Oncoplastia extrema: Cirugia conservadora en cáncer de mama multicéntrico. Presentación de caso clínico / Extreme oncoplasty: Conservative surgery in multicentric breast cancer. Presentation of a clinical case

Introducción: La conservación mamaria ha evolucionado y cada vez se utilizan más técnicas oncoplásticas para obtener mejores resultados cosméticos. Además, esta técnica permite escisiones más amplias que previenen deformidades mamarias mediante la reconstrucción de grandes defectos de resección. Objetivo: Reportar con un caso clínico: estrategia quirúrgica de una paciente con cáncer de mama multicéntrico y cirugía conservadora oncoplástica de la mama. Caso clínico: Paciente de 72 años con mamas grandes ptosis Grado III con diagnóstico de carcinoma mamario derecho multicéntrico Estadio IA mT1cNO luminal A anatómico - pronóstico. Deseos de la paciente de conservar la mama. Se decide mastoplastía terapéutica + Ganglio centinela (GC) + mastopexia y reducción contralateral. Anatomía patológica: mama derecha: carcinoma ductal infiltrante multicéntrico GC 0/3. Mama izquierda: 1 foco de 2 mm carcinoma lobulillar infiltrante de tipo clásico score II de nottingham. margen libre. Inmunohistoquimica: RE 95%, RP 95%, Her2 neu negativo score 0 y ki67 2%. Se discute caso en unidad de mastología, se decide: Radioterapia 3D bilateral y hormonoterapia con anastrazole. Discusión: La Mastoplastia terapéutica es considerada una opción de tratamiento estándar para pacientes seleccionados garantizando la conservación mamaria con seguridad oncológica aceptable, adecuados resultados estéticos y similar supervivencia. Permite la escisión tumoral con márgenes de resección más amplios y resultados oncológicos y estéticos aceptables. Creemos que es esencial brindar información precisa para ayudar a la paciente en la toma de decisiones sobre las consecuencias específicas de cualquier técnica oncoplástica. Conclusiones: La oncoplastia extrema debe ser utilizada solamente para casos seleccionados. Deberá ser evaluado por un equipo multidisicplinario, idealmente en el contexto de una unidad de mastología integrada por: cirujanos mastólogos, radioterapeutas, imagenólogos, oncólogos, anatomopatólogos y psicooncólogos. La técnica quirúrgica debe ser realizada preferentemente por cirujanos de mama con formación oncoplástica y reconstructiva de la mama(AU)

Introduction: Breast conservation has evolved and more oncoplastic techniques are used to obtain better cosmetic results. In addition, this technique allows wider excisions that prevents breast deformities by reconstructing large resection defects. Objetivo: Report with a clinical case: surgical strategy of multicentric breast cancer and oncoplastic breast-conservative surgery. Clinical case: A 72-year-old patient with large breasts with Grade III ptosis diagnosed with multicentric right mammary carcinoma Stage IA mT1cNO luminal A anatomical - prognosis. The patient's wishes to preserve the breast. Therapeutic mastoplasty + sentinel node (SLN) + mastopexy and contralateral reduction was decided. Pathology: right breast: multicentric infiltrating ductal carcinoma GC 0/3. Left breast: 1 focus of 2-mm infiltrating lobular carcinoma of the classic Nottingham score II type. free margin. Inmunohystochemistry: RE 95%, RP 95%, Her2 neu negative score 0 and ki67 2%. The case is discussed in the mastology unit, and we decided: Bilateral 3D radiotherapy and hormone therapy with anastrozole. Discussion: Therapeutic mastoplasty is considered a standard treatment option for selected patients, guaranteeing breast conservation with acceptable oncological safety, adequate aesthetic results and similar survival. It allows tumor excision with wider resection margins and acceptable oncological and cosmetic results. We believe that it is to provide accurate information to help the patient in making essential decisions about the specific consequences of any oncoplastic technique. Conclusions: Extreme oncoplasty should only be used for selected cases. It must be evaluated by a multidisciplinary team, ideally in the context of a mastology unit made up of: breast surgeons, radiotherapists, imaging specialists, oncologists, pathologists and psycho-oncologists. The surgical technique should preferably be performed by breast surgeons with oncoplastic and reconstructive training of the breast(AU)

Reticulohistiocitosis multicéntrica reveladora de cáncer de mama ductal in situ: reporte de caso con aspectos dermatoscópicos e histopatológicos / Multicentric reticulohistiocytosis revealing a ductal breast cancer in situ: a case report with dermoscopic and histopathological findings

