Neumotórax espontáneo persistente como manifestación primaria de sarcoma sinovial pleuropulmonar primario: a propósito de un caso / Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

El sarcoma sinovial pleuropulmonar (SSPP) es un tumor primario de pulmón, maligno, infrecuente en pediatría (prevalencia 0,1-0,5 %) que afecta predominantemente a adolescentes y adultos jóvenes. Se ha descrito una sobrevida global cercana al 30 % a los 5 años. Se reporta el caso de un paciente de 12 años de edad, previamente sano, que presentó tos, dolor torácico y disnea de comienzo súbito, como manifestación inicial de neumotórax izquierdo, el que persistió a los 4 días y requirió resección quirúrgica de lesión bullosa pulmonar. Se realizó diagnóstico histológico de sarcoma sinovial pleuropulmonar confirmado por estudio molecular, que evidenció la translocación cromosómica entre el cromosoma X y el 18: t(X;18) (p11.2;q11.2) de la pieza quirúrgica extirpada. Ante pacientes con neumotórax persistente o recidivante, es importante descartar causas secundarias, entre ellas, sarcoma sinovial pleuropulmonar. Su ominoso pronóstico determina la necesidad de arribar a un diagnóstico temprano e implementar un tratamiento agresivo

Pleuropulmonary synovial sarcoma (PPSS) is a primary malignancy of the lung, uncommon in pediatrics (prevalence: 0.1­0.5%) that predominantly affects adolescents and young adults. Overall survival has been reported to be close to 30% at 5 years. Here we report the case of a previously healthy 12-year-old male patient who presented with cough, chest pain, and dyspnea of sudden onset as initial manifestation of left pneumothorax, which persisted after 4 days and required surgical resection of pulmonary bullous lesion. A histological diagnosis of pleuropulmonary synovial sarcoma was made and confirmed by molecular study, which showed chromosomal translocation between chromosomes X and 18: t(X;18) (p11.2;q11.2) in the surgical specimen removed. In patients with persistent or recurrent pneumothorax, it is important to rule out secondary causes, including pleuropulmonary synovial sarcoma. Such poor prognosis determines the need for early diagnosis and aggressive treatment.

Preliminary Recommendations on the Timing of Lung Surgery after Novel Coronavirus Infection in Patients with Pulmonary Nodules and Lung Cancer / 中国肺癌杂志

In recent years, the corona virus disease 2019 (COVID-19) pandemic has had a huge impact on the global medical, political and economic fields. Since the beginning of the COVID-19 epidemic, our understanding of the impact of COVID-19 has grown exponentially. Recently, the COVID-19 epidemic has changed rapidly in China, and there has been controversy over how to carry out surgical operations for patients with lung neoplastic lesions. Some studies have shown that lung cancer patients undergoing surgery are more likely to experience respiratory failure and perioperative death after contracting COVID-19 than the general population, however, delays in cancer treatment are also associated with increased mortality among these patients. In particular, the novel coronavirus Omikron variant has a higher transmissibility and may escape the immunity obtained through the previous novel coronavirus infection and vaccination. In order to minimize the risk of novel coronavirus infection in surgical patients, it is necessary to develop new treatment guidelines, expert consensus and preventive measures. However, the current rapid change of the epidemic situation has led to insufficient time and evidence to develop guidelines and consensus. Therefore, thoracic surgeons need to evaluate specific patient populations at higher risk of severe complications before surgery and weigh the benefit of surgical treatment against the risk of novel coronavirus infection. We try to give some recommendations on lung surgery during the current domestic epidemic situation based on the guidelines and consensus of oncology and thoracic surgery organizations in different regions on lung surgery.
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Effects of radiation therapy on tumor microenvironment: an updated review / 中华医学杂志(英文版)

Cancer is a major threat to human health and causes death worldwide. Research on the role of radiotherapy (RT) in the treatment of cancer is progressing; however, RT not only causes fatal DNA damage to tumor cells, but also affects the interactions between tumor cells and different components of the tumor microenvironment (TME), including immune cells, fibroblasts, macrophages, extracellular matrix, and some soluble products. Some cancer cells can survive radiation and have shown strong resistance to radiation through interaction with the TME. Currently, the complex relationships between the tumor cells and cellular components that play major roles in various TMEs are poorly understood. This review explores the relationship between RT and cell-cell communication in the TME from the perspective of immunity and hypoxia and aims to identify new RT biomarkers and treatment methods in lung cancer to improve the current status of unstable RT effect and provide a theoretical basis for further lung cancer RT sensitization research in the future.

