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 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 en pediatría: una rara entidad / Pediatric solitary bone plasmacytoma: a rare entity

El plasmocitoma en edad pediátrica es poco frecuente. Se presenta a una adolescente de 14 años que ingresó por una cuadriplejia fláccida asociada a una tumoración a nivel de cráneo y columna cervical. En el estudio histológico se diagnosticó un plasmocitoma en ambos sitios, sin infiltración medular. Recibió tratamiento con radio y quimioterapia. A los tres años se encontró una nueva lesión a nivel del ovario izquierdo, que coincidió histológicamente con el diagnóstico inicial. El plasmocitoma solitario de hueso se describe como el más común en edad pediátrica con tendencia a la recidiva tumora(AU)

Plasmacytoma in the pediatric age group is rare. We present a 14-year-old girl admitted with a flaccid quadriplegia associated with a tumor at the level of the skull and cervical spine. In the histological study plasmacytoma was diagnosed in both sites without bone marrow infiltration. The patient was treated with radiation and chemotherapy. After three years a new lesion on the left ovary was found, which histologically coincided with the initial diagnosis. Solitary bone plasmacytoma is described as the most common in pediatric patients prone to tumor recurrence(AU)

Plasmocitoma de la mama / Breast plasmocytoma

El plasmocitoma de la mama es muy infrecuente y en la mayoría de los casos aparece asociado a mieloma múltiple. Se describe una paciente de 42 años de edad con diagnóstico conocido de plasmocitoma solitario del cuello asociado a mieloma múltiple, que 11 meses después de alcanzada la remisión se presenta con una tumoración en la mama derecha sin manifestaciones sistémicas de mieloma. El estudio histológico e inmunohistoquímico del tumor confirmaron el diagnóstico de plasmocitoma. El diagnóstico diferencial en ocasiones es difícil y debe hacerse con tumores primarios y secundarios de la mama. El tratamiento de elección es la radioterapia local siempre que no existan manifestaciones sistémicas de la enfermedad subyacente(AU)

Breast plasmocytoma is infrequent and in most of the cases is associated to multiple myeloma. We describe a 42 year old woman with a diagnosis of solitary plasmocytoma of the neck associated to multiple myeloma that went into remission and eleven months later she presented a right breast lump which was confirmed by core biopsy and immunohistochemical study to be a plasmocytoma. Sometimes differential diagnosis is difficult and should be performed in primary and secondary breast tumours. The election treatment is radiotherapy if there are not manifestations of a subyacent sistemic disease(AU)

Multifocal extramedullary plasmocytoma of the sinonasal region: a case report and literature review / Plasmocitoma multifocal extramedular de la región sinonasal: reporte de un caso y revisión de la literatura

We report on a patient who presented to ENT services with right side epistaxis, frontal lobe headache, right infraorbital pain and the feeling of having a stuffy nose. CT and MRI were performed and later a biopsy confirmed the presence of sinonasal plasmocytoma. The Head and Neck oncology committee recommended radiotherapy as the choice of treatment. At the present time the patient is being followed on scheduled medical visits.

Se reporta el caso de un paciente que se presentó en el servicio de Otorrinolaringología con epistaxis, cefalea del lóbulo frontal derecho, dolor infraorbitario derecho y la sensación de tener la nariz tapada. Se realizaron TC y RNM, y luego una biopsia confirmó la presencia de un plasmocitoma nasosinusal. El comité de oncología de cabeza y cuello recomendó radioterapia como tratamiento de elección. En la actualidad, el paciente está en siguimiento y control en las visitas médicas regulares.

Plasmablastic extramedullary plasmacytoma.

Extramedullary plasmactyoma is the solitary, soft tissue form of plasma cell neoplasm but lack the defining features of medullary or multiple myeloma. The diagnosis is difficult to make in routine practice setting due to the morphological and immunohistochemical overlap with plasmablastic lymphoma. We report a case of plasmablastic extramedullary plasmacytoma in a 52-year-old in the mandibular lingual gingiva and discuss its differential from plasmablastic lymphoma. The gingival mass regressed with primary radiotherapy.

Resección quirúrgica de plasmocitoma solitario del esternón asociado a cirugía de revascularización miocárdica: seguimiento a largo plazo / Surgical resection of solitary plasmacytoma of the sternum associated with coronary artery bypass grafting surgery: long-term follow up

The plasmacytomas tumors are caused by a malignant proliferation of plasma cells. Primary tumors of the sternum are uncommon, being this kind of tumors exceptional. We present the case of a 72 years old man with a sternal plasmacytoma, that after 2 years of receiving radiotherapy treatment, had tumor growth, associated with coronary artery disease. In this case, surgical resection of solitary sternal plasmacytoma was performed associated with coronary artery bypass grafting surgery. The chest wall reconstruction was done with a polypropylene mesh. More than 5 years long-term follow up was completed and the patient is asymptomatic and free of malignant proliferation of plasma cells.

Los plasmocitomas son tumores originados por una proliferación neoplásica de células plasmáticas. Los tumores primarios de esternón son infrecuentes, siendo los plasmocitomas solitarios de esternón excepcionales. Presentamos el caso de un hombre de 72 años con un plasmocitoma esternal que después de 2 años post tratamiento con radioterapia presentó aumento de tamaño tumoral asociado a enfermedad coronaria. Se realizó resección quirúrgica del plasmocitoma solitario del esternón asociada a cirugía de revascularización miocárdica con 4 bypass coronarios. La pared torácica fue reconstituida con una malla de polipropileno. A más de 5 años de seguimiento el paciente se encuentra asintomático cardiovascular y libre proliferación maligna de células plasmáticas.

