Analysis of completion intraoperative venography during first rib resection for venous thoracic outlet syndrome.

BACKGROUND: We evaluated the impact of completion intraoperative venography on clinical outcomes for axillosubclavian vein (AxSCV) thrombosis owing to venous thoracic outlet syndrome (vTOS). METHODS: We performed a retrospective, single-center review of all patients with vTOS treated with first rib resection (FRR) and intraoperative venography from 2011 to 2023. We reviewed intraoperative venographic films to classify findings and collected demographics, clinical and perioperative variables, and clinical outcomes. Primary end points were symptomatic relief and primary patency at 3 months and 1 year. Secondary end points were time free from symptoms, reintervention rate, perioperative complications, and mortality. RESULTS: Fifty-one AxSCVs (49 patients; mean age, 31.3 ± 12.6 years; 52.9% female) were treated for vTOS with FRR and external venolysis followed by completion intraoperative venography with a mean follow up of 15.5 ± 13.5 months. Before FRR, 32 underwent catheter-directed thrombolysis (62.7%). Completion intraoperative venography identified 16 patients with no stenosis (group 1, 31.3%), 17 with no stenosis after angioplasty (group 2, 33.3%), 10 with residual stenosis after angioplasty (group 3, 19.7%), and 8 with complete occlusion (group 4, 15.7%). The overall symptomatic relief was 44 of 51 (86.3%) and did not differ between venographic classifications (group 1, 14 of 16; group 2, 13 of 17; group 3, 10 of 10; and group 4, 7 of 8; log-rank test, P = .5). The overall 3-month and 1-year primary patency was 42 of 43 (97.7%) and 32 of 33 (97.0%), respectively (group 1, 16 of 16 and 9 of 9; group 2, 16 of 17 and 12 of 13; group 3, 10 of 10, 5 of 5; group 4, primary patency not obtained). There was one asymptomatic rethrombosis that resolved with anticoagulation, and three patients underwent reintervention with venous angioplasty for significant symptom recurrence an average 2.89 ± 1.7 months after FRR. CONCLUSIONS: Our single-center retrospective study demonstrates that FRR with completion intraoperative venography has excellent symptomatic relief and short- and mid-term patency despite residual venous stenosis and complete occlusion. Although completion intraoperative venographic classification did not correlate with adverse outcomes, this protocol yielded excellent results and provides important clinical data for postoperative management. Our results also support a conservative approach to AxSCV occlusion identified after FRR.

Perianal Paget's disease metachronically associated with adenocarcinoma

Introduction: This article describes an unusual case of perianal Paget's Disease in a 42-year-old man, highlighting distinct characteristics about more typical cases. Case report: 42-year-old male patient with persistent perianal lesion, recurrent abscesses, and discomfort. Magnetic resonance imaging indicated a possible fistulous path. Biopsy revealed secondary anal Paget's disease and adenocarcinoma. Chemotherapy and radiotherapy treatment demonstrated a positive response. Discussion: Perianal Paget's Disease is a rare condition of the anal canal that is part of the Extramammary Paget's Disease (EMPD) group. Symptoms include itching, pain, perianal bleeding, and skin lesions. Diagnosis can be challenging and usually involves performing a histopathological biopsy associated with an immunohistochemical study. Treatment varies and may involve surgery, radiotherapy, and chemotherapy. Due to the rarity of the disease, the best therapeutic approach is not yet well defined, and follow-up is essential due to the risk of recurrence. Conclusion: Perianal Paget's Disease is rare and requires differentiation from other conditions. Early detection is essential to prevent malignancy. (AU)

Are there clinical and subclinical/pathological forms of Paget's disease of the breast?

Introduction: Breast disease management has changed over recent decades, related to molecular subtype, oncoplastic surgery and targeted therapies. Nevertheless, literature on Paget's disease of the breast (PDB), initially described as a clinical entity and now considered a multifocal/multicentric disease. Methods: PDB was classified as clinical in the presence of areolar abnormalities and as subclinical/pathological in all other cases. Clinical and prognostic data were evaluated and compared between the different presentation forms. Statistics comprised descriptive analysis, inter-group comparison (chi-square and Mann-Whitney tests) and overall and cancer-specific survival rates (Kaplan-Meier method and the log-rank test). Results: Of 85 patients included in this series, PDB was clinical in 58.8%. Overall, 27.1% had stage 0 and 92.9% had multifocal/multicentric disease. Most patients (83.5%) had the HER2 or luminal HER2 molecular subtype. Patients with clinical PDB had a higher rate of in situ disease (p=0.028) and were more likely to undergo breast-conserving surgery (p<0.001). Most of the 43 patients with HER2 invasive disease received anti-HER therapy. Mean follow-up time was 71.2 ± 43.3 months. Cancer-specific actuarial survival at 60 and 120 months was 92.3% and 83.1%, respectively. Survival was unaffected by the clinical form of PDB (p=0.275), anti-HER therapy (p=0.509) or oncoplastic surgery (p=0.821). Conversely, clinical stage affected survival significantly (p ≤ 0.001). Conclusion: PDB is a rare condition associated with multifocality/multicentricity and HER2 overexpression. Cases of clinical disease and those of subclinical/pathological disease differ significantly. Further studies are required to evaluate the clinical/areolar disease and the impact of advances in breast disease management on PDB.