Perianal Paget 's disease complicated with lung adenocarcinoma and anal canal carcinoma: A case report and literature review / 中南大学学报(医学版)

Perianal Paget's disease (PPD) is a rare malignant cutaneous tumor. This paper reported a case of PPD complicated by lung adenocarcinoma and anal canal cancer. The patient, a 76-year-old female, had been experiencing recurrent lower abdominal pain and perianal pruritus for the past 5 years. Upon physical examination, a cauliflower-like neoplasm in size of 5 cm×6 cm was observed on the right perianal skin, with local skin ulceration and a small amount of fluid discharge. The left perianal skin was also involved. In thoracoknee position, a hard mass was palpable in the rectal submucosa at 5-6 points 2 cm from the anal verge. Chest CT revealed multiple lesions in both lungs, indication of metastatic tumors. Further evaluation with fluorodeoxyglucose positron emission tomography and computed tomography (FDG-PET/CT) indicated multiple hypermetabolic nodules in the lungs, hypermetabolic lymph nodes throughout the body, early FDG uptake in a small patch of skin on the left hip, and increased FDG uptake in the anorectal region. Histopathological examination confirmed the diagnosis of lung adenocarcinoma. This resulted in the patient being diagnosed with PPD, lung adenocarcinoma, anal canal cancer, and systemic multiple lymph node metastasis. The combination of PPD with gastrointestinal tumors and other metachronous malignant tumors is highly prevalent. Colonoscopy, FDG-PET/CT, histopathology, and immunohistochemistry play crucial roles in early identification of local lymph node and distant involvement, facilitating the evaluation of potential malignant tumors and differential diagnosis. Treating methods for PPD are currently diverse, including postoperative combined or single chemotherapy, radiotherapy, targeted therapy, and photodynamic therapy. As trerapeutical options continue to develop, the extent and efficacy of surgery need to be reassessed.

Cáncer de pulmón, ¿cuál es la mejor estrategia para cambiar los desenlaces? / Lung cancer: ¿what is the best strategy to change outcomes?

En el día a día de la atención en salud vemos cómo el cáncer de pulmón sigue siendo una de las patologías oncológicas con peor pronóstico: su tasa global de sobrevida de solo el 20,0 %. Esto se debe, en parte, a que en la mayoría de los casos su diagnóstico se hace en estadios avanzados, cuando ya no hay muchas opciones de tratamiento, más allá de medidas paliativas. A diferencia de otros tipos de cáncer ­como el de cuello uterino, el de mama o el de próstata­, en los cuales existen estrategias de tamizaje y prevención asequibles y que favorecen un mejor pronóstico, en el de pulmón no ha sido posible implementar a gran escala tales intervenciones, lo cual ha favorecido el pobre pronóstico de la enfermedad y ha contribuido a que siga siendo la principal causa de muerte por cáncer en el mundo (1).

In the day-to-day health care we see how lung cancer continues to be one of the oncological pathologies with the worst prognosis: its overall survival rate of only 20.0%. This is due, in part, to the fact that in most cases its diagnosis is made in advanced stages, when there are no longer many treatment options, beyond palliative measures. Unlike other types of cancer ­such as cervical, breast or prostate cancer­, in which there are affordable screening and prevention strategies that favor a better prognosis, in lung cancer it has not been possible to implement such interventions on a large scale, which has favored the poor prognosis of the disease and has contributed to its continuing to be the leading cause of death from cancer in the world (1).

