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.

Prognostic Value of R-ISS Staging Combined with "Multiple-Hits" in Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To analyze the prognostic value of R-ISS staging combined with "Multiple-Hits" in patients with multiple myeloma (MM), and to detected the effect of different "Multiple- Hits" combinations to the prognosis of the patients.@*METHODS@#The 220 MM patients treated in the hematology department of People's Hospital of Xinjiang Uygur Autonomous Region from April 2013 to October 2019 were enrolled and retrospective analyzed. All the patients were detected by FISH. The effects of R-ISS staging combined with "Multiple-Hits" and different "Multiple-Hits" combinations to the prognosis of the patients were compared.@*RESULTS@#For the patients at R-ISS stage II and III, the median progression-free survival (PFS) time, overall survival (OS) time and duration of response (DOR) time in "Multiple-Hits" patients were all shorter than those without high-risk cytogenetic abnormality (HRCA) and those with only one type of HRCA (P<0.05), while the TTR (time to response) was significantly prolonged (P<0.05). For the prognosis of the patients among the three different "Multiple-Hits" combinations(1q21+ combined with del(17p), 1q21+ combined with t(14;16) and combined 1q21+ combined with t(4;14)), 1q21+ combined with del(17p) showed the worst prognosis.@*CONCLUSION@#The patients with Different "Multiple-Hits" combinations shows different prognosis. The R-ISS staging combination with "Multiple-Hits" is more conducive to accurately judging the prognosis of MM patients.

Mieloma múltiple: enfermedad ósea extensa en una paciente joven / Myeloma multiple: extensive bone disease in a young patient

El mieloma múltiple (MM) es un tumor de proliferación clonal de plasmocitos en la médula ósea (MO). Hasta ahora no es curable1,2. Puede presentarse como una enfermedad indolente o con manifestaciones clínicas como insuficiencia renal, anemia y lesiones osteolíticas1. Se presenta el caso de una paciente femenina de 46 años, quien padecía dolor en la región del brazo izquierdo, acompañado por dolores óseos generalizados. Al examen físico se observó en el tercio proximal de la región humeral izquierda y hombro ipsilateral, gran tumoración que deformaba la anatomía local, indurada, inmóvil y dolorosa. Presentaba anemia severa (Hb. 6 g/dL), cuantificación de ß2 Microglobulina 4,23 mg/L (VR 0,80 ­ 3,0 mg/L) y rastreo óseo radiológico con múltiples lesiones líticas. En la muestra de médula ósea se encontró infiltración de 80 % de células plasmáticas mono- clonales kappa. Se le diagnosticó discrasia de células plasmáticas tipo MM monoclonal kappa sintomático, estadio II (ISS), con enfermedad ósea extensa y un gran plasmocitoma humeral izquier- do. Se indicó tratamiento de inducción de la remisión con el esquema VCD (bortezomib, ciclofosfamida y dexametasona). Adicionalmente ácido zoledrónico. Posteriormente se modificó a bortezomib, talidomida y prednisona. Luego del tratamiento antineoplásico, refirió acalmia completa del dolor con mejoría de la movilidad. Este caso clínico se trata de una presentación inusual de MM debido a la edad de la paciente y a la extensa enfermedad ósea. Llamó la atención la ausencia de niveles elevados de la cadena liviana kappa de las inmunoglobulinas libres en suero. Por la edad de la paciente y la ausencia de co-morbilidades significativas, es candidata para trasplante de células progenitoras hematopoyéticas (TCPH)(AU)

