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Ectopic Cervical Thymoma in a Patient Diagnosed With Graves Disease: A Systematic Literature Review.The Journal of Clinical Endocrinology... Apr 2024Thymomas are benign thymic epithelial neoplasms, rarely found outside the anterior mediastinum. Although hyperthyroid states have been associated with thymic...
Thymomas are benign thymic epithelial neoplasms, rarely found outside the anterior mediastinum. Although hyperthyroid states have been associated with thymic hyperplasia, only 3 thymoma cases have been previously reported in patients with Graves disease (GD), all within the anterior mediastinum. Here, we report a case of ectopic cervical thymoma in a 22-year-old female patient previously treated for GD. The patient underwent ultrasonography, computed tomography, inconclusive fine-needle aspiration, and ultimately gross dissection for diagnostic workup and definitive treatment of an anterior neck mass, producing a 2.5 × 2.3 × 1.5-cm entity consistent with Masaoka stage I and type B2 thymoma per World Health Organization classification. The patient underwent an uncomplicated subsequent clinical course, with no adjuvant radiotherapy administered. After conducting a systematic literature review, we conclude that of the 109 cases of ectopic cervical thymoma reported, this is the first to describe a case of ectopic cervical thymoma in a patient with a past medical history of GD. For GD patients in stable euthyroid remission with the persistent or recurrent presence of an anterior neck mass, the extrathyroidal origin of the mass should always be considered, including the exceptional presence of a cervical ectopic thymoma.
Topics: Female; Humans; Young Adult; Adult; Thymoma; Thymus Neoplasms; Neck; Tomography, X-Ray Computed; Graves Disease
PubMed: 37897424
DOI: 10.1210/clinem/dgad635 -
Endocrine Nov 2023Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome that combines endocrine and non-endocrine tumors. Thymic neuroendocrine tumors are uncommon components that...
BACKGROUND
Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome that combines endocrine and non-endocrine tumors. Thymic neuroendocrine tumors are uncommon components that predict poor prognosis in patients with MEN1. We aimed to summarize the clinical characteristics of thymoma in MEN1 by reviewing the current reports from the literature.
METHODS
A patient with multiple endocrine neoplasia type 1 (parathyroid hyperplasia, pituitary adenoma, and insulinoma) was found to have a 2 × 1.5 cm thymic mass during long-term follow-up. Thoracoscope surgery was performed, and a histopathology examination revealed WHO Type B3 thymoma. A pathogenic mutation of c.783 + 1G > A in the MEN1 gene was identified. We further searched PubMed and EMBASE for thymoma in association with MEN1.
RESULTS
A comprehensive overview of the literature concerning characteristics of MEN1-related thymoma was summarized. Clinical characteristics and differences between thymoma and thymic carcinoid are highlighted.
CONