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International Journal of Molecular... Mar 2024Recently, the fifth edition of the WHO classification recognized the thoracic -deficient undifferentiated tumor (SMARCA4-UT) as a separate entity from conventional... (Review)
Review
Recently, the fifth edition of the WHO classification recognized the thoracic -deficient undifferentiated tumor (SMARCA4-UT) as a separate entity from conventional non-small cell lung cancer with deficiency because of the different clinicopathological characteristics of these two diseases. SMARCA4-UT mainly occurs in young to middle-aged adults and involves a large mass compressing the tissues surrounding the mediastinum and lung parenchyma. Unfortunately, SMARCA4-UT shows a high probability of recurrence after upfront surgery as well as radiotherapy resistance; moreover, chemotherapy has low efficacy. Moreover, given the recent classification of SMARCA4-UT, no data concerning specific clinical trials are currently available. However, several case reports show immunotherapy efficacy in patients with this disease not only in a metastatic setting but also in a neoadjuvant manner, supporting the development of clinical trials. In addition, preclinical data and initial clinical experiences suggest that inhibiting pathways such as CDK4/6, AURKA, ATR, and EZH2 may be a promising therapeutic approach to SMARCA4-UT.
Topics: Adult; Middle Aged; Humans; Carcinoma, Non-Small-Cell Lung; Lung Neoplasms; Sarcoma; Biomarkers, Tumor; Mutation; DNA Helicases; Nuclear Proteins; Transcription Factors
PubMed: 38542211
DOI: 10.3390/ijms25063237 -
International Journal of Surgical... Sep 2023Thymic epithelial neoplasms are the most common tumors of thymic origin but are overall rare in the general population. Their morphologic diversity, ranging from low... (Review)
Review
Thymic epithelial neoplasms are the most common tumors of thymic origin but are overall rare in the general population. Their morphologic diversity, ranging from low grade to overtly malignant lesions, along with various histologic growth patterns make them a diagnostically challenging group of tumors. Very occasionally, thymomas and thymic carcinomas may develop in combination with other benign or malignant lesions of thymic origin, further complicating the diagnostic process. The focus of this review lies on the spectrum of thymic epithelial tumors that present with other thymic lesions in the same tumor mass, such as multilocular thymic cysts, neuroendocrine neoplasms, lymphomas, and germ cell tumors among others. Awareness of the existence of such unusual tumors may not only aid in their diagnosis but may also have implications for prognostic and therapeutic purposes.
Topics: Humans; Thymoma; Thymus Neoplasms; Lymphoma; Neoplasms, Glandular and Epithelial
PubMed: 36036356
DOI: 10.1177/10668969221118324 -
Journal of Surgical Case Reports May 2024A 72-year-old female presented with 2 years of pro-gradient pain in the upper thoracic spine radiating to the left arm and leg. MRI revealed a 2.7 × 2.0 × 12 cm...
A 72-year-old female presented with 2 years of pro-gradient pain in the upper thoracic spine radiating to the left arm and leg. MRI revealed a 2.7 × 2.0 × 12 cm paravertebral mass at T2/T3, extending into the foraminal and epidural nerves with extensive dural sac contact in the left hemithorax. The removed tumour was surprisingly soft for a schwannoma or chordoma. However, after the surgery, histopathology revealed the presence of brachyury protein (T-box transcription factor T), which is characteristic of a chordoma. While chordomas are extremely rare, it is important that they are kept in mind for the differential diagnosis of a posterior mediastinal mass. Successful treatment can only be achieved through a complete en bloc resection. This can often be complex due to their location along the spine. This case report aims to highlight the features and treatment of this rare disease.
PubMed: 38721258
DOI: 10.1093/jscr/rjae299 -
Cancer Cytopathology Nov 2023The objectives of this study were to investigate the utility of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA)/endoscopic ultrasound-guided...
BACKGROUND
The objectives of this study were to investigate the utility of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA)/endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) for the diagnosis of amyloidosis coupled with the feasibility of mass spectrometry (MS) for amyloid subtyping.
METHODS
All patients who had amyloid diagnosed by EBUS-TBNA/EUS-FNA at two tertiary care centers from 2011 to 2020 were retrieved along with the MS subtype, clinical findings, and outcomes.
RESULTS
Eight patients were included: seven underwent EBUS-TBNA of mediastinal lymph nodes, and one underwent EUS-FNA of a periportal lymph node. Ages ranged from 37 to 79 years (median, 69 years), with equal numbers of men and women. Presenting clinical history included one case each of follicular lymphoma, lymphoplasmacytic lymphoma, rheumatoid arthritis, possible sarcoid, cirrhosis, and chronic renal insufficiency, and one case each of suspected pulmonary and cardiac amyloidosis. All cases showed waxy, amorphous material on direct smears (n = 5) or ThinPrep slides (n = 3), which were confirmed as amyloid on Congo Red staining. Immunohistochemistry showed dominant lambda staining in two of three cases. MS was performed in all cases and identified five of the light-chain (AL) type, one of the heavy-chain/AL type, and two suggestive of AL amyloidosis. Bone marrow biopsy performed in seven patients demonstrated that three had monoclonal plasma cells and one had lymphoplasmacytic lymphoma. Two of four patients with systemic amyloidosis received chemotherapy and remained alive, whereas three with localized disease remained stable under observation.
