Amiloidose nodular - relato de rara apresentação da amiloidose localizada cutânea / Nodular amyloidosis - report of a rare presentation of cutaneous localized amyloidosis

Contexto: Amiloidose é um grupo de doenças caracterizadas pelo depósito de proteínas fibrilares, denominadas substância amiloide. Podem ser divididas em formas localizadas ou sistêmicas, sendo que dentre as localizadas, a forma nodular é a mais rara. Descrição do caso: Relatamos o caso de amiloidose primária localizada cutânea nodular que se apresentou com nódulos violáceos no dorso, e placas acastanhadas na região cervical há 8 anos sem evidências de envolvimento sistêmico. Discussão: Como cerca de 1% a 7% dos casos de amiloidose nodular localizada cutânea podem evoluir com envolvimento sistêmico, o seguimento dos pacientes faz-se necessário. O tratamento não é obrigatório, a retirada das lesões pode ser feita se o paciente o desejar, contudo as recidivas são frequentes. Conclusões: Mesmo possuindo baixa prevalência, a amiloidose nodular deve ser reconhecida pelo risco de progredir para acometimento sistêmico e associação com discrasias plasmocitárias, como mieloma múltiplo.

Plasmocitoma extramedular solitario rinosinusal: reporte de caso y revisión de literatura / Solitary extramedullary plasmacytoma of the rhinosinusal cavity: a case report and literature review

Resumen El plasmocitoma extramedular solitario (PES) es una neooplasia maligna infrecuente caracterizada por una proliferación aislada de células plasmáticas monoclonales en tejido extramedular. La localización más frecuente es en cabeza y cuello con predominio en el territorio rinosinusal, sin embargo, estas lesiones malignas representan menos del 1% de los tumores de esta región anatómica. El diagnostico requiere una alta sospecha clínica, análisis histopatológico acucioso, estudios serológicos y exámenes radiológicos sistémicos de acuerdo a los criterios diagnósticos establecidos en la literatura internacional. Se analiza el caso de un paciente masculino con un PES que se presentó como un tumor de fosa nasal derecha y obstrucción nasal de meses de evolución con hallazgos clínicos e imagenológicos inespecíficos. El diagnóstico definitivo se realizó mediante biopsia endoscópica nasal y estudio histopatológico. El tratamiento fue abordado de manera multidisciplinaria entre otorrinolaringología, hematología y radiooncología. De acuerdo a las guías internacionales, se decidió realizar radioterapia localizada con buen resultado clínico precoz. El PES requiere un abordaje multidisciplinario para lograr un diagnóstico y tratamiento oportuno, siendo imprescindible la exclusión del mieloma múltiple debido a las diferencias terapéuticas y en pronóstico clínico. El tratamiento puede realizarse con radioterapia y/o cirugía, siendo la radioterapia el pilar de tratamiento.

Abstract Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm characterized by isolated proliferation of monoclonal plasma cells in extramedullary tissue. The most frequent location is in the head and neck with a predominance in the rhinosinusal territory; however, these malignant lesions represent less than 1% of the tumors in this anatomical region. The diagnosis requires a high clinical suspicion, careful histopathological analysis, serological studies and systemic radiological examinations according to the diagnostic criteria established in the international literature. We analyze the case of a male patient with SEP that presented as a tumor of the right nostril and nasal obstruction of months of evolution with nonspecific clinical and imaging findings. The definitive diagnosis was made by nasal endoscopic biopsy and histopathological study. The treatment was approached by multidisciplinary teamwork. According to international guidelines, it was decided to perform localized radiotherapy with good early clinical results. SEP requires a multidisciplinary approach to achieve a timely diagnosis and treatment, being essential exclusion of multiple myeloma due to the therapeutic differences and prognosis. Treatment can be done with radiation therapy and/or surgery; radiation therapy is the mainstay of treatment.

Incidental Finding of Multiple Synchronous Neoplasms in Explanted Liver After Transplantation: A Case Report.

