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Background: Parasellar plasmacytomas are rare neurosurgical entities. Intrinsic characteristics of these tumors, such as adjacent bone erosion and symptoms resulting from invasion and mass effect, may lead to the possibility of a solitary extramedullary plasmacytoma (SEP) as a differential diagnosis. Case Description: We present the case of a 39-year-old male with a 1-month history of bilateral decreased visual acuity, retroocular pulsating pain, and chromatic vision loss. A computed tomography scan of the head revealed a parasellar lesion causing chiasmatic compression, as well as clival, orbital, sphenoidal, and ethmoidal invasion. A combined transorbital and endonasal endoscopic approach was found suitable, and gross total resection was achieved. Histological analysis of the lesion established the diagnosis of a SEP. After radiotherapy, a new magnetic resonance imaging was performed, revealing a recurrence of the lesion with a high grade of invasion. The patient was treated with palliative radiotherapy, as surgical resection did not seem feasible. Conclusion: Surgical resection and radiotherapy may achieve remission of these lesions; however, recurrence rates remain high despite any treatment modality. Patients with this condition must be followed up with a multidisciplinary team due to the high risk of multiple myeloma progression.
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Plasma cell neoplasms are common, accounting for more than 1% of all malignancies. Its most common form is multiple myeloma, but others, such as extramedullary plasmacytoma (EMP), exist. Spinal cord compression secondary to these pathologies is not uncommon, however, adjacent bone involvement is usually present. Spinal cord compression caused by isolated epidural EMP is extremely rare, with only one case reported to date. We describe the case of a 75-year-old female patient that presented with paraparesis, due to an isolated epidural EMP associated with light chain amyloidosis. She was treated with surgical decompression and neoadjuvant chemotherapy, and is currently with a 15-month disease-free period.
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Multiple myeloma (MM) is a hematological malignancy characterized by unregulated and clonal proliferation of plasma cells in the bone marrow; these cells produce and secrete an anomalous monoclonal immunoglobulin, or a fragment of this, called M protein. The clinical manifestations of MM result from the proliferation of these plasmocytes, the excessive production of monoclonal immunoglobulin and the suppression of normal humoral immunity, leading to hypercalcemia, bone destruction, renal failure, suppression of hematopoiesis and humoral immunity, increasing the risk for the development of infections. The increase in life expectancy of the world population led to a concomitant increase in the prevalence of MM, a pathology that usually affects the elderly population. The aim of this review is to update the reader on epidemiology, diagnostic criteria, differential diagnosis with other monoclonal gammopathies, systemic treatment and prognosis of MM.
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El plasmocitoma extramedular es una neoplasia infrecuente y el compromiso mamario es en extremo raro, con alrededor de 83 casos reportados desde 1928. Se presenta el caso de una paciente de 63 años con antecedente de mieloma múltiple, con cuadro clínico de un mes de evolución de masa blanda de 4.5 cm en la mama derecha, indolora, con progresión rápida. Se realizó ecografía mamaria con reporte Bi-RADS 4, por sospecha de malignidad se realizó biopsia y fue remitida al servicio de mastología. El estudio básico de patología e inmunohistoquímica concluye que la lesión corresponde a un plasmocitoma extramedular y luego de múltiples líneas de tratamiento oncológico el plasmocitoma mamario presenta una excelente respuesta clínica e imagenológica. Se hace la descripción del caso y la revisión de literatura.
Extramedullary plasmacytoma is a rare neoplasm, and involvement of the breast is extremely uncommon, with approximately 83 cases reported since 1928. We present the case of a 63-year-old patient with a history of multiple myeloma, who presented with a one-month history of a rapidly progressing, painless, soft mass measuring 4.5 cm in the right breast. Breast ultrasound showed a Bi-RADS 4 lesion, and due to suspicion of malignancy, a biopsy was performed and the patient was referred to the mastology department. Basic pathological and immunohistochemical studies concluded that the injury corresponded to an extramedullary plasmacytoma. After multiple lines of oncological treatment, the breast plasmacytoma showed an excellent clinical and imaging response. The case is described, and a literature review is presented.
