Successful Treatment of Refractory Orbital Plasmacytoma with Chimeric Antigen Receptor T Cell Therapy: A Case Report and Review of the Literature.

Orbital plasmacytoma is a rare plasma cell tumor that may arise as an aggressive form of extramedullary multiple myeloma. Treatment modalities include surgical excision, radiation, and chemotherapy. Chimeric antigen receptor T cell therapy is currently reserved for refractory disease. The authors present a case of a 69-year-old woman with an extensive orbital plasmacytoma refractory to multimodal therapy who was treated with idecabtagene vicleucel chimeric antigen receptor T cell therapy. Four days after infusion, the patient exhibited grade 1 cytokine release syndrome, which resolved with tocilizumab. The orbital plasmacytoma significantly decreased in size 1 month after treatment and demonstrated complete serological response and sustained tumor burden reduction at 10-month follow-up. This case highlights the efficacy of chimeric antigen receptor T cell therapy for refractory orbital plasmacytoma and calls attention to potential inflammatory toxicities.

Skeletal muscle extramedullary plasmacytoma transformed into plasmablastic plasmacytoma: a case report.

BACKGROUND: Extramedullary plasmacytoma (EMP) is a rare plasma cell malignancy, especially when the tumor originates in skeletal muscle. Plasmablastic plasmacytoma is an anaplastic round cell tumor with highly malignancy and poor prognosis. To date, there have been no reports on the transformation of skeletal muscle EMP into plasmablastic plasmacytoma. Therefore, the diagnosis, treatment, and prognosis of cases of this pathologic transformation are unclear. CASE PRESENTATION: This article reports a case of an elderly male patient who presented with a painless mass in the right calf and was diagnosed with EMP by puncture pathology. Complete remission was obtained after sequential chemoradiotherapy. 6 months later, another puncture was performed due to plasmablastic plasmacytoma multiple distant metastases, and the pathology showed that EMP was transformed to plasmablastic plasmacytoma. Despite aggressive antitumor therapy, the disease continued to deteriorate, and the patient ultimately died of respiratory failure. CONCLUSION: The transformation of EMP into plasmablastic plasmacytoma is very rare, and its diagnosis and treatment require the participation of both experienced pathologists and clinicians. We report this case in order to raise clinicians' awareness of the diagnosis and treatment of EMP and its transformation to plasmablastic plasmacytoma, and to avoid misdiagnosis and underdiagnosis.

[Consensus for the diagnosis and management of extramedullary plasmacytoma in China(2024)].

Extramedullary plasmacytoma (EMP) is a special type of malignant plasmacytosis, which is complex and heterogeneous. Most EMP patients have poor prognosis and lack a stratified prognostic system or ideal treatment strategy supported by evidence-based medical evidence, which cannot meet clinical needs. In order to improve the understanding of this disease entity, Plasma Cell Disease Group, Chinese Society of Hematology, Chinese Medical Association and Chinese Myeloma Committee-Chinese Hematology Association developed the "Chinese Expert Consensus on the diagnosis and treatment of extramedullary plasmacytoma", which aims to standardize the clinical diagnosis and treatment of EMP and ultimately improve the overall survival of patients with plasmacytoma.

Recurrence of solitary plasmacytoma in the liver 10 years after the onset of multiple bone lesions.

A 79-year-old man presented with a history of solitary plasmacytoma in the bone 10 years ago. Chemoradiotherapy was effective, and remission was maintained with intermittent treatment at relapse of the bone lesions. One year after the last treatment, a follow-up computed tomography (CT) scan revealed multiple liver masses, and a liver biopsy revealed plasmacytoma. There was no clonal plasma cell infiltration in the bone marrow, and the final diagnosis was solitary plasmacytomas of the liver. Although liver involvement is known in relapsed refractory multiple myeloma, solitary plasmacytoma in the relapsed stage confined to the liver is rare, and all previous reports have been from the initial presentation. To the best of our knowledge, this is the first recurrent case of solitary plasmacytoma of the liver.

Skull base plasmacytoma in young patients aged below 40 years: Radiological perspectives and clinical outcomes.