Pneumocystis jirovecii Pneumonia in Patients with Lung Cancer: A Review / 中国肺癌杂志

In recent years, with the widespread use of immunodepressant agents, Pneumocystis jirovecii pneumonia (PJP) has been significantly found in non-human immunodeficiency virus (HIV) patients, such as those with malignancies, post-transplantation and autoimmune diseases. Although the risk factors and management of PJP have been extensively studied in the hematologic tumor and post-transplant populations, the research on real tumor cases is insufficient. Lung cancer has been the most common tumor with the highest number of incidence and death worldwide, and the prognosis of lung cancer patients infected with PJP is poor in clinical practice. By reviewing the previous studies, this paper summarized the epidemiology and clinical manifestations of PJP in lung cancer patients, the risk factors and possible mechanisms of PJP infection in lung cancer patients, diagnosis and prevention, and other research progresses to provide reference for clinical application.
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Construction and validation of a nomogram for predicting the risk of secondary peripheral neuropathy in patients with advanced lung cancer / 浙江大学学报·医学版

OBJECTIVE@#To construct and validate a nomogram for predicting the risk of secondary peripheral neuropathy in patients with advanced lung cancer.@*METHODS@#The sociodemographic and clinical data of 335 patients with advanced lung cancer admitted to Department of Respiratory, the First Affiliated Hospital of Zhejiang University School of Medicine from May 2020 to May 2021 were retrospectively collected. Pearson correlation analysis, univariate and multivariate logistic regression analyses were used to identify the risk factors of secondary peripheral neuropathy in patients with advanced lung cancer. A nomogram was constructed according to the contribution of each risk factor to secondary peripheral neuropathy, and the receiver operating characteristic (ROC) curve, Calibration curve and clinical decision curve were used to evaluate differentiation, calibration, and the clinical utility of the model. The nomogram was further validated with data from 64 patients with advanced lung cancer admitted between June 2021 and August 2021.@*RESULTS@#The incidences of secondary peripheral neuropathy in two series of patients were 34.93% (117/335) and 40.63% (26/64), respectively. The results showed that drinking history ( OR=3.650, 95% CI: 1.523-8.746), comorbid diabetes ( OR=3.753, 95% CI: 1.396-10.086), chemotherapy ( OR=2.887, 95% CI: 1.046-7.970), targeted therapy ( OR=8.671, 95% CI: 4.107-18.306), immunotherapy ( OR=2.603, 95% CI: 1.337-5.065) and abnormal liver and kidney function ( OR=12.409, 95% CI: 4.739-32.489) were independent risk factors for secondary peripheral neuropathy (all P<0.05). A nomogram was constructed based on the above risk factors. The area under the ROC curve (AUC) of the nomogram for predicting the secondary peripheral neuropathy was 0.913 (95% CI: 0.882-0.944); and sensitivity, specificity, positive and negative predictive values were 85.47%, 81.65%, 71.43% and 91.28%, respectively. The Calibration curve and clinical decision curve showed good calibration and clinical utility. External validation results showed that the AUC was 0.764 (95% CI: 0.638-0.869); and sensitivity, specificity, positive and negative predictive values were 79.28%, 85.79%, 73.25% and 85.82%, respectively.@*CONCLUSIONS@#Advanced lung cancer patients have a high risk of secondary peripheral neuropathy after anticancer therapy. Drinking history, comorbid diabetes, chemotherapy, targeted therapy, immunotherapy, abnormal liver and kidney function are independent risk factors. The nomogram prediction model constructed in the study is effective and may be used for the risk assessment of secondary peripheral neuropathy in patients with advanced lung cancer.