Multiple myeloma (MM) is a tumor of clonal proliferation of plasma cells in the bone marrow (BM). Until now it is not curable1,2. It can present as without symptoms or with clinical manifestations such as renal failure, anemia and osteolytic lesions1. We describe the case of a 46-year-old female patient, who complained of pain in her left arm, and, also, by generalized bone pain. On physical examination a large tumor was present in the proximal third of the left humeral region and ipsilateral shoulder, it was hard, painful and immo- bile. She had severe anemia (Hb 6 g / dL), quantification of ß2 Microglobulin 4.23 mg / L (VR 0.80 - 3.0 mg /L) and the radiological bone survey showed multiple lytic lesions. In the bone marrow sample, an infiltration of 80 % kappa monoclonal plasma cells was found. Her diagnosis was MM-type plasma cell dyscrasia, symptomatic kappa, stage II (ISS), with extensive bone disease and a large left humeral plasmacytoma. Remission induction therapy was indicated with the VCD scheme (bortezomib, cyclophosphamide and dexa- methasone). Additionally zoledronic acid was administered. Subsequently, it was modified to bortezomib, thalidomide and prednisone. After antineoplastic treatment, she referred pain relief with improvement of mobility. This clinical case is an unusual presentation of MM due to the age of the patient and extensive bone disease. The absence of high levels of the kappa light chain of free immunoglobulins in serum attracted attention. Due to the age of the patient and the absence of significant comorbidities, she is a candidate for trans- plantation of hematopoietic stem cells(AU)

Hipertensión pulmonar como presentación en mieloma múltiple / Pulmonary hypertension as presentation in multiple myeloma

El mieloma múltiple es una enfermedad oncohematológica, que representa el 15% de las enfermedades hematológicas malignas. La edad media de aparición es entre los 65-70 años, siendo muy poco frecuente en pacientes jóvenes; 2% son menores de 40 años. Presentamos el caso de una mujer de 36 años con antecedente de tabaquismo de 20 paquetes año. Consultó por disnea asociada a signos de insuficiencia cardíaca derecha, anemia, proteinuria, elevación de reactantes de fase aguda y patrón sugestivo de restricción moderadamente grave en la espirometría y caída de la capacidad de difusión de monóxido de carbono (DLco). El ecocardiograma doppler evidenció dilatación de cavidades derechas y signos de hipertensión pulmonar que se confirmó con cateterismo cardiaco derecho. En busca de la etiología se arribó al diagnóstico de mieloma múltiple.

Multiple myeloma is a hematologic disease, which accounts for 15% of hematologic malignancies. The average age of onset is between 65-70 years and is very rare in young patients, as 2% are under 40 years old. We present a case of 36-year-old women with history of 20 pack years (p/y) smoking, who complaints of dyspnea associated with signs of right cardiac overload, anemia, proteinuria, elevated acute phase reactants and spirometry pattern suggestive of moderately-severe restriction and severe drop in diffusing capacity for carbon monoxide (DLCO). Echocardiogram evidence dilated right heart cavities and signs of pulmonary hypertension which is confirmed by right heart catheterization. In search of the etiology we arrive to the diagnosis of multiple myeloma.

Generalized erythematous and scaly plaques and papules: a rare case of Rosai-Dorfman disease accompanied by multiple myeloma

Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.

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.

Análisis costo-efectividad de lenalidomida en combinación con dexametasona frente a bortezomib combinado con dexametasona en el tratamiento en segunda línea del mieloma múltiple en Chile / Cost-effectiveness of lenalidomide in combination with dexamethasone compared to bortezomib in combination with dexamethasone for the second-line treatment of multiple myeloma in Chile

CONTEXTO: El mieloma múltiple es una neoplasia de las células plasmáticas de la medula ósea. Las terapias disponibles no son curativas y la mayoría de los pacientes se vuelve refractario al tratamiento. Agentes como lenalidomida y bortezomib han demostrado su eficacia en el tratamien-to en segunda línea de estos pacientes. OBJETIVO: Evaluar el costo-efectividad de la combinación lenalidomida/dexametasona frente a bortezomib/dexametasona en pacientes con mieloma múltiple, no candidatos a trasplante, previamente tratados con bortezomib, desde la perspectiva del sistema nacional de salud chileno. METODOLOGÍA: Se empleó un modelo de Markov que simula la evolución de una cohorte de pacientes a través de cuatro estados de salud (preprogresión en tratamiento, preprogresión sin tratamiento, progresión o muerte) en un horizonte temporal de 25 años. Los datos de eficacia, uso de recursos y frecuencia de efectos adversos fueron extraídos de los ensayos sobre mieloma múltiple MM-009 y MM-010 y de un estudio retrospectivo de retratamiento con bortezomib. Todos los parámetros fueron validados por expertos. Se aplicó una tasa de descuento en costos y beneficios de 3%. La robustez de los resultados fue evaluada mediante un análisis de sensibilidad univariante y probabilístico. RESULTADOS: El tratamiento con lenalidomida/dexametasona proporciona 1,41 años de vida y 0,83 años de vida ajustados por calidad incrementales respecto a bortezomib/dexametasona, con un costo incremental de 11 864 597,86 pesos chilenos (19 589,86 dólares). La ratio de cos-to-efectividad y costo-utilidad incremental se cifró en 8 410 266,92 pesos chilenos (13 886,35 dólares) por año de vida ganado y 14 271 896,16 pesos chilenos (23 564,59 dólares) por año de vida ajustado por calidad respectivamente. CONCLUSIÓN: La lenalidomida/dexametasona representa una alternativa potencialmente costo-efectiva, desde la perspectiva del sistema nacional de salud chileno, para el tratamiento en segunda línea de pacientes con mieloma múltiple no candidatos a trasplante.