La reticulohistiocitosis multicéntrica es una enfermedad inflamatoria, una histiocitosis de células no Langerhans, poco frecuente y de etiología desconocida. Se clasifica como multicéntrica al presentar compromiso multisistémico. La enfermedad afecta predominantemente a la piel y las articulaciones, pero es posible la afectación visceral. Las manifestaciones cutáneas se caracterizan por múltiples nódulos y pápulas de color eritemato-marronáceas, pruriginosas en la cara, manos, cuello y tronco. Se asocia a enfermedades autoinmunes y neoplasias malignas, observándose entre el 20 y el 30% de los pacientes con reticulohistiocitosis multicéntrica. Su diagnóstico se realiza sobre la base de la histopatología de tejidos afectados. Al ser una enfermedad poco reportada, no existe tratamiento estandarizado. Se reporta un caso de reticulohistiocitosis multicéntrica como manifestación paraneoplásica a un cáncer ductal de mama, siendo tratadas con éxito, sin recidivas luego de dos años de seguimiento. Pocos casos se han reportado en la literatura de reticulohistiocitosis multicéntrica asociado a cáncer mamario.

Multicentric reticulohistiocytosis is a rare non-Langerhans cell histiocytosis of unknown etiology. It is classified as multicentric because of multisystem involvement. The disease predominantly affects the skin and joints, but visceral involvement is possible. Multiple erythematous-brownish, pruritic nodules and papules on the face, hands, neck, and trunk are characteristic. It is associated with autoimmune diseases, or malignant neoplasms are seen in 20% to 30% of patients with multicentric reticulohistiocytosis. The diagnosis is based on histopathology of affected tissues. As it is an underreported disease, there is no standardized treatment. A case of multicentric reticulohistiocytosis is reported as a paraneoplastic manifestation of ductal breast cancer, being successfully treated with no recurrence after two years of follow-up. Few cases of multicentric reticulohistiocytosis associated with breast cancer have been reported in the literature.

Knowledge and perception of health-care professionals on clinical trials in India: A pan-Indian cross-sectional survey

Purpose: The purpose of the study was to evaluate the knowledge and perception of health-care professionals (HCPs), such as doctors/surgeons, pharmacists, nurses, optometrists, and lab technicians, on clinical trials (CTs) in India. Methods: The study was a pan-Indian cross-sectional survey initiated by the Indian Ophthalmology Clinical Trial Network (IOCTN) by using a previously validated questionnaire for three months of data collection. An online survey was used to record information regarding demographics, CT knowledge, and CT perception among HCPs. Results: A total of 630 responses were recorded from HCPs: 207 doctors and surgeons, 159 pharmacists, and 264 laboratory technicians, nurses, and optometrists across India. Over 90% of HCPs had a clear knowledge on the purpose of CTs, the informed consent (IC) process, ethical approval by the Drugs Controller General of India (DCGI). About 80% and 90% were aware of confidentiality of patients, voluntariness of participation, and good clinical practice. Surprisingly, less than 50% had lesser knowledge regarding monetary incentives of CT participants (CTPs). A slightly positive perception was observed regarding the potential benefits of CTPs, compensation related to injury, and importance of obtaining IC. Less than 50% had a negative perception that monetary compensation to CTPs led to bias and deprivation of standard treatments. However, no significant difference was observed between other aspects of demographics and perception regarding CTs. Conclusion: We observed doctors and surgeons to be having the highest regarding CTs, followed by pharmacists. The survey highlighted the necessity of scheduling awareness programs among the HCPs, which would improve their misconceptions and perception of CTs while interacting with patients for CT enrollment.

Challenges faced by frontline health managers during the implementation of COVID-19 related policies in India: A qualitative analysis

Background & objectives: The COVID-19 pandemic exposed the strengths and weaknesses of the healthcare systems across the world. Many directives, guidelines and policies for pandemic control were laid down centrally for its implementation; however, its translation at the periphery needs to be analyzed for future planning and implementation of public health activities. Hence, the objectives of this study were to identify the challenges faced by frontline health managers in selected States in India during the pandemic with regard to implementation of the COVID-19-related policies at the district level and also to assess the challenges faced by the them in adapting the centrally laid down COVID-19 guidelines as per the local needs of the district. Methods: A qualitative study using the grounded theory approach was conducted among frontline district-level managers from eight different States belonging to the north, south, east and west zones of India. The districts across the country were selected based on their vulnerability index, and in-depth interviews were conducted among the frontline managers to assess the challenges faced by them in carrying out COVID-19 related activities. Recorded data were transcribed verbatim, manually coded and thematically analyzed. Results: Challenges faced in implementing quarantine rules were numerous, and it was also compounded by stigma attached with the disease. The need for adapting the guidelines as per local considerations, inclusion of components of financial management at local level, management of tribal and vulnerable populations and migrants in COVID context were strongly suggested. The need to increase human resource in general and specifically data managers and operators was quoted as definite requirement. Interpretation & conclusions: The COVID-19 guidelines provided by the Centre were found to be useful at district levels. However, there was a need to make some operational and administrative modifications in order to implement these guidelines locally and to ensure their acceptability.