O plasmocitoma extramedular é neoplasia pouco frequente e o acometimento mamário extremamente raro, com cerca de 83 casos relatados desde 1928. Apresentamos o caso de uma doente de 63 anos com antecedentes de mieloma múltiplo, com quadro clínico com um mês de evolução de uma massa mole de 4,5 cm na mama direita, indolor, de rápida evolução. Foi realizada ultrassonografia mamária com laudo Bi-RADS 4, realiza-da biópsia por suspeita de malignidade e encaminhada ao serviço de mastologia. O estudo básico de patologia e imuno-histoquímica conclui que a lesão corresponde a um plasmocitoma extramedular e após múltiplas linhas de tratamento oncológico, o plasmocitoma mamário apresenta uma excelente resposta clínica e imagiológica. É feita a descrição do caso e revisão da literatura.
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Humanos , FemininoRESUMO
Abstract Multiple myeloma (MM) is a hematological malignancy characterized by unregulated and clonal proliferation of plasma cells in the bone marrow; these cells produce and secrete an anomalous monoclonal immunoglobulin, or a fragment of this, called M protein. The clinical manifestations of MM result from the proliferation of these plasmocytes, the excessive production of monoclonal immunoglobulin and the suppression of normal humoral immunity, leading to hypercalcemia, bone destruction, renal failure, suppression of hematopoiesis and humoral immunity, increasing the risk for the development of infections. The increase in life expectancy of the world population led to a concomitant increase in the prevalence of MM, a pathology that usually affects the elderly population. The aim of this review is to update the reader on epidemiology, diagnostic criteria, differential diagnosis with other monoclonal gam-mopathies, systemic treatment and prognosis of MM.
Resumo O mieloma múltiplo (MM) constitui neoplasia maligna de origem hematológica caracterizada pela proliferação desregulada e clonal de plasmócitos na medula óssea; estas células produzem e secretam imunoglobulina monoclonal anômala, ou um fragmento desta, denominado proteína M. As manifestações clínicas do MM decorrem da proliferação destes plasmócitos, da produção excessiva de imunoglobulina monoclonal e da supressão da imunidade humoral normal, levando à hipercalcemia, destruição óssea, insuficiência renal, supressão da hematopoiese e da imunidade humoral,aumentandooriscoparaodesenvolvimento de infecções. O aumento na expectativa de vida da população mundial levou a concomitante incremento na prevalência do MM, patologia que habitualmente acomete a população idosa. O objetivo desta revisão é atualizar o leitor sobre a epidemiologia, critérios diagnósticos, diagnóstico diferencial com outras gamopatias monoclonais, tratamento sistêmico e prognóstico do MM.
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Humanos , Masculino , Feminino , Procedimentos Ortopédicos , Difosfonatos/uso terapêutico , Procedimentos Cirúrgicos Profiláticos , Fraturas Espontâneas/diagnóstico por imagem , Mieloma Múltiplo/radioterapiaRESUMO
Abstract Cutaneous manifestations occur during the course of hematologic malignancies and precede, follow, or are late events in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, and immunosuppression resulting from the hematologic neoplasia itself or its treatment. The dermatologist must be aware of these conditions, which can help both in the diagnosis of the underlying disease and in the reduction of patient morbidity. This review (part I) addresses skin lesions associated with direct infiltration by systemic hematologic malignancies.
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Cutaneous manifestations occur during the course of hematologic malignancies and precede, follow, or are late events in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, and immunosuppression resulting from the hematologic neoplasia itself or its treatment. The dermatologist must be aware of these conditions, which can help both in the diagnosis of the underlying disease and in the reduction of patient morbidity. This review (part I) addresses skin lesions associated with direct infiltration by systemic hematologic malignancies.
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Neoplasias Hematológicas , Dermatopatias , Neoplasias Cutâneas , Humanos , Dermatopatias/patologia , Neoplasias Hematológicas/complicações , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/patologiaRESUMO
Metastases to the breast from non-mammary origin are rare. The majority of these lesions appear as secondary manifestations of melanoma and lymphoma, followed by lung carcinomas, gynecological carcinomas, and sarcomas. There has been a steady trend of an increase in diagnosis of intramammary metastases owing to the current advances in imaging technology. Imaging features depend on the type of primary neoplasm and route of dissemination, some of which resemble primary breast cancer and benign breast entities. There are certain imaging features that raise the level of suspicion for metastases in the correct clinical context. However, imaging manifestations of intramammary metastases do not always comply with the known classic patterns. The aim of this review is to clarify these features, emphasizing radiologic-pathologic correlation and a multidisciplinary approach, since most cases are found in patients with advanced disease.