Informe de un caso clínico con síndrome de Paget-Schroetter / Report of a clinical case with Paget-Schroetter Syndrome

Introducción: El síndrome de Paget-Schroetter (SPS) es una trombosis venosa profunda primaria del complejo venoso subclavio-axilar que ocurre después del uso repetitivo y extenuante de los hombros y los brazos. Muestra una incidencia de 1 por 100 000 personas al año. Se informa con mayor frecuencia en atletas jóvenes. Objetivo: Presentar un caso inusual de síndrome de Paget-Schroetter en un individuo joven no deportista. Caso clínico: Varón militar activo de 24 años de edad, sin antecedentes patológicos personales, que ingresó con inflamación del miembro superior izquierdo de 24 horas de evolución. Presentó una trombosis de la vena cefálica izquierda después de un esfuerzo físico de carga y descarga. Tras descartar trastornos secundarios de hipercoagulabilidad se le diagnosticó un SPS. Se le informó de la opción de intervención quirúrgica, pero la rechazó. El diagnóstico fue confirmado con ecografía Doppler y tratado con anticoagulación endovenosa al inicio y luego por vía oral durante 6 meses. Durante el seguimiento no se evidenció trombosis crónica de la vena cefálica izquierda ni formación de intervalo de colaterales vasculares. Conclusiones: El SPS es una condición clínica que necesita un alto índice de sospecha y un diagnóstico oportuno, por tanto, los médicos deben estar atentos a esta rara entidad para su reconocimiento temprano y derivación oportuna a cirugía vascular(AU)

Introduction: Paget-Schroetter syndrome (PSS) is a primary deep vein thrombosis of the subclavian-axillary venous complex that occurs after repetitive and strenuous use of the shoulders and arms. It shows an incidence of 1 per 100,000 people per year. It is reported more frequently in young athletes. Objective: To present an unusual case of Paget-Schroetter syndrome in a young non-athlete individual. Clinical case: 24-year-old active military man with no personal pathological history is presented, who was admitted with inflammation of the left upper limb of 24 hours of evolution. He presented a thrombosis of the left cephalic vein after a physical effort of loading and unloading. After ruling out secondary hypercoagulability disorders, he was diagnosed with SPS. He was informed of the option of surgical intervention, but he declined it. The diagnosis was confirmed with Doppler ultrasound and treated with intravenous anticoagulation at the beginning, and then orally for 6 months. During the follow-up of the patient, there was no evidence of chronic thrombosis of the left cephalic vein or interval formation of vascular collaterals. Conclusions: SPS is a clinical condition that requires a high index of suspicion and prompt diagnosis, therefore, physicians must be attentive to this rare entity for early recognition and timely referral to vascular surgery(AU)

Oncoplastic surgery for Paget's disease of the breast.

Introduction: Paget's disease of the breast (PDB) is a rare nipple entity associated with multifocality. Due to its location, resection of the entire nipple-areolar complex is necessary. Historically central quadrantectomy and mastectomy have the surgical treatments of choice. The feasibility of oncoplastic breast surgery (OBS) for PDB is unknown. Methods: This was a retrospective study performed in a Brazilian oncological hospital. We evaluated the factors related to the performance of OBS in PDB. In addition, the impact of OBS on local recurrence and survival was analysed. Comparisons were made between groups using the chi-square test, Mann-Whitney U test, and Kaplan-Meier method. To assess the impact factor of the variables on the performance of OBS, logistic regression was performed. Results: Eighty-five patients were evaluated. OBS was performed in 69.4% (n=59), and of these, 16 (27.2%) were symmetrized with contralateral surgery. Mastectomy without reconstruction was performed in 28.3% of the patients. The primary procedure performed was mastectomy with reconstruction (n=38; 44.7%), and the preferential technique for immediate reconstruction was skin-sparing mastectomy with prosthesis; for late reconstruction, the preferred technique was using the latissimus dorsi. Breast conserving-surgery was performed in 27.0% (n=23), primarily using the plug-flap technique (OBS). Age was associated with the use of OBS; as patients aged 40-49 exhibited a higher rate of OBS (p = 0.002; odds ratio 3.22). OBS did not influence local recurrence (p=1.000), overall survival (p=0.185), or cancer-specific survival (p=0.418). Conclusion: OBS improves options related to surgical treatment in PDB without affecting local recurrence or survival rates.