CONCLUSIONS
EBUS-TBNA/EUS-FNA is effective for amyloidosis diagnosis and provides adequate material for ancillary tests, including MS, which can identify the precursor amyloidogenic protein, leading to appropriate patient management.
Topics: Male; Humans; Female; Adult; Middle Aged; Aged; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Tertiary Care Centers; Tertiary Healthcare; Bronchoscopy; Mediastinum; Lung Neoplasms; Lymph Nodes; Amyloidosis; Lymphoma; Neoplasm Staging
PubMed: 37641237
DOI: 10.1002/cncy.22751 -
International Journal of Surgery Case... Sep 2023Bronchogenic cysts (BCs) are rare congenital lesions that originate from the tracheobronchial bud and can be found in any organ derived from the embryonic foregut....
INTRODUCTION
Bronchogenic cysts (BCs) are rare congenital lesions that originate from the tracheobronchial bud and can be found in any organ derived from the embryonic foregut. Complete surgical excision is the treatment of choice, and the definitive diagnosis is established by histopathological examination.
PRESENTATION OF THE CASE
41-year-old female with diastolic arterial hypertension and a heterogeneous mass, with multiple calcifications and a solid component with well-defined regular contours. A biochemical study revealed no functionality. Intraoperatively we noticed an adrenal gland-dependent mass with a cystic component and an area of sebaceous content and histological examination confirmed a bronchogenic cyst.
DISCUSSION
The majority of cases of BCs are asymptomatic. They can be intrapulmonary, mediastinal or ectopic, being the left adrenal region the most common retroperitoneal location. Symptomatic cysts should always be surgically resected either by thoracotomy or a minimally invasive technique, which has been shown to improve postoperative discomfort and shorten hospital stay with reliable postoperative outcomes.
CONCLUSION
Despite their low incidence, it is important to consider BCs in the differential diagnosis of retroperitoneal masses, particularly in the left para-adrenal region.
PubMed: 37603914
DOI: 10.1016/j.ijscr.2023.108706 -
Journal of Thoracic Disease Sep 2023The management of large mediastinal tumors requires a complex multidisciplinary approach, particularly in the perioperative setting due to increased risk of hemodynamic... (Review)
Review
BACKGROUND AND OBJECTIVE
The management of large mediastinal tumors requires a complex multidisciplinary approach, particularly in the perioperative setting due to increased risk of hemodynamic compromise. The utilization of extracorporeal membrane oxygenation (ECMO) provides a useful adjunct in the surgical management for biopsy and resection of these mediastinal masses. The objective of this article is to review indications and implementation of ECMO in the surgical management of mediastinal disease.
METHODS
A literature review of the PubMed database was completed evaluating articles discussing 'extracorporeal circulation', 'cardiopulmonary bypass', 'anesthesia', 'mediastinal disease', and 'mediastinal cancer'. These articles were evaluated for contribution to the discussion of indications and implementation of ECMO in the management of these patients.
KEY CONTENT AND FINDINGS
Large mediastinal tumors place patients at risk of hemodynamic collapse on induction of anesthesia due to compression of vascular structures, tracheobronchial tree and creation of V/Q mismatch. Patients may be stratified regarding their risk of perioperative complications by evaluation of postural symptoms, cross sectional imaging findings and pulmonary function tests. Those patients at elevated perioperative risk may benefit from the utilization of ECMO, most commonly veno-arterial (V-A) ECMO. Guidewires or ECMO cannulas may be placed under local anesthesia prior to induction. Those patients with hemodynamic compromise may receive mechanical circulatory support to allow completion of the operation.
CONCLUSIONS
The use of a multidisciplinary team consisting of surgeons, anesthesiologists, perfusionists and OR team is critical to the success in the use of ECMO in the resection of mediastinal masses. With diligent preparation, these high-risk patients may be optimally managed at the time of resection.
PubMed: 37868893
DOI: 10.21037/jtd-22-1391 -
International Journal of Surgical... Oct 2023Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) is a recently described aggressive neoplasm of young smokers defined by inactivating mutations and...
CONTEXT
Thoracic SMARCA4-deficient undifferentiated tumor (SMARCA4-UT) is a recently described aggressive neoplasm of young smokers defined by inactivating mutations and characterized by cells with rhabdoid morphology, high mitotic activity, and abundant necrosis.
OBJECTIVE
Describe and compare 3 unusual presentations of SMARCA4-UT in older adults, including one presenting as a metastatic lesion mimicking a primary bone sarcoma. Discuss the molecular characteristics of SMARCA4-UT and their relationship to nonsmall-cell lung carcinomas with .
DESIGN
Three patients with SMARCA4-UTs were identified utilizing a natural language search in CoPath. hematoxylin and eosin sections from all patients as well as Papanicolaou-stained slides and Diff-Quik-stained slides for the first patient were examined. A broad range of immunostains, including BRG1/SMARCA4, were evaluated. Molecular testing was performed via next-generation sequencing.
RESULTS
The 3 patients were aged 58, 70, and 70 years. All had a significant smoking history. The first patient presented with an iliac bone mass and mediastinal lymphadenopathy, the second with mediastinal adenopathy, and the third with a paratracheal mass. All 3 tumors showed a diffuse proliferation of pleomorphic, rhabdoid cells with high mitotic activity and tumor necrosis. SMARCA4 was lost in all 3 tumors by immunohistochemistry. Molecular testing revealed alterations in the first 2.
CONCLUSIONS
Thoracic SMARCA4-UT should be considered in the differential diagnosis of pleomorphic rhabdoid tumors in older adults with a smoking history. Although most present as lung and/or mediastinal masses, they may occasionally present as a metastasis and mimic an undifferentiated sarcoma, representing a potential diagnostic pitfall.
PubMed: 37903457
DOI: 10.1177/10668969231206350 -
The American Surgeon Jul 2023Lymphangiomas of pancreatic origin are rare and can often be found with abdominal symptoms or incidentally on imaging. A 46-years-old female presented with epigastric...
Lymphangiomas of pancreatic origin are rare and can often be found with abdominal symptoms or incidentally on imaging. A 46-years-old female presented with epigastric abdominal pain and vomiting, and twenty-pound weight loss over 3 months. Computed tomography of the abdomen showed a septated mass of the distal pancreas measuring 25.4 cm in largest diameter and extending superiorly into the mediastinum via a hiatal hernia. Endoscopic ultrasound demonstrated abutment but no invasion of the esophagus and stomach. She underwent an exploratory laparotomy with retroperitoneal mass excision, distal pancreatectomy, splenectomy, and hiatal hernia repair with Dor fundoplication. Pathology was consistent with a cystic lymphangioma. Patient was discharged on postoperative day 3 and remained recurrence free for over a year.
Topics: Female; Humans; Middle Aged; Diaphragm; Pancreatic Neoplasms; Pancreas; Lymphangioma; Pancreatectomy
PubMed: 37501311
DOI: 10.1177/00031348231157912 -
The Journal of Thoracic and... Sep 2023
Topics: Humans; Heart; Heart Neoplasms; Mediastinal Neoplasms; Thymus Neoplasms; Sarcoma
PubMed: 34862054
DOI: 10.1016/j.jtcvs.2021.11.010 -
Journal of Thoracic Disease Jul 2023Resuscitative therapies for respiratory and cardiac failure are lifesaving and extended by using extracorporeal life support (ECLS) as mechanical circulatory support... (Review)
Review
BACKGROUND AND OBJECTIVE
Resuscitative therapies for respiratory and cardiac failure are lifesaving and extended by using extracorporeal life support (ECLS) as mechanical circulatory support (MSC). This review informs the debate to identify the life-threatening thoracic emergencies in which patients may be cannulated for ECLS support.
METHODS
An advanced search was performed in PubMed, Embase, Google Scholar, and references query, assessed in June 2022, identified 761 records. Among them, 74 publications in English were included in the current narrative review.
KEY CONTENT AND FINDINGS
ECLS is an additional tool for organ support in life-threatening thoracic emergencies. It provides bridging to recovery or to decision about destination as definitive therapy, intervention, or surgery. Non-traumatic emergencies include mediastinal mass, acute lung injury (ALI), aspiration, embolisms, acute and chronic heart failure. However, based on the current evidence, trauma, and especially blunt thoracic trauma, is one of the main indications for ECLS use in thoracic emergencies, among others in chest wall fractures, blunt and penetrating lung injuries. ECLS use is always individualized to patient's needs, injury pattern and kind of organ failure, circulatory arrest inclusive, depending on if respiratory or cardiac and circulatory support is needed. Further, ECLS offers the possibility for fast volume resuscitation and rewarming, thus preventing the lethal of trauma: hypothermia, hypoperfusion and acidosis. Anticoagulation may be omitted for some hours or days. Interdisciplinary cooperation between the intensivists, surgeons, anesthesiologists, emergency medical services, an appropriately organized and trained staff, equipment resources and logistical planning are essential for successful outcomes.
CONCLUSIONS
ECLS use in selected life-threatening thoracic emergencies is increasing. The summarized findings appeal to policymakers, and we hope that our summary of recommendations may impact clinical practice and research.
PubMed: 37559625
DOI: 10.21037/jtd-22-1307