Recent Research Progress of Extramedullary Plasmacytoma --Review / 中国实验血液学杂志

Extramedullary plasma cell tumor (EMP) is a kind of plasma cell tumor, and its pathogenesis is not completely clear. According to whether it is independent of myeloma disease, it can be divided into primary and secondary EMP, which have different biological and clinical characteristics. Primary EMP has low invasion, fewer cytogenetic and molecular genetic abnormalities and good prognosis, and surgery and / or radiotherapy are the mainly treatments. Secondary EMP, as the extramedullary invasive progression of multiple myeloma (MM), is often accompanied by high-risk cellular and molecular genetic abnormalities and poor prognosis, chemotherapy, immunotherapy and hematopoietic stem cell transplantation are the mainly treatment. This paper reviews the latest research progress of EMP in the pathogenesis, cytogenetics molecular genetics and treatment, so as to provide reference for clinical work.

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.

Clinical analysis of 10 cases with extramedullary plasmacytoma of the head and neck / 中华耳鼻咽喉头颈外科杂志

Objective: To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. Methods: A retrospective analysis was conducted on 10 cases with extramedullary plasmacytoma of the head and neck who were admitted to Henan Tumor Hospital from January 2005 to January 2020. Among the 10 patients, 6 were male and 4 were female. The average age at diagnosis was 56.3 years old (34-74 years old). Among them, 3 cases were located in the nasal cavity, 2 cases in the nasopharynx, 1 case in the sinuses, 2 cases in the larynx, 1 case in the oropharynx, and 1 case in the cervical lymph nodes. Treatments were administered according to tumor size and resection extent. Complete surgical excision (negative margins) was preferred, followed by adjuvant radiotherapy or radiotherapy alone. The clinical characteristics, diagnosis, treatment and prognosis of EMP were analyzed. Results: The patients' symptoms were not specific, frequently with local obstruction symptom and localized masses. All patients were confirmed pathologically as suffering from monoclonal plasmacytoma, with negative bone marrow biopsy and negative skeletal survey. Five patients received surgery, 3 received radiotherapy, and 2 received surgery with additional radiation. The follow-up time was 16-125 months, with a median of 92 months. Two patients developed into multiple myeloma. One patient who received radiotherapy after surgery relapsed after 7 years of follow-up and again received surgical treatment, with no evidence of second recurrence. The remaining patients had no recurrence or progression. Conclusion: Extramedullary plasmacytoma of the head and neck has a good prognosis. Surgical treatment can be considered for completely resectable lesions.

Huge Sellar Plasmacytoma as Differential Diagnosis of Invasive Pituitary Adenoma: A Case Report

Sellar plasmacytomas are rare tumors arising from plasma cells. They are often misdiagnosed as adenomas.We report the case of a 63-year-old woman with headache, cranial nerve III palsy and decreased visual acuity. Imaging revealed an extensive lesion centered on the clivus, extending to the cavernous sinus bilaterally and into the sphenoid sinus. The hormonal tests were compatible with panhypopituitarism and mild hyperprolactinemia. The first hypothesis was invasive pituitary adenoma. Partial resection was achieved, and the immunohistochemical evaluation was compatible with plasmacytoma. After a few weeks, she developed lumbar and hip pain, and the imaging confirming osteolytic lesions. The final diagnosis was multiple myeloma.

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)

Plasmocitoma solitario de costilla como presentación inicial de mieloma múltiple: reporte de un caso / Lone rib plasmacytoma as a presentation of initial multiple myeloma: a case report

Resumen Introducción: El 50% de los tumores de pared torácica son malignos, dentro de los que destaca el plasmocitoma de costilla. Objetivo: Presentar un caso clínico que debutó inicialmente como un plasmocitoma de costilla, y que terminó presentándose como mieloma múltiple. Materiales y Método: Registro clínico de un paciente sometido a resección de tumor de parrilla costal. Resultados: Paciente masculino de 58 años, con un año de dolor costal, asociado a aumento de volumen a nivel de la octava costilla derecha en línea media axilar, indurada. TC de tórax que demuestra imagen sugerente de plasmocitoma de 79 × 44 mm. Se realiza resección quirúrgica, con instalación de malla de prolene en el defecto. Biopsia diferida con compromiso neoplásico por lesión monoclonal de células plasmáticas. Se complementa estudio con biopsia de médula ósea confirmando mieloma múltiple. Se inicia tratamiento con quimioterapia adyuvante. Conclusiones: El plasmocitoma óseo solitario es una entidad de baja frecuencia, que se asocia a la presencia de mieloma múltiple. Es por esto que al momento de la sospecha se hace necesario descartar su presencia, con el fin de mejorar el pronóstico del paciente.