Enfermedad de Paget pigmentada de la mama simuladora de melanoma cutáneo / Pigmented mammary Paget's disease mimicking cutaneous melanoma

Resumen ANTECEDENTES: La enfermedad de Paget pigmentada de la mama es una variante poco frecuente de este padecimiento cutáneo, una dermatosis que afecta al complejo areola-pezón de manera típicamente unilateral. La importancia del reconocimiento de esta dermatosis pigmentada inespecífica es que forma parte del diagnóstico diferencial del melanoma cutáneo, por manifestarse como una mácula pigmentada irregular, cuyo estudio histopatológico muestra una proliferación de melanocitos en los estratos suprabasales de la epidermis, fagocitosis de melanina y melanófagos en dermis, hallazgos muy similares al melanoma cutáneo, además de la coexistencia de células claras malignas, características de la enfermedad de Paget. La inmunohistoquímica es una técnica auxiliar en la dermatopatología, que forma parte del proceso diagnóstico de los pacientes para lograr el diagnóstico certero que, al correlacionar la clínica y el estudio histopatológico, le permite al dermatólogo tratar a los pacientes con enfermedad de Paget pigmentada de la mama junto con un equipo multidisciplinario para la búsqueda, diagnóstico y, en su caso, tratamiento quirúrgico de las neoplasias subyacentes que suelen relacionarse con esta variante pigmentada. CASO CLÍNICO: Paciente de 35 años con una lesión pigmentada que afectaba el complejo areola-pezón derecho. Con la correlación histopatológica e inmunohistoquímica se estableció el diagnóstico de enfermedad de Paget pigmentada de la mama y posterior al procedimiento ginecológico, se asoció esta dermatosis con un adenocarcinoma microinvasor triple negativo. CONCLUSIONES: La enfermedad de Paget pigmentada de la mama es un diagnóstico complejo que requiere correlación clínico-patológica y estudios de extensión para valorar su asociación con neoplasias subyacentes.

Abstract BACKGROUND: Pigmented Mammary Paget's Disease is a rare variant of this skin condition, an unilateral dermatosis that typically affects the areola-nipple complex. The importance of recognizing this nonspecific pigmented dermatosis resides in its differential diagnosis of cutaneous melanoma, as it presents as an irregular pigmented macula. The histopathological study shows proliferation of melanocytes in the suprabasal layers of epidermis, phagocytosis of melanin and melanophages in dermis. These findings are very similar to cutaneous melanoma, in addition to the presence of malignant clear epitelial cells that are characteristic of Paget's disease. Immunohistochemistry is part of the approach of patients with lesions that shows proliferating melanocytes to rule out other neoplasms. Making an accurate diagnosis by correlating the clinical, histopathological study and immunohistochemistry allows the dermatologist to approach patients with mammary pigmented mammary Paget's disease with a multidisciplinary team for the diagnosis and surgical treatment of the underlying neoplasms that are usually related to this pigmented variant. CASE REPORT: We report the case of a 35 years old woman with a pigmented lesion that affected the right areola-nipple complex. With the histopathological and immunohistochemical correlation, the diagnosis of pigmented mammary Paget's disease was made and after the gynecological approach, this dermatosis was associated with a triple negative microinvasive adenocarcinoma. CONCLUSION: Pigmented mammary Paget's disease is a complicated diagnosis that requires clinicopathological correlation and extension studies to assess its association to underlying neoplasms.

Clinical features, diagnostic evaluation and responses to treatment in Paget's disease of bone in a center specialized in bone metabolism / Características clínicas, evaluación diagnóstica y respuestas al tratamiento de la enfermedad de Paget ósea en un centro especializado en metabolismo óseo

Abstract The epidemiology of Paget's disease of bone (PDB) has changed in the last years but there is no update data on its clinical presentation, diagnosis and management in Latin America. Our aim was to describe its clinical features, diagnostic evaluation and responses to treatment in a group of PDB patients treated between June 2012 and December 2019 in an institution specialized in bone diseases, in Buenos Aires, Argentina. The frequency of PDB (180/10 714) was 1.68%. Median age was 67 (range 39-97) years and 59.5% were women. Most patients were asymptomatic (58.6%) and had monostotic disease (54.3%). Favorable responses were obtained in all patients who were treated with zoledronate (n = 36), in 10 out of 14 treated with pamidronate, in 9 out of 10 who received intravenous ibandronate and in 12 out of 13 who received oral bisphosphonates. The response rates were not significantly different when we compared monostotic vs. polyostotic disease. Among the biochemical parameters, mean values of bone specific and total alkaline phosphatase, and C-terminal cross-linked telopeptide of type I collagen decreased significantly after treatment with bisphosphonates. It seems that our results reflect the change in PDB epidemiology towards a more indolent disease. In the future, this would probably allow physicians to use lower doses of bisphosphonates than the ones historically recommended for these patients.

Resumen La epidemiología de la en fermedad de Paget ósea (EPO) ha cambiado en los últimos años. Son necesarios datos actualizados sobre su forma de presentación clínica, diagnóstico y tratamiento en nuestra región. Nuestro objetivo fue describir las características clínicas, evaluación diagnóstica y respuestas al tratamiento de un grupo de pacientes con EPO en un centro especializado en salud ósea de Buenos Aires, Argentina. Se evaluaron todos los pacientes que fueron atendidos en nuestra institución por enfermedades óseas entre junio de 2012 y diciembre de 2019. La frecuencia de EPO (180/10 714) fue de 1.68%. La mediana de edad fue de 67 (rango 39-97) años. El 59.5% eran mujeres. La mayoría se encontraba asintomático (58.6%) y tenían enfermedad monostótica (54.3%). Se objetivaron respuestas favorables en todos los que recibieron zoledronato (n = 36), en 10 de 14 pacientes que recibieron pamidronato, en 9 de 10 que utilizaron ibandronato endovenoso y en 12 de 13 con bifosfonatos orales. Los porcentajes de respuesta no variaron significativamente entre pacientes con formas monostóticas y poliostóticas. Entre los parámetros bioquímicos, los valores de fosfatasa alcalina total y ósea y de β cross-laps disminuyeron significativamente luego del tratamiento con bifosfonatos. Nuestros resultados reflejarían un cambio en la epidemiología de la EPO hacia una forma de presentación más indolente. Esto permitiría probablemente el uso de dosis más bajas de bifosfonatos que las históricamente recomendadas para estos pacientes.