TAFRO syndrome mimicking systemic lupus erythematosus: Case report and literature review

TAFRO syndrome is a very rare disease, with less than 100 cases reported in the literature. It is classified as a type of idiopathic multicentric Castleman disease, but it has clinical, paraclinical, and histopathological characteristics that differentiate between TAFRO and idiopathic forms of Castleman disease not otherwise specified. However, it is a challenging exclusion diagnosis. TAFRO syndrome is characterized by systemic inflammatory involvement, often severe, which can present with kidney failure, and become a severe disease with a high mortality rate. The clinical manifestations of TAFRO can be confused with hematology malignancies or various autoimmune diseases. Although there are some reports of TAFRO syndrome associated with autoimmune compromise, there is no published consensus for the diagnosis or treatment. The case presented is a patient who meets the criteria to be classified as SLE, and with manifestations with significant clinical involvement, but with no improvement with standard treatment. It was found that the patient's systemic involvement was due to TAFRO, and that therefore the TAFRO syndrome could simulate SLE, something previously not described in the literature.

El síndrome TAFRO es una enfermedad muy poco común, con menos de 100 casos reportados en la literatura. Se clasifica como un tipo de enfermedad de Castleman multicéntrica idiopática, pero tiene características clínicas, paraclínicas e histopatológicas que permiten diferenciarla de las formas de la enfermedad Castleman idiopática no clasificadas de otra manera; sin embargo, es un diagnóstico de exclusión difícil de hacer. El síndrome TAFRO se caracteriza por compromiso inflamatorio sistémico, en muchas ocasiones severo, que puede presentarse con falla renal y convertirse en una enfermedad grave, con una alta tasa de mortalidad. Las manifestaciones clínicas de TAFRO pueden confundirse con neoplasias hematológicas o varias enfermedades autoinmunes. En la literatura existen algunos reportes de síndrome TAFRO asociados con compromiso autoinmune, pero no se ha publicado un consenso para su diagnóstico ni para su tratamiento. El caso que se presenta es un paciente que cumple con los criterios para ser clasificado como LES, que tenía manifestaciones con gran compromiso clínico, pero sin mejoría con el tratamiento estándar. Se encontró que el compromiso sistémico del paciente era por TAFRO y que, por lo tanto, el síndrome TAFRO podría simular LES, algo no descrito previamente en la literatura.

Reticulohistiocitosis multicéntrica: una gran simuladora dentro de la Reumatología / Multicentric reticulohistiocytosis: a great pretender within Rheumatology

La reticulohistiocitosis multicéntrica (RHM) es una histiocitosis de células no Langerhans, multisistémica, rara, caracterizada por afectación cutánea y articular, que poco frecuentemente afecta a otros órganos. El diagnóstico se basa en hallazgos clínicos y resultados de biopsias de piel o sinovial, sin describirse un parámetro de laboratorio específico. Realizamos una revisión de la literatura en relación a las características clínicas de esta entidad desde el punto de vista reumatológico.

Multicentric reticulohistiocytosis (MHR) is a rare, multisystem, non-Langerhans cell histiocytosis characterized by skin and joint involvement, and rarely involving other organs. Diagnosis is based on clinical findings and results of skin or synovial biopsies; a specific laboratory parameter has not been described. We conducted a review of the literature regarding the clinical characteristics of this entity from a rheumatological point of view.

Does focal heterogeneity affect survival in postoperative ipsilateral multifocal and multicentric breast cancers?

SUMMARY OBJECTIVE: In multicentric/multifocal breast tumors, there may be immunological and histological differences between foci that may affect survival and treatment choice. We aimed to evaluate the effect of focal heterogeneity seen in multicentric/multifocal breast tumors on survival. METHODS: We retrospectively collected and analyzed the clinicopathological data of 89 female patients with multifocal/multicentric breast cancer, whose surgical and medical treatment was completed and who were followed up for 5 years. RESULTS: Of all patients, 29.2% (26/89) were heterogeneous. Heterogeneity of these foci was as follows: histologic heterogeneity of index foci (mix type): 15.7% (14/89), histologic heterogeneity of inter-foci: 7.9% (7/89), and immunohistochemical heterogeneity of inter-foci: 10.1% (9/89). When additional foci were evaluated, oncological therapy was changed for 3 (3.3%) of 89 patients. Heterogeneity does not have a significant (p>0.05) effect on recurrence and survival in multicentric/multifocal breast cancers. Pathological N stage is an independent risk factor for disease-free survival (hazard ratio=2.29, 95% confidence interval=1.39-3.76, p=0.001). CONCLUSIONS: In multifocal/multicentric breast cancers, less than 4% of patients may experience heterogeneity requiring change in the therapeutic decision. However, heterogeneity does not have a significant effect on recurrence and survival in multifocal/multicentric breast cancers. The pathological N stage is an independent risk factor for disease-free survival.

