Solitary Plasmacytoma of Mandible – A Rare Dyscrasia- Case Report and Review of Literature

Introduction: The plasma cell neoplasms may present in soft tissue as extramedullary plasmacytomas, bone as a solitary plas- macytoma of bone, or as part of the multifocal disseminated disease multiple myeloma. Aim of study: The study aims to report solitary plasmacytoma in the gnatic bone oral cavity, which is also mimicking as malig- nant neoplasm of bone, seen in a female patient. Case Report: A 38-year-old female patient reported to the outpatient department of our hospital complaining of pain and swelling over the left lower one-third region of the face for one month CBCT analysis shows a hypodense area involving 35 regions extending towards ascending rami of the mandible. Conclusion: Plasmacytoma, despite being a lesion with slow, asymptomatic growth, can assume large volumes, making proper treatment difficult. When there is no bone involvement and it is diagnosed early, the success of treatment is generally higher. The treatment of choice is radiotherapy, with good results for the remission of the lesion

Plasmocitoma extramedular solitario rinosinusal: reporte de caso y revisión de literatura / Solitary extramedullary plasmacytoma of the rhinosinusal cavity: a case report and literature review

Resumen El plasmocitoma extramedular solitario (PES) es una neooplasia maligna infrecuente caracterizada por una proliferación aislada de células plasmáticas monoclonales en tejido extramedular. La localización más frecuente es en cabeza y cuello con predominio en el territorio rinosinusal, sin embargo, estas lesiones malignas representan menos del 1% de los tumores de esta región anatómica. El diagnostico requiere una alta sospecha clínica, análisis histopatológico acucioso, estudios serológicos y exámenes radiológicos sistémicos de acuerdo a los criterios diagnósticos establecidos en la literatura internacional. Se analiza el caso de un paciente masculino con un PES que se presentó como un tumor de fosa nasal derecha y obstrucción nasal de meses de evolución con hallazgos clínicos e imagenológicos inespecíficos. El diagnóstico definitivo se realizó mediante biopsia endoscópica nasal y estudio histopatológico. El tratamiento fue abordado de manera multidisciplinaria entre otorrinolaringología, hematología y radiooncología. De acuerdo a las guías internacionales, se decidió realizar radioterapia localizada con buen resultado clínico precoz. El PES requiere un abordaje multidisciplinario para lograr un diagnóstico y tratamiento oportuno, siendo imprescindible la exclusión del mieloma múltiple debido a las diferencias terapéuticas y en pronóstico clínico. El tratamiento puede realizarse con radioterapia y/o cirugía, siendo la radioterapia el pilar de tratamiento.

Abstract Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm characterized by isolated proliferation of monoclonal plasma cells in extramedullary tissue. The most frequent location is in the head and neck with a predominance in the rhinosinusal territory; however, these malignant lesions represent less than 1% of the tumors in this anatomical region. The diagnosis requires a high clinical suspicion, careful histopathological analysis, serological studies and systemic radiological examinations according to the diagnostic criteria established in the international literature. We analyze the case of a male patient with SEP that presented as a tumor of the right nostril and nasal obstruction of months of evolution with nonspecific clinical and imaging findings. The definitive diagnosis was made by nasal endoscopic biopsy and histopathological study. The treatment was approached by multidisciplinary teamwork. According to international guidelines, it was decided to perform localized radiotherapy with good early clinical results. SEP requires a multidisciplinary approach to achieve a timely diagnosis and treatment, being essential exclusion of multiple myeloma due to the therapeutic differences and prognosis. Treatment can be done with radiation therapy and/or surgery; radiation therapy is the mainstay of treatment.

Plasmocitoma extramedular de canal auditivo externo: caso clínico / Extramedullary plasmacytoma of external auditory canal: a case report

Resumen El plasmocitoma extramedular es una neoplasia de células plasmáticas poco frecuente, que se ubica en 80% a 90% de los casos en la cabeza o cuello. Esta neoplasia representa menos del 1% de toda la patología maligna de cabeza y cuello. Dada la poca frecuencia de la patología y la escasez de casos publicados, esta entidad presenta una gran dificultad clínica y terapéutica. El diagnóstico se basa en el análisis histológico con inmunohistoquímica de la muestra obtenida, y el tratamiento varía según la ubicación, donde se puede realizar radioterapia o cirugía. En este artículo presentamos el caso de una paciente de 56 años, con antecedentes de otorrea de larga data en oído derecho, al examen físico presentaba un tumor que obstruía todo el conducto auditivo externo. Se realizó exéresis tumoral y la biopsia evidenció un plasmocitoma.

