specific patient population, services and structure. Positron emission tomography pet for staging of solitary. Extramedullary plasmacytoma in rectum and anal canal. Apr 21, 2020 bone metastasis occurs when cancer cells spread from their original site to a bone. The radiation is similar to an xray but more powerful. Jan 27, 2020 lynne eldrige, md, is a lung cancer physician, patient advocate, and awardwinning author of avoiding cancer one day at a time. Dna is the chemical that carries the instructions for nearly everything our cells do. Some patients diagnosed with a solitary plasmacytoma will go on to develop myeloma. Treatment may be considered if percentage of clonal plasma cells is.
Multiple solitary plasmacytoma with multifocal bone. In radiation therapy, there is focusing of radiation on the solitary plasmacytoma to destroy the abnormal cancerous cells. Diagnosis of a solitary plasmacytoma requires a very thorough evaluation. Solitary plasmacytoma sp is characterized by a mass of neoplastic monoclonal plasma cells in either bone sbp or soft tissue without evidence of systemic disease attributing to myeloma. Solitary bone plasmacytoma and extramedullary plasmacytoma. They then need additional treatment, such as chemotherapy. Surgical treatment for a giant solitary plasmacytoma with. Diagnosis, treatment, and response assessment in solitary. Radical radiotherapy has formed the backbone of sp treatment for many years with current guidelines recommending its use as a frontline treatment 1. The modern principles outlining the supportive role of palliative rt in multiple myeloma in an era.
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. Multiple myeloma commonly is associated with conditions and symptoms referable to the thorax. Treatment of the plasmacytoma led to regression of both the poems syndrome and the skin lesion and enlarged lymph nodes. Solitary and extramedullary plasmacytoma page 1 of 4. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary. A solitary plasmacytoma is a single cancer site that may involve the bone osseous or other tissues extraosseous or extramedullary. Seven patients have not shown evidence of dissemination, and five are alive 1. Ovarian plasmacytomas are a unique and unusual presentation of extramedullary plasmacytomas emp. Cerebral or brain ischemia is a condition that occurs when there isnt enough blood flow to the brain. We report a case of a 62yearold woman presenting with blurred vision of the right eye and tenderness of the right temporal region, which was interpreted as temporal arteritis. Full text full text is available as a scanned copy of the original print version. The most common treatment for both for both types of solitary plasmacytoma is radiotherapy. Jan 16, 2018 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.
This may possibly be combined with a stem cell transplant. Radiotherapy for solitary plasmacytoma and multiple myeloma. Solitary plasmacytoma of the spine associated with. This study was undertaken to investigate the clinical value in staging patients with plasmacytoma pc using positron emission tomography pet. Pneumonitis and thoracic pain are not unusual as a result of altered immunologic competence and skeletal defects. Fdgpet was done for staging in 11 patients and for restaging in 4 patients. Chemotherapy is indicated only in cases of disseminated disease 11,12. Solitary plasmacytoma of the mandible a rare entity.
Solitary plasmacytoma is a localized collection of monoclonal plasma cells. Progression to disseminated disease in the form of multiple myeloma occurs in 17% to 32% of cases. 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. The aim of treatment is to direct enough radiation to the tumour to kill as many of the plasmacytoma cells as possible, while minimising damage to nearby tissues. Extremedullaire, plasmacytome, sinus maxillaire, solitaire, cote introduction 3, 4 plasma cell neoplasms constitute a continuum of disease spectrum which have been classified as multiple myeloma mm, solitary plasmacytoma sp and extramedullary plasmacytoma emp. Mandibular location of bone plasmacytoma is very rare and diagnosis is first histological. This is possible because by definition, solitary plasmacytomas are localised. The primary treatment for most patients will be radiotherapy, but surgery may also be required, where close. Treatment included radiotherapy 18 of 19 andor surgery 11 of 19 and chemotherapy eight of 19. The most common of these is spb, accounting for 35% of all plasma cell. In 2011 spring time i was diagnosed with a solitary plasmacytoma in vertebra l1.
