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.

Cavidad oral como sitio de asiento de enfermedades oncohematológicas. Plasmocitoma extramedular: a propósito de dos casos / The oral cavity as a reservoir of oncohematological diseases. Extramedullary plasmocytoma: a propos of two cases

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)

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 mamario y cáncer de mama: a propósito de un caso / Breast Plasmacytoma and breast cancer: a case report

El plasmocitoma mamario es una neoplasia de células plasmáticas extremadamente infrecuente, con menos de cincuenta casos descritos en el último siglo. Por este motivo, apenas se dispone de datos acerca del abordaje, tratamiento y seguimiento más convenientes. Presentamos el caso de una paciente de 70 años que debutó con un plasmocitoma mamario y que un año después fue diagnosticada de un carcinoma mamario lobulillar ipsilateral. La asociación entre plasmocitoma y cáncer de mama no está descrita en la literatura, por lo que es muy complicado establecer un vínculo entre ambas entidades. Sin embargo, el abordaje terapéutico del plasmocitoma podría comprometer el tratamiento ulterior de un cáncer de mama, por lo que el tratamiento idóneo en estos casos sea probablemente la cirugía.

Breast plasmocytoma is an extremely rare plasma cell neoplasm, with less than 50 cases reported in the last century. This is the reason why we barely have data about optimal management, treatment and follow-up. We hereby report the case of a 70 year old woman diagnosed with breast plasmocytoma that developed lobular breast cancer a year later. The link between plasmocytoma and breast cancer has not been previously established. However, breast plasmocytoma treatment could compromise latter breast cancer approach, so probably the most suitable strategy in these cases should be breast surgery.Conclusions: There are clinical characteristics associated with complications in women with surgical management abortion in our center, such as admission diagnosis, unplanned pregnancy, previous abortion and type of evacuation. There are limitations regarding the quantity and quality of information, however, our results allow us to know the profile of patients treated for abortion in our center.

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)

Solitary plasmacytoma of the jaws: therapeutical considerations and prognosis based on a case reports systematic survey / Solitary plasmacytoma of the jaws: therapeutical considerations and prognosis based on a case reports systematic survey

Abstract Introduction: Solitary plasmacytoma is a rare malignant tumor of plasma cells with no evidence of systemic proliferation. There are two known subtypes: extramedullary solitary plasmacytoma and solitary bone plasmacytoma. The etiology is still unknown. Both lesions present a risk of progression to multiple myeloma. A number of approaches have been used for treatment of solitary plasmacytoma. Objective: To carry out a systematic review of the case reports described in the literature, focusing on therapeutic and prognostic aspects. Methods: A search of clinical case reports was performed in the PubMed database using Mesh Terms related to "plasmacytoma" under the following criteria: type of study (case report), articles in English language, conducted in humans, with no publication date limits. Results: Of the 216 articles found, only 21 articles met the pre-established inclusion criteria. Conclusion: The occurrence of solitary bone plasmacytoma in the bones of the face is a rare condition prevalent between the 4th and 6th decades of life, located in the posterior region of the mandible in most cases. Histopathological examination and systemic investigation are mandatory for confirmation of diagnosis.

Resumo: Introdução: O plasmocitoma solitário é um tumor maligno raro de células plasmáticas sem evidência de proliferação sistêmica e engloba dois subtipos: plasmocitoma solitário extramedular e plasmocitoma solitário ósseo. A etiologia ainda é desconhecida. Ambas as lesões apresentam risco de progressão para mieloma múltiplo. Uma série de abordagens tem sido usada para seu tratamento. Objetivo: Realizar uma revisão sistemática da literatura com enfoque nos aspectos terapêuticos e prognósticos. Método: Realizou-se uma busca de relatos de caso clínico na base de dados PubMed com termos de busca relacionados com "plasmocitoma" sob os seguintes critérios: tipo de estudo (relato de caso), artigos na língua inglesa, estudos realizados apenas em humanos, sem limites de data de publicação. Resultados: Dos 216 artigos encontrados, apenas 21 preencheram os critérios de inclusão pré-estabelecidos. Conclusão: A ocorrência de plasmocitoma solitário ósseo nos ossos da face é uma condição rara prevalente entre a 4a e a 6a décadas de vida, localizada na região posterior de mandíbula na maioria dos casos. O exame histopatológico e a investigação sistêmica são mandatórios para confirmação do diagnóstico.