Abstract Extramedullary plasmacytoma is a rare plasma cell neoplasm, affecting 80% to 90% of the head or neck. This neoplasm represents less than 1% of all malignant head and neck pathology. Given the infrequency of the pathology and the scarcity of published cases, this entity presents great clinical and therapeutic difficulty. Diagnosis is based on histology and immunohistochemistry and treatment varies depending on the location, where radiation therapy or surgery can be performed. In this article, we present the case of a 56-year-old patient with a history of long-standing otorrhea in the right ear. Physical examination presented a tumor that obstructed the entire external auditory canal. Tumor excision was performed, and the biopsy revealed a plasmacytoma.

Plasmacytoma of the cervix / Philippine Journal of Obstetrics and Gynecology

@#Plasmacytoma is a rare disease entity that represents only 5%‒10% of all plasma cell neoplasms. It is rarely found in the female genital tract. There are 8 identified case reports on plasmacytoma of the cervix. The clinical symptoms are nonspecific and biopsy with immunohistochemistry is used to diagnose. Due to the paucity of cases, there is no standard treatment modality. We present a case of a 34-year-old patient who had a history of postcoital bleeding. Biopsy and immunohistochemistry were done which confirmed the diagnosis of plasmacytoma. Total abdominal hysterectomy, bilateral salpingectomy with transposition of ovaries was the chosen treatment option.

Plasmocitoma extramedular de laringe: a propósito de un caso / Extramedullary plasmacytoma of the larynx: a case report

Resumen Los plasmocitomas solitarios son una rara forma de presentación de neoplasias de células plasmáticas. Deben ser diferenciados del mieloma múltiple en el momento del diagnóstico y realizar un seguimiento estricto por el riesgo de transformación a mieloma. La localización de los mismos en la laringe es muy inusual, dado que sólo se han publicado 56 casos. Se presenta el caso de una paciente con un plasmocitoma extramedular de laringe que se trató mediante cirugía y radioterapia. Se revisan los criterios diagnósticos y los problemas terapéuticos.

Abstract Solitary plasmacytomas are a rare form of plasma cell neoplasms. They should be differentiated from multiple myeloma at the time of diagnosis and strictly monitored for the risk of transformation to myeloma. Their location in the larynx is very unusual, since only 56 cases have been published. We present the case of a patient with extramedullary plasmacytoma of the larynx that has been treated with surgery and radiotherapy. We reviewed the literature for diagnostic criteria and therapeutic problems.

Plasmocitoma solitario intracraneal: una presentación inusual / Intracranial solitary plasmacytoma: an unusual presentation

Background: Intracranial solitary plasmacytoma is extremely rare and its location in the clivus is unusual; its clinical presentation is very varied and the diagnostic images are similar to the other tumors in skull-base. Case presentation: 47-year-old woman with initial diagnosis of pituitary macroadenoma, attended with headache, nausea, vomiting, diplopia in the right eye and paralysis of her abducent nerve. She was reoperated by transsphenoidal endoscopic approach, concluding in solitary clivus plasmacytoma by biopsy and immunohistochemistry. Conclusions: Clivus is an unusual location for intracranial plasmacytoma. Early diagnosis, complete study and careful follow-up are required due to its high progression to multiple myeloma(AU)

Introducción: El plasmocitoma solitario intracraneal es extremadamente raro y su localización en el clivus es inusual; su presentación clínica es muy variada y las imágenes diagnósticas son similares a las de otros tumores de la base del cráneo. Reporte de caso: Se presenta el caso de una mujer de 47 años con diagnóstico inicial de macroadenoma hipofisiario, que cursó con cefalea, náuseas, vómitos, diplopía en ojo derecho y parálisis de su nervio abducente. Fue reintervenida por vía transesfenoidal endoscópica, concluyendo en plasmocitoma solitario del clivus por biopsia e inmunohistoquímica. Conclusiones: El clivus es una localización inusual para plasmocitoma intracraneal, se requiere un diagnóstico precoz, estudio completo y seguimiento cuidadoso por su alta progresión a mieloma múltiple(AU)

Applications of one-stage total spondylectomy by anterior and posterior approaches for solitary plasmacytoma of cervical spine / 中国修复重建外科杂志

Objective: To investigate the feasibility of one-stage total spondylectomy by anterior and posterior approaches for treating solitary plasmacytoma (SP) of cervical spine.