A 57 year old woman was referred to our department with a presumed diagnosis of a nonfunctioning pituitary macroadenoma. Cerebral ischemia symptoms, treatment, recovery columbia. Thus, this clinically distinctive syndrome can reveal the poems syndrome at a curable stage. Solitary plasmacytoma, extramedullary plasmacytoma, myeloma. Pn shakuntala was involved in manuscript preparation of the observations, patient care, reference hunting, and final editing. Solitary mesenteric plasmacytoma revealed by an acute. Diagnosis and management of solitary plasmacytoma of bone. Immunoglobulin free light chains and solitary plasmacytoma of. In some patients, a bone marrow aspiration can detect a low monoclonal plasma cell infiltration which indicates a high risk of early progression to an overt myeloma disease. The purpose of this study was to determine the ct and mr imaging features of sep of the nasal tract. The follow up consisted of blood and urine tests every third month and plain xrays. Softtissue or nonosseous extramedullary plasmacytoma emp solitary bone plasmacytoma sbp multifocal form of multiple myeloma multiple myeloma plasmablastic sarcoma t. If it is plasma cell neoplasm consistent with a plasmacytoma or multiple myeloma, then the case would be reportable.
Emp involving the ovary is usually large at the time of presentation, more likely. Local radiotherapy is the treatment of choice for solitary bone plasmacytome sbp. The urinary tract is rarely involved, with only 9 solitary plasmacytomas of the kidney described. The median age of occurrence is 5070 years with a male. 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. Multiple solitary plasmacytoma msp is a rare plasma cell dyscrasia, characterised by multiple lesions of neoplastic monoclonal plasma cells. If the diagnosis is plasmacytoma and there is no additional information to indicate if its a plasmacytoma of the bone or an extramedullary plasmacytoma, default to plasmacytoma of the bone and assign histology, 973. In patients with either solitary plasmacytoma of bone or asymptomatic mm, systemic treatment should be deferred until there is evidence of disease progression. Solitary bone and extramedullary plasmacytomas are rare plasma cell proliferative disorders. Solitary extramedullary myeloma plasmacytoma of the upper part of the respiratory tract and airway.
Plasma cell neoplasms including multiple myeloma treatment. Their diagnosis is based on histologic confirmation of monoclonal plasma cell infiltration of a single disease site and on the exclusion of systemic myeloma. May 02, 2012 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. These guidelines were developed by a working group of clinical. In this issue of blood, 2 studies, one by paiva et al for the spanish myeloma study group and one by hill et al for the leeds group uk, showed independently that flow cytometry of the bone marrow could identify patients with solitary plasmacytomas sps at high risk for progression to active multiple myeloma mm. This study reports a case of an extramedullary plasmacytoma of the. Radiation therapy for solitary plasmacytoma and multiple. Radiotherapy involves focusing radiation similar to xrays on the plasmacytoma to kill the abnormal cells. Solitary plasmacytoma comprises 24% of cases of plasma cell myeloma.
Solitary plasmacytoma symptoms, diagnosis, treatments and. Nov 27, 2017 if the plasmacytoma is present as an inconspicuous solitary mass of abnormal plasma cells, then it is known as solitary plasmacytoma, i. Patients with extramedullary plasmacytomas and osteosclerotic lesions were excluded. Diagnosis and management of solitary extramedullary.
Such patients usually become free of symptoms after local radiotherapy. The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Bone metastasis can occur in any bone but more commonly occurs in the spine, pelvis. Additional information is now available on the role of serum free light chains and pet scanning.
Solitary sellar plasmacytomas are exceedingly rare and difficult to distinguish from other pituitary tumors. The alliance for clinical trials in oncology, a national clinical trials network supported by the national cancer institute nci, is sponsoring a clinical trial for patients with solitary plasmacytoma of bone spb. In this study, a total of 12 patients diagnosed with spb were enrolled. Get a printable copy pdf file of the complete article 2. Solitary plasmacytomas represent approximately 5% of all patients with myeloma. Overall and diseasefree survival kaplanmeier method in patients with solitary plasmacytoma of bone. Malignant bone tumours of the spine are extremely rare solitary bone plasmacytoma. The treatment was orthopedic surgery and 25 rounds of radiation.
Extramedullary plasmacytoma is a plasma cell tumor that grows. They didnt go for a new biopsy at the site of the tumour. Primary extramedullary plasmacytoma is composed of a monoclonal proliferation of cells with plasmacytic differentiation in an extramedullary site. But some types of cancer are particularly likely to spread to bone, including breast cancer and prostate cancer. Both diseases represent a single localized process without. Pdf diagnosis, treatment, and response assessment in.