BACKGROUND: Multiple myeloma is a hematologic malignancy affecting bone marrow derived plasma cells. Current therapies are not able to eradicate the disease and most patients become refractory to the treatment. Lenalidomide and bortezomib have proved effective in the second-line treatment of these patients. OBJECTIVE: To evaluate the cost-effectiveness of lenalidomide in combination with dexamethasone compared to bortezomib in combination with dexamethasone in patients with multiple myeloma previously treated with bortezomib, from the perspective of the Chilean National Health Service. METHODOLOGY: A four-state Markov model (preprogression on treatment; preprogression off treatment, progression and death) was used to simulate the evolution of a cohort of multiple myeloma patients over a 25-year time horizon. Efficacy data, resource use and frequency of adverse events were extracted from MM009/010 studies and a retrospective analysis of retreatment with bortezomib. All inputs were validated by experts. A 3% annual discount rate was used for costs and health outcomes. The robustness of the results was evaluated through univariate and probabilistic sensitivity analyses. RESULTS: Lenalidomide in combination with dexamethasone treatment provided 1.41 incremental life years and 0.83 incremental quality-adjusted life years in comparison with bortezomib in combination with dexamethasone, with an incremental cost of 11 864 597.86 CLP (19 589.86 US$). The incremental cost-effectiveness and cost-utility ratio were estimated at 8 410 266.92 CLP (13 886,35 US$) / incremental life year and 14 271 896.16 CLP (23 564,59 US$)/incremental quality-adjusted life years, respectively. CONCLUSIONS: Lenalidomide in combination with dexamethasone represents a potentially cost-effective alternative for the second-line treatment of patients with multiple myeloma who are not eligible for transplantation, from the perspective of the Chilean National Health Service.

TRIM56 Suppresses Multiple Myeloma Progression by Activating TLR3/TRIF Signaling

PURPOSE: Tripartite-motif-containing protein 56 (TRIM56) has been found to exhibit a broad antiviral activity, depending upon E3 ligase activity. Here, we attempted to evaluate the function of TRIM56 in multiple myeloma (MM) and its underlying molecular basis. MATERIALS AND METHODS: TRIM56 expression at the mRNA and protein level was measured by qRT PCR and western blot analysis. 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) and flow cytometry analysis was performed to investigate the effect of TRIM56 on MM cell proliferation and apoptosis. The concentrations of interferon (IFN)-β, interleukin (IL)-6, and tumor necrosis factor-α in MM cell culture supernatants were detected with respective commercial ELISA kits. Western blot was employed to determine the effect of TRIM56 on toll-like receptor 3 (TLR3)/toll-IL-1 receptor (TIR) domain-containing adaptor inducing IFN-β (TRIF) signaling pathway. RESULTS: TRIM56 expression was prominently decreased in MM cells. Poly (dA:dT)-induced TRIM56 overexpression in U266 cells suppressed proliferation, induced apoptosis, and enhanced inflammatory cytokine production, while TRIM56 knockdown improved growth, diminished apoptosis, and inhibited inflammatory cytokine secretion in RPMI8226 cells. Moreover, TRIM56 knockdown blocked TLR3 signaling pathway. Furthermore, poly (I:C), a TLR3 agonist, markedly abolished TRIM56 depletion-induced increase of proliferation, decrease of apoptosis, and reduction of inflammatory factor in MM cells. CONCLUSION: TRIM56 may act as a tumor suppressor in MM through activation of TLR3/TRIF signaling pathway, contributing to a better understanding of the molecular mechanism of TRIM56 involvement in MM pathogenesis and providing a promising therapy strategy for patients with MM.