Imaging diagnosis of solitary plasmacytoma in bone / 实用放射学杂志

Objective To investigate the imaging findings of solitary plasmacytoma in bone(SPB)to improve the diagnostic accuracy. Methods The imaging data of 22 cases with SPB confirmed by pathology were reviewed retrospectively,including DR(n=15),CT (n=20)and MRI(n=11).8 patients were examined by DR,CT and MRI.Results The tumors were located at vertebrae(n=9),humerus (n=1),scapula(n=2),sternum(n=1),ilium(n=3),pubis(n=1),ribs(n=2),frontal bone(n=1)and mandible(n=2).The lesions showed as expansive growth and punched-out like bone destruction with well-defined margins on DR and CT.Soft tissue masses(n=7) or bony ridge(n=11)could be observed.No periosteal reaction was shown in any cases.One case was misdiagnosed as odontoma, which demonstrated as patchy high-density lesion in the mandible.The lesions were of same signal or slightly hyper-intensity on T1WI and hyper-intensity on T2WI and DWI compared with the muscle signal.There was soft tissue infiltration without obvious peritumoral edema.The lesions combined with bony ridge were shown as"mini brain"sign."Cuff sign"could be seen in 4 cases in the vertebrae. The tumors enhancement was obvious after injection of contrast agent.Conclusion The diagnosis of SPB is relatively difficult,and should be comprehensively analyzed by combining imaging features,clinical and laboratory examinations.

Huge Extramedullary Plasmacytoma of the Retroperitoneum / 대한내과학회지

Extramedullary plasmacytoma (EMP) represent 3% of plasma cell neoplasms. Approximately 80-90% of EMPs involve the mucosa-associated lymphoid tissue of the upper airways and 75% of these involve the nasal and paranasal regions, while renal or retroperitoneal infiltration is very rare. EMPs are highly radiosensitive, with excellent results. The local control rate of radiotherapy can reach 90-97% and the 5-year overall survival rate can be 57-61%. EMP has an indolent course and the prognosis is generally favorable, with 70% of the patients remaining disease free for 10 years. However, about 30% may progress to plasma cell myeloma or relapse. Here, we report a case of a huge retroperitoneal solitary extramedullary plasmacytoma that grew rapidly.

Prognostic factors in solitary plasmacytoma / 中国肿瘤临床

Objective:To investigate the clinical features, treatment strategies, and relative prognostic factors in 66 patients with solitary plasmacytoma (SP). Methods:The data of 644 patients, who were diagnosed with pathologically proven plasmacytoma in Tianjin Medical University Cancer Institute and Hospital between June 2000 and October 2012, were collected. Sixty-six of these patients (10.25%) were evaluated as SP, including 45 solitary bone plasmacytoma (SBP) and 21 extramedullary plasmacytoma (EMP). Results:SBP and EMP were the two clinical subsets of SP revealing the location of the lesion. SBP mostly occurred in the axial skeleton, whereas EMP was most frequently observed in the upper respiratory tract. The differences among tumor size, serum M-protein, and serumβ2-microglobulin exhibited statistical significance. Conclusion:Large tumor size (≥5 cm), positive serum M-protein, and serumβ2-microglobulin were the factors that affected the prognosis of SBP patients. Radiotherapy and serumβ2-microglobulin>3.5 mg/L were the favorable prognostic factors for EMP patients.