Reticulohistiocitosis multicéntrica: una gran simuladora dentro de la Reumatología / Multicentric reticulohistiocytosis: a great pretender within Rheumatology

La reticulohistiocitosis multicéntrica (RHM) es una histiocitosis de células no Langerhans, multisistémica, rara, caracterizada por afectación cutánea y articular, que poco frecuentemente afecta a otros órganos. El diagnóstico se basa en hallazgos clínicos y resultados de biopsias de piel o sinovial, sin describirse un parámetro de laboratorio específico. Realizamos una revisión de la literatura en relación a las características clínicas de esta entidad desde el punto de vista reumatológico.

Multicentric reticulohistiocytosis (MHR) is a rare, multisystem, non-Langerhans cell histiocytosis characterized by skin and joint involvement, and rarely involving other organs. Diagnosis is based on clinical findings and results of skin or synovial biopsies; a specific laboratory parameter has not been described. We conducted a review of the literature regarding the clinical characteristics of this entity from a rheumatological point of view.

Paraneoplastic pemphigus as an atypical presentation of Castleman disease / Pénfigo paraneoplásico como presentación atípica de la enfermedad de Castleman

Abstract Castleman disease is a non-clonal lymphoproliferative disorder with a broad range of clinical manifestations. We present the case of a male patient with a clinical picture of asthenia, adynamia, hyporexia, weight loss, oral and genital ulcers, and red, itchy eyes. The physical exam showed conjunctival redness and oral and genital ulcers. Computed axial tomography with contrast of the chest and abdomen revealed multiple enlarged mediastinal and retroperitoneal lymph nodes, and a solid 94x51 mm retroperitoneal mass. A biopsy of the mass was taken, which reported the hyaline vascular variant of Castleman disease. A scrotal lesion biopsy was also ordered, with a histopathological analysis compatible with pemphigus. In addition, direct immunofluorescence was positive in the epidermal intercelullar spaces, as well as immunoprecipitation with anti-desmoglein, anti-desmoplakin, anti-envoplakin and pemphigoid ampule antigen. Thus, the presence of multicentric Castleman disease associated with paraneoplastic pemphigus was established. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1964)

Resumen La enfermedad de Castleman es un trastorno linfoproliferativo no clonal con amplia gama de manifestaciones clínicas. Se presenta el caso de paciente masculino con cuadro clínico consistente en astenia, adinamia, hiporexia, pérdida ponderal, úlceras orales y genitales, prurito ocular e hi peremia conjuntival. El examen físico evidenció hiperemia conjuntival, úlceras orales y genitales. La tomografía axial computarizada contrastada de tórax y abdomen reveló múltiples adenopatías mediastinales, retroperitoneales y masa sólida de 94 x 51 milímetros de localización retroperitoneal. Se realizó biopsia de la masa previamente descrita, que reportó enfermedad de Castleman variante hialino vascular. También se indicó biopsia de lesión escrotal cuyo análisis histopatológico fue compatible con pénfigo, además la fluorescencia inmunológica directa fue positiva en los espacios intercelulares de la epidermis al igual que inmunoprecipitación con anticuerpos anti-desmogleina, anti-desmoplaquina, anti-envoplaquina y antígeno del penfigoide ampollar. Por lo anteriormente descrito se definió la existencia de enfermedad de Castleman multicéntrica asociada a pénfigo paraneoplásico. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1964)

Clinicopathological features and possible pathogenesis of Castleman disease / 中国肿瘤临床

Castleman disease (CD) refers to a group of uncommon lymph node proliferative disorders that have highly clinically heterogeneous and obvious pathological features. The etiology and pathogenesis of CD remain unclear. The pathogenesis of unicentric CD (UCD) is most likely driven by clonal proliferation and acquired mutations of tumor stromal cells. Idiopathic multicentric CD (iMCD) intersects with a variety of diseases in terms of its clinical manifestation and pathological features including autoimmune factors, paraneoplastic syndrome, viral infectious factors and inflammatory factor disorders. The pathogenesis of iMCD is probably the common pathway of immune dysregulation and cytokine increase caused by the combined action of multiple etiologies. Here, we aim to summarize the latest reported etiology and pathogenesis of CD, aiming to deepen the understanding of this disease.