Plasmocitoma intraconal e infiltração uveal em paciente portadora de mieloma múltiplo / Intraconal plasmocytoma and uveal infiltration in a patient with multiple myeloma

Resumo Mieloma múltiplo (MM) é uma neoplasia que cursa com a proliferação desordenada de clones de plasmócitos, produzindo imunoglobulina monoclonal e normalmente se apresenta como lesões osteolíticas. Em alguns casos, porém, esta doença apresenta-se como massas, chamadas de plasmocitomas. O acometimento ocular e orbitário é incomum nesta patologia. Neste trabalho, descrevemos o caso de uma paciente de 63 anos com diagnóstico prévio de MM que evoluiu com um plasmocitoma intraconal em olho direito, bem como uma massa vascularizada câmara anterior proveniente de infiltração uveal. Essas lesões foram correlacionadas MM e culminaram com a perda visual no olho acometido. Não foi encontrado na literatura relatos de plasmocitoma intraconal.

Abstract Multiple myeloma (MM) leads to disorderly proliferation of plasma cells clones, producing monoclonal immunoglobulin and commonly presents osteolytic lesions. In some cases, however, masses called plasmocytomas are found. Ocular and orbital involvement is unusual in this pathology. In this paper, we describe a case of a 63 year-old patient with previous diagnostic of MM that evolved an intraconal plasmocytoma in the right eye, as well as a vascularized mass in the anterior chamber from uveal infiltration. These lesions were correlated to MM e lead to visual loss in the affected eye. Reports of intraconal plasmocytoma have not been found in literature.

Plasmocitoma: desafio diagnostico em paciente idosa com apresentação clínica atípica. relato de caso / Difficult to diagnose plasmocytoma with atypical clinical presentation in an older patient: a case report

INTRODUÇÃO E OBJETIVO: O plasmocitoma é uma proliferação neoplásica de um clone de plasmócitos que produzem imunoglobulina monoclonal. O plasmocitoma solitário ósseo é definido pela presença de um plasmocitoma na ausência de múltiplas lesões osteolíticas ou outros achados compatíveis com mieloma múltiplo. O presente artigo relata o caso de uma paciente idosa que apresentou plasmocitoma ósseo solitário de esterno de difícil diagnóstico devido a manifestações clínicas atípicas e possível evolução para mieloma múltiplo. RELATO DO CASO: Paciente de 74 anos, do sexo feminino. Procurou atendimento médico por confusão mental, taquidispneia e dor torácica precordial em queimação, além de história de queda com trauma da região anterior do tórax. Na internação hospitalar evoluiu com hiponatremia refratária ao tratamento, tromboflebite superficial em membro superior esquerdo, celulite periorbitária, endoftalmite e osteomielite no esterno. A eletroforese de proteínas séricas teve como resultado o aumento policlonal na região das gamaglobulinas. A biópsia da lesão considerou achado compatível com plasmocitoma. Tomografia computadorizada de esqueleto não evidenciou lesões adicionais. Apresentou uma evolução ruim com insuficiência renal crônica agudizada por provável síndrome de lise tumoral, indo a óbito. CONCLUSÃO: O estudo vem ressaltar a importância da hipótese diagnóstica de plasmocitoma/mieloma múltiplo frente a um paciente idoso, com manifestação clínica diversificada, como anemia, insuficiência renal, fratura patológica e infecções de repetição, em vista de sua evolução e prognóstico.

INTRODUCTION AND OBJECTIVE: Plasmacytoma is a neoplastic proliferation of a plasma cell clone which produces monoclonal immunoglobulin. Solitary plasmacytoma of bone is defined by the presence of a single plasmacytoma in the absence of multiple osteolytic lesions or other findings compatible with multiple myeloma. We report an older patient with solitary plasmacytoma of the sternum associated with atypical clinical findings which complicated diagnosis and possible progression to multiple myeloma. CASE REPORT: A 74-year-old woman sought medical care due to mental confusion, tachydyspnea, and chest pain after a fall with blunt trauma to the anterior chest. During admission, the patient developed refractory hyponatremia, superficial thrombophlebitis in the left upper limb, periorbital cellulitis, endophthalmitis, and sternal osteomyelitis. Serum protein electrophoresis showed a polyclonal increase in gamma globulins. Biopsy result was compatible with plasmacytoma. A computed tomography scan showed no additional lesions. Progression was poor, with chronic renal failure exacerbated by probable tumor lysis syndrome, resulting in death. CONCLUSION: The present findings emphasize the importance of considering a diagnostic hypothesis of plasmacytoma/multiple myeloma in older patients with a set of varied clinical signs, such as anemia, renal failure, pathological fracture, and repetitive infections