Introduction: Up to 50% of chest wall tumors are malignant; among which rib plasmocytoma stand out. Aim: Showcase a clinical case that debuted as a rib plasmacytoma, and that ended up presenting as Multiple Myeloma. Materials and Method: Records of a patient with resection of chest wall tumor. Results: Male patient of 58 years, with one year of costal pain, associated with an indurated increase in volume at the level of the eighth right rib in the mid-axillary line. Chest CT scan demonstrated a suggestive image of plasmacytoma of 79 × 44 mm. Surgical resection was performed, with prolene mesh installation in the defect. Biopsy showed neoplastic compromise due to monoclonal lesion of plasma cells. Study is complemented with bone marrow biopsy confirming multiple myeloma. The patient was treated with adjuvant chemotherapy. Conclusions: Solitary bone plasmacytoma is a low frequency entity, which is associated with the presence of multiple myeloma. At the moment of suspicion, it is necessary to rule out their presence, in order to improve the patient's prognosis.

Plasmocitoma extramedular, intracanal extradural, posterior a artrodesis cervical en un paciente con mieloma múltiple / Extramedullary, intracanal epidural plasmacytoma after cervical arthrodesis in a patient with multiple myeloma

El plasmocitoma extradural a nivel cervical es una de las patologías menos frecuentes dentro de los tumores extramedulares del mieloma múltiple; así mismo, es infrecuente que cause compresión medular o de las raíces nerviosas. Se presenta este caso por su gravedad y baja frecuencia en un paciente con diagnóstico de mieloma múltiple en tratamiento, que concurre con síntomas neurológicos (parestesia y paresia braquial bilateral) en la semana posterior a la cirugía de liberación meningorradicular y artrodesis cervical (C6-C7), en el mismo nivel de aparición de un plasmocitoma intracanal, extradural diagnosticado por resonancia magnética y confirmado con el resultado de la biopsia intraquirúrgica. Se ha publicado un caso similar al presentado, pero con la diferencia de que el paciente debutó con cuadriparesia. Se conoce también un caso de plasmocitoma originado en la médula espinal de la región torácica. En nuestro paciente, al igual que en un caso reportado con mieloma múltiple asintomático con manifestación primaria de plasmocitoma extramedular, la evolución de la enfermedad fue rápida, y el paciente falleció en menos de un año desde el diagnóstico. Nivel de Evidencia: IV.

Epidural plasmacytoma at the cervical spine is one of the less common extramedullary tumors in multiple myeloma. Likewise compression of the spinal cord or the nerve roots is uncommon. The following case is presented due to its rarity and severity. A patient diagnosed with and being treated for multiple myeloma presented with neurological symptoms (paresthesia and bilateral upper extremity weakness) a week after decompression and C6-C7 fusion, at the same level of an intracanal, epidural plasmacytoma detected by magnetic resonance and confirmed by intraoperative biopsy. A similar case was published, however it differs in that the patient presented with quadriplegia. There is also a reported case of plasmacytoma originating in the thoracic region of the spinal cord. In our patient, similar to the previously reported case of asymptomatic multiple myeloma with primary manifestation of extramedullary plasmacytoma, progression of the disease was rapid and the patient died less than one year from diagnosis. Level of Evidence: IV.

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.

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.

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.

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

Plasmocitoma dural solitario: reporte de un caso / Solitary dural plasmacytoma: a case report

Se presenta el caso de una paciente con una ocupante de espacio frontal izquierda simulando un meningioma de la convexidad, con diagnóstico histopatológico de plasmocitoma. Los plasmocitomas durales son extremadamente raros, siendo una forma inusual de mielomatosis con solo 15 casos publicados. En el presente trabajo se hace una revisión de la literatura y discusión de su diagnóstico terapéutico y pronóstico

Plasmocitoma intracraniano solitário / Solitary intracranial plasmacytoma

Os autores relatam caso de plasmocitoma solitário, sem manifestaçao sistêmica da doença, em paciente de 43 anos, com história de cefaléia parietal esquerda. Sao discutidos os aspectos tomográficos e a conduta, bem como a revisao de literatura, visto ser este o primeiro caso publicado na literatura nacional.

Plasmocitoma localizado de rinofaringe / Located plasmocytoma of the rhinopharynx

Apresentam-se um caso de plasmocitoma extramedular recidivado de rinofaringe. Após uma cirurgia anterior em outra Instituiçäo da qual ficou assintomático por 6 anos, compareceu ao nosso Serviço com extensa recidiva, sendo considerado entäo, passível de tratamento quimioterápico, de onde tomou destino ignorado. Discutem-se a raridade do mieloma solitário, para seu diagnóstico, os critérios de Bichel e Kirketerp, ao lado da extrema raridade de sua localizaçäo nasofaríngea