Clinical features, diagnostic evaluation and responses to treatment in Paget's disease of bone in a center specialized in bone metabolism.

The epidemiology of Paget's disease of bone (PDB) has changed in the last years but there is no update data on its clinical presentation, diagnosis and management in Latin America. Our aim was to describe its clinical features, diagnostic evaluation and responses to treatment in a group of PDB patients treated between June 2012 and December 2019 in an institution specialized in bone diseases, in Buenos Aires, Argentina. The frequency of PDB (180/10 714) was 1.68%. Median age was 67 (range 39-97) years and 59.5% were women. Most patients were asymptomatic (58.6%) and had monostotic disease (54.3%). Favorable responses were obtained in all patients who were treated with zoledronate (n = 36), in 10 out of 14 treated with pamidronate, in 9 out of 10 who received intravenous ibandronate and in 12 out of 13 who received oral bisphosphonates. The response rates were not significantly different when we compared monostotic vs. polyostotic disease. Among the biochemical parameters, mean values of bone specific and total alkaline phosphatase, and C-terminal crosslinked telopeptide of type I collagen decreased significantly after treatment with bisphosphonates. It seems that our results reflect the change in PDB epidemiology towards a more indolent disease. In the future, this would probably allow physicians to use lower doses of bisphosphonates than the ones historically recommended for these patients.

La epidemiología de la enfermedad de Paget ósea (EPO) ha cambiado en los últimos años. Son necesarios datos actualizados sobre su forma de presentación clínica, diagnóstico y tratamiento en nuestra región. Nuestro objetivo fue describir las características clínicas, evaluación diagnóstica y respuestas al tratamiento de un grupo de pacientes con EPO en un centro especializado en salud ósea de Buenos Aires, Argentina. Se evaluaron todos los pacientes que fueron atendidos en nuestra institución por enfermedades óseas entre junio de 2012 y diciembre de 2019. La frecuencia de EPO (180/10 714) fue de 1.68%. La mediana de edad fue de 67 (rango 39-97) años. El 59.5% eran mujeres. La mayoría se encontraba asintomático (58.6%) y tenían enfermedad monostótica (54.3%). Se objetivaron respuestas favorables en todos los que recibieron zoledronato (n = 36), en 10 de 14 pacientes que recibieron pamidronato, en 9 de 10 que utilizaron ibandronato endovenoso y en 12 de 13 con bifosfonatos orales. Los porcentajes de respuesta no variaron significativamente entre pacientes con formas monostóticas y poliostóticas. Entre los parámetros bioquímicos, los valores de fosfatasa alcalina total y ósea y de Β cross-laps disminuyeron significativamente luego del tratamiento con bifosfonatos. Nuestros resultados reflejarían un cambio en la epidemiología de la EPO hacia una forma de presentación más indolente. Esto permitiría probablemente el uso de dosis más bajas de bifosfonatos que las históricamente recomendadas para estos pacientes.

Enfermedad de Paget vulvar recurrente con respuesta a quimioterapia con carboplatino paclitaxel; reporte de un caso y revisión de la literatura / Recurrent vulvar paget disease responding to carboplatin paclitaxel chemotherapy: case report and literature review

La enfermedad de Paget vulvar es sumamente rara, representa menos del 1% de las neoplasias vulvares. Generalmente tiene un curso crónico y es altamente recurrente. La cirugía es el estándar de tratamiento; sin embargo, los decepcionantes resultados del tratamiento quirúrgico y la alta morbilidad, han llevado a buscar otras alternativas de tratamiento incluyendo radioterapia, vaporizaciones con láser de CO2 y quimioterapia entre otros. Todas ellas con resultados prometedores, aunque hay una necesidad de estudios prospectivos para explorar estos enfoques. Presentamos el caso de una paciente con enfermedad de Paget vulvar recurrente con muy buena respuesta a la quimioterapia sistémica con carboplatino mas paclitaxel

Enfermedad de Paget bilateral de la mama. Presentación inusual de un caso

RESUMEN La enfermedad de Paget de la mama se encuentra entre las llamadas formas especiales del cáncer de mama. Su incidencia es de solo el 1 al 1,5 % del total de los cánceres mamarios que se diagnostican. Fundamentalmente aparece en mujeres en la quinta o sexta décadas de la vida. Se considera extremadamente rara en su forma bilateral. Se presenta el caso de una paciente de 32 años de edad que acudió a consulta provincial de mastología por presentar lesiones escamosas en el complejo areola pezón de ambas mamas, más acentuadas en la derecha. Se le realizó biopsia con aspiración con aguja fina y se diagnostica enfermedad mamaria de Paget bilateral. La enfermedad de Paget de la mama es una enfermedad de rara aparición y en el caso que se presenta aparecen dos características pocos comunes, el tratarse de una paciente joven y la localización bilateral de la enfermedad, lo cual hace de más valor su publicación para conocimiento del personal médico dedicado a la atención de estas afecciones.

ABSTRACT Paget's disease of the breast is among the so-called special forms of breast cancer. Its incidence is only 1 to 1.5 % of all breast cancers that are diagnosed. It mainly appears in women in the fifth or sixth decade of life. It is considered extremely rare in its bilateral form. The case of a 32-years-old female patient of urban origin who attended the provincial mastology clinic for presenting scaly lesions in the nipple areola complex of both breasts, more accentuated on the right side. She underwent a fine needle aspiration biopsy and was diagnosed with bilateral Paget's disease of the breast. Paget's disease of the breast is a disease of rare appearance throughout the world and in the case that it occurs, two uncommon characteristics appear, the fact that it is a young patient and the bilateral location of the disease, which makes it more valuable its publication for the knowledge of medical personnel dedicated to the care of these conditions.

Enfermedad de Paget extramamaria perianal, tratada con imiquimod tópico / Atypical presentation of perianal extramammary Paget's disease, treated with topical imiquimod

RESUMEN La enfermedad de Paget extramamaria (EPEM) perianal es un adenocarcinoma intraepitelial del margen anal de muy baja frecuencia. La forma primaria representa una neoplasia de origen apocrino, mientras que la forma secundaria representa una diseminación pagetoide de una neoplasia maligna en general anorectal. Debido a su similitud clínica con otras patologías de presentación frecuente, se requiere de un alto índice de sospecha para diagnosticarla. La resección quirúrgica amplia o la cirugía micrográfica de Mohs se considera comúnmente el pilar del tratamiento con altas tasas de recurrencia. Estudios previos han demostrado buena respuesta en la EPEM vulvar con imiquimod 5% en crema, pero su eficacia no ha sido bien descrita en la afectación perianal. En este artículo presentamos un caso de EPEM perianal primario con sobreinfección con HPV, al que se le indicó tratamiento tópico con imiquimod.

ABSTRACT Perianal extramammary Paget disease is a very low frequency intraepithelial adenocarcinoma of the anal margin. The primary form represents a neoplasm of apocrine origin, while the secondary form represents a pagetoid spread of a generally anorectal malignant neoplasm. Due to its clinical similarity with other pathologies with frequent presentation, a high index of suspicion is required to diagnose it. Wide surgical resection or Mohs micrographic surgery is commonly considered the mainstay of treatment with high recurrence rates. Previous studies have shown a good response in vulvar extramammary Paget disease with imiquimod 5% cream, but its efficacy has not been well described in perianal involvement. In this article we present a case of primary perianal extramammary Paget disease with HPV superinfection, which received topical treatment with imiquimod.

Misdiagnosis of Paget's Disease of Bone in a Congenital Generalized Lipodystrophy Patient: Case Report.

Paget's disease of bone (PDB) is a common skeleton disorder in which the diagnosis is suggested by radiological analyses. Congenital generalized lipodystrophy (CGL) is a rare, but a radiologic differential diagnosis of Paget's disease. Patients present total or almost total lack of subcutaneous adipose tissue, leptin deficiency, and precocious ectopic lipid accumulation, which lead to intense insulin resistance, poorly controlled diabetes mellitus, and hypertriglyceridemia. CGL subtypes 1 and 2 present sclerosis and osteolytic lesions that can resemble "pagetic" lesions. The clinical correlation is, therefore, essential. We report a CGL patient with bone lesions in which the radiographic findings led to a misdiagnosis of PDB. This case report brings awareness to CGL, a life-threating condition. Its early recognition is essential to avoid clinical complications and premature death. Therefore, it is important to consider CGL as PDB's differential diagnosis, especially in countries with high prevalence of this rare disease, such as Brazil.

Enfermedad de Paget mamaria: revisión de la literatura / Paget's disease of the breast: review of the literature

Resumen La enfermedad de Paget mamaria es una neoplasia infrecuente de la mama, muchas veces de difícil diagnóstico y mal pronóstico. El objetivo es revisar los aspectos fundamentales clínicos, diagnósticos, así como los avances más recientes en su tratamiento. Se realizó una búsqueda bibliográfica a través de PUBMED, GOOGLE SCHOLAR y UPTODATE, de los últimos 20 años. Fueron seleccionados e incluidos en la revisión 38 artículos. La enfermedad de Paget mamaria es una neoplasia maligna infrecuente del complejo areola pezón de características inflamatorias al examen físico, asociado en un 85% de los casos a un cáncer de mama subyacente, en el 50% de los casos no identificable al examen físico, de difícil diagnóstico, cuya sospecha clínica se confirma con imágenes, biopsia e inmunohistoquímica. El tratamiento es mastectomía versus cirugía conservadora y radioterapia. Un mal enfoque clínico, retrasa el diagnóstico y empeora la sobrevida. MÉD.UIS.2021;34(1): 45-53.