A multicentric study on the newly developed reconstruction locking plate for midshaft clavicular fracture / Journal of Rural Medicine

Objectives: To examine the efficacy and clinical and radiological outcomes of the use of a streamlined clavicle plate® (MEIRA, Aichi, Japan) for midshaft clavicular fractures.Methods: This was a retrospective cohort study of 155 patients with displaced midshaft clavicular fractures treated using a streamlined clavicle plate between 2015 and 2019 in 18 hospitals across Japan. A questionnaire regarding bone union and postoperative complications was used, and 136 cases were followed up for one year or until bone union. Plate fitting was evaluated retrospectively using surgical records, radiographic findings, and surgeon’s opinion.Results: During surgery, plate bending was needed in 19 cases (12.3%), poor fitting was observed in 8 cases (5.2%), and bone union was achieved in 133 cases (97.8%). Total implantation failure, including plate breakage and screw loosening, occurred in 10 cases (6.5%) from the intraoperative to postoperative period. Subjective complications were observed in 26 cases (16.8%): incongruity around the surgical scar or in the anterior chest in 23, and contracture of the shoulder in three. Plate removal was performed in 66 cases (48.5%) per patient’s request.Conclusion: The use of a streamlined clavicle plate is effective for midshaft fractures of the clavicle, and the success rates of bone union and implantation using this approach are comparable to those of other existing plates.

Impact of point-of-care cardiopulmonary ultrasound on treatment change in critically ill patients: assessment of 1 913 cases in a multicentric, prospective study / 中华超声影像学杂志

Objective:To describe the features of point-of-care cardiopulmonary ultrasound (POCUS) in the critically ill patients and analyze the independent factors associated with treatment changes after POCUS assessment.Methods:This was a prospective multicentric observational study from January to December 2018 in 13 intensive care units (ICU) in China. Consecutive patients admitted to the ICU were enrolled, POCUS were performed within the first 24 h of admission. The POCUS parameters included acute or chronic cardiac abnormality, diameter of inferior vena cava (IVC) at end-expiration, right ventricular systolic function, systolic and diastolic function of left ventricle (LV) and lung ultrasound score. The general features of patients and performers were recorded. Based on the treatment proposed by the performer before and after POCUS assessment, the patients were divided into treatment changed and unchanged groups. Factors associated with treatment changes were identified by multiple logistic regression analysis.Results:Totally 1 913 patients were enrolled including 322 (16.8%) patients with shock, 638 (33.3%) patients with respiratory failure, 139 (7.3%) patients with both shock and respiratory failure and 814 (42.6%) perioperative patients. POCUS had contributed to treatment changes in 1 204 (62.9%) patients, including 867 (72.0%) cases involved fluid management. Univariate analysis showed that there were significant differences in general characteristics of patients, performers and POCUS parameters between groups(all P<0.05). Logistic regression analysis showed that independent factors of treatment changes included ministry of education or university affiliated teaching hospitals (vs.general hospitals, OR=1.891, 95% CI=1.314-2.722, P<0.001 and OR=1.644, 95% CI=1.152-2.347, P=0.006 separately), middle and senior title performers (vs.primary title, OR=2.112, 95% CI=1.358-3.284, P=0.001, OR=3.271, 95% CI=2.129-5.025, P<0.001 separately), mechanical ventilation (vs.without, OR=0.488, 95% CI 0.381-0.626, P<0.001), IVC diameter ≤1 cm (vs.1-2 cm, OR=0.317, 95% CI 0.231-0.434, P<0.001), LV ejecting fraction <50% (vs.≥50%, OR=0.328, 95% CI=0.210-0.512, P<0.001), lung ultrasound score of 6-12 and >12 points(vs.score ≤ 6 points, OR=0.237, 95% CI=0.178-0.315, P<0.001 and OR=0.619, 95% CI=0.457-0.837, P=0.002 separately). Conclusions:POCUS assessment contributes to treatment changes, most of which involves fluid management strategy, in 62.9% critical patients. The independent influencing factors associated with POCUS parameters includes IVC diameter at end-expiratory, LV systolic function and lung ultrasound score. The performers′ title and hospital grade also have a noticeable effect.

Multicentric reticulohistiocytosis: A case report / 北京大学学报(医学版)