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Carcinoma , Melanoma , Humanos , Mama/diagnóstico por imagem , Melanoma/diagnóstico por imagemRESUMO
BACKGROUND: The objective was to review a decade of plasmacytoma (PC) and multiple myeloma (MM) data from French Guiana, and to study its spatial and temporal trends. METHODS: This was a retrospective study of MM and PC between January 2005 and December 2014 using cancer registry data, including age-standardized incidence and mortality rates. RESULTS: There were 110 cases of PC and MM (62 women and 48 men), representing the eighth most frequent malignancy in French Guiana. PC and MM were much more common in females. In men, 79% of cases occurred at ≥55 years, and in women, 90% of cases occurred at ≥50 years. The median age at diagnosis was 60 years for men and 66 years for women, while it was 72 years for men and 75 years for women in mainland France. The incidence rate standardized to the world population was 5.9 patients of PC and MM per 100,000 men/year and 7.8 per 100,000 women/year. CONCLUSIONS: In our territory, the incidence of PC and MM was higher and patients were diagnosed at a substantially younger age than in mainland France. Women had a greater incidence than men, and there was an increasing temporal trend of incidence among women. African ancestry and the frequency of obesity, notably among women, could have contributed to this observation.
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RESUMEN Presentamos el caso de un paciente afectado por un mieloma múltiple refractario a diversas líneas de tratamiento, que ingresó por hemoptisis causada por la aparición de un plasmocitoma en la tráquea. El hallazgo se produjo por broncoscopia y el diagnós tico y tratamiento se realizó mediante técnicas endoscópicas, con muy buen resultado funcional. El caso es de interés por su escasa frecuencia, así como para dar a conocer a la comunidad este tipo de presentación atípica y su posible manejo.
ABSTRACT We present the case of a patient affected by multiple myeloma refractory to various lines of treatment who was admitted due to hemoptysis caused by the appearance of a plasmacytoma in the trachea. The finding was obtained from a bronchoscopy, and the diagnosis and treatment were made by means of endoscopic techniques, with a very good functional result. This case is of interest because it is unusual and also because it allows us to raise awareness among the community of this atypical presentation and possible managemen
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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.
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Humanos , Masculino , Pessoa de Meia-Idade , Plasmocitoma/cirurgia , Plasmocitoma/diagnóstico , Neoplasias Encefálicas/cirurgia , Neoplasias Encefálicas/diagnóstico , Neoplasias dos Seios Paranasais/cirurgia , Neoplasias dos Seios Paranasais/diagnóstico , Neoplasias Nasais/cirurgia , Neoplasias Nasais/diagnóstico , Plasmocitoma/radioterapia , Biópsia , Neoplasias Encefálicas/radioterapia , Neoplasias dos Seios Paranasais/radioterapia , Tomografia Computadorizada por Raios X , Neoplasias Nasais/radioterapia , Resultado do TratamentoRESUMO
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.
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Humanos , Feminino , Pessoa de Meia-Idade , Plasmocitoma/cirurgia , Plasmocitoma/patologia , Plasmocitoma/diagnóstico por imagem , Neoplasias da Orelha/cirurgia , Neoplasias da Orelha/patologia , Neoplasias da Orelha/diagnóstico por imagem , Plasmócitos , Espectroscopia de Ressonância Magnética , Tomografia Computadorizada por Raios X , Diagnóstico Diferencial , Orelha , Meato Acústico ExternoRESUMO
Importance: Adenopathy and extensive skin patch overlying plasmacytoma syndrome is a paraneoplastic syndrome characterized by a cutaneous vascular patch overlying a plasmacytoma and systemic manifestations. It is thought to be an early stage of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome, which is a rare, but potentially fatal multisystemic disease that is associated with plasma cell dyscrasia. Thus, a high index of suspicion is required to identify patients with adenopathy and extensive skin patch overlying plasmacytoma as they may present with early polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes, which is curable if detected early. Objective: To report additional cases of adenopathy and extensive skin patch overlying plasmacytoma syndrome, describe dermatoscopic and histologic findings of the cutaneous patch and review all up to date literature on adenopathy and extensive skin patch overlying plasmacytoma syndrome. Design: Case series from a single tertiary care center. Participants: Here, we present the second case series of three patients with adenopathy and extensive skin patch overlying plasmacytoma syndrome who all meet the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. The diagnosis was suspected based on the presence of the violaceous cutaneous patch along with symptoms of systemic involvement (fatigue, weight loss, weakness). Dermoscopy revealing regular dilated parallel capillaries was suggestive of a benign/reactive vascular process. Histopathology in all three cases showed reactive vascular proliferation with a characteristic 90° branching. To date only 20 cases of adenopathy and extensive skin patch overlying plasmacytoma have been published, including ours. All patients presented with cutaneous lesions (violaceous patch and others) and most, at least 15/20, met the diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. When clinical follow-up was reported, most patients had a favorable prognosis with partial or complete symptom resolution following treatment of the underlying plasmocytoma.