The clinicopathological study of 18 solitary plasmacytoma patients / 中国医师进修杂志

Objective To investigate the diagnosis,differential diagnosis,treatment and prognosis of solitary plasmacytoma (SP) and summarize its clinical features for pathological and clinical application.Methods Reviewed the data of 18 patients diagnosed with SP from January 2005 to August 2013.The clinical features,diagnosis,treatment and prognosis were analyzed retrospectively.Results All 18 cases were pathologically confirmed with SP and met the diagnostic criteria of SP.In 18 patients,12 patients were male and 6 patients were female.Four cases of lesion were located at lumbar,each 2 cases of lesion were located at mandible and ribs,each 1 case of lesion was located at sacrum and occipital bone,3 cases of lesion were located at nasal cavity and maxillary sinus,2 cases of lesion was located at pars nasalis pharyngis,each 1 case of lesion was located at parotid,parasternal and peritoneal cavity.All patients were followed up for 54 months,2 patients were died,3 patients were local recurrence,2 patients were transformed to multiple myeloma,others were survival with no tumor.Conclusions As early as possible to diagnose is the key to treat SP.The final diagnosis of SP is based on the histopathological findings and the optimal treatment for patients with SP is surgery combined with moderate dose radiotherapy.

Rapid Progression of Solitary Plasmacytoma to Multiple Myeloma in Lumbar Vertebra

The prognosis of solitary plasmacytoma varies greatly, with some patients recovering after surgical removal or local fractional radiation therapy, and others progressing to multiple myeloma years later. Primary detection of progression to multiple myeloma is important in the treatment of solitary plasmacytoma. There have been several analyses of the risk factors involved in the early progression to multiple myeloma. We describe one case of solitary plasmacytoma of the lumbar vertebra that was treated with surgical decompression with stabilization and additional radiotherapy. The patient had no factors associated with rapid progression to multiple myeloma such as age, size, immunologic results, pathological findings, and serum free light chain ratio at the time of diagnosis. However, his condition progressed to multiple myeloma less than two months after the initial diagnosis of solitary plasmacytoma. We suggest that surgeons should be vigilant in watching for rapid progression to multiple myeloma even in case that the patient with solitary plasmacytoma has no risk factors for rapid progression to multiple myeloma.

Solitary Plasmacytoma of Mandible: A rare case report.

Solitary plasmacytoma is a rare condition affecting the jaws which manifests itself as a single osteolytic lesion without plasmocytosis of bone marrow and constitutes approximately 3% of all plasma containing tumors. It is different from multiple myeloma in terms of its clinical behaviour and prognosis. The most common clinical presentation is local bone pain and lesion on alveolar ridge and affects mandible frequently than maxilla. We report a rare case of solitary plasmacytoma of mandible, diagnosed on the basis of distinct radiological and histopathological findings, that has been treated by subtotal hemimandibulectomy. The review of literature concerning clinical, histological and radiological features, as well as the proper management concerning this tumour is included.

A Case of Multiple Solitary Plasmacytoma Recurring in Multiple Visceral Organs / 대한내과학회지

Multiple solitary plasmacytoma is a very rare disease entity, which occurs in up to 5% of patients with solitary plasmacytomas. We report an atypical case of multiple solitary plasmacytoma that recurred in multiple visceral organs without any evidence of bone marrow involvement. A 68-year-old male presented with voiding difficulty. Twenty months earlier, he had been placed on local radiotherapy for solitary plasmacytomas in the right 6th rib and right iliac bone. Recurrences were noted 14 and 12 months later in several ribs and the 5th cervical vertebra, respectively. These were well controlled with local radiotherapy and conventional systemic chemotherapy. He had multiple soft tissue masses in the stomach, pancreas, pelvic cavity, and right buttock. An endoscopic biopsy of the gastric mass confirmed the diagnosis of plasmacytoma. Local radiotherapy to the pelvic mass and systemic therapy consisting of bortezomib and dexamethasone were given, and he has been well for 8 months.