Plasmacytoma in the oral cavity: a case report / Plasmocitoma en la cavidad oral: reporte de caso

The plasma cell neoplasms may present in soft tissue as extramedullary plasmacytoma (EMP), in bone as a solitary plasmacytoma of bone (SPB), or as part of the multifocal disseminated disease multiple myeloma (MM). The EMP is rare, comprising around 3 percent of all plasma cell neoplasm. The majority (80 percent) occurs in the head and neck region. In this study we report a case of a man, 70 years old, melanoderm, with a lesion of the oral cavity. Upon physical examination, a lesion was found that extended throughout the posterior upper alveolar ridge, as far as the maxillary tuber on the left side, extending towards the palate. Radiographic examination, complementary laboratory exams were performed. Based on the conclusive symptoms of plasmacytoma, the patient was referred to the hematology service for treatment with local radiotherapy. The patient responded satisfactorily to the treatment, and after 15 months, all clinical symptoms of the lesion in the oral cavity had disappeared.

La neoplasia de células plasmáticas se presenta en los tejidos blandos como plasmocitoma extramedular (EMP), en el hueso como plasmocitoma solitario (SPB), o como parte de una enfermedad diseminada, el mieloma múltiple (MM). El EMP es poco frecuente, manifestandose en alrededor de un 3 por ciento. Se presentan generalmente (80 por ciento) en las regiones de cabeza y cuello. En este reporte de caso, un hombre de 70 años, melanoderma, presentaba una lesion en la cavidad oral. En el examen físico se observó la lesión a lo largo de la parte posterior del reborde alveolar superior, en la tuberosidad maxilar en el lado izquierdo hacia el paladar. Se realizaron exámenes radiográficos y de laboratorio. Con base en los signos de plasmocitoma, el paciente fue referido a tratamiento con radioterapia. El tratamiento fue muy exitoso y después de 15 meses se observó que el paciente no presentaba los signos clínicos característicos de la lesión neoplásica.

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)

Plasmocitoma extramedular nasal: presentacíon de un caso y revisión de literatura / Extramedullary plasmocytoma: case report and literature review

Extramedullary plasmocytoma is a rare plasmatic cell neoplasia which mostly compromises the head and neck (80-90%). On initial presentation they must be differentiated from multiple myeloma This may prove to be difficult because a varying percentage may be associated at a later date with the development of multiple myeloma. We describe a case of extramedullary plasmacytoma in the nose and we discussed and review the literature.

Plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma: case report / Plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo: relato de caso

The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.

Os autores relatam um raro caso de plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo em uma paciente de 42 anos. A lesão foi removida cirurgicamente e o exame anatomopatológico, aliado à imuno-histoquímica, revelou positividade para imunoglobulina G e cadeias leve tipo Kappa. Após extensa avaliação sistêmica, nenhuma anormalidade foi observada, sendo feito o diagnóstico de plasmocitoma solitário ósseo. A paciente foi submetida à radioterapia, permanecendo livre da doença por 5 anos e 6 meses até 2007, quando apresentou uma fratura patológica devido à infiltração plasmocitária na coluna lombar, sendo feito diagnóstico de mieloma múltiplo. Uma vez que o plasmocitoma solitário ósseo pode ser a primeira manifestação do mieloma múltiplo, esses pacientes necessitam de adequado diagnóstico e seguimento a longo prazo, pois o tratamento e prognóstico dessas duas condições é diferente.

High dose-rate brachytherapy as a treatment option in primary tracheal tumors

OBJETIVO: Apresentar a experiência do tratamento de 4 pacientes com tumores primários de traquéia, não operados, submetidos à braquiterapia endobrônquica de alta taxa de dose. PACIENTES E MÉTODOS: Dois casos de carcinoma espinocelular, uma recidiva de carcinoma adenóide cístico e uma recidiva de plasmocitoma primário da traquéia. Todos receberam braquiterapia endobrônquica, exclusiva ou como reforço de dose da radioterapia externa. Foram administradas 3 ou 4 frações de 7,5 Gy cada, calculados a 1 cm de profundidade. O seguimento foi considerado a partir do término da braquiterapia. RESULTADOS: Em todos os casos houve resposta completa. Dois pacientes com carcinoma espinocelular evoluíram a óbito em 6 e 33 meses após a braquiterapia, o primeiro, sem evidência de doença e o outro por recidiva local, respectivamente. As outras 2 pacientes encontravam-se vivas após 64 e 110 meses de seguimento, sem evidência de doença. Esses 2 casos apresentaram estenose traqueal em 22 e 69 meses após a braquiterapia, sendo necessária a colocação de prótese traqueal apenas na paciente com carcinoma adenóide cístico. CONCLUSÕES: A braquiterapia endobrônquica de alta taxa de dose pode ser utilizada tanto como reforço de dose da irradiação externa quanto em recidivas. O controle local obtido em 3 de 4 pacientes indica que casos individuais podem se beneficiar desse procedimento. Sobrevida a longo prazo pode ser observada, principalmente nos casos de histologia adenóide cística.

Lymph node involvement in plasmacytoma

A case of plasmacytoma of the spine with multiple sites of involvement at presentation is reported. The sites involved were T4, T5, T9 levels and the jugular foramen. The patient had no evidence of bone marrow involvement or paraprotein either in the serum or urine. Resections of the tumour from T4 and T5 levels was performed, followed by radiation therapy. The patient developed a recurrence seven and half years after the initial presentation. The recurrence occurred at the right clavicle and in the soft tissue around it. This was followed by multiple recurrences at the same site with involvement of the supraclavicular lymphnodes inspite of aggressive chemotherapy, radiation therapy and autologous stem cell transplant. Fine Needle Aspiration cytology and histology showed a typical plasma cell neoplasm with characterstic nuclear features, immunohistochemistry was compatible with a plasmacytoma