Abstract Paget's disease of the breast is an uncommon neoplasm of the breast, many times difficult to diagnose and of poor prognosis. The objective is to review the fundamental clinical and diagnostic aspects, as well as the most recent advances in its treatment. A bibliographic search of the last 20 years was carried out through PUBMED, GOOGLE SCHOLAR and UPTODATE. 38 articles were selected and included in the review. Mammary paget disease is a rare malignant neoplasm of the nipple areola complex with inflammatory features on physical examination, associated in 85% of cases with underlying breast cancer, in 50% of cases not recognizable on physical exam, difficult to diagnose, whose clinical suspicion is confirmed by imaging, biopsy and immunohistochemistry. Treatment is mastectomy versus conservative surgery and radiotherapy. A poor clinical approach delays the diagnosis and worsens survival rate. MÉD.UIS.2021;34(1): 45-53.

Resección de la primera costilla por videotoracoscopía en síndrome de Paget-Schroetter / Resection of the first rib by video thoracoscopy in Paget-Schroetter syndrome

Resumen El síndrome del opérculo torácico se refiere a una serie de signos y síntomas que se producen por la compresión del paquete vásculo-nervioso en la unión costo-clavicular. El síndrome de Paget-Schroetter (SPS) se define como la trombosis primaria, espontánea o de esfuerzo de la vena subclavia. Las vías de abordaje quirúrgicas tradicionales utilizadas para descomprimir el opérculo torácico son la trans axilar y las claviculares (supra e infra). El objetivo del estudio fue describir nuestra experiencia en la resección de la primera costilla por videotoracoscopía (VATS). Este es un estudio descriptivo observacional utilizando una base de datos prospectiva con análisis retrospectivo desde enero de 2017 a marzo de 2020. Se incluyeron 9 pacientes con diagnóstico de SPS en los que se resecó la primera costilla por VATS. En un paciente el procedimiento fue bilateral por presentar trombosis espontánea en ambas venas subclavias. De los 9, 6 eran mujeres. La edad media fue de 30.7 ± 10.7 años. La estadía hospitalaria media fue de 3.1 ± 0.5 días. Uno fue re-operado por hemotórax. No se detectaron recurrencias en el seguimiento a mediano-largo plazo. La resección de la primera costilla por VATS es un procedimiento seguro y factible. La misma, a diferencia de los abordajes tradicionales, puede ser resecada bajo visión directa de todos los elementos del opérculo torácico. Sin embargo, esta técnica requiere un manejo avanzado en cirugía toracoscópica.

Abstract Thoracic outlet syndrome (TOS) refers to a number of signs and symptoms that arise from compression of the neurovascular bundle at the costoclavicular junction. Paget-Schroetter syndrome is defined as the primary, spontaneous or effort thrombosis of the subclavian vein. The supraclavicular and trans-axillary approaches are currently the most commonly used for first rib resection. The aim of this article was to describe our experience in a minimally invasive approach (VATS) of first rib resection for primary venous thoracic outlet and the associated outcomes. This is a descriptive observational study using a retrospective analysis of a prospective database from January 2017 to March 2020. Nine patients underwent video thoracoscopic first rib resection due to PagetSchroetter syndrome (one bilateral procedure). Ten thoracoscopic first rib resections were performed. There were 6 female and 3 male patients, with a mean age of 30.7 ± 10.7 years. The mean length of hospital stay was 3.1 ± 0.5 days. No complications were recorded intraoperatively. One patient had to be re-operated because of hemothorax. There were no recurrences in a follow-up of at least 12 months. VATS resection of the first rib is a safe and feasible procedure and can be performed under direct vision of thoracic outlet elements. However, the technique requires experience with thoracoscopic surgery. The outcomes associated with our technique are comparable with the outcomes related to other current standards of care.

[Resection of the first rib by video thoracoscopy in Paget-Schroetter syndrome]. / Resección de la primera costilla por videotoracoscopía en síndrome de Paget-Schroetter.

Thoracic outlet syndrome (TOS) refers to a number of signs and symptoms that arise from compression of the neurovascular bundle at the costoclavicular junction. Paget-Schroetter syndrome is defined as the primary, spontaneous or effort thrombosis of the subclavian vein. The supraclavicular and trans-axillary approaches are currently the most commonly used for first rib resection. The aim of this article was to describe our experience in a minimally invasive approach (VATS) of first rib resection for primary venous thoracic outlet and the associated outcomes. This is a descriptive observational study using a retrospective analysis of a prospective database from January 2017 to March 2020. Nine patients underwent video thoracoscopic first rib resection due to Paget- Schroetter syndrome (one bilateral procedure). Ten thoracoscopic first rib resections were performed. There were 6 female and 3 male patients, with a mean age of 30.7 ± 10.7 years. The mean length of hospital stay was 3.1 ± 0.5 days. No complications were recorded intraoperatively. One patient had to be re-operated because of hemothorax. There were no recurrences in a follow-up of at least 12 months. VATS resection of the first rib is a safe and feasible procedure and can be performed under direct vision of thoracic outlet elements. However, the technique requires experience with thoracoscopic surgery. The outcomes associated with our technique are comparable with the outcomes related to other current standards of care.

