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.

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

Las neoplasias malignas de la cavidad oral en gran medida (90%) consisten en carcinoma de células escamosas que surgen de la mucosa de revestimiento. El 10% restantes de neoplasias malignas orales de un grupo heterogéneo de tumores de diferente etiología. Presentamos dos casos de patología oncohematológica: Mieloma Múltiple (AU)

Malignant neoplasms of the oral cavity largely (90%) consist of squamous cell carcinoma arising from the lining mucosa. e remaining 10% of oral malignancies from a heterogeneous group of tumors of different etiology. We present two cases of oncohematological pathology: Multiple Myeloma (AU)

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.

Solitary plasmacytoma of mandible: an unusual bilateral presentation

Plasmacytoma is a neoplastic proliferation of monoclonal plasma cells, which can present clinically as solitary bone neoplasm, extramedullary plasmacytoma, and multiple myeloma. The biological behavior of these tumors is variable from periods of clinical latency to rapid growth and progression from localized forms to more disseminated multiple myeloma. We present the case of solitary plasmacytoma of the mandible with rare bilateral involvement in a 65-year-old female patient. This paper highlights the importance of understanding the maxillofacial manifestations of the disease by the dentist for early diagnosis and thus better prognosis.

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.

Extramedullary Manifestation of Multiple Myeloma in the Oral Cavity.

BACKGROUND: Extramedullary plasmacytomas occurs in about 20% of multiple myeloma (MM) recurrences. Extramedullary disease seems to respond poorly to thalidomide and has adverse prognostic implication. When disease recurs in the oral cavity with soft tissue infiltration, some authors defend upfront surgical excision prior to radiotherapy with the aim of achieving better local control. We describe herein such an atypical case of recurrence from MM, with complete local response after 2 cycles of chemotherapy. Unfortunately, disease progressed later on, and the patient died after 9 months post-recurrence. This emphasizes the prognostic impact of extramedullary disease manifestation in MM.

Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos / Myasthenia gravis and its association with lymphoproliferative disorders: a case series

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

Canine Cutaneous Plasmacytosis: 21 Cases (2005-2015).

BACKGROUND: Cutaneous plasmacytosis (CP) is a syndrome of multiple cutaneous plasma cell tumors, in the absence of multiple myeloma. Although rare in both humans and dogs, treatment recommendations are usually extrapolated from multiple myeloma protocols. To date, no case series of CP have been described in the veterinary literature. HYPOTHESIS/OBJECTIVES: To describe clinical presentation, determine treatment response rates and duration, and report overall survival of dogs with CP. ANIMALS: Twenty-one client-owned dogs with CP. METHODS: Medical records of 21 dogs with CP were reviewed. Diagnosis was based on histopathologic evaluation of at least 1 representative cutaneous or subcutaneous lesion in dogs with ≥3 lesions. Dogs with suspicion of multiple myeloma were excluded. RESULTS: The most commonly affected breeds were the golden (5/21) and Labrador retriever (3/21). Fourteen of 21 dogs had >10 lesions, with some having >100. Lesions commonly were described as round, raised, pink-to-red, and variably alopecic or ulcerated. The most commonly used drug protocol was combined melphalan and prednisone, with an overall response rate (ORR) of 73.7% (14/19 dogs). Single-agent lomustine was associated with a similar ORR of 71.4% (5/7 dogs). For all treatments combined, the median progression-free interval after the first treatment was 153 days. The median survival time from the first treatment was 542 days. CONCLUSIONS AND CLINICAL IMPORTANCE: Alkylating agents were effective in inducing remission of CP; corticosteroids, melphalan, and lomustine were the most commonly used drugs. Survival times were similar to those reported in dogs with multiple myeloma treated with alkylating agents.

[Myasthenia gravis and its association with lymphoproliferative disorders: a case series]. / Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos.

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

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.