BACKGROUND: Plasmacytoma of the skull base is a rare manifestation of plasma cell neoplasm with only a few cases documented in literature involving young adults. Plasmacytoma can be an isolated solitary lesion or a secondary manifestation of multiple myeloma (MM). In this study, we report the clinical and radiological characteristics, management, and outcomes of patients under the age of 40 who presented with skull base plasmacytoma and associated neurological manifestations. Additionally, we share our experience in treating a rare case of skull base plasmacytoma diagnosed during pregnancy, in which the patient exhibited a favorable response to myeloma treatment initiated after delivery. CASE SERIES: Four patients were identified, comprising one pregnant female and three male patients, with a median age of 36 years (range 33-37 years). The main presenting symptoms were headache, dizziness, and cranial nerve palsy. All patients received underwent systemic myeloma therapy and radiotherapy with three patients also underwent autologous stem cell transplantation (ASCT). Notably, all patients achieved complete remission. CONCLUSION: Skull base plasmacytoma represents a rare manifestation of plasma cell neoplasms, underscoring the importance of considering it in the differential diagnosis of skull base lesions to ensure early intervention and avoid potential serious complications. Throughout our series, the cornerstone of therapy involved radiotherapy, systemic myeloma therapy, and ASCT, all of which elicited a favorable response in every case.

Solitary bone plasmacytoma: Long-term clinical outcomes in a single center.

BACKGROUND: A solitary plasmacytoma is classified into a solitary plasmacytoma of the bone (SBP) and a solitary extramedullary (soft tissue mass) plasmacytoma, based on the site of the lesion. Despite the high local control rate with radiotherapy, approximately half of patients' conditions progress to multiple myeloma (MM) within 3-5 years after diagnosis, with SBP having a worse prognosis. PATIENTS AND METHODS: We retrospectively assessed the treatment and outcomes of patients with SBP in a hospital in China from 2008 to 2021. Twenty-four patients treated over 13 years with SBP were enrolled in this retrospective study. RESULTS: The most common sites for SBP were the axial skeleton and femur. The M protein was detected in 11 patients (46 %), of which 8 (33 %) had light chains, 2 (8 %) had immunoglobulin G kappa and 1 (4 %) had immunoglobulin D kappa. Flow cytometry revealed that 5 patients (21 %) had minimal bone marrow involvement. The treatment included chemotherapy, surgery, and radiotherapy in 18 (75 %), 12 (50 %), and 9 (38 %) patients, respectively, of whom 13 (54 %) received combined treatment. Over a median follow-up period of 67.2 months, 9 patients (38 %) developed MM in a median time of 101.5 months. The 5- and 10-year progression-free survival rates were 67.3 % and 37.4 %, respectively. One patient died due to pneumonia without progression and the other died due to relapse. CONCLUSION: This study confirmed the high rate of progression of SBP to MM, indicating a need for adjunct chemotherapy for the management of SBP.

Plasmocitoma de mama: presentación de un caso clínico y revisión de la literatura / Breast plasmocitoma: case report and literature review

El plasmocitoma es una neoplasia de células plasmáticas infrecuente, cuyo escenario clínico se presenta como evidencia de diseminación del mieloma múltiple o como una tumoración solitaria extramedular, siendo el compromiso mamario extremadamente raro (63 casos reportados a partir de 1928). Presentamos el caso de una paciente mujer de 37 años con plasmocitoma en mama derecha multifocal, describiendo las características clínicas, imagenológicas, anatomopatológicas, así como el tratamiento y el seguimiento

Plasmacytoma is an uncommon plasma cell neoplasm, whose clinical behaviour is taken as evidence of disseminated multiple myeloma or an extramedullary solitary tumour. Breast involvement is extremely rare (63 cases reported since 1928). We present the case of a 37-year-old woman with a multifocal right breast plasmacytoma and describe the clinical, imaging and histologic findings, as well as treatment and follow-up

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 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 ovárico bilateral detectado por 18F-FDG PET/TC / Bilateral ovarian plasmacytoma detected on 18F-FDG PET/CT

No disponible

Plasmocitoma óseo solitario / Solitary bone plasmocytoma

El dolor lumbar crónico constituye un motivo frecuente de consulta y, aunque en la mayoría de las ocasiones presenta un carácter mecánico, en un 1-5 % demuestra un origen neoplásico .El plasmocitoma óseo solitario es un tumor de células plasmáticas poco habitual que asienta principalmente en la columna vertebral, provocando dolor sobre todo a nivel torácico y/o lumbar. Se presenta el caso de una mujer de 78 años con dolor lumbar crónico y síndrome de POEMS, en la que se halló una imagen ósea lítica en una radiografía lumbar, correspondiente a un plasmocitoma óseo solitario que se confirmó mediante biopsia (AU)

Chronic low back pain is a common reason for visiting the doctor and although in most cases it has a mechanical character, 1-5% are of neoplasic origin. Solitary bone plasmacytoma is a rare plasma cell tumor located mainly in the spine, causing pain especially in the thoracic and lumbar region. We report the case of a 78 year old woman with chronic back pain and POEMS syndrome, in which a lytic bone image was found in a lumbar radiography corresponding to a solitary bone plasmacytoma confirmed by biopsy (AU)

Plasmocitoma pulmonar primario: aportación de 3 casos / Three Cases of Primary Pulmonary Plasmacytoma

El plasmocitoma extramedular es un tumor de células plasmáticas que es más frecuente en el aparato respiratorio superior. La localización pulmonar es rara y puede presentarse como enfermedad limitada al pulmón y ganglios regionales, o bien como enfermedad diseminada. Cuando se localiza sólo en el aparato respiratorio inferior (plasmocitoma pulmonar primario), el diagnóstico es difícil y generalmente se realiza en la pieza quirúrgica. Aportamos 3 casos, con la peculiaridad en 2 de ellos de que el diagnóstico se confirmó mediante biopsia bronquial. Otros datos relevantes son la presencia de paraproteína en sangre y orina en 2 de los casos, y el seguimiento durante 10 y 15 años sin recidiva de la enfermedad(AU)

Extramedullary plasmacytoma is a plasma cell malignancy that most commonly occurs in the upper respiratory tract. Plasmocytoma located in the lung is an unusual finding, and in such cases the disease may be confined to the lung and regional lymph nodes or may be disseminated. When only located in the lower respiratory tract (primary pulmonary plasmacytoma), diagnosis is difficult and is usually based on the excised tissue. We present 3 cases, 2 of which were particularly unusual in that diagnosis was confirmed by bronchial biopsy. Other important findings included the detection of paraprotein in the blood and urine of 2 of the patients, and follow-ups over 10 and 15 years without recurrence of the disease in 2 of the cases(AU)

Plasmocitoma extramedular de nasofainge / Extramedullary plasmacytoma of the nasopharinx. Typical versus atypical presentation

Los plasmocitomas extramedulares son neoplasias de células plasmáticas poco frecuentes. El 80% de estos tumores se originan en el territorio ORL. Presentamos un caso típico de una mujer con un tumor único en nasofaringe. Actualmente está en remisión completa tras cirugía y radioterapia. También presentamos un caso muy atípico de una mujer con un tumor en nasofaringe asociado a una adenopatía metastásica cervical y un foco de plasmocitoma óseo. En este caso debió realizarse tratamiento sistémico con quimioterapia. Realizamos una actualización sobre el tema

Extramedullary plasmacytoma are rafe plasma cell neoplasias. Eighty percent of these tumours grow in the ENT region. We report a typical case of a woman with a single tumour in the nasopharynx. Actually she is in complete remission after surgical and radiotherapic treatment. We algo report an atypical case of a woman with a tumour in the nasopharynx associated with a cervical metastatic adenopathy and a plasmacytoma of bone. the treatment of this wide spread disease was systemic chemotherapy. An update on this subject is done

The POEMS syndrome

The POEMS syndrome is an unusual disorder manifesting primary as peripheral sensorimotor neuropathy in association with a monoclonal gammopathy. The importance of its recognition is that significant clinical improvement may result from localization and treatment of the underlying plasma cell tumour. AU)

The POEMS syndrome

The POEMS syndrome is an unusual disorder manifesting primary as peripheral sensorimotor neuropathy in association with a monoclonal gammopathy. The importance of its recognition is that significant clinical improvement may result from localization and treatment of the underlying plasma cell tumour.

Plasmocitoma cutáneo primario

Se presenta el caso de un hombre que consultó por una masa tumoral de un año de evolución, localizada en la mejilla derecha que resulto ser un plasmocitoma cutáneo primario. Se muestran los resultados de los examenes y se comentan las características de los palasmocitomas, haciendo enfasis en la rareza de esta lesión de la cual aparecen hasta ahora solamente once casos descritos en la literatura médica