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Hormones (Athens, Greece) Mar 2022In this article, we present a case of neuroendocrine neoplasm of unknown primary origin (UPO NEN), which is a rare cause of ectopic Cushing's syndrome (ECS) presenting... (Review)
Review
BACKGROUND
In this article, we present a case of neuroendocrine neoplasm of unknown primary origin (UPO NEN), which is a rare cause of ectopic Cushing's syndrome (ECS) presenting numerous challenges, together with a literature review.
CASE REPORT
A 43-year-old male patient presented with clinical features consistent with Cushing's syndrome (CS) and adrenocorticotropic hormone (ACTH)-dependent hypercortisolemia. Despite a suspicious lesion on pituitary MRI, the high-dose dexamethasone suppression test and bilateral inferior petrosal sinus sampling results were not compatible with Cushing's disease. Bilateral non-homogeneous opacities were observed in the thorax CT of the patient, who also had a history of COVID-19 infection, but no tumoral lesion was detected. When Ga-SSTR PET/CT and FDG-PET/CT were performed, multiple metastatic foci were detected in mediastinal and hilar lymph nodes and the axial skeleton. Paratracheal-subcarinal lymph nodes were excised mediastinoscopically, and the diagnosis of NEN was made. Histopathological findings indicated that the possible origin was an atypical pulmonary carcinoid with a low Ki-67 labeling index. After controlling hypercortisolemia, a regimen of somatostatin analogs and capecitabine plus temozolomide was decided upon as treatment by a multidisciplinary council.
CONCLUSION
This is a challenging case of UPO NEN presenting with ECS and confounding factors, such as previous infection and incidental lesions, during the diagnosis process. The case in question highlighted the fact that atypical pulmonary carcinoid with a low proliferation index may cause visible metastases even when radiologically undetectable.
Topics: ACTH Syndrome, Ectopic; Adrenocorticotropic Hormone; Adult; COVID-19; Carcinoid Tumor; Cushing Syndrome; Humans; Lung Neoplasms; Male; Neoplasms, Unknown Primary; Neuroendocrine Tumors; Positron Emission Tomography Computed Tomography
PubMed: 34478059
DOI: 10.1007/s42000-021-00316-z -
BMC Endocrine Disorders May 2021Manifestations of hypokalaemia in ectopic adrenocorticotropic hormonesyndrome(EAS) vary from mild muscle weakness to life-threatening arrhythmia. Herein, we present a...
BACKGROUND
Manifestations of hypokalaemia in ectopic adrenocorticotropic hormonesyndrome(EAS) vary from mild muscle weakness to life-threatening arrhythmia. Herein, we present a rare case of EAS with concomitant rhabdomyolysis(RM) as a result of intractable hypokalaemia.
CASE PRESENTATION
A 64-year-old man was admitted for limb weakness and facial hyperpigmentation for 2 weeks. Lab tests revealed intractable hypokalaemia (lowest at 1.8 mmol/L) and metabolic alkalosis. The diagnosis of RM was based on a creatine kinase(CK)level of 5 times the upper limit. The elevated CK and myohemoglobin (Mb) levels returned to within the normal range after the alleviation of hypokalaemia. The patient was diagnosed with ACTH-dependent Cushing's syndrome (CS) based on unsuppressed serum cortisol after a low-dose dexamethasone suppression test(LDDST) and remarkably elevated ACTH levels. The diagnosis of EAS was made based on the results of a high-dose dexamethasone suppression test(HDDST) and bilateral inferior petrosal sinus sampling(BIPSS). Multiple lymph nodes in the left supraclavicular fossa, right root of neck, mediastinum and bilateral hili of the lung were found with abnormal uptake of Ga-DOTA-NOC. Mediastinoscopic lymph node biopsy was performed. The pathological diagnosis was small-cell and large-cell neuroendocrine carcinoma with positive ACTH staining. The patient was prescribed mifepristone and received one cycle of chemotherapy. The patient could not tolerate subsequent chemotherapy and died of dyscrasia.