Liver transplantation is the only potentially curative treatment for patients with end-stage liver disease. After the procedure, histopathologic analysis of the liver explant may reveal neoplasms that were not previously diagnosed in preoperative imaging examinations. This incidental finding of primary liver neoplasms in the explant is not an uncommon situation in liver transplant, and hepatocarcinomas and cholangiocarcinomas are the types of tumors most frequently encountered in this scenario. These are the most common primary neoplasms of the liver, and liver transplantation is often a curative treatment for these types of tumors when they are in their earlier stages. In contrast, liver plasmacytoma is a rare type of plasma cell neoplasm, consisting of a single mass of monoclonal plasma cells, which is treated primarily by radiotherapy and is seldom encountered in the setting of liver transplant. We report the case of a patient who underwent liver transplantation for the treatment of cryptogenic cirrhosis, with no preoperative diagnosis of liver tumors. Analysis of the liver explant revealed the presence of three synchronous neoplasms with different histologic origins: a 27-mm hepatocellular carcinoma, a 17-mm intrahepatic cholangiocarcinoma, and a 25-mm solitary hepatic plasmacytoma. The patient received no further adjuvant treatment and remained well and with no signs of disease recurrence over an observation period of 44 months. We found no previous report in the literature of the synchronous presence of these three types of liver neoplasms.

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.

Plasmocitoma extramedular em cão positivo para leishmaniose: Relato de caso / Extramedullary plasmacytoma in a dog positive for leishmaniasis: case report

Os plasmocitomas extramedulares são uma proliferação excessiva de células plasmocíticas, que pode acometer nos cães, o sistema cutâneo e mucocutâneo. Classificados como benignos, já que por sua vez possuem um baixo potencial metastático, porém o local onde se infiltra se comporta de forma agressiva. A formação dos nódulos é isolada, mas podem ser múltiplos, apresentam coloração avermelhada e possuem tamanhos médios de 1 a 2 cm. O histopatológico evidencia plasmócitos, em arranjo de cordão e entremeados a uma discreta quantidade de tecido conjuntivo. O objetivo deste relato é identificar e diagnosticar um caso de plasmocitoma extramedular em cão positivo para leishmaniose.(AU)

The extramedullary plasmacytomas are an excessive proliferation of plasmacytic cells, which can affect the cutaneous and mucocutaneous systems in dogs. Classified as benign, as they in turn have a low metastatic potential, but the site where they infiltrate behaves aggressively. The formation of nodules is isolated, but they can be multiple, present with a reddish color and have average sizes of 1 to 2 cm. Histopathology shows plasma cells, in a cord arrangement and intermingled with a small amount of connective tissue. The purpose of this report is to identify and diagnose a case of oral plasmacytoma in a dog.(AU)

Safety and Feasibility Analysis of a Prospective Trial on Stereotactic Body Radiotherapy for Solitary Bone Plasmacytoma.

BACKGROUND: There have been reports on the use of hypofractionated stereotactic body radiotherapy (SBRT) for bone plasmacytomas, but no prospective data are available. We present the initial analysis of an ongoing prospective protocol on SBRT addressing the feasibility and safety of this treatment for solitary bone plasmacytomas. PATIENTS AND METHODS: A prospective cohort of SBRT for solitary bone plasmacytoma was developed. Patients could receive different doses depending on the index bone, from single fraction for skull base lesions, 24 Gy in 3 fractions for spine lesions, and 30 Gy in 5 fractions for other bones. Overall survival, bone events, local control, and progression to multiple myeloma (MM) were measured and compared to our retrospective cohort of patients treated with conformal standard-dose radiotherapy. Quality of life was assessed via the EORTC QLQ-C30 questionnaire, and toxicities were assessed by the CTCAE v5.0 criteria. After 1 year or the inclusion of 5-10 patients, a feasibility and safety analysis was programmed. RESULTS: Between April 2018 and April 2019, 5 patients were included. All were male, with a median age of 53.1 years. The median follow-up was 21.8 months. No patient had local progression, bone event, or died. Two patients had progressions to MM. The mean survival free of progression to MM was 18.6 months, compared to 19 months in the retrospective cohort; median values were not reached. There were no grade 3 toxicities. CONCLUSION: SBRT for plasmacytoma is safe and feasible. More robust data are awaited.

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)

Plasmacytoma and plasma cell myeloma affecting the jaws: A multi-institutional collaborative study.

BACKGROUND: Plasma cell neoplasms are characterized by the proliferation of a single clone of plasma cells with production of a monoclonal immunoglobulin. They can manifest as a single lesion (plasmacytoma) or as multiple lesions (multiple myeloma). METHODS: Paraffin-embedded tissue blocks of patients microscopically diagnosed with plasma cell neoplasms in the jaws were retrieved from five pathology files. Data including clinical, radiographic, microscopic and immunohistochemical findings, treatment employed and follow-up status were retrieved from the pathology reports. RESULTS: Fifty-two cases were retrieved (mean age: 59.4 years) without sex predilection. The mandible was the most affected site (67.3%), usually associated with pain and/or paresthesia (53.8%). Lesions in other bones besides the jaws were reported for 24 patients (46.2%). Radiographically, tumours usually presented as poorly defined osteolytic lesions with unilocular or multilocular images, while microscopy revealed diffuse proliferation of neoplastic plasma cells with nuclear displacement and abundant eosinophilic cytoplasm. Two cases were classified as anaplastic, and amyloid deposits were found in two other cases. Immunohistochemistry was positive for plasma cell markers and negative for CD20 and CD3, and monoclonality for kappa light chain predominated. The overall survival rate after 5 years of follow-up was 26.6%. CONCLUSION: Plasma cell neoplasms are aggressive tumours with a poor prognosis and involvement of the jaws may be the first complaint of the patient. Thus, oral pathologists, head and neck surgeons and dentists should be aware of their clinical, radiographic and microscopic manifestations.

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

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

Plasmocitoma extramedular de terceira pálpebra em gato / Extramedullary plasmacytoma of the third eyelid in a cat

Background: Extramedullary plasmacytoma is a neoplasm originating from plasmacytes with benign behavior, althoughit can present malignant characteristics such as local invasion and metastases in some cases. Primary extramedullary plasmacytoma in the third eyelid is rare in humans and animals and has not yet been described in cats. Thus, the goal of thepresent study is to describe the cytological and histopathological findings of an extramedullary plasmacytoma in a catsthird eyelid.Case: A 5-year-old female feline patient with a history of ocular alteration for approximately 30 days was referred tothe ophthalmology service. Upon ophthalmic examination of the left eye, an increase in volume with tissue proliferationwas identified in the third eyelids conjunctiva. A collection of material for cytopathological examination was performed.Numerous plasmocytes and atypical lymphocytes with anisocytosis and anisokaryosis were observed, with a possible neoplastic injury being suggested and referred to the patient for surgical removal of the third eyelid. In the histopathologicalanalysis, it was possible to observe several plasma cells and vascular neoformation along the entire conjunctive marginbelow the epithelium. The glands, serous and mucous, present in the fragment, revealed areas of intervening plasmacyticinfiltrate and a large area close to the hyaline cartilage containing intense plasmacytic inflammatory infiltrate with rareneutrophils and macrophages. There was also a structural breakdown of glandular components and lymphocyte cells withmitosis figures and discrete cellular pleomorphism.Discussion: Plasmacytoma is a rare neoplasm in both dogs and cats. The anatomical regions most frequently affected arethe digits, lips, and pinna. Concerning the 3rd eyelid tumor involvement, numerous...(AU)

Plasmocitoma extramedular de terceira pálpebra em gato / Extramedullary plasmacytoma of the third eyelid in a cat