Fine needle aspiration cytology in feline skeletal muscle as a diagnostic tool for extramedullary plasmacytoma / Citologia aspirativa por agulha fina no músculo esquelético felino como ferramenta diagnóstica para o plasmocitoma extramedular

Extramedullary noncutaneous plasmacytoma (ENP) is a myeloproliferative disorder of plasma cells that rarely affects cats. This paper describes an ENP case revealed by fine needle aspiration cytology (FNAC) of the mass in the skeletal muscle of an 8-month-old, male, mixed breed cat, which had a nodule in the left hind limb. The rapid immunoassay test confirmed the presence of feline leukemia virus (FeLV). The animal necropsy macroscopically showed the nodule came from the semimembranosus muscle. Histopathological examination ratified the cytological findings. Thus, this paper alerts to the existence of plasmacytoma located in the skeletal muscle of feline species. FNAC is a quick and efficient method for diagnosis of ENP.(AU)

O plasmocitoma extramedular (PEM) não cutâneo é um distúrbio mieloproliferativo de plasmócitos que raramente acomete felinos. Este trabalho descreve um caso de PEM no músculo esquelético de um gato, macho, sem raça definida, de oito meses de idade, que apresentava um aumento de volume no membro pélvico esquerdo. A citologia aspirativa por agulha fina (CAAF) da massa revelou tratar-se de PEM. O teste imunoenzimático rápido confirmou a presença do vírus da leucemia felina (FeLV). Na necropsia do animal, macroscopicamente, percebeu-se que o nódulo era originário do músculo semimembranoso. O exame histopatológico ratificou os achados citológicos. Desta forma, alerta-se para a existência de plasmocitoma com localização em músculo esquelético na espécie felina, sendo a CAAF um método alternativo rápido e eficaz para o seu diagnóstico.(AU)

Plasmocitoma extramedular del tabique nasal: reporte de un caso y revisión bibliográfica / Extramedullary plasmacytoma nasal septum: case report and literature review

Los plasmocitomas son tumores malignos caracterizados por proliferación anormal de células plasmáticas monoclonales. Éstos pueden originarse tanto en hueso como en tejidos blandos, denominados plasmocitomas óseos solitarios y plasmocitomas extra-medulares (PEM), respectivamente. Estos últimos representan menos del 1% de toda la patología maligna de cabeza y cuello, sin embargo, el reporte de estos casos resulta provechoso frente a la poca casuística al respecto. Presentamos el caso de plasmocitoma extramedular del septo nasal en un varón de 74 años con dificultad respiratoria nasal progresiva y frecuente epistaxis del lado izquierdo. A la especuloscopía nasal se observó en el vestíbulo una elevación tumoral de superficie lisa, grisácea, sésil. La biopsia mostró que era un plasmocitoma. Estudios posteriores descartaron la presencia de mieloma múltiple. Esto confirmó el diagnóstico de PEM. La masa fue completamente disecada, se indicó radioterapia y el paciente no tuvo adherencia al tratamiento. Sobre el mismo lecho al año siguiente reaparece lesión de similares características. Conclusión: Plasmocitoma extramedular del tabique nasal es una entidad rara, de una larga historia natural y que representa un desafío diagnóstico y terapéutico. Dependiendo de la resecabilidad de la lesión, la terapia combinada mediante cirugía y radioterapia es el tratamiento de elección.

Plasmacytomas are malignant tumors characterized by abnormal proliferation of monoclonal plasma cells. They may originate in bone and soft tissue , called solitary bone plasmacytoma and extramedullary plasmacytomas (EMP) respectively. The latter represents less than 1% of all malignant disease of the head and neck, however, the report of these cases it is useful to cope with the shallow casuistics thereon. We report a case of EMP of the nasal septum in a man of 74 years with progressive nasal breathing difficulties and frequent epistaxis on the left side. A nasal speculoscopy was observed in tumor lift lobby smooth, gray, sessile surface. The biopsy showed it was a plasmacytoma. Subsequent studies reject the presence of multiple myeloma. This confirmed the diagnosis of EMP. The mass was completely dissected, radiation therapy is indicated and the patient had no adherence. Upon the same place a similar injury returns. Conclusion: Septum extramedullary plasmacytoma is a rare entity of a long natural history and represents a diagnostic and therapeutic challenge. Depending on the resectability of the lesion, combination therapy with surgery and radiation therapy is the treatment of choice.