CONCLUSIONS
RM is a rare complication of EAS with insidious onset and atypical clinical manifestations. Serum potassium levels should be vigilantly monitored to avoid RM in EAS.
Topics: ACTH Syndrome, Ectopic; Carcinoma, Neuroendocrine; Fatal Outcome; Humans; Hypokalemia; Male; Middle Aged; Radiography, Thoracic; Rhabdomyolysis
PubMed: 33971870
DOI: 10.1186/s12902-021-00755-0 -
ClinicoEconomics and Outcomes Research... 2021To describe the distribution of diagnostic procedures, rates of complications, and total cost of biopsies for patients with lung cancer.
PURPOSE
To describe the distribution of diagnostic procedures, rates of complications, and total cost of biopsies for patients with lung cancer.
PATIENTS AND METHODS
Observational study using data from IBM Marketscan Databases for continuously insured adult patients with a primary lung cancer diagnosis and treatment between July 2013 and June 2017. Costs of lung cancer diagnosis covered 6 months prior to index biopsy through treatment. Costs of chest CT scans, biopsy, and post-procedural complications were estimated from total payments. Costs of biopsies incidental to inpatient admissions were estimated by comparable outpatient biopsies.
RESULTS
The database included 22,870 patients who had a total of 37,160 biopsies, of which 16,009 (43.1%) were percutaneous, 14,997 (40.4%) bronchoscopic, 4072 (11.0%) surgical and 2082 (5.6%) mediastinoscopic. Multiple biopsies were performed on 41.9% of patients. The most common complications among patients receiving only one type of biopsy were pneumothorax (1304 patients, 8.4%), bleeding (744 patients, 4.8%) and intubation (400 patients, 2.6%). However, most complications did not require interventions that would add to costs. Median total costs were highest for inpatient surgical biopsies ($29,988) and lowest for outpatient percutaneous biopsies ($1028). Repeat biopsies of the same type increased costs by 40-80%. Complications account for 13% of total costs.
CONCLUSION
Costs of biopsies to confirm lung cancer diagnosis vary substantially by type of biopsy and setting. Multiple biopsies, inpatient procedures and complications result in higher costs.
PubMed: 33762834
DOI: 10.2147/CEOR.S295494 -
Translational Lung Cancer Research Jan 2021The staging of mediastinal lymph nodes for lung cancer is crucial for planning treatments or reinterventions. In potentially curable patients the aim of mediastinal... (Review)
Review
The staging of mediastinal lymph nodes for lung cancer is crucial for planning treatments or reinterventions. In potentially curable patients the aim of mediastinal staging is to exclude the presence of malignancy in mediastinal lymph nodes with a high level of accuracy while also considering clinical factors and the balance of the benefits and risks of tissue sampling techniques. Mediastinal staging is based on computed tomography (CT) and positron emission tomography (PET) and can be sufficient when no mediastinal abnormalities are present and the probability of unforeseen N2 disease is low. In the case of bulky lymph nodes with a high probability of malignancy in PET-CT, tissue confirmation is not normally required. If mediastinal sampling is needed it can be achieved by endosonographic techniques, including endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) or a combination of the two. Positive results do not need further confirmation. In the case of negative results, surgical techniques still play a role in the selected cases discussed by multidisciplinary lung cancer committees. New mediastinal surgical techniques including video-assisted cervical mediastinoscopy (VACM), video-assisted mediastinoscopic lymphadenectomy (VAMLA), and transcervical extended mediastinal lymphadenectomy (TEMLA) have been shown to be useful in selected patients. Final pathological staging is based on lymph node removal during surgery and can be achieved by taking one of two approaches: lymph node sampling or systematic lymph node sampling. The accuracy of PET-CT and mediastinal endosonography is lower for mediastinal restaging than it is for surgical techniques; their false positive and false negative (FN) rate is high and so, they require histological confirmation. Here we explain and revise the results from the most recent studies and current international guidelines.