El síndrome del opérculo torácico se refiere a una serie de signos y síntomas que se producen por la compresión del paquete vásculo-nervioso en la unión costo-clavicular. El síndrome de Paget- Schroetter (SPS) se define como la trombosis primaria, espontánea o de esfuerzo de la vena subclavia. Las vías de abordaje quirúrgicas tradicionales utilizadas para descomprimir el opérculo torácico son la trans axilar y las claviculares (supra e infra). El objetivo del estudio fue describir nuestra experiencia en la resección de la primera costilla por videotoracoscopía (VATS). Este es un estudio descriptivo observacional utilizando una base de datos prospectiva con análisis retrospectivo desde enero de 2017 a marzo de 2020. Se incluyeron 9 pacientes con diagnóstico de SPS en los que se resecó la primera costilla por VATS. En un paciente el procedimiento fue bilateral por presentar trombosis espontánea en ambas venas subclavias. De los 9, 6 eran mujeres. La edad media fue de 30.7 ± 10.7 años. La estadía hospitalaria media fue de 3.1 ± 0.5 días. Uno fue re-operado por hemotórax. No se detectaron recurrencias en el seguimiento a mediano-largo plazo. La resección de la primera costilla por VATS es un procedimiento seguro y factible. La misma, a diferencia de los abordajes tradicionales, puede ser resecada bajo visión directa de todos los elementos del opérculo torácico. Sin embargo, esta técnica requiere un manejo avanzado en cirugía toracoscópica.

Paget's disease of the jaws: Histopathological features of a series of 31 cases.

The aim of the present study was to analyze the histopathological features of Paget's disease of the jaws observed in a series comprising 31 cases. The study comprised all cases of Paget's disease of the jaws filed in the archives of the Surgical Pathology Laboratory of the Oral Pathology Department, School of Dentistry, University of Buenos Aires, between 1960 and 2018. Their microscopic features were evaluated, and available clinical data and radiographic studies were analyzed. Paget's disease of the jaws accounted for 0.05% of retrieved oral-maxillofacial pathologies. Microscopically, all cases showed lamellar bone trabeculae with the characteristic mosaic pattern. Twenty cases (64%) showed osteoblastic-osteoclastic activity, and all showed areas of necrosis. Cemento-osseous trabeculae were observed in 15 cases (48%), and cementicles were observed in 13 (42%). Osteomyelitis was seen in 11 cases (35%), all of which showed cemento-osseous trabeculae with a mosaic structure, sclerosis and necrosis, and chronic inflammation with abscess formation. Mean age was 61 years (44-85 years); 19 cases were women. Localization was the maxilla in 13 cases (42%), and the disease involved other skeletal bones in five cases. To our knowledge, this is the largest series of Paget's disease of the jaws reported to date. Paget's disease is infrequent in the jaws and has distinct histopathological features that not only differ from those observed at other skeletal sites but also require differential diagnosis from other pathologies affecting the jaws exclusively.

El objetivo del presente trabajo fue analizar las características histopatológicas de la enfermedad de Paget en los maxilares en una serie de 31 casos. El estudio comprendió todos los casos de enfermedad de Paget de los maxilares provenientes del Laboratorio de Patología Quirúrgica de la Cátedra de Anatomía Patológica de la Facultad de Odontología de la Universidad de Buenos Aires, entre 1960 y 2018. Se evaluaron las características microscópicas y se analizaron los datos clínicos y estudios radiográficos disponibles. La enfermedad de Paget en los maxilares representó el 0,05% de las patologías buco-maxilofaciales. Microscópicamente, todos los casos mostraron trabéculas óseas laminares con el característico patrón en mosaico. Veinte casos (64%) mostraron actividad osteoblástica-clástica y todos los casos mostraron necrosis focal. En 15 casos (48%) las trabéculas presentaron aspecto cemento-óseo y en 13 casos (42%) se observaron cementículos. Once casos (35%) presentaron cuadros osteomielíticos y todos ellos mostraron trabéculas cemento-óseas con estructura en mosaico, esclerosis y necrosis, e infiltrado inflamatorio crónico con formación de abscesos. La media de edad fue 61 años (44-85 años), y 19 fueron mujeres. Trece casos (42%) se localizaron en maxilar superior y 5 casos presentaron compromiso de otros huesos. A nuestro entender, esta es la serie más grande de enfermedad de Paget en los maxilares reportada hasta la fecha. La enfermedad de Paget es poco frecuente en los maxilares y presenta características histopatológicas que además de ser diferentes a las observadas en otros sitios del esqueleto plantean el diagnóstico diferencial con otras entidades que se presentan exclusivamente en los maxilares.