A 65-year-old woman developed erythema, papules and nodules over the body. Some nodules of her auricles and hands like string beads. Besides, she suffered from symmetrical swelling and pain of multiple joints, morning stiffness with deformity of joints; She had elevated erythrocyte sedimentation rate and C reactive protein levels; Her rheumatoid factor and antinuclear antibody were positive; Joints destruction was found with X-ray imaging; Skin pathology showed Dermal infiltrate of abundant histiocytes, part of them with a ground-glass appearance; A CD68 immunohistochemical stain was positive and the cells were negative for S100, CD1a. These findings were diagnostic evidences of multicentric reticulohistiocytosis (MRH). The patient received high-dose of glucocorticoids combinated with immunosuppressive agents, and achieved a satisfactory effect. MRH was a rare multisystem disease characterized by papulonodular mucocutaneous and destructive arthritis, and its pathogeny was not yet completely understood. The typical lesions of MRH were hard papules or nodules that usually occured on the hands, face and arms. Classic coral bead appearance from periungual cutaneous nodules that were characteristic of MRH. MRH was an inflammatory joint disease, affecting almost all the appendicular joints and characterized by joint multiple, symmetrical, destructive, progressive disability. Joints destruction of the distal interphalangeal joints was a unique feature of MRH. In addition to skin and joints, it could also involve other systems. There were no diagnostic laboratory markers for MRH. Laboratory examinations had often been found to be non-specific. Imageological examination mainly showed bone and joint destruction. Skin biopsy was the best test to diagnose MRH, the typical histopathological findings included an infiltrate with histiocytes and multinucleated giant cells with a ground-glass appearing in eosinophilic cytoplasm, and the immunohistochemical stain was positive for CD68. The diagnosis was typically made based on the clinical presentation, supportive radiographic findings and skin biopsy. MRH was easily possible to mistake for other more common autoimmune conditions, such as rheumatoid arthritis, psoriatic arthritis, osteoarthritis, and dermatomyositis, but the distinctive clinical, radiographic, and histologic features could aid in differentiating these diseases. MRH could mimic other rheumatic diseases, besides, it could also coexist with cancer or other autoimmune disorders. There was no standardized treatment for MRH. However, Nonsteroidal anti-inflammatory drugs, glucocorticoid, Immunosuppressant, biologic medications, and bisphosphonates had been used with varying degrees of curative effect. Treatment with glucocorticoid combined with immunosuppressants were effective for rash and arthritis, early use of them should be strongly considered, and refractory cases could be treated with biological agents. By reporting a MRH case and reviewing literature, this paper aims to help the clinicians improve the understanding of this rare disease, and suggests that when one diagnosis cannot explain the whole picture of the disease, and further evidence should be sought to confirm the diagnosis.

Comprehensive treatment for multicentric giant cell tumors of the pelvis and spine using apatinib: A case report and literature review

Background: There are no standardized treatments for giant cell tumors of the bone (GCTB) in rare locations such as the spine and pelvis or for those that are inoperable and recurrent, let alone for multicentric GCTB. This study reports a novel case of multicentric GCTB treated with a promising antiangiogenic drug, apatinib, a small-molecule tyrosine kinase inhibitor. The efficacy of apatinib in the treatment of GCTB has not been reported previously. Patients and Methods: A 27-year-old female presented with two giant cell tumors of the spine and sacrum–ilium diagnosed on December 15, 2016. Surgery and selective arterial embolization (SAE) were not reasonable options for this patient, and denosumab was unavailable; therefore, the antiangiogenic drug apatinib and the osteoclast inhibitor zoledronic acid were administered. Apatinib was initially administered at a dose of 850 mg daily, which was decreased to 425 mg daily after 7 months, and then increased again to 635 mg after 11 months. The patient was prescribed a maintenance dose of 500 mg daily after 16 months. The patient reported side effects of Grades I–III nausea, vomiting, and Grades II–III hand–foot syndrome. The patient underwent SAE at 26 months, and at that time, she was switched to denosumab instead of zoledronic acid. Results: The patient showed noticeable symptomatic improvement and visibly reduced tumor size after the first month of treatment. Computed tomography in the 4th month identified a partial response based on the RECIST criteria. The patient has achieved an objective reduction in tumor size at 32 months. Conclusions: Comprehensive treatment including apatinib represents a potential new treatment strategy for inoperable GCTB, with tolerable side effects. However, further clinical trials are now necessary to confirm an effective dose and determine the efficacy and safety of apatinib in the treatment of GCTB

Orthotic management with a customized humeral brace for Gorham-Stout disease of the humerus: A case report

@#Gorham-Stout disease is characterized by massive osteolysis or "vanishing bone" on radiograph. Due to its rarity, no standard Physical Medicine and Rehabilitation (PM&R) management has been published. With this comes the dilemma of managing another case of vanishing right humerus in a 13 year-old male, right handed student, with normal growth and development. To date, this could be the third documented case in the Philippines, but the first with humeral involvement, and the first to manage using a customized humeral brace. The absence of the right humerus affects the bimanual overhead and tabletop activities of the patient, for which a custom-made humeral orthosis was provided to manage the limited activities. There were improvements in activities such as writing, card turning, stacking, and lifting objects of variable weights, as well as with hand dexterity as evidenced by the standardized hand function tests done prior and post brace fitting. Being a rare bone disease with no standard management and unpredictable course, cases are managed symptomatically. For this case of an absent humerus significantly affecting upper extremity function, orthotic management is one aspect that could be recommended to achieve positive functional outcomes.