Consideraciones radiológicas del cáncer de pulmón y Covid-19: una mirada oncológica / Radiological considerations of lung cancer and Covid-19: a oncology look

Patients with SARS-Cov-2 infection and lung cancer have an unfavorable prognosis, characterized by higher rates of respiratory failure, use of invasive mechanical ventilation and higher mortality rates. Due to similarities in affected organs in Covid-19 and lung cancer, the radiological accurate diagnosis has become a challenge for physicians. Radiologic findings of lung cancer, such as parenchymal consolidation, spiculation and microlobulations are not specific. Imaging findings of Covid-19 in patients with lung cancer consist of multiple patchy multifocal bilateral ground-glass opacities and consolidations, being hardly distinguishable from an underlying lung malignancy. Differential radiological diagnosis in patients with lung cancer and Covid-19 must include pneumonitis and lung toxicity caused by chemotherapy, target therapies and radiotherapy. Follow-up and simulation tomography in radiotherapy have become an unexpected ally in the early detection of Covid-19 in asymptomatic stages in lung cancer patients. Patients with lung cancer should have particular considerations due to their high risk and the adverse effects of systemic therapies and radiotherapy. (AU)

Control Exitoso del dolor oncológico crónico por cáncer de pulmón con el bloqueo del plano del erector espinal (BPEE) en un servicio de neumología. Reporte de caso / Successful control of chronic cancer pain from lung cancer with erector spinae plane (ESP) block in a pulmonology department. Case report

Cáncer pain is defined as an unpleasant sensory and emotional experience. 90% of patients with terminal cáncer present pain. An alternative for the management of pain located in the thorax is erector spinae plane (ESP) block guided by real-time ultra- sound. However, its use in the management of oncological chest pain is not very common, with few reports in this regard, with most of these reports always performed by anesthesiologists. We describe our first experience performing a real-time guided ESP block using ultrasound performed by a trained pulmonologist in a 64 years-old male with lung adenocarcinoma stage IVa with sternum metastases and multiple left costal arches associated with left pleural effusion due to pleural metastases. We found ESP block to be safe for the patient as well as effective in reducing pain measured by Visual Analogic Score and was able to reduce the use of systemic analgesics.

El dolor es una experiencia sensorial y emocional desagradable. El 90% de los pacientes con cáncer terminal presentan dolor y una alternativa para el tratamiento del dolor localizado en el tórax es el bloqueo del plano del erector espinal de la columna (BPEE) guiado por ultrasonido en tiempo real. Sin embargo, su uso en el tratamiento del dolor oncológico no es común y hay pocos informes al respecto, la mayoría realizados por anestesiólogos. Describimos nuestra primera experiencia realizando un BPEE guiado en tiempo real con ultrasonido y realizado por un neumólogo en un varón de 64 años con adenocarcinoma de pulmón en estadio IVa, con metástasis en el esternón y múltiples arcos costales izquierdos y derrame pleural izquierdo debido a metástasis pleural. Descubrimos que el bloqueo BPEE fue seguro y efectivo para reducir el dolor medido por la puntuación de Escala Visual Analógica y fue capaz de reducir el uso de analgésicos sistémicos.

Revisión bibliográfica sobre la COVID 19 en pacientes con cáncer de pulmón / Bibliographic review on COVID 19 in patients with lung cancer

RESUMEN La COVID 19 es una enfermedad pandémica producida por el virus SARS-CoV-2, tiene dentro de los grupos vulnerables al cáncer de pulmón por presentar una inmunodepresión adquirida por los tratamientos oncoespecíficos administrados y esto conlleva a una mayor exposición a complicaciones si se contrae esta terrible infección que azota al mundo en la actualidad. El objetivo fue exponer los riesgos y complicaciones que tienen los pacientes con cáncer de pulmón que reciben tratamientos oncoespecíficos si se infectan con el SARS-COV-2. Se realizó una revisión sistemática de los principales artículos publicados en inglés y en español por autores cubanos y extranjeros en revistas de alto impacto a nivel mundial, información reportada por la Organización Mundial de la Salud, la red de Infomed y el Ministerio de Salud Pública de Cuba. Se concluyó que los pacientes con cáncer de pulmón no presentan un riesgo superior a la población general para contraer la COVID 19, sí existe cierta evidencia de que estos pacientes puedan sufrir una infección más grave si la adquieren (AU).