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BACKGROUND: Solitary bone plasmacytoma is a plasmatic cell dyscrasia; its presentation in the posterior fossa is very rare. CASE DESCRIPTION: We present two cases, a 59-year-old male and a 50-year-old female, both with heterogeneous clinical presentation. One had symptoms compatible with endocranial hypertension, and the other presented with a hemispheric cerebellar syndrome and ipsilateral trigeminal neuralgia. They were both related to an intraosseous tumor of the occipital region near the torcula with large extension to the posterior fossa. The diagnosis of a plasma cell neoplasm arising from the diploe of the squamous portion of the occipital bone was confirmed with immunohistochemistry. CONCLUSION: The treatment for a cranial tumor that is suspected to be a solitary bone plasmacytoma requires a multidisciplinary team to diagnose, plan a total resection, and after surgery continue with the follow-up of the patient. Solitary bone plasmacytoma should be considered as a differential diagnosis for a tumor that produces cancellous bone widening without sclerotic borders.
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BACKGROUND: Despite mainly benign, exophytic subcutaneous cranial masses present with a myriad of differential diagnosis possibilities, ranging from simple, superficial lesions to complex lesions involving the central nervous system. Although the gold standard imaging modality for the diagnosis of these lesions is magnetic resonance imaging, Doppler Ultrasonography can be a useful, inexpensive, and available tool for evaluation of lesions that could potentially be safely treated in the primary care setting, and lesions that would demand advanced neurosurgical care. CASE DESCRIPTION: This patient presented with a complex exophytic plasmocytoma that was first diagnosed and erroneously approached as a subcutaneous lipoma with surgical resection in an outpatient surgical setting. This interpretive approach resulted in the failure of the procedure due to significant hemorrhage. The patient was immediately referred to neurosurgical care and transferred to our center. Admission doppler ultrasound imaging revealed absence of the frontal bone, the enriched and profuse vascularization, allowing further and proper diagnostic approach and treatment. CONCLUSION: Ultrasound could be a reliable, fast, and simple imaging method aiding practitioners to perform a better workup for patients with exophytic subcutaneous cranial masses.
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Plasmacytoma is a neoplastic proliferation of monoclonal plasma cells, which can present clinically as solitary bone neoplasm, extramedullary plasmacytoma, and multiple myeloma. The biological behavior of these tumors is variable from periods of clinical latency to rapid growth and progression from localized forms to more disseminated multiple myeloma. We present the case of solitary plasmacytoma of the mandible with rare bilateral involvement in a 65-year-old female patient. This paper highlights the importance of understanding the maxillofacial manifestations of the disease by the dentist for early diagnosis and thus better prognosis.
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BACKGROUND: There have been reports on the use of hypofractionated stereotactic body radiotherapy (SBRT) for bone plasmacytomas, but no prospective data are available. We present the initial analysis of an ongoing prospective protocol on SBRT addressing the feasibility and safety of this treatment for solitary bone plasmacytomas. PATIENTS AND METHODS: A prospective cohort of SBRT for solitary bone plasmacytoma was developed. Patients could receive different doses depending on the index bone, from single fraction for skull base lesions, 24 Gy in 3 fractions for spine lesions, and 30 Gy in 5 fractions for other bones. Overall survival, bone events, local control, and progression to multiple myeloma (MM) were measured and compared to our retrospective cohort of patients treated with conformal standard-dose radiotherapy. Quality of life was assessed via the EORTC QLQ-C30 questionnaire, and toxicities were assessed by the CTCAE v5.0 criteria. After 1 year or the inclusion of 5-10 patients, a feasibility and safety analysis was programmed. RESULTS: Between April 2018 and April 2019, 5 patients were included. All were male, with a median age of 53.1 years. The median follow-up was 21.8 months. No patient had local progression, bone event, or died. Two patients had progressions to MM. The mean survival free of progression to MM was 18.6 months, compared to 19 months in the retrospective cohort; median values were not reached. There were no grade 3 toxicities. CONCLUSION: SBRT for plasmacytoma is safe and feasible. More robust data are awaited.