Impact of focality on prognostication of early and operable breast carcinomas of no special type

Background and Objectives: Multifocal/multicentric (MF/MC) breast carcinomas are not uncommon and its prognostic significance debated. We attempted to analyze the association of focality and prognostic factors in operated pT1 and pT2 breast carcinomas of no special type (NST). Materials and Methods: Retrospectively identified pathologically proven 124 unifocal (UF) and 49 MF/MC pT1 and pT2 breast carcinomas of NST over the past three years were compared in terms of clinical and pathological factors. Results: The patients with MF/MC NST tumors were more likely to undergo radical surgery (P = 0.028). The tumors showed higher incidence of lymphovascular invasion (P = 0.024), perineural invasion (P = 0.046), ductal carcinoma in situ component (P < 0.001), higher number of positive axillary lymph nodes (P < 0.001), and higher anatomical staging (P = 0.048) when compared to the UF counterparts. Morphological intertumoral heterogeneity was noted in MF/MC tumors in 16 of 49 cases (32.65%). Conclusion: Most published studies on MF breast cancers have included all histological types and varying definitions. We included only pathologically defined stages and a single histological type to ensure “purity” of the groups. Higher anatomic staging and morphological interlesional heterogeneity suggest that early MF/MC tumors represent multiple primaries with a different biology. Careful consideration of features of each focus needs to be considered when deciding appropriate adjuvant therapy and for accurately prognosticating these patients. Immunohistochemical and morphological (grade) heterogeneity between the different foci may pose problems with “prognostic stage grouping” these tumors according to the American Joint Committee on Cancer staging system (8th edition).

Advance in diagnosis and treatment of idiopathic multicentric Castleman disease / 中国肿瘤临床

Castleman disease (CD) is a rare lymphoproliferative disease that is clinically classified into unicentric Castleman disease (UCD) and multicentric Castleman disease (MCD). According to the status of human herpes virus-8 (HHV-8) infection, MCD is further classified into HHV-8-positive MCD, and HHV-8-negative MCD, which is also called idiopathic multicentric Castleman disease (iMCD). There are standard treatment options for both UCD and HHV-8-positive MCD, but there has been no uniform standard of diagnosis and treatment for iMCD, which mainly relied on the experience of clinicians. In recent years, Castleman Disease Collaborative Network (CDCN) has newly defined the concept and diagnostic criteria of iMCD, and a comprehensive guidance on the treatment of iMCD was established in November 2018. In this paper, we try to review the current treatment options and advances of iMCD, which might help Chinese clinicians to diagnose and treat this rare disease.

Corneoconjunctival manifestations of lymphoma in three dogs

An 8-year-old Shih Tzu, a 5-year-old Maltese, and a 10-year-old Maltese presented with conjunctival hyperemia and peripheral corneal edema. Severe conjunctival thickening with varying degrees of corneal extension was observed. Cytological examination showed many large lymphocytes with malignant changes in the conjunctiva which was consistent with findings in fine-needle aspiration samples taken from regional lymph nodes. They were diagnosed as having Stage V multicentric lymphoma. When conjunctival thickening is observed in canine patients with multicentric lymphoma, conjunctival metastasis with infiltration of neoplastic lymphoid cells should be included in the differential diagnosis.

Pathological features of 78 metastatic or multicentric neoplasms involving the central nervous system in dogs / Aspectos patológicos e distribuição de 78 neoplasias metastáticas ou multicêntricas envolvendo o sistema nervoso central de caninos

Secondary neoplasms affecting the central nervous system (CNS) may occur through blood flow or direct extension. This condition occurrence has increased both due to a longer life expectancy of dogs, as well as with the employment of chemotherapeutics, which may increase the survival period and, thus, the odds of the occurrence of metastasis. The aim of this study was to analyze the epidemiological features, such as the age, breed and sex of the animals affected, as well as the gross and microscopic findings of the metastasis from neoplasms involving the CNS of 78 dogs, and, based on the data, estimate the occurrence and frequency of the metastatic or multicentric neoplasms. Females (71.79%) were most affected than males, with an average age of 9.5-years-old and a median of 10-years-old. Most of the cases had a multifocal distribution (73.07%), and epithelial neoplasms (50%) were the most common. Telencephalon was the most affected neuroanatomical region (61.1%). Mammary neoplasms were the most frequent (47.44%), followed by hemangiosarcoma (19.23%), lymphoma (10.26%) and melanoma (6.41%). Less common neoplasms included osteosarcoma, histiocytic sarcoma and lung carcinoma (2.56%). Mammary neoplasms were mainly composed of anaplastic and micropapillary carcinomas, of which only multifocal microscopic lesions were observed.(AU)