Background: Extramedullary plasmacytoma is a neoplasm originating from plasmacytes with benign behavior, althoughit can present malignant characteristics such as local invasion and metastases in some cases. Primary extramedullary plasmacytoma in the third eyelid is rare in humans and animals and has not yet been described in cats. Thus, the goal of thepresent study is to describe the cytological and histopathological findings of an extramedullary plasmacytoma in a cat’sthird eyelid.Case: A 5-year-old female feline patient with a history of ocular alteration for approximately 30 days was referred tothe ophthalmology service. Upon ophthalmic examination of the left eye, an increase in volume with tissue proliferationwas identified in the third eyelid’s conjunctiva. A collection of material for cytopathological examination was performed.Numerous plasmocytes and atypical lymphocytes with anisocytosis and anisokaryosis were observed, with a possible neoplastic injury being suggested and referred to the patient for surgical removal of the third eyelid. In the histopathologicalanalysis, it was possible to observe several plasma cells and vascular neoformation along the entire conjunctive marginbelow the epithelium. The glands, serous and mucous, present in the fragment, revealed areas of intervening plasmacyticinfiltrate and a large area close to the hyaline cartilage containing intense plasmacytic inflammatory infiltrate with rareneutrophils and macrophages. There was also a structural breakdown of glandular components and lymphocyte cells withmitosis figures and discrete cellular pleomorphism.Discussion: Plasmacytoma is a rare neoplasm in both dogs and cats. The anatomical regions most frequently affected arethe digits, lips, and pinna. Concerning the 3rd eyelid tumor involvement, numerous...

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 extramedular oral em felino doméstico / Oral extramedullary plasmocytoma in domestic cat

Plasmocitomas são geralmente incomuns em cães, representando cerca de 3% das neoplasias, e, em gatos, esse número é ainda menor, sendo consideradas raras e observadas em aproximadamente 1% dos casos. Objetiva-se com este trabalho apresentar um relato de caso de uma doença muito pouco relatada na oncologia veterinária, o plasmocitoma extramedular oral em felinos domésticos. A metodologia empregada para a construção deste trabalho deu-se por meio de: consulta a referências bibliográficas; observação e acompanhamento dos processos realizados no âmbito hospitalar, além da consulta ao prontuário médico do paciente. Embora muito descrita na literatura a utilização da cirurgia de mandibulectomia ou maxilectomia na terapia de plasmocitomas orais em cães, são escassos os relatos do emprego da técnica em gatos. Porém, é certo afirmar que a eletroquimioterapia, associada à quimioterapia antineoplásica adjuvante, promoveu uma ótima sobrevida (407 dias), sem comprometimento na qualidade de vida do paciente, mostrando que, mesmo com um tratamento menos invasivo e conservador, foi possível cuidar do paciente de maneira efetiva. Uma grande dificuldade, durante todo o processo, foi a falta de relatos científicos específicos para o plasmocitoma oral em felinos. As neoplasias orais em felinos e, principalmente, as originadas por plasmócitos precisam ser mais estudadas e relatadas, promovendo maior conhecimento técnico e científico a todos os profissionais da área.

Plasmocytomas are generally uncommon in dogs, representing about 3% of neoplasms, and in cats, this number is even lower, being considered rare and observed in approximately 1% of cases. The aim of this work is to present a case report of a disease that is rarely reported in veterinary oncology: oral extramedullary plasmacytoma in domestic cats. The methodology used for the construction of this work was given through: consultation of bibliographic references; observation and monitoring of processes performed in the hospital, in addition to consulting the patient's medical record. Although the use of mandibulectomy or maxillectomy surgery to treat oral plasmacytomas in dogs is widely described in the literature, there are few reports on the use of the technique in cats. However, it is true to say that electrochemotherapy, associated with adjuvant antineoplastic chemotherapy, promoted an excellent survival (407 days), without compromising the patient's quality of life, showing that even with a less invasive and conservative treatment it was possible to care for the patient in a way effective. A major difficulty throughout the process was the lack of specific scientific reports for oral plasmacytoma in felines. Oral neoplasms in felines and mainly those originating from plasmocytes need to be further studied and reported, promoting greater technical and scientific knowledge to all professionals in the field.