Plasmocitoma extramedular primario colorrectal / Primary extramedullary colorectal plasmacytoma

Aim: To present a patient with an uncommon primary extramedullary plasmocytoma in the colon. Case report: We report a case of a 64 year old female with wasting syndrome and low-gastrointestinal bleeding. Histology described colon and rectum with plasma cell infiltration in the muscle layer. The immunohistochemistry showed CD45 (+) and lambda (+). Infiltration of bone marrow and other places with a thoraco-abdominal-pelvic scan, blood tests, bone survey and bone marrow biopsy was discarded. Surgical management was performed by laparoscopic low anterior resection with end colostomy. Conclusion: Primary plasmacytoma of colon is a rare clinical entity that is a challenge for the pathologist and surgeon, but early diagnosis contributes to early handling.

Objetivo: Presentar un caso de esta poco frecuente patología en el colon. Caso clínico: Se reporta el caso de una paciente mujer de 64 años con síndrome consuntivo y sangrado digestivo bajo. La anatomía patológica describe colon y recto con infiltración de células plasmáticas en la capa muscular. La inmunohistoquímica demostró CD45 (+) y lambda (+). Se descartó la infiltración de médula ósea y otros lugares con una tomografía abdomino-pélvica, análisis de sangre, radiografía de cráneo y biopsia de médula ósea. El manejo quirúrgico se realizó mediante resección anterior baja laparoscópica con colostomía terminal. Conclusión: El plasmocitoma primario de colon es una entidad clínica rara que constituye un reto para el patólogo y cirujano, pero su diagnóstico oportuno contribuye a un manejo temprano.

Plasmocitoma solitário do osso: relato e discussão de um caso de acometimento incomum / Solitary plasmacytoma of the bone: report and discussion of a case of involvement unusual

Plasmocitoma é uma proliferação clonal e neoplásica de células plasmáticas. Paciente, sexo feminino, 51 anos, sem história de tabagismo e consumo de álcool mostrou leve inflamação e inchaço na gengiva vestibular do dente 48 com mobilidade grau I. Radiograficamente verificou--se a presença de imagem radiolúcida multilocular na região do ângulo mandibular. Durante a cirurgia confirmou-se a presença de uma lesão destacável, friável, contendo líquido acastanhado e purulento em seu interior. A lesão foi totalmente removida em conjunto com o dente 48. Ao exame histopatológico (HE), foi identificada uma neoplasia maligna caracterizada pela proliferação de células plasmocitoides pleomórficas com núcleos intensamente hipercromáticos. Estas células exibiam núcleo basofílico com notável distribuição irregular da cromatina. Para confirmar o diagnóstico procedeu a avaliação imuno-histoquímica, que mostrou positividade para Plasm cell e Lambda e negatividade para Kappa. Com base nestes resultados concluiu-se o diagnóstico de plasmocitoma solitário do osso.

Plasmacytoma is a clonal proliferation and neoplastic of plasma cells. Patient, female, 51 years old, no history of smoking and alcohol consumption showed mild inflammation and swelling in the oral gingiva of the tooth 48 with mobility grade I. Radiographically verified the presence of radiolucent and multilocular image in the region of the mandibular angle. During surgery confirmed the presence of a lesion detachable, friable, browned and containing purulent fluid inside. This lesion was removed fully together with the tooth 48. Histopathology (HE) was identified a malignant neoplasm characterized by proliferation of plasmacytoid cells intensely hyperchromatic and pleomorphic. These cells exhibited large basophilic nucleus with remarkable irregular chromatin distribution. To confirm the diagnosis proceeded immunohistochemistry evaluation which showed positivity for Plasma Cell and Lambda and negativity of Kappa. Based on these findings concluded the diagnosis of plasmacytoma.

Extramedullary Plasmacytoma of Breast.

Solitary plasmacytomas are more frequent in males than females. We are describing a solitary extramedullary plasmacytoma of the breast in a 50 year old woman on fine needle aspiration cytology and confirmed on histology. During 6 months of fallow up, no other involvement was detected in the bone marrow or in any other site. Extramedullary plasmacytoma occur in lymph node, lung, thyroid, gastrointestinal tract testis, and skin. Extramedullary plasmacytoma of breast is a rare lesion. Especially those are not associated with multipal myeloma. Most reported cases represent secondary involvement or initial manifestation of the multipal myeloma.