SUMMARY COVID-19 is a pandemic disease produced by SARS-CoV-2 virus; the group of patients with lung cancer is vulnerable to this disease because of presenting an acquired immune depression due to administered oncospecific treatments, leading to higher exposition to complications if the patient gets this terrible disease striking worldwide nowadays. The objective of this review was exposing the risk and complications affronted by patients suffering lung cancer with oncospecific treatment if they get infected by SARS-CoV-2. The authors carried out a systematic review of the main articles published in Spanish and English by Cuban and foreign authors in high impact journals around the world, information reported by the World Health Organization, INFOMED and the Ministry of Public Health of Cuba. It was concluded that patients with lung cancer are not at a higher risk of catching COVID-19 than general population; it does exist certain evidence of that these patients could suffer a more serious infection if they get the disease (AU).

Remitting Seronegative Symmetrical Synovitis with Pitting Edema associated with Lung Malignancy:Case Report and Literature Review / 中国医学科学院学报

Remitting seronegative symmetrical synovitis with pitting edema(RS3PE),the inflammatory arthritis attacking mainly elderly males,is characterized by symmetrical synovitis with pitting edema of the dorsum of hands and feet and the absence of rheumatoid factor.RS3PE commonly accompanies malignant tumor,infections and other diseases.Here we report a case of RS3PE associated with lung malignancy and review other six cases to summarize the clinical features,treatment and prognosis.

Simultaneous thoracoscopic approach in a patient with long-standing persistent atrial fibrillation and primary lung cancer: the first described case

Abstract Cardiac rhythm disorders are common in many patients with cancer. The management of synchronous long-standing persistent atrial fibrillation and pulmonary lesions remains a serious surgical dilemma due to the lack of clinical data and surgical guidelines. To the best of our knowledge, this is the first described case of simultaneous thoracoscopic pulmonary segmentectomy and left atrial posterior wall and pulmonary vein isolation combined with left atrial appendage resection in a patient with early-stage primary lung cancer and long-standing persistent atrial fibrillation.

Erythroderma as first manifestation of squamous cell lung cancer: rare case report

Abstract Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.

Lung cancer and the COVID-19 pandemic: recommendations from the brazilian thoracic oncology group

New cases of the novel coronavirus disease 2019 (COVID-19), also known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), continue to rise worldwide following the declaration of a pandemic by the World Health Organization (WHO). The current pandemic has completely altered the workflow of health services worldwide. However, even during this critical period, patients with other diseases, like cancer, need to be properly treated. A few reports have shown that mortality due to SARS-CoV-2 is higher in elderly patients and those with other active comorbidities, including cancer. Patients with lung cancer are at risk of pulmonary complications from COVID-19, and as such, the risk/benefit ratio of local and systemic anticancer treatment has to be considered. For each patient, several factors, including age, comorbidities, and immunosuppression, as well as the number of hospital visits for treatment, can influence this risk. The number of cases is rising exponentially in Brazil, and it is important to consider the local characteristics when approaching the pandemic. In this regard, the Brazilian Thoracic Oncology Group has developed recommendations to guide decisions in lung cancer treatment during the SARS-CoV-2 pandemic. Due to the scarcity of relevant data, discussions based on disease stage, evaluation of surgical treatment, radiotherapy techniques, systemic therapy, follow-up, and supportive care were carried out, and specific suggestions issued. All recommendations seek to reduce contagion risk by decreasing the number of medical visits and hospitalization, and in the case of immunosuppression, by adapting treatment schemes when possible. This statement should be adjusted according to the reality of each service, and can be revised as new data become available.

Hipertiroidismo como clave diagnóstica de un coriocarcinoma avanzado: Una asociación inusual / Hyperthyroidism as a diagnostic key to advanced choriocarcinoma. An unusual association