Cartografía de heterotopías psicoactivas: una mirada a los discursos médicos, jurídicos y sociales sobre los usos de drogas / Cartography of psychoactive heterotopias: a look at the medical, legal and social discourses regarding drug use

Este artículo traza un mapa del control social de las drogas a partir de las políticas del espacio, de acuerdo al concepto foucaultiano de heterotopía. En primer lugar, se describe una breve genealogía de los usos de sustancias psicotrópicas en los diversos tiempos y culturas hasta la llegada del paradigma prohibicionista, atendiendo al modo en que el poder ha señalado, separado y encerrado determinados rituales y usos del placer en emplazamientos físicos y simbólicos. Este itinerario se centra en el contexto español para establecer un diálogo entre las distintas políticas del espacio que se han sucedido y superpuesto en la construcción y gestión de un problema que deviene objeto de la mirada, la mecánica y los discursos médicos, jurídicos y sociales. Así, se analizan las intersecciones de los emplazamientos liminares de consumo con el paradigma de la reducción de daños, así como las estrategias terapéuticas con prescripción farmacológica, desde los programas de metadona hasta los más recientes de heroína.

This article traces a map of the social control of drugs through the politics of space, according to the Foucaultian concept of "heterotopia." Firstly, a brief genealogy of the use of psychotropic substances in different times and cultures is described, up to the introduction of the prohibitionist paradigm. Attention is paid to the way in which power has marked, separated and enclosed certain rituals and uses of pleasure in physical and symbolic sites. The itinerary is focused on the Spanish context to establish a dialogue between the various policies of space that have come into being and have overlapped in the construction and management of a problem which has been rendered an object to the gazes, mechanics and discourses of the medical, legal, and social fields. In this way, the intersections between the liminal spaces of drug use and the harm reduction paradigm are analyzed, including therapeutic strategies with prescribed drugs, from methadone programs to the new heroin programs.

Survival analysis in patients with metastatic spinal disease: the influence of surgery, histology, clinical and neurologic status / Análise de sobrevida em pacientes com doença metastática espinhal: influência da cirurgia, histologia, status clínico e neurológico

Spine is the most common site for skeletal metastasis in patients with malignancy. Vertebral involvement quantification, neurological status, general health status and primary tumor histology are factors to set surgical planning and therapeutic targets. We evaluated the impact of general clinical and neurological status, histologic type and surgery in survival. Method : The study sample consisted of consecutive patients admitted from July 2010 to January 2013 for treatment. Results : Sixty eight patients were evaluated. 23 were female and 45 were male. Main primary neoplasic sites were: breast, prostate, lung/pleura and linfoproliferative. Thirty three out of 68 received surgical treatment, 2 received percutaneous biopsy and 33 had nonsurgical treatment. Survival : Log Rank curves revealed no statistical significant difference according to histological type, surgical approach and Frankel Score. Karnofsky Score was statistically different. Conclusion : Histological type and clinical status were statistically associated with life expectancy in vertebral metastatic disease. .

A coluna vertebral é o sítio mais comum de metastases ósseas. A quantificação do acometimento vertebral, o status neurológico, status clínico e histologia do tumor primário são fatores importantes para planejamento cirúrgico e metas terapêuticas. Nós avaliamos o impacto do status clinico geral e neurológico, tipo histológico e cirurgia na sobrevida de pacientes com metástases espinhais. Método : A amostra consistiu de pacientes consecutivamente admitidos de Julho de 2010 a Janeiro de 2013. Resultados : Sessenta e oito pacientes foram avaliados. 23 eram mulheres e 45 eram homens. Os principais sítios primários foram mama, próstata, pulmão e linfoproliferativos. Trinta e três realizaram tratamento cirúrgico, 2 realizaram biópsia percutânea e 33 tiveram tratamento conservador e radioterapia. Conclusão As curvas Log Rank não revelaram significância quanto à cirurgia e escore de Frankel, mas revelaram associação com Karnofsky e tipo histológico. .