Solitary Extramedullary Plasmacytoma in the Gastrointestinal Tract: Report of Two Cases and Review of Literature / 대한소화기학회지

Solitary extramedullary plasmacytoma (EMP) is a plasma cell neoplasm without bone marrow involvement. EMPs are rare in the gastrointestinal (GI) tract. We report two cases of primary EMP, one in the transverse colon and the other in the stomach. In the first case, a mass on the transverse colon was found on colonoscopy. The patient underwent left hemicolectomy and has been followed-up for 3 years without recurrence postoperatively. The latter case had several masses in the stomach. The patient underwent bypass surgery and has received supportive care for 1 month. Histopathologic specimens of both the cases showed a monoclonal lambda chain EMP. Subsequent investigations included a bone marrow biopsy, serum IgA, IgG, IgM and serum protein electrophoresis, and the results were negative for multiple myeloma in both the cases. Solitary EMP in the GI tract can be mistaken for colon cancer or stomach cancer on endoscopy; therefore, a sufficient number of biopsy specimens can help diagnose solitary EMPs. Surgical resection alone or with radiation therapy in cases with positive surgical margin is currently the only treatment for solitary EMP in the GI tract. Further study is necessary to determine disease prognosis and to investigate other treatment methods.

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.

Extramedullary nasal plasmacytoma: literature review and a rare case report

INTRODUCTION: Extramedullary plasmacytoma is a plasma cell tumor that grows within any of the soft tissues of the organism. Similar to all plasmacytic dyscrasia, the disease is extremely rare. AIM: This study reports a case of an extramedullary plasmacytoma of the nasal cavity and provides a literature review on the topic. CASE REPORT: A 51-year-old woman presented at our tertiary university hospital with a 6-month history of progressive nasal obstruction, predominantly to the right side, and self-limiting epistaxis. Examination revealed a large pale-reddish tumor within the right nasal cavity. Anatomopathological analysis showed features consistent with a plasmacytoma diagnosis, which was subsequently confirmed by immunohistochemical techniques. Further assessment revealed the solitary nature of the condition, consistent with extramedullary plasmacytoma. Radiotherapy was initiated, which led to partial regression of the symptoms. The tumor was surgically removed by using a mid-facial degloving approach. The patient evolved with a naso-oral fistula and underwent 3 corrective surgeries. No evidence of associated systemic disease was found after 5 years of follow-up. CONCLUSIONS: This case report, which describes a rare tumor of the nasal cavity, is expected to improve the recognition and referral of this condition by ear, nose, and throat (ENT) specialists for multidisciplinary management and long-term follow-up.

Plasmocitoma da glândula tireoide: relato de caso de uma rara neoplasia / Primary plasmacytoma of the thyroid gland: a case report of a rare neoplasia

Extramedullary plasmacytoma comprises 3%-5% of all plasma cell neoplasms, and approximately 80% of the cases occur in the upper respiratory tract. Primary thyroid plasmacytomas (PTP) are rare tumors. The authors report a case of PTP in a male patient with dyspnea and dysphagia. Physical examination and computerized tomography (CT) scan revealed a solid tumor affecting the thyroid gland, measuring 12 cm in its greatest dimension. Surgical biopsy was performed. Microscopy revealed a hypercellular malignant neoplasm composed of round plasmacytoid cells arranged in solid nests, which showed a positive immunoexpression for CD138, epithelial membrane antigen (AME), kappa light chains and multiple myeloma oncogene 1 (MUM1). The diagnosis of PTP was accordingly established.

O plasmocitoma extramedular constitui 3%-5% de todas as neoplasias de plasmócitos e aproximadamente 80% dos casos ocorrem no trato respiratório superior. Plasmocitomas primários da tireoide (TPP) são tumores raros. Os autores relatam um caso de TPP em um paciente masculino referindo dispneia e disfagia. O exame físico e a tomografia computadorizada revelaram tumor sólido comprometendo a glândula tireoide, medindo 12 cm na maior dimensão, o qual foi submetido à biópsia cirúrgica. À microscopia, foi identificada neoplasia maligna hipercelular constituída por células redondas/plasmocitoides dispostas em ninhos sólidos, as quais exibiam imunoexpressão positiva para CD138, antígeno da membrana epitelial (AME), cadeias leves kappa e oncogene mieloma múltiplo 1 (MUM1). O diagnóstico de TPP foi, então, estabelecido.

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.