El hipertiroidismo es una condición relativamente frecuente con múltiples etiologías. La más común es la enfermedad de Graves, seguida del bocio multinodular y el adenoma tóxico. La asociación entre hipertiroidismo y cáncer es infrecuente en la práctica clínica. Presentamos el caso de un varón de 42 años con síntomas de hipertiroidismo de dos meses de evolución. Al examen físico se constató una marcada hepatomegalia de consistencia duro pétrea. El examen de testículos se reveló normal. Se llevó a cabo el diagnóstico de hipertiroidismo a través del dosaje hormonal. Los estudios por imágenes mostraron la presencia de múltiples lesiones sólidas compatibles con metástasis hepáticas. Luego de descartar las causas habituales de hipertiroidismo y las neoplasias primarias de la glándula tiroides, se consideró la posibilidad de mimetismo molecular a través de la producción ectópica de gonadotrofina coriónica humana. Se obtuvieron valores críticamente elevados de esta hormona y en un segundo tiempo se confirmó el diagnóstico histológico de coriocarcinoma a través de una biopsia hepática. Consideramos que el reconocimiento de este mecanismo poco frecuente de hipertiroidismo, puede ser una clave diagnóstica para arribar rápidamente al diagnóstico correcto, particularmente en los tumores extragonadales.

Hyperthyroidism is a relatively frequent condition with multiple causes. The most common cause is Graves' disease; followed by hyperthyroid multinodular goiter and toxic adenoma. Association between hyperthyroidism and cancer is infrequent in daily practice. We present the case of a 42-year-old man who developed severe symptoms of hyperthyroidism within a period of two months. Physical examination revealed significant hepatomegaly. Testicular examination proved normal. Imaging studies showed the presence of multiple hepatic solid lesions consistent with metastases. After discarding the most common causes of hyperthyroidism and primary thyroid gland neoplasm, the possibility of molecular mimicry was considered through human chorionic gonadotrophin production. Critical high values of this hormone were found and choriocarcinoma histological diagnosis was confirmed through a liver biopsy. We consider that the recognition of this rare mechanism of hyperthyroidism may be a clue permitting a faster diagnosis, particularly when extragonadal tumors are present.

Primary pulmonary undifferentiated pleomorphic sarcoma (PPUPS)

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade pleomorphic neoplasm with no identifiable line(s) of differentiation using currently available diagnostic techniques. Therefore, it is essentially a diagnosis of exclusion, which requires generous tissue sampling, adequate contextually interpreted immunohistochemistry, and relevant molecular studies. UPS is a common soft tissue sarcoma (historically one of the entities referred to as malignant fibrous histiocytoma (MFH)), which can develop in various organs, but lung involvement is usually due to metastasis. Primary Pulmonary UPS (PPUPS) is exceptionally rare and here we present a 66-year-old man who presented with anemia and weight loss, found to have a 17 cm right lung mass with invasion to the chest wall and diaphragm. Extensive sampling and immunohistochemistry studies failed to reveal any line of differentiation. Upon exclusion of a possible extrapulmonary origin, a diagnosis of PPUPS was rendered. In addition, we reviewed all 84 previously reported cases of PPUPS/PPMFH in the literature since 1979 and summarized the clinical information.

Tuberculosis and lung cancer / Tuberculosis y cáncer de pulmón

Abstract: Objective: To describe the epidemiological studies about the relationship between lung cancer (LC) and pulmonary tuberculosis (Tb) and its possible molecular mechanisms. Materials and methods: We reviewed research databases in search of publications that included keywords LC and Tb. Results: It has been proposed that chronic inflammation in the lungs due to Tb could cause clastogenic activity in the DNA of bronchial epithelium. Another possibility is lateral gene transfer; since Mycobacterium tuberculosis (MTb) is an intracellular organism, bacterial DNA could integrate to bronchial epithelial cells inducing neoplastic transformation. Conclusions: There are epidemiological reports, particularly from Asian countries, which confirm a relationship between LC and Tb. MTb could play an active role in cellular transformation and it is important to elucidate the mechanism involved.

Resumen: Objetivo: Describir los estudios que documentan la relación entre el cáncer de pulmón (CP) y la tuberculosis pulmonar (Tb) y sus posibles mecanismos moleculares. Material y métodos: Se revisaron bases de datos de publicaciones, usando como palabras clave CP y Tb. Resultados: Se ha propuesto que la inflamación crónica en el pulmón provocada por la Tb podría producir actividad clastogénica. Otra posibilidad es la transferencia lateral de genes; Mycobacterium tuberculosis (MTb) es un organismo intracelular facultativo cuyo DNA podría integrarse al material genético del epitelio bronquial induciendo transformación neoplásica. Conclusión: Existen evidencias epidemiológicas, particularmente en países asiáticos, que documentan la relación entre CP y Tb. MTb podría desempeñar un papel activo en la transformación neoplásica cuyo mecanismo debe de ser elucidado.