The panel on the left shows the remission time in 61 patients with solitary plasmacytoma of bone treated at the md anderson cancer center, according to disappearance and persistence of myeloma protein or nonsecretory myeloma. To make a diagnosis of solitary plasmacytoma of bone or extramedullary site, other plasma cell tumours or multiple myeloma must be excluded. The aesop adenopathy and extensive skin patch overlying a p. Spb is a rare less than 450 cases annually in the us occurrence of myeloma in a single bone without evidence of myeloma elsewhere in the body absence of monoclonal plasma cells. Radiotherapy appears to be an effective modality of treatment of solitary plasmacytoma. When treated with radiotherapy, prognosis of the patients was excellent. Solitary plasmacytoma is a rare tumor and observed in 37% of patients with plasma cell neoplasms. Original article the outcome and analysis of different. The median age at presentation was 53 years and there was a predominance of males. The current treatment of choice is radiotherapy because these tumors are highly radiosensitive. If a vertebra is sufficiently weakened by the plasmacytoma, a painful compression fracture may result.
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. Solitary and extramedullary plasmacytoma md anderson. Nearly all types of cancer can spread metastasize to the bones. Surgery was performed to all patients with spb in combination with radiation therapy or chemotherapy, or both. Extramedullary plasmacytoma is a malignant plasma cell dyscrasia that is most commonly found in the head and neck. Fifteen patients with known pc underwent pet using f18 fluorodeoxyglucose fdgpet.
In this article, the authors presented a 40yearold man who had a painless protruding mass over the midline of the posterior head region. Transsphenoidal treatment of nonneoplastic intra sellar cysts. Given the rarity of sp there is a lack of prospective clinical trial data on which to base treatment decisions. Five patients have developed multiple myeloma mm, four within three years and one after twelve years. The treatment used most commonly for both types of plasmacytoma is radiotherapy.
The international lymphoma radiation oncology group presents a standardized approach to the use and implementation of definitive rt in solitary plasmacytomas. These entities are diagnosed by demonstrating infiltration of a monoclonal plasma cell population in a single bone lesion or presence of plasma cells involving a soft tissue mass, respectively. Regarding the treatment of extramedullary plasmacytoma in the head and neck, the choice of radiotherapy or surgery alone is controversial in the published data. Jan 15, 20 the role of single agent carboplatin in extramedullary plasmacytoma of ovary as an adjuvant treatment needs to be explored. 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. Pn shakuntala 1, sr praveen 1, b shankaranand 2, k rajshekar 1, k umadevi 1 and ud bafna 1. Radiation therapy for treating solitary plasmacytoma. Apr 26, 2006 we report an uncommon case of an intrasellar plasmacytoma presenting as a nonfunctional invasive pituitary macroadenoma as the first manifestation of multiple myeloma. Solitary plasmacytoma is the rarest type of plasma cell neoplasm, and the anaplastic form is even more uncommon. The diagnosis and management of patients with solitary plasmacytoma requires the same range of clinical and laboratory expertise as for patients with mm uk myeloma forum, 2001. In contrast, sep is nearly always truly localized and has a high cure rate with local treatment.
Since the previous guideline several new articles on prognostic factors, treatment and outcome in patients with solitary plasmacytoma of bone have been published. Plasmacytoma most commonly originates in bone and predominantly affects older patients. Dec 31, 2019 a plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue extramedullary. A brain magnetic resonance imaging mri revealed an enhanced mass. Biopsy confirmation of a monoclonal plasma cell infiltration from a single site is required for diagnosis. Oct 31, 2012 plasmacytoma is a rare disease, which afflicts 2 to 3 people per every 100,000 of the general population. 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. Sep 15, 2000 such patients usually become free of symptoms after local radiotherapy. The clinical features and the results of treatment of twelve patients with solitary plasmacytoma of bone spb are reported. Corticosteroids and immunomodulatory drugs may be used. No doseresponse relationship is observed, and high local control rates are achieved with limited portals.