Extramedullary plasmacytoma in a captive collared peccary (Pecari tajacu) / Plasmocitoma extramedular em cateto criado em cativeiro (Pecari tajacu)

An extramedullary plasmacytoma case in a captive collared peccary (Pecari tajacu) is reported. The animal, a female aging three years old, had a medical history of diffusely distributed skin and mucocutaneous junction lesions, associated with swollen lymph nodes. Clinical examination and complementary exams (complete blood count, biochemical analysis, skin scraping to search mites and fungal culture) were performed. Thirty days after examination, the animal died. At necropsy, multiple consistent nodules, aseptic pustules and swollen lymph nodes were found. On histological exams of the skin and oral mucosa, we observed a large number of round cells forming masses organized in nests, sheets, and cords of cells in a well-vascularized fibrovascular tissue. Neoplastic plasma cells infiltrated between the fibers and the lamina propria of smooth muscle. Spaces among the cell masses were filled with some eosinophil and fluid. Most of the cells were well differentiated, presenting a perinuclear clear zone. In some points, the cells were pleomorphic. The plasma cells presented eccentric, basophilic and spherical nuclei, showing a dense to organized chromatin with distinct nucleoli. Binucleate cells were observed, but multinucleated giant cells were rare. Oral mucosa and lymph nodes tested by immunohistochemical analyses were positive for Mb-1, with a multifocal distribution. In regard to Bcl-2, the neoplastic cells were intermittent weakly positive. So, an extramedullary plasmacytoma was diagnosed in the collared peccary considering the location, the histopathological and immunohistochemical findings.(AU)

Um caso de plasmocitoma extramedular em cateto criado em cativeiro (Pecari tajacu) está sendo relatado. O animal, uma fêmea com três anos de idade, apresentou um histórico médico de lesões de pele, envolvendo junção mucocutânea e de aumento de linfonodos. Exame clínico e exames complementares (hemograma completo, análise bioquímica, raspado de pele para pesquisa de ácaros e cultura fúngica) foram realizados. Após 30 dias, o animal morreu. Na necropsia, verificou-se a presença de múltiplos nódulos, pústulas assépticas e aumento dos linfonodos. Na pele e mucosa oral, histologicamente as massas consistiam em ninhos, lençóis e cordões de células redondas, e um estroma fibrovascular bem vascularizado. Os plasmócitos foram observados infiltrados entre as fibras e músculo liso da lâmina própria. Espaços contendo eosinófilos, fluido e células livres estavam presentes na massa. A maioria das células estava bem diferenciada, com uma zona perinuclear clara, mas algumas células demonstraram-se pleomórficas. Os plasmócitos apresentavam núcleo excêntrico, redondo, basófilo, e pontilhado, com cromatina variando de densa a grosseiramente organizada e nucléolos distintos. O citoplasma finamente granular de anfifílico para basofílico. As células foram interpretadas como plasmócitos neoplásicos. Células binucleadas foram observadas e células gigantes multinucleadas eram raras. Na imunohistoquímica de tecidos da mucosa oral e de linfonodos observou-se positividade moderada e multifocal para Mb-1. As células tumorais revelaram positividade fraca e intermitente para Bcl-2. Com base na localização, achados histopatológicos e imuno-histoquímicos, um plasmocitoma extramedular foi diagnosticado.(AU)

Extramedullary plasmacytoma in a captive collared peccary (Pecari tajacu) / Plasmocitoma extramedular em cateto criado em cativeiro (Pecari tajacu)

An extramedullary plasmacytoma case in a captive collared peccary (Pecari tajacu) is reported. The animal, a female aging three years old, had a medical history of diffusely distributed skin and mucocutaneous junction lesions, associated with swollen lymph nodes. Clinical examination and complementary exams (complete blood count, biochemical analysis, skin scraping to search mites and fungal culture) were performed. Thirty days after examination, the animal died. At necropsy, multiple consistent nodules, aseptic pustules and swollen lymph nodes were found. On histological exams of the skin and oral mucosa, we observed a large number of round cells forming masses organized in nests, sheets, and cords of cells in a well-vascularized fibrovascular tissue. Neoplastic plasma cells infiltrated between the fibers and the lamina propria of smooth muscle. Spaces among the cell masses were filled with some eosinophil and fluid. Most of the cells were well differentiated, presenting a perinuclear clear zone. In some points, the cells were pleomorphic. The plasma cells presented eccentric, basophilic and spherical nuclei, showing a dense to organized chromatin with distinct nucleoli. Binucleate cells were observed, but multinucleated giant cells were rare. Oral mucosa and lymph nodes tested by immunohistochemical analyses were positive for Mb-1, with a multifocal distribution. In regard to Bcl-2, the neoplastic cells were intermittent weakly positive. So, an extramedullary plasmacytoma was diagnosed in the collared peccary considering the location, the histopathological and immunohistochemical findings.(AU)