The 4Ds of ectopic ACTH syndrome: diagnostic dilemmas of a difficult disease

SUMMARY Cushing's syndrome (CS) is an uncommon condition that leads to high morbidity and mortality. The majority of endogenous CS is caused by excessive ACTH secretion, mainly due to a pituitary tumor - the so-called Cushing's disease (CD) - followed by ectopic ACTH syndrome (EAS), an extra-pituitary tumor that produces ACTH; adrenal causes of CS are even rarer. Several methods are used to differentiate the two main etiologies: specific laboratory tests and imaging procedures, and bilateral inferior petrosal sinus sampling (BIPSS) for ACTH determination; however, identification of the source of ACTH overproduction is often a challenge. We report the case of a 28-year-old woman with clinical and laboratory findings consistent with ACTH-dependent CS. All tests were mostly definite, but several confounding factors provoked an extended delay in identifying the origin of ACTH secretion, prompting a worsening of her clinical condition, with difficulty controlling hyperglycemia, hypokalemia, and hypertension. During this period, clinical treatment was decisive, and measurement of morning salivary cortisol was a differential for monitoring cortisol levels. This report shows that clinical reasoning, experience and use of recent methods of nuclear medicine were decisive for the elucidation of the case.

Linfangioleiomiomatosis pulmonar, una rara enfermedad pulmonar: presentación de un caso clínico / Pulmonary lymphangioleiomyomatosis, a rare lung disease: presentation of a clinical case

Introducción: La linfangioleiomiomatosis Pulmonar (LAM) es una rara y progresiva enfermedad; caracterizada por proliferación excesiva de células musculares lisas a partir de vasos linfáticos, sanguíneos y vías aéreas. En conjunto al anormal crecimiento celular descrito, se aprecia degeneración quística difusa del parénquima pulmonar, lo que puede reflejarse desde cuadros completamente asintomáticos hasta el deterioro severo del intercambio gaseoso con insuficiencia respiratoria fulminante. Descripción del caso: Paciente femenino de 41 años de edad, con cuadro clínico consistente en tos seca ocasional, asociada a dolor leve de características pleuríticas en 'puntada de costado ' derecha. Ante la no mejoría clínica, se indica estudio imagenológico donde se demuestra neumotorax espontáneo derecho. En estudio tomográfico se aprecian además lesiones pulmonares quísticas. El estudio anátomo-patológico demuestra cambios estructurales que se reportan compatibles con LAM. Conclusión: Dada la simplicidad de los síntomas con que la LAM puede debutar, su confirmación diagnóstica se genera en fases avanzadas de la enfermedad, cuando el daño pulmonar importante conlleva a la aparición de factores clínicos con mayor repercusión sobre el estado general de los pacientes por lo que la realización de estudios imagenológicos tempranos gana vital importancia.

Introduction: Pulmonary lymphangioleiomyomatosis (LAM) is a rare and progressive disease; characterized by airway, lymphatic and blood vessels-smooth muscle cells excessive proliferation. Added to the abnormal cell growth, parenchymal cystic degeneration is present, which can be reflected initially as a asymptomatic course and can progress to severe gaseous exchange deterioration and fulminating respiratory insufficiency. Case description: A 41-year-old female patient with a clinical course consisting of occasional dry cough, associated with mild pleuritic pain on the right side of thorax. As no improvement was achieved, thoracic imaging study was performed, where a right pneumothorax was found. Tomography images showed multiple lung cystic lesions. Anatomopathological study reports structural changes compatible with LAM. Conclusion: Given the simplicity of the symptoms that LAM can debut with, its diagnostic confirmation is generated in advanced stages of the disease, when the important pulmonary damage leads to the appearance of clinical factors with greater impact on the general state of patients so early thoracic imaging studies gain vital importance.