Are the binary typology models of alcoholism valid in polydrug abusers ?

Objective: To evaluate the dichotomy of type I/II and type A/B alcoholism typologies in opiate-dependent patients with a comorbid alcohol dependence problem (ODP-AP). Methods: The validity assessment process comprised the information regarding the history of alcohol use (internal validity), cognitive-behavioral variables regarding substance use (external validity), and indicators of treatment during 6-month follow-up (predictive validity). Results: ODP-AP subjects classified as type II/B presented an early and much more severe drinking problem and a worse clinical prognosis when considering opiate treatment variables as compared with ODP-AP subjects defined as type I/A. Furthermore, type II/B patients endorse more general positive beliefs and expectancies related to the effect of alcohol and tend to drink heavily across several intra- and interpersonal situations as compared with type I/A patients. Conclusions: These findings confirm two different forms of alcohol dependence, recognized as a low-severity/vulnerability subgroup and a high-severity/vulnerability subgroup, in an opiate-dependent population with a lifetime diagnosis of alcohol dependence. .

Quantitative ultrasound at the hand phalanges in patients with bisphosphonate-related osteonecrosis of the jaws

Patients with bisphosphonate-related osteonecrosis of the jaws (BRONJ) who received intravenous or oral bisphosphonates (BP) were selected for determination of their bone microarchitecture as a risk predictor of BRONJ development. The diagnosis of BRONJ was made based on clinical and radiographic findings. The control group consisted of healthy patients. All patients underwent quantitative and qualitative ultrasound measurements of bone at the hand phalanges carried out using the DBM Sonic BP. Ultrasound bone profile index (UBPI), amplitude-dependent speed of sound (AD-SoS), bone biophysics profile (BBP), and bone transmission time (BTT) were measured. The BRONJ group consisted of 17 patients (62 ± 4.24; range: 45-82); 10 (58.8%) were male and seven (41.1%) were female, of whom 11 (64.7%) suffered from multiple myeloma, three (17.6%) from osteoporosis, one (5.8%) from prostate cancer, one (5.8%) from kidney cancer, and one (5.8%) from leukemia. Fourteen (82.3%) of them received intravenous BP whereas three (17.6%) received oral BP. Nine (9/17; 52.9%) patients developed bone exposure: two in the maxilla and seven in the mandible. Regarding quantitative parameters, Ad-SoS was low in the BRONJ group, but not significant. The UBPI score was significantly reduced in BRONJ patients with exposed bone when compared to controls (0.47 ± 0.12 vs. 0.70 ± 0.15; p = 0.004). The present study demonstrated that quantitative ultrasound was able to show bone microarchitecture alterations in BRONJ patients, and suggests that these analyses may be an important tool for early detection of bone degeneration associated with BRONJ.

Bone Cement Augmentation Procedures for Spinal Pathologic Fractures by Multiple Myeloma

Efficacy and safety of bone cement augmentations for spinal pathologic fractures related to multiple myeloma, and usefulness of radionuclide studies for surgical decision were retrospectively evaluated. Forty eight vertebrae from 27 patients for bone cement augmentation procedures and 48 vertebrae from 29 patients for conservative treatment were enrolled. Clinical results using visual analogue scale (VAS) and Oswestry disability index (ODI), and radiologic results were assessed. For clinical decisions on treatment of spinal pathologic fracture, bone scan or single photon emission computed tomography was done for 20 patients who underwent surgery. Mean follow-up was 16.8 months. In terms of clinical results, immediate pain relief was superior in the operated group to that in the conservative group. ODI, maintenance of vertebral height and local kyphotic angle at the last follow-up were superior in the operated group in comparison to the conservative group. At one year follow-up, cumulative survival rate were 77.4% and 74.7% in the operated and conservative groups, respectively (log rank test> 0.05). Leakage of bone cement was noted at 10 treated vertebrae. Bone cement augmentations presented short-term pain relief for spinal pathologic fractures by myeloma with relative safety in highly selected patients, and radionuclide imaging studies were useful for the surgical decision on these procedures.

Lesiones cutáneas en mieloma múltiple: Descripción de un caso y revisión de la literatura / Cutaneous involvement in multiple myeloma: Report of one case

Cutaneous involvement in multiple myeloma (MM) is extremely rare. It may correspond to plasmocytomas, secondary amyloidosis, paraneoplastic syndromes or drug reactions. We report a 75-year-old female with skin lesions, corresponding to extramedullary plasmacytomas (EPs), as a manifestation of MM progression. Most EPs appear in the upper airway and gastrointestinal tract, but have also been described in the genitourinary tract, lung, kidney, thyroid and in 2% of cases, in the skin. Cutaneous lesions are generally associated with terminal stages of the disease and a poor prognosis.

Case for diagnosis

Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.

Mieloma múltiple: la radiografía panorámica como un primer hallazgo de diagnóstico / Multiple myeloma: ray wide as a first discovery of diagnosis

Mieloma múltiple (MM), también conocido como Mieloma de células plasmáticas, es una proliferación tumoral de plasmocitos que infiltran la médula hematopoyética, de causa desconocida. Recursos de imagen son ampliamente utilizados en clínica estomatológica y los profesionales de esta área puede ser el primero en ser solicitado, por lo tanto, es importante la detección de la afectación que puede tener carácter sistémico en la preservación de la salud del paciente. Bajo estas condiciones, la literatura tiene pruebas de la ocurrencia del mieloma múltiple. En este reporte, una paciente mujer, de raza negra, de 53 años de edad fue remitida al Departamento de Estomatología por un odontólogo, por la presentación de las lesiones radiolúcidas en la mandíbula. Los hallazgos radiográficos panorámicos incluyen múltiples lesiones mal definidas radiolúcidas y la gammagrafía ósea mostró múltiples áreas de captación del radiotrazador en la mandíbula, la pelvis, la columna vertebral, el cráneo, la clavícula y las costillas. La paciente fue remitida a un comité de tumores multidisciplinar, y ha tolerado el tratamiento y su seguimiento desde el año 2009. Debido al comportamiento de esta enfermedad, el cuidado clínico y la evaluación radiológica deben llevarse a cabo, proporcionando información precisa y guías de tratamiento de los pacientes.

Multiple myeloma (MM), also called plasma cell myeloma, is a monoclonal neoplasic proliferation of plasma cells of bone marrow derivation with an unknown cause. Imaging resources are widely used in clinical stomatology and the professionals of this area may be the first to be requested, thus, it is important the detection of involvement that may have systemic character in preserving the patient's health. Under these conditions, the literature has proof the occurrence of the multiple myeloma. In this report the 53-year-old melanoderm woman was referred to the Stomatology Department by a dentist on presentation of radiolucent lesions in the mandible. At the panoramic radiographic findings included multiple ill-defined radiolucent lesions and bone scintigraphy showed multiple areas of radiotracer uptake in the jaw, pelvis, spine, skull, clavicle, and ribs. The patient was referred to a multidisciplinary tumor board, and she has been tolerating her treatment well and following up since 2009. Due to the behavior of this disease, careful clinical and radiological evaluation must be carried out, which provides accurate information and guides treatment of patients.

Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma / Amiloidose sistemica primaria, cutis laxa adquirida e mucinose cutanea em paciente com mieloma multiplo

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Mulher de 57 anos, com equimose periorbitária, frouxidão cutânea nas dobras, polineuropatia e síndrome do túnel do carpo bilateral.O exame histopatológico da lesão axilar revelou fragmentação de fibras elásticas, porém a coloração de von Kossa foi negativa;o diagnóstico foi de cútis laxa. Amiloidose sistêmica primária foi confirmada pela presença de material amilóide no exame histopatológico da pálpebra, além de púrpura, polineuropatia, síndrome do túnel do carpo bilateral e gamopatia monoclonal. Foi diagnosticada como portadora de mieloma múltiplo por apresentar 40% de plasmócitos na medula óssea, componente M urinário e anemia. A paciente evoluiu com bolhas de conteúdo citrino, cujo exame histopatológico mostrou mucinose. Os diagnósticos finais foram: amiloidose sistêmica primária, cútis laxa adquirida e mucinose, todos vinculados ao mieloma múltiplo.