Anaplastic extraosseous (extramedullary) plasmacytoma in the right nostril.

Extraosseous (extramedullary) plasmacytomas are rare plasma cell neoplasms that can result in an erroneous and/or delayed diagnosis as often they are not considered in the differential diagnosis due to their rarity. Furthermore, the anaplastic type is one of the most difficult to recognize in biopsies. We report the case of a patient with an extraosseous plasmacytoma occluded in the right nostril. Its prompt and accurate diagnosis resulted in early treatment and a good outcome, despite the tumour being anaplastic with the risk of progressing into a plasma cell myeloma. The patient shows no recurrence or disease progression after 10 years of follow-up. Our case highlights the clinical and pathological characteristics of this rare disorder that should be considered in order to improve diagnostic criteria and thus early treatment. We also reviewed the pertinent literature.

Plasmocitoma extramedular de laringe: a propósito de un caso / Extramedullary plasmacytoma of the larynx: a case report

Resumen Los plasmocitomas solitarios son una rara forma de presentación de neoplasias de células plasmáticas. Deben ser diferenciados del mieloma múltiple en el momento del diagnóstico y realizar un seguimiento estricto por el riesgo de transformación a mieloma. La localización de los mismos en la laringe es muy inusual, dado que sólo se han publicado 56 casos. Se presenta el caso de una paciente con un plasmocitoma extramedular de laringe que se trató mediante cirugía y radioterapia. Se revisan los criterios diagnósticos y los problemas terapéuticos.

Abstract Solitary plasmacytomas are a rare form of plasma cell neoplasms. They should be differentiated from multiple myeloma at the time of diagnosis and strictly monitored for the risk of transformation to myeloma. Their location in the larynx is very unusual, since only 56 cases have been published. We present the case of a patient with extramedullary plasmacytoma of the larynx that has been treated with surgery and radiotherapy. We reviewed the literature for diagnostic criteria and therapeutic problems.

Plasmocitoma mamario y cáncer de mama: a propósito de un caso / Breast Plasmacytoma and breast cancer: a case report

El plasmocitoma mamario es una neoplasia de células plasmáticas extremadamente infrecuente, con menos de cincuenta casos descritos en el último siglo. Por este motivo, apenas se dispone de datos acerca del abordaje, tratamiento y seguimiento más convenientes. Presentamos el caso de una paciente de 70 años que debutó con un plasmocitoma mamario y que un año después fue diagnosticada de un carcinoma mamario lobulillar ipsilateral. La asociación entre plasmocitoma y cáncer de mama no está descrita en la literatura, por lo que es muy complicado establecer un vínculo entre ambas entidades. Sin embargo, el abordaje terapéutico del plasmocitoma podría comprometer el tratamiento ulterior de un cáncer de mama, por lo que el tratamiento idóneo en estos casos sea probablemente la cirugía.

Breast plasmocytoma is an extremely rare plasma cell neoplasm, with less than 50 cases reported in the last century. This is the reason why we barely have data about optimal management, treatment and follow-up. We hereby report the case of a 70 year old woman diagnosed with breast plasmocytoma that developed lobular breast cancer a year later. The link between plasmocytoma and breast cancer has not been previously established. However, breast plasmocytoma treatment could compromise latter breast cancer approach, so probably the most suitable strategy in these cases should be breast surgery.Conclusions: There are clinical characteristics associated with complications in women with surgical management abortion in our center, such as admission diagnosis, unplanned pregnancy, previous abortion and type of evacuation. There are limitations regarding the quantity and quality of information, however, our results allow us to know the profile of patients treated for abortion in our center.