Plasmocitoma solitário ósseo: relato de caso / Solitary bone plasmocitoma: case report

JUSTIFICATIVA E OBJETIVOS: Plasmocitoma é um tumor maligno, originado da proliferação irreversível e autônoma dos plasmócitos, podendo se apresentar como massa circunscrita ou infiltração difusa. O plasmocitoma ósseo solitário é raro. Embora a idade dos pacientes seja variável, é incomum antes dos 30 anos. Tem predomínio no sexo masculino em proporção 3:1 e sua principal localização é a coluna vertebral. No plasmocitoma solitário, a eletroforese de proteínas séricas, o mielograma e as análises laboratoriais e radiológicas não apresentam evidências de doença sistêmica. O objetivo deste estudo foi relatar o caso de plasmocitoma solitário ósseo, localizado no osso esterno, enfocando aspectos essenciais na sua caracterização.RELATO DO CASO: Paciente do sexo masculino, 45 anos, encaminhado ao Serviço de Cirurgia Torácica devido ao surgimento de massa na região do manúbrio esternal, associado à dor local. Realizou tomografia computadorizada (TC) do tórax que mostrou extensa lesão lítica no osso esterno. A avaliação laboratorial incluindo hemograma, eletrólitos, eletroforese de proteínas séricas, análise quantitativa de imunoglobulinas séricas estavam todos dentro da normalidade. A pesquisa de proteína de Bence Jones foi negativa e a avaliação radiológica do crânio e ossos longos e a cintilografia óssea não mostrava outras lesões líticas. A biópsia de medula óssea foi normal. O paciente foi submetido à biópsia incisional da lesão que confirmou o diagnóstico de plasmocitoma solitário ósseo.CONCLUSÃO: Plasmocitoma solitário apesar de raro deve ser lembrado como diagnóstico diferencial de outras afecções para que o tratamento correto seja instituído, objetivando controle precoce da doença e melhor sobrevida.(AU)

BACKGROUND AND OBJECTIVES: Plasmacytoma is a malignant tumor originated of irreversible and independent proliferation of plasma cells. It's able to present itself as a circumscribed mass or diffuse infiltration. Solitary bone plasmacytoma is a rare tumor. Although the age of the patients is changeable, it is uncommon before the 30 years. It has predominance in men in ratio 3:1 and the main localization is the vertebral column. In solitary plasmacytoma, the serum protein electrophoresis, the bone marrow biopsy, as well as the laboratorial and radiological analyses and bone cintilography do not show evidences of systemic disease. The objective of this study is to report the case of a solitary bone plasmacytoma located in sternum, focusing essential aspects in the characterization of this entity. CASE REPORT: A male patient, 45-year-old, directed to the Thoracic Surgery Service due to the sprouting of a mass in sternum associated to local pain. In the Thoracic Computadorized Tomography (CT) Scan was noticed extensive litic injury in sternum. The laboratorial evaluation including hemogram, electrolytes, serum protein electrophoresis, serum quantitative immunoglobulin's analysis were normal. The Bence Jones protein was negative and the radiological evaluation of the skull and long bones did not show other litic injuries. The bone marrow biopsy was normal. The patient was submitted to a biopsy that ensuring the diagnosis of solitary bone plasmacytoma. CONCLUSION: Although solitary is a rare disease it should be remembered for differential diagnosis, objectifying a correct treatment and a better control of the disease.(AU)

Analysis of imaging presentation of solitary plasmacytoma in 17 cases / 重庆医科大学学报

Objective:To improve diagnostic ability by the evaluation of imaging appearances of solitary plasmacytoma.Meth-ods:Seventeen patients with solitary plasmacytoma confirmed by biopsy or operation were included in our study,whose clinical features and diagnostic imaging appearances were included.Results:Single lesion was found in all cases;Lesions of vertebra in 5 cases,flat bone in 5 cases,pipe bone in 2 cases and outside the bone in 5 cases.The Lesions in bone showed expansive osteolytic with clear border,soft tissue mass nearby,and thin and broken cortical.Vertebra lesions showed lytic destruction and compressed fracture.MRI showed slightly hypointense in T1WI and hyperintense in T2WI with markedly homogeneous enhancement.Lesions outside the bone appeared soft tissue mass with similar density to muscle in CT and X ray,and isointen-sity in T1WI and T2WI with markedly homogeneous enhancement.Lesions in ECT showed medium-to-high nuclide concentration,and in PET/CT showed the increase of glucose metabolize,with SUV 6.3～8.6.About one month after effective chemical therapy,soft tissue mass shrank obviously and new bone appeared,with the decrease of glucose metabolize,and SUV under 2.0.Conclusion:Solitary plasmacytoma often occurs in male,and in younger age and lacks symptoms in the whole body compared with multiple myeloma.Lesions in different regions show different manifestations,but all borders are well defined.CT and MRI can depict the extent of solitary plasmacytoma of bone clearly,but MRI was better in showing bone marrow infiltration range,and showing compression of spinal cord.PET/CT are more suitable in follow-up therapy.