Paget's disease of the jaws: Histopathological features of a series of 31 cases / Enfermedad de Paget en los maxilares. Características histopatológicas de una serie de 31 casos

ABSTRACT The aim of the present study was to analyze the histopathological features of Paget's disease of the jaws observed in a series comprising 31 cases. The study comprised all cases of Paget's disease of the jaws filed in the archives of the Surgical Pathology Laboratory of the Oral Pathology Department, School of Dentistry, University of Buenos Aires, between 1960 and 2018. Their microscopic features were evaluated, and available clinical data and radiographic studies were analyzed. Paget's disease of the jaws accounted for 0.05% of retrieved oral-maxillofacial pathologies. Microscopically, all cases showed lamellar bone trabeculae with the characteristic mosaic pattern. Twenty cases (64%) showed osteoblastic-osteoclastic activity, and all showed areas of necrosis. Cemento-osseous trabeculae were observed in 15 cases (48%), and cementicles were observed in 13 (42%). Osteomyelitis was seen in 11 cases (35%), all of which showed cemento-osseous trabeculae with a mosaic structure, sclerosis and necrosis, and chronic inflammation with abscess formation. Mean age was 61 years (44-85 years); 19 cases were women. Localization was the maxilla in 13 cases (42%), and the disease involved other skeletal bones in five cases. To our knowledge, this is the largest series of Paget's disease of the jaws reported to date. Paget's disease is infrequent in the jaws and has distinct histopathological features that not only differ from those observed at other skeletal sites but also require differential diagnosis from other pathologies affecting the jaws exclusively.

RESUMEN El objetivo del presente trabajo fue analizar las características histopatológicas de la enfermedad de Paget en los maxilares en una serie de 31 casos. El estudio comprendió todos los casos de enfermedad de Paget de los maxilares provenientes del Laboratorio de Patología Quirúrgica de la Cátedra de Anatomía Patológica de la Facultad de Odontología de la Universidad de Buenos Aires, entre 1960 y 2018. Se evaluaron las características microscópicas y se analizaron los datos clínicos y estudios radiográficos disponibles. La enfermedad de Paget en los maxilares representó el 0,05% de las patologías buco-maxilofaciales. Microscópicamente, todos los casos mostraron trabéculas óseas laminares con el característico patrón en mosaico. Veinte casos (64%) mostraron actividad osteoblástica-clástica y todos los casos mostraron necrosis focal. En 15 casos (48%) las trabéculas presentaron aspecto cemento-óseo y en 13 casos (42%) se observaron cementículos. Once casos (35%) presentaron cuadros osteomielíticos y todos ellos mostraron trabéculas cemento-óseas con estructura en mosaico, esclerosis y necrosis, e infiltrado inflamatorio crónico con formación de abscesos. La media de edad fue 61 años (44- 85 años), y 19 fueron mujeres. Trece casos (42%) se localizaron en maxilar superior y 5 casos presentaron compromiso de otros huesos. A nuestro entender, esta es la serie más grande de enfermedad de Paget en los maxilares reportada hasta la fecha. La enfermedad de Paget es poco frecuente en los maxilares y presenta características histopatológicas que además de ser diferentes a las observadas en otros sitios del esqueleto plantean el diagnóstico diferencial con otras entidades que se presentan exclusivamente en los maxilares.

Case for diagnosis. Phagedenic ulcer on the thorax

Abstract Paget's disease is a rare disorder of the nipple and/or the areola that is characterized by an erythematosquamous lesion and is often associated with in situ or invasive breast carcinoma. The authors present an atypical, exuberant case that had evolved over eight years, emphasizing the importance of early diagnosis.

Case for diagnosis. Phagedenic ulcer on the thorax.

Paget's disease is a rare disorder of the nipple and/or the areola that is characterized by an erythematosquamous lesion and is often associated with in situ or invasive breast carcinoma. The authors present an atypical, exuberant case that had evolved over eight years, emphasizing the importance of early diagnosis.

Enfermedad de Paget de hueso esporádica. Serie de casos y revisión de la literatura / Sporadic Paget's disease of the bone. Case series and literature review

RESUMEN La enfermedad de Paget ósea es una enfermedad metabólica del hueso de etiología no esclarecida, que se caracteriza por una fase de resorción aumentada seguida por una fase de osteoformación aberrante. Es frecuente en Europa, Norteamérica, Nueva Zelanda y Australia, pero infrecuente en Asia, Medio Oriente y África. En población colombiana hay reportes de casos. Generalmente cursa asintomática y se diagnostica incidentalmente por hallazgos radiográficos o fosfatasa alcalina elevada. El uso de bifosfonatos favorece el control del recambio óseo y permite prevenir complicaciones como las fracturas. Se presenta una serie de casos en Colombia y una revisión de la literatura.

A B S T R A C T Paget's disease of the bone is a metabolic bone disease of unknown origin, and is characterised by an increased phase of resorption, followed by an aberrant osteoformation phase. It is common in Europe, North America, New Zealand, and Australia, but infrequent in Asia, the Middle East, Africa, and in the Colombian population there are case reports. It is usually asymptomatic and is diagnosed incidentally by radiographic findings or an elevated alkaline phosphatase. The use of bisphosphonates favours the control of bone turnover and prevents complications such as fractures. A series of cases in Colombia is presented, along with a review of the literature.