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Neoplasias Ósseas/radioterapia , Plasmocitoma/radioterapia , Radiocirurgia , Adulto , Idoso , Neoplasias Ósseas/complicações , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/psicologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/etiologia , Plasmocitoma/complicações , Plasmocitoma/mortalidade , Plasmocitoma/psicologia , Intervalo Livre de Progressão , Estudos Prospectivos , Qualidade de Vida , Doses de Radiação , Radiocirurgia/efeitos adversos , Inquéritos e QuestionáriosRESUMO
Las neoplasias malignas de la cavidad oral en gran medida (90%) consisten en carcinoma de células escamosas que surgen de la mucosa de revestimiento. El 10% restantes de neoplasias malignas orales de un grupo heterogéneo de tumores de diferente etiología. Presentamos dos casos de patología oncohematológica: Mieloma Múltiple (AU)
Malignant neoplasms of the oral cavity largely (90%) consist of squamous cell carcinoma arising from the lining mucosa. e remaining 10% of oral malignancies from a heterogeneous group of tumors of different etiology. We present two cases of oncohematological pathology: Multiple Myeloma (AU)
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Humanos , Masculino , Pessoa de Meia-Idade , Plasmocitoma/diagnóstico , Plasmocitoma/patologia , Plasmocitoma/diagnóstico por imagem , Neoplasias Bucais/diagnóstico , Radioterapia , Biópsia/métodos , Tomografia Computadorizada por Raios X , Procedimentos Cirúrgicos Bucais/métodos , Difosfonatos/uso terapêutico , Seio Maxilar/cirurgia , Mieloma MúltiploRESUMO
Multiple myeloma is characterized by malignant proliferation of clonal plasma cells. Usually, appears as a generalized disease but it can present as solitary bone plasmacytoma or a solitary soft tissue mass or extramedullary plasmacytoma. In the case of extramedullary involvement, it could present as soft tissue plasmacytomas and the prognosis is poor. The 18F-FDG PET/CT could be a valuable tool for characterization of the medullary and extramedullary involvement. We present a case of F18-FDG PET/CT with extramedullary involvement with soft tissue plasmacytomas in the setting of MM.
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BACKGROUND: Plasma cell neoplasms are characterized by the proliferation of a single clone of plasma cells with production of a monoclonal immunoglobulin. They can manifest as a single lesion (plasmacytoma) or as multiple lesions (multiple myeloma). METHODS: Paraffin-embedded tissue blocks of patients microscopically diagnosed with plasma cell neoplasms in the jaws were retrieved from five pathology files. Data including clinical, radiographic, microscopic and immunohistochemical findings, treatment employed and follow-up status were retrieved from the pathology reports. RESULTS: Fifty-two cases were retrieved (mean age: 59.4 years) without sex predilection. The mandible was the most affected site (67.3%), usually associated with pain and/or paresthesia (53.8%). Lesions in other bones besides the jaws were reported for 24 patients (46.2%). Radiographically, tumours usually presented as poorly defined osteolytic lesions with unilocular or multilocular images, while microscopy revealed diffuse proliferation of neoplastic plasma cells with nuclear displacement and abundant eosinophilic cytoplasm. Two cases were classified as anaplastic, and amyloid deposits were found in two other cases. Immunohistochemistry was positive for plasma cell markers and negative for CD20 and CD3, and monoclonality for kappa light chain predominated. The overall survival rate after 5 years of follow-up was 26.6%. CONCLUSION: Plasma cell neoplasms are aggressive tumours with a poor prognosis and involvement of the jaws may be the first complaint of the patient. Thus, oral pathologists, head and neck surgeons and dentists should be aware of their clinical, radiographic and microscopic manifestations.