Um caso de plasmocitoma extramedular em cateto criado em cativeiro (Pecari tajacu) está sendo relatado. O animal, uma fêmea com três anos de idade, apresentou um histórico médico de lesões de pele, envolvendo junção mucocutânea e de aumento de linfonodos. Exame clínico e exames complementares (hemograma completo, análise bioquímica, raspado de pele para pesquisa de ácaros e cultura fúngica) foram realizados. Após 30 dias, o animal morreu. Na necropsia, verificou-se a presença de múltiplos nódulos, pústulas assépticas e aumento dos linfonodos. Na pele e mucosa oral, histologicamente as massas consistiam em ninhos, lençóis e cordões de células redondas, e um estroma fibrovascular bem vascularizado. Os plasmócitos foram observados infiltrados entre as fibras e músculo liso da lâmina própria. Espaços contendo eosinófilos, fluido e células livres estavam presentes na massa. A maioria das células estava bem diferenciada, com uma zona perinuclear clara, mas algumas células demonstraram-se pleomórficas. Os plasmócitos apresentavam núcleo excêntrico, redondo, basófilo, e pontilhado, com cromatina variando de densa a grosseiramente organizada e nucléolos distintos. O citoplasma finamente granular de anfifílico para basofílico. As células foram interpretadas como plasmócitos neoplásicos. Células binucleadas foram observadas e células gigantes multinucleadas eram raras. Na imunohistoquímica de tecidos da mucosa oral e de linfonodos observou-se positividade moderada e multifocal para Mb-1. As células tumorais revelaram positividade fraca e intermitente para Bcl-2. Com base na localização, achados histopatológicos e imuno-histoquímicos, um plasmocitoma extramedular foi diagnosticado.(AU)

[Intracranial tumor behavior of plasma cell neoplasms. Report of 2 cases and literature review]. / Comportamiento tumoral de neoplasias intracraneales de células plasmáticas. Reporte de 2 casos y revisión de la bibliografía.

BACKGROUND: Multiple myeloma is a plasmatic cell neoplasm that is characterized by skeletal destruction, renal failure, anemia and hypercalcemia. The skull plasmacytomas represent less than 1% of the head and neck tumors, they can be the primary lesion or occur as a secondary manifestation of multiple myeloma in 20-30% of the patients, or they can even manifest several years later after the diagnosis of plasmacytoma. Although some of the lesions may be surgically accessible, the aggressive natural behavior will complicate the evolution of the patients. We present two cases of Mexican women with intracranial plasmacytomas, one of them associated with multiple myeloma. CLINICAL CASES: The first case was a 24 year-old woman diagnosed with a multiple myeloma with plasmacytic-plasmablastic bone infiltration that was removed in 90%. She presented a local recurrence that required a second intervention for removal. The second case was a 62 year-old female with a malignant intracranial tumor of plasma cells that was totally resected. Both patients received adjuvant treatment based on chemotherapy and radiation therapy with favorable results. The patients died at 5 and 1.5 years respectively due to renal failure secondary to systemic disease. CONCLUSIONS: We propose chemotherapy and radiation therapy as an essential part of treatment for this condition, as the aggressive behavior of the neoplasms can complicate the evolution, despite being surgically accessible.

Antecedentes: el mieloma múltiple es una neoplasia de células plasmáticas caracterizada por destrucción ósea, insuficiencia renal, anemia e hipercalcemia. Los plasmacitomas de los huesos del cráneo representan menos de 1% de los tumores de cabeza y cuello. Se manifiestan como lesión primaria o secundaria a mieloma múltiple en 20-30%, incluso pueden aparecer varios años después del diagnóstico. Los autores comunicamos dos casos de pacientes mexicanas con lesiones plasmocíticas intracraneales, asociadas con mieloma múltiple.Casos clínicos: el primer caso es el de una paciente de 24 años de edad, con diagnóstico de mieloma múltiple e infiltración ósea que fue extirpado en 90%. Experimentó una recidiva local que requirió otra intervención para su remoción. El segundo caso es el de una mujer de 62 años de edad con un tumor intracraneal de células plasmáticas que se resecó en su totalidad. Ambas recibieron terapia adyuvante con quimio y radioterapia con resultados favorables. Las pacientes fallecieron a los 5 y 1.5 años, respectivamente, por insuficiencia renal como consecuencia de la enfermedad sistémica. Conclusiones: se propone a la quimioterapia y radioterapia como parte esencial del tratamiento de este tumor porque su comportamiento natural agresivo puede complicar la evolución, a pesar de ser accesibles quirúrgicamente.