POEMS Syndrome Diagnosis in a Patient with Mixed Polyneuropathy: Case Report.

POEMS syndrome is a rare condition of paraneoplasic origin characterized by the presence of a sensorimotor polyneuropathy associated with the presence of a proliferative disorder of plasmatic monoclonal cells and overproduction of vascular endothelial growth factor. The acronym "POEMS" represents multisystem findings including polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder and skin changes; nevertheless, clinical presentation is heterogeneous. We describe a clinical case, the diagnostic and therapeutic approach in a patient with sensorimotor polyneuropathy in whom POEMS syndrome was diagnosed; to understand this pathology, its clinical and paraclinical manifestations in order to make a diagnosis or to avoid a delayed one and to provide an adequate treatment.

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 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 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)

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)

Plasmocitoma solitario de la base craneal: Reporte de tres pacientes / Solitary cranial base plasmocytoma: Three cases report

Plasmocytomas constitute a group of malignant neoplasm arise from clonal plasmatic cells being solitary extramedular form infrequent. Here we report three patients with solitary anterior and middle cranial base plasmocytoma. The importance of these tumors in differential diagnoses of cranial base lesions and the role of endoscopic endonasal biopsy before deciding definitive clinical approach was emphasized.

Los plasmocitomas constituyen neoplasias malignas de las células plasmáticas clónales. La forma extramedular solitaria en la base craneal es infrecuente. Se reportan tres pacientes con plasmocitoma solitario de la base craneal anterior y media. Se enfatiza en la importancia de esta afección en el diagnóstico diferencial de las lesiones de base de cráneo así como la biopsia endonasal endoscópica antes de decidir el tratamiento definitivo.

Progresión de plasmocitoma óseo a mieloma múltiple y reporte de un caso / Progression of plasmacytoma of bone to multiple mieloma and a case report

El plasmocitoma óseo solitario y el mieloma múltiple son dos gammapatías monoclonales que pertenecen al mismo grupo de alteraciones neoplásicas de células plasmáticas. En ocasiones el plasmocitoma precede al mieloma. La localización esternal de un plasmocitoma es excepcional. Se reporta el caso de una persona de sexo masculino de 75 años, con dolor moderado en la región dorsal acompañado de disnea de medianos esfuerzos. La tomografía reveló lesiones osteolíticas localizadas en el tercio inferior del esternón, con características radiológicas de un plasmocitoma óseo solitario, además se observan lesiones en la 5ta. costilla izquierda y escápulas. La biopsia de médula ósea evidenció una plasmocitosis medular del 45 % y la inmunoelectroforesis, una concentración de 4210 mg/dl de Inmunoglobulina G. Se hace el diagnóstico de mieloma múltiple y se analizan las alteraciones que lo hicieron evolucionar hasta ese punto con el fin de tener presente la posibilidad de la evolución a mieloma múltiple en aquellos pacientes con plasmocitoma óseo. Se expone el siguiente caso con el objetivo de mejorar la calidad de vida de los pacientes a través del diagnóstico oportuno del plasmocitoma óseo, logrando así evitar la evolución a mieloma múltiple, y resultar en una mayor supervivencia.

Solitary plasmacytoma of bone and multiple myeloma are two monoclonal gammapathies that belong to the same group of neoplastic alterations of plasma cells. The plasmacytoma occasionally precedes myeloma. The sternal location of plasmocytoma is rare. This is the case of a man aged 75 years, with moderate pain in the dorsal region and short of breath when making medium efforts. The axial tomography showed osteolytic lesions in the lower third of sternum with radiological characteristics of a solitary plasmocytoma of bone in addition to lesions in the 5th left rib and scapulas. The bone marrow biopsy revealed 45% medullary plasmocytosis whereas immunoelectrophoresis showed Ig G concentration of 4210 mg/dl. Multiple myeloma was diagnosed and an analysis was made on the alterations leading to this result, with the purpose of bearing in mind the possibility of progression to a multiple myeloma in those patients with plasmocytoma of bone. The following case was presented in order to improve the quality of life of patients through timely diagnosis of plasmocytoma of bone and to avoid progression to multiple myeloma, thus achieving higher survival rates.

Cutaneous Manifestations in POEMS Syndrome: Case Report and Review.

The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24-44% of patients.

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.

Primary testicular plasmocytoma: A five year follow-up.

The testicular plasmocytoma represents only 5% of the non-germinative cell testicular tumors, and accounts for only 2% of all plasma cell neoplasms. Approximately, 50 cases of testicular plasmocytoma have been reported in medical literature; however, only 9 of these are isolated tumors without previous history or progression to multiple myeloma. A 47-year-old patient, presenting progressive and painless growth of the right testicle in the last four years, underwent surgical treatment in another hospital two years ago, to correct a hydrocele in the same testicle with no improvement at all. Sonography showed a tumor with the following measurements for the right testicle: 84 × 59 × 80 mm. The tumor marker values were all normal. An abdominal computed tomography found no evidence of retroperitoneal lymph nodes invasion. The patient underwent a right radical orchiectomy. Pathologic analysis revealed a malignant neoplasia described as a plasmocytoma (solitary myeloma) that produces immunoglobulin's kappa light chain. After five years of follow-up, there were no signs of metastasis or local recurrence in the exams. Case report and review of literature have been presented here.

Extramedullary plasmacytoma of the larynx

Introduction: The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx. Objective: To describe a case of extramedullary plasmocytoma of the larynx, with literature review. Case Report: Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma. Conclusion: Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate...

Extramedullary plasmacytoma of the larynx.

INTRODUCTION: The extramedullary plasmocytoma is one of the localized forms of malignancy of the plasma cells, which has multiple myeloma main diagnosis. Its main site to the head and neck, but with a rare presentation in the larynx. OBJECTIVE: To describe a case of extramedullary plasmocytoma of the larynx, with literature review. CASE REPORT: Patient female, 49, referring to intermittent dysphonia for 01 years with progressive worsening associated with vocal fatigue and vocal effort, with reddish lesion, smooth edges fold left ventricular endoscopy. Being subjected to excisional biopsy diagnosed with extramedullary histopathological plasmocytoma. CONCLUSION: Extramedullary Plasmocytoma must be considered in the differential diagnosis of rare tumors of the larynx. It is essential after the diagnosis of multiple myeloma research and a "follow up" appropriate.

Plasmocitoma solitario de cabeza: presentación de dos casos

Se trata de un tumor de baja frecuencia constituido de células plasmáticas que pueden aparecer a nivel de cabeza y cuello. Estos constituyen menos del 1 por ciento de las neoplasias malignas de esta región. Deben ser diferenciados del mieloma múltiple, lo que puede ser dificultoso porque un variable porcentaje va asociado a un desarrollo más tardío de esta patología. Describimos dos casos de plasmocitoma solitario parietal. La clínica, diagnóstico y los problemas terapéuticos(radioterapia, cirugía o combinación de ambos) son discutidos y revisados en la literatura

Solitary plasmocytoma is a rare plasmactic cell tumor occurring in the head and neck. These constitute, less than 1% of all head and neck malignancies. They must be differentiated from multiple myeloma because a varying percentage may be associated at a later date with the development of multiple myeloma. We describe two cases of plasmocytomas, both in parietal bone. The clinical, diagnosis and therapeutic problems (radiation, surgery or a combination of both) are discussed and reviewed in the literature