Neoplasias secundárias envolvendo o sistema nervoso central (SNC) podem ocorrer por via hematógena ou por extensão direta. A ocorrência desta condição vem aumentando devido à expectativa de vida mais longa dos cães, bem como através do uso de quimioterápicos, os quais podem ampliar a sobrevida e, portanto, as chances para a ocorrência de metástases. O objetivo deste estudo foi analisar os aspectos epidemiológicos, como idade, raça e sexo dos animais afetados, bem como caracterizar os achados macroscópicos e microscópicos de neoplasmas com metástases envolvendo o SNC de 78 caninos e, baseado nestes dados, estimar a ocorrência e a frequência de neoplasias metastáticas ou multicêntricas. Fêmeas (71,79%) foram mais afetadas do que machos, com uma idade média de 9,5 anos e mediana de 10 anos. A maior parte dos casos apresentava distribuição multifocal (73,07%), e neoplasias de origem epitelial (50%) foram as mais frequentes. A localização neuroanatômica mais afetada foi o telencéfalo (61,1%). As neoplasias mamárias foram as mais frequentes (47,44%), seguidas por hemangiossarcoma (19,23%), linfoma (10,26%) e melanoma (6,41%). Osteossarcoma, sarcoma histiocítico e carcinoma pulmonar (2,56%) foram neoplasias menos frequentes. Os tipos de neoplasmas mamários mais observados foram o carcinoma anaplásico e o carcinoma micropapilar, nesses dois tipos as lesões eram multifocais e observadas somente na microscopia.(AU)

Aspectos epidemiológicos, clínicos e anatomopatológicos do linfoma folicular em cães / Epidemiological, clinical and pathological aspects of follicular lymphoma in dogs

Linfomas foliculares são uma rara forma de distúrbio linfoproliferativo descrita em medicina veterinária. Juntamente com a não reconhecida ocorrência dos linfomas de Hodgkin em cães, essa é a maior diferença acerca de linfoma entre humanos e cães. O objetivo deste artigo é descrever os achados epidemiológicos, clínicos e anatomopatológicos vistos em cinco cães com linfoma folicular. Destes, dois eram machos (40%) e três eram fêmeas (60%). A idade dos cães afetados variou de 11 a 13 anos. Quatro dos cinco (80%) cães eram de raça pura e um (20%) não tinha raça definida. Todos os cães apresentaram linfadenomegalia generalizada e esplenomegalia, o que incluiu os casos como linfoma multicêntrico. Na necropsia, os linfonodos e o baço demonstraram um padrão nodular à superfície de corte, caracterizado por dezenas a centenas de nódulos brancos, multifocais ou coalescentes e de tamanhos variáveis. Na superfície natural do baço, frequentemente (4/5, 80%), havia miríades de pontos brancos, multifocais ou coalescentes, de tamanhos variáveis. Na histopatologia, os tumores foram confirmados como linfomas foliculares. Todos os casos eram Grau III, sendo dois (40%) incluídos como IIIa e outros três (60%) como IIIb. Em um caso (1/5, 20%), o linfoma folicular foi considerado como IIIb variante de pequenos centroblastos semelhantes aos linfócitos neoplásicos vistos no linfoma de Burkitt. Os linfomas foram validados como tendo origem em células B através da imuno-histoquímica, utilizando anticorpos anti-CD20. Os casos de linfomas foliculares descritos comportaram-se de forma agressiva e levaram os pacientes à morte.(AU)

Follicular lymphomas are a rare form of lymphoproliferative disorder described in veterinary medicine. Together with the probable non-existence of Hodgkin's lymphomas in dogs, this is the biggest difference about lymphoma between humans and dogs. The aim of this article is to describe the epidemiological, clinical and anatomopathological findings observed in five dogs with follicular lymphoma. Of the five dogs with follicular lymphoma, two were male (40%) and three were female (60%). The age of affected dogs ranged from 11 to 13 years. Four of the five (80%) dogs were purebred and one (20%) had no defined breed. All dogs presented generalized lymphadenomegaly and splenomegaly, which included cases as multicentric lymphoma. At necropsy, the lymph nodes and the spleen demonstrated a nodular pattern at the cut surface, characterized by tens to hundreds of white nodules of variable size, multifocal or coalescing. On the natural surface of the spleen, often (4/5, 80%), there were myriads of white, multifocal or coalescing dots of varying sizes. In histopathology, the tumors were confirmed as follicular lymphomas. All cases were Grade III, two (40%) included as IIIa and three (60%) as IIIb. In one case (1/5, 20%), follicular lymphoma was considered as a IIIb variant of small centroblasts similar to the neoplastic lymphocytes seen in Burkitt's lymphoma. Lymphomas were validated as having origin in B cells through immunohistochemistry, using anti-CD20 antibody. The cases of follicular lymphomas described behaved aggressively and led the patients to death.(AU)