Plasmacytoma is diagnosed by a tissue biopsy or bone marrow biopsy. A case of extramedullary plasmacytoma emp in a 50year old woman and another. Spinal plasmacytomas may cause dull, aching pain as they destroy normal bone. Korolkowa et al reported that 40% occur in the nasal cavity and paranasal sinus, 20% in the nasopharynx, and 18% in the oropharynx1,2. She reported a 3 month history of headaches and a 2 week history of sudden onset of right facial. Tumour sizes were larger in patients with bone involvement p 0 003. Solitary plasmacytoma of bone and asymptomatic multiple. Solitary or extramedullary plasmacytoma memorial sloan. However, 70 percent of people with solitary plasmacytoma eventually develop multiple myeloma. However, if the neoplasm can be completely surgically resected, the longterm results have been reported to be equal to those involving radiation alone 5 galieni p. Spinal plasmacytomas may cause other symptoms that vary depending on the tumors size and location. Plasmacytoma of the thyroid europe pmc article europe. A type of cancer from plasma cells where the plasma cells multiply uncontrollably.
The treatment is generally given over several days to reduce sideeffects. Know the causes, symptoms, treatment and prognosis of solitary plasmacytoma. Guidelines on the diagnosis and management of solitary. In patients with mm without symptoms, the diagnosis is made on the basis of screening laboratory tests. Two rare presentations of plasma cell neoplasm bioline. Get detailed treatment information in this summary for clinicians. Risk of progression of solitary bone plasmacytoma to myeloma using a risk stratification model that incorporates the serum free light chain flc ratio measured at baseline a or 1 to 2 years following diagnosis b and the persistence of serum monoclonal protein 1 to 2 years following diagnosis. Pdf solitary bone plasmacytoma sbp is a rare entity characterized by.
Solitary plasmacytoma multiple myeloma texas oncology. Pdf solitary extramedullary plasmacytoma of the bladder. Plasma cell neoplasms are a spectrum of disorders composed of multiple myeloma, plasmacytoma, extramedullary plasmacytoma, and plasma cell leukemia. Direct immuno fluorescence study showed no more immune complex deposits. Plasma cell neoplasms including multiple myeloma treatment include observation, chemotherapy, radiation, stem cell rescue, targeted, and supportive therapies. Plasmacytoma is a plasma cell dyscrasia in which a plasma cell tumour grows within soft tissue. Between 1956 and 1978, nine patients with solitary plasmacytoma of bone spb and seven with extramedullary plasmacytoma emp were treated at the university of washington hospital and swedish tumor institute. Surgical and medical staging were performed on the present case. Solitary extramedullary plasmacytoma of the bladder. For spb and extramedullary plasmacytoma the distinction is the presence of only one lesion. In general, the risk of progression to myeloma is higher in patients with a solitary bone plasmacytoma sbp compared to those with. Solitary plasmacytoma of bone and asymptomatic multiple myeloma. It differs from multiple myeloma by the lack of hypercalcaemia, renal insufficiency, anaemia and pathological monoclonal plasmocytosis on a. Solitary intracranial plasmacytoma pdf free download.
Ct and mr imaging of solitary extramedullary plasmacytoma. It is an infrequent form of plasma cell neoplasm and represents 5% to 10% of all plasma cell neoplasms according to the literature 15. Plasmacytoma solitary mandible radiotherapy mots cles. Plasmacytoma the spine hospital at the neurological. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma.
Mari gowda road, bengaluru 560029, karnataka, india. Solitary plasmacytoma accounts for 5% of the plasma cell neoplasm. A case report and literature article pdf available in oncology williston park, n. Solitary plasmacytoma is a rare plasma cell dyscrasia, classified as solitary bone plasmacytoma or solitary extramedullary plasmacytoma. Doctors at columbia neurosurgery in new york will discover the symptoms and causes in order to successfully treat cerebral ischemia. The common presentation of sbp is in the axial skeleton, whereas the extramedullary plasmacytoma. The primary treatment for most patients will be radiotherapy, but. The treatment that is used most commonly for both types of plasmacytoma is radiotherapy. A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue extramedullary. Why some patients develop multiple myeloma and others a single plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor. Rathkes cleft cystintracranial plasmacytoma case reports 11. Discrete intrathoracic tumors related to myeloma plasmacytomas, however, are relatively rare and only 33 cases have been reported in the literature from 1911 through 1960. Solitary plasmacytoma of the skull, a single malignant monoclonal plasma cell proliferation without systemic involvement, is rare and often misdiagnosed by radiological examinations only.
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