Retrospective analysis of the relation between the clinical features and prognosis of 12 solitary plasmacytoma of bone patients / 中国癌症杂志

Background and purpose:Solitary plasmacytoma of bone(SBP) is a rare disease,reports releveant to this disease are rarely seen. The purpose of this study was to investigate the relation between the clinical features and the prognosis of solitary plasmacytoma of bone. Methods:We reviewed the data of 12 patients diagnosed with solitary plasmacytoma of bone from 1998 to 2007 in Peking University third hospital,the clinical features,treatment and prognosis of the patients were analyzed retrospectively. Results:The age ranged from 37-71 years(mean 49.6 years) ,the male/female ratio was 3 to 1. Immunophenotype analysis showed that 11(91.6%) cases were positive for CD79a,10(83.3%) positive forVS38C,and all negative for CD20. With 12 to 87 months follow-up(average 40?22 months) ,three cases(33%) developed to multiple myeloma,two of them died from infection,the median survival time was 73 months,the 3 year and 5 year survival rate were 90 percent and 75 percent respectively. Conclusion:Middle and old male are more likely to develop SP. The prognosis is good,but some of them can progress to multiple myeloma.

Solitary Dural Plasmacytoma: Case Report

The authors present a case of solitary dural plasmacytoma. A 43-year-old woman was admitted our department due to headache. Brain magnetic resonance(MR) image showed homogeneous enhanced lesion at the left occipital area. We performed surgical excision and it was confirmed plasmacytoma, histologically. The solitary dural plasmacytomas are exceedingly rare. Before this case report, only 17 cases had been reported in the literature

Solitary Plasmacytoma of Iliac Bone Rapidly Progressed to Multiple Myeloma / 대한내과학회지

Solitary osseous plasmacytoma accounts for 3-5% of plasma cell tumor and are assumed to have a fairly good prognosis, with long duration of relapse free survival after local irradiation. A 64 year old woman with a lytic lesion involving left iliac bone was diagnosed as a solitary plasmacytoma, with a negative work-up for coexisting plasma cell disorders. Three months after irradiation of 5,400 cGy, the patient was readmitted with hypercalcemia and mass in left forehead and left gingiva. New multiple osteolytic lesions were developed. A aspiration cytology of forehead mass and curetted specimen of right femur due to pathologic fracture revealed plasmacytoma. MRI of thoracolumbar spine revealed abnormal high signal intensity in the L2 body, T8 and T9 vertebrae. Serum protein electropheresis revealed monoclonal gammopathy of IgG-kappa type. This aggressive case of solitary plasmacytoma, evolving into multiple myeloma after brief duration of remission, is in sharp contrast with the natural course of a solitary plasmacytoma.

Cytologic Features of Plasmacytoma of the Ovary and Breast Occurred in a Patient with Solitary Plasmacytoma of Vertebra / 대한세포병리학회지

A case of plasmacytoma of the ovary and breast, which developed in a patient with a solitary plasmacytoma in the lumbar vertebra for nine months, was diagnosed cytologically and histologically. Enlargement of the right ovary and multiple palpable masses in the right and left breast were already present at six months after the diagnosis of vertebral solitary plasmacytoma. At eight months, plasma cell leukemia developed, and nine months the enlarged both ovaries, replaced by yellowish-gray solid tumors showed infiltration of immature plasma cells. The cytologic features of the ovarian tumors were same with those of the breast tumor. The tumor cells were of predominantly immature plasma cells with one or more nuclei. Some mature plasma cell had an eccentric nucleus with single nucleolus and peripherally clumped chromatin. Binucleated or multinucleated giant cells were often present. Histologically, sheets of poorly differentiated plasmacytoid tumor cells were separated by strands of hyaline fibrous tissue. On immunohistochemical stains, the tumor cells showed strong reactivity for lambda-light chain but no reaction for kappa-light chain, cytokeratin, or leukocyte common antigen.