PubMed: 33569331
DOI: 10.21037/tlcr.2020.03.08 -
Journal of Thoracic Disease Jan 2021Esophagectomy via transcervical incision inflatable single-port mediastinoscope combined with laparoscopy as a safe and feasible minimally invasive technique has gained...
BACKGROUND
Esophagectomy via transcervical incision inflatable single-port mediastinoscope combined with laparoscopy as a safe and feasible minimally invasive technique has gained attention recently. But the occurrence of Intraoperative events is inevitable. It's necessary to investigate and discuss the intraoperative events and countermeasures during operation.
METHODS
Intraoperative events were retrospectively reviewed in 60 patients who underwent esophagectomy via transcervical incision inflatable single-port mediastinoscope combined with laparoscopy in the recent 3 years.
RESULTS
There was no perioperative death and no aortic or bronchial injury. Bronchial artery injury occurred in 2 cases (3.34%), bronchial artery combined with azygos vein hemorrhage occurred in 1 case (1.67%). The pleura were injured in 3 cases (5%). Recurrent laryngeal nerve injury was noticed in 7 cases (11.67%). Thoracic duct injury occurred in 1 case (1.67%).
CONCLUSIONS
As a new surgical method, esophagectomy via transcervical incision inflatable single-port mediastinoscope combined with laparoscopy is considered safe and feasible, but requires improvement when compared with traditional surgical methods. Due to the influence of surgical space and with experienced surgeons, the incidence of intraoperative events such as intraoperative bleeding and thoracic duct injury is not dominant when compared with the traditional surgical methods. Thoracic surgeons should continuously improve their clinical knowledge as well as skills. Careful preoperative examination and evaluation of the patients, being familiar with the anatomical structure and various methods, wise selection of energy devices and calmly dealing with all kinds of events are the key factors for successful surgeries with fewer intraoperative events.
PubMed: 33569193
DOI: 10.21037/jtd-20-2331 -
Thoracic Cancer Feb 2021Cervical mediastinoscopy is useful for diagnosing lung and mediastinal disease. Ultrasound is a safe real-time diagnostic tool widely employed in many surgical fields....
BACKGROUND
Cervical mediastinoscopy is useful for diagnosing lung and mediastinal disease. Ultrasound is a safe real-time diagnostic tool widely employed in many surgical fields. Ultrasound was used in cervical mediastinoscopy in our cohort with satisfactory results. This study investigated the safety, feasibility, and availability of video-assisted mediastinoscopy (VAM) combined with ultrasound for mediastinal lymph node biopsy.
METHODS
A total of 87 cases involving cervical mediastinal lymph node biopsy performed from November 2015 to May 2020, with complete clinical and pathological information, were retrospectively analyzed in the Department of Thoracic Surgery at Beijing Chaoyang Hospital. The cohort was divided into two groups: ultrasound-guided biopsy under video-assisted mediastinoscopy (UVAM) (44 cases) and routine VAM (43 cases). Operation time, biopsy number and nodal stations, postoperative complications, pathological conditions, and surgical difficulty were compared between the two nodal stations.
RESULTS
UVAM was significantly shorter and more lymph node specimens were obtained than with VAM. There was one case of fatal bleeding and two cases of right recurrent laryngeal nerve injury in the VAM group, and no postoperative complications in the UVAM group.
CONCLUSIONS
When used with cervical VAM, ultrasound guidance assists physicians assess the space between lymph nodes, surrounding tissues, and large vessels systematically, making biopsy safer and easier, improving lymph node sampling, and decreasing postoperative complications. Furthermore, surgeons can easily learn and master this method.
KEY POINTS
Significant findings of the study: Ultrasound was used in combination with cervical mediastinoscopy and the results showed that ultrasound guidance makes biopsy in patients safer and easier, improves lymph node sampling, and decreases postoperative complications.
WHAT THIS STUDY ADDS
Surgeons can easily learn and master this method.
Topics: Adult; Aged; Female; Humans; Image-Guided Biopsy; Lymph Node Excision; Lymph Nodes; Male; Mediastinoscopy; Mediastinum; Middle Aged
PubMed: 33141499
DOI: 10.1111/1759-7714.13717 -
Breast (Edinburgh, Scotland) Dec 2020To assess the predictive value of F-fluorodeoxyglucose positron-emission tomography (FDG-PET/CT) in detecting mediastinal lymph node metastasis with histopathologic...
BACKGROUND
To assess the predictive value of F-fluorodeoxyglucose positron-emission tomography (FDG-PET/CT) in detecting mediastinal lymph node metastasis with histopathologic verification in breast cancer (BC) patients.
MATERIALS AND METHODS
Between February 2012 and October 2019, 37 BC patients who underwent histopathological verification for FDG-PET positive mediastinal lymph nodes were retrospectively analyzed. Nine patients (24%) were screened before beginning treatment, while 27 (76%) were screened at the time of disease progression, an average of 39 months after completion of initial treatment.
RESULTS
The histopathologic diagnosis revealed lymph node metastasis from BC in 15 patients (40%) and benign disease in 22 patients (60%). The standardized uptake value (SUV) of mediastinal lymph nodes was significantly higher in patients with lymph node metastasis compared to those with benign histology (9.0 ± 3.5 vs. 5.9 ± 2.4; P = 0.007). The cut-off value of SUV after the ROC curve analysis for pathological lymph node metastasis was 6.4. Two of the 15 patients with mediastinal SUV ≤ 6.4 and 13 of the 22 patients with SUV > 6.4 had lymph node metastasis. Age and pathological findings were prognostic factors for overall survival in univariate analysis. The treatment decision was changed in 19 patients (51%) after mediastinoscopic evaluation of the entire cohort.
CONCLUSIONS
This is the first study to support the need for pathologic confirmation of a positive PET/CT result following evaluation of mediastinal lymph nodes for staging BC, either at initial diagnosis or at the time of progression. Treatment decisions were consequently altered for nearly half of the patients.
Topics: Adult; Aged; Breast Neoplasms; Female; Fluorodeoxyglucose F18; Humans; Lymph Nodes; Lymphatic Metastasis; Mediastinum; Middle Aged; Positron Emission Tomography Computed Tomography; Predictive Value of Tests; Radiopharmaceuticals; Retrospective Studies
PubMed: 33125983
DOI: 10.1016/j.breast.2020.10.011 -
Surgical Case Reports Sep 2020Choriocarcinomas are usually classified as either gestational or non-gestational. Primary choriocarcinomas in the gastrointestinal tract, especially primary...
BACKGROUND
Choriocarcinomas are usually classified as either gestational or non-gestational. Primary choriocarcinomas in the gastrointestinal tract, especially primary choriocarcinomas in the esophagus, are extremely rare. We report a case of a rare primary esophageal choriocarcinoma mixed with squamous cell carcinoma-like components in association with Barrett's adenocarcinoma.
CASE PRESENTATION
A 58-year-old man visited the hospital, complaining of hematemesis and tarry stools. In emergency upper gastrointestinal endoscopy, a bleeding esophageal tumor was observed. Additionally, a contrast computed tomography (CT) scan showed a large hypervascular tumor 4.8 cm in diameter in the left kidney. He came to our institution for further examination and treatment of the esophageal tumor and kidney lesion. The patient had an easy bleeding elevated tumor 2 cm in diameter at the left wall of the middle thoracic esophagus and a left renal carcinoma. Histopathological diagnosis of the biopsy specimen of the esophageal tumor was a poorly differentiated carcinoma. However, a precise histological type diagnosis could not be obtained. In June 2016, mediastinoscopic transhiatal esophagectomy and posterior mediastinal gastric tube reconstruction were performed to treat his esophageal tumor. Histopathologically, most of the tumor comprised hCG-positive syncytiotrophoblasts. Therefore, we confirmed it as a primary esophageal choriocarcinoma. Furthermore, the tumor contained a poorly differentiated squamous cell carcinoma-like component that was also diagnosed as a choriocarcinoma using immunohistochemical staining and there was a small Barrett's esophageal adenocarcinoma lesion in the Barrett's epithelium near the tumor. Three months after surgery, a CT scan demonstrated multiple lung metastatic nodules and multiple intrahepatic masses. Needle biopsy from the lung nodule showed a choriocarcinoma. Despite chemotherapy, the metastatic choriocarcinoma regrew rapidly and multiple bone metastases appeared. He died because of his esophageal choriocarcinoma 13 months after primary resection.
CONCLUSIONS
We encountered an extremely rare case of esophageal choriocarcinoma combined with squamous cell carcinoma-like components in association with a simultaneous Barrett's adenocarcinoma that we followed for the entire course of his disease, from resection to end of life. Esophageal choriocarcinomas are rare with peculiar characteristics and very poor prognoses. Additional cases are needed to establish an appropriate future treatment.
PubMed: 32990826
DOI: 10.1186/s40792-020-00990-y -
Lung India : Official Organ of Indian... 2020A 49-year-old male was evaluated in our hospital for chronic cough, cervical lymphadenopathy, and a testicular mass. As a part of the management, he underwent a cervical...
A 49-year-old male was evaluated in our hospital for chronic cough, cervical lymphadenopathy, and a testicular mass. As a part of the management, he underwent a cervical lymph node biopsy, which showed metastatic deposits from papillary carcinoma thyroid. Subsequently, he underwent orchidectomy for suspected testicular malignancy, but the biopsy showed discrete granulomatous inflammation consistent with sarcoidosis. This was followed by total thyroidectomy with neck node dissection. Nodal histopathological examination, this time, revealed a single node containing both malignancy and granulomas. Prior to the thyroid surgery, he underwent a mediastinoscopic sampling of the mediastinal nodes, which also showed granulomatous inflammation consistent with sarcoidosis. Sarcoidosis affecting the genitourinary system is a rare entity. The coexistence of sarcoidosis and thyroid malignancy poses a diagnostic challenge. A thorough review of the literature was done, and there are no reports from India on the above association. This is a unique case, which could possibly suggest an association between sarcoidosis and malignancy and highlights the importance of obtaining a tissue diagnosis in such cases.
PubMed: 32883906
DOI: 10.4103/lungindia.lungindia_378_19 -
International Journal of Surgery Case... 2020An esophageal diverticulum is a rare condition, and surgery is indicated if symptomatic. We successfully performed mediastinoscopic esophagectomy for a giant esophageal...
INTRODUCTION
An esophageal diverticulum is a rare condition, and surgery is indicated if symptomatic. We successfully performed mediastinoscopic esophagectomy for a giant esophageal diverticulum with stenosis.
PRESENTATION OF CASE
A 63-year-old man visited our hospital because of dysphagia. He had been pointed out an esophageal diverticulum at a local hospital 13 years before visiting our hospital. Upper gastrointestinal endoscopy revealed an esophageal diverticulum at the lower thoracic esophagus and the structural stenosis in the anal side of the diverticulum. Computed tomography showed a 54 mm esophageal diverticulum at the lower thoracic esophagus. Esophagectomy was required because of the structural stenosis. His medical history included chronic obstructive pulmonary disorder. So, we chose the mediastinal approach to avoid a respiratory complication. We performed mediastinoscopic esophagectomy and esophagogastrostomy via the retrosternal route. The postoperative course was good. At 9 months postoperation, there were no symptoms.
DISCUSSION
Recently, laparoscopic diverticulectomy with myotomy and fundoplication has been considered the best approach in most cases. In the case with the structural stenosis, esophagectomy may be required.
CONCLUSION
Mediastinoscopic esophagectomy for the patient with poor respiratory function and who need esophagectomy could be an effective and noninvasive candidate procedure.
PubMed: 32698269
DOI: 10.1016/j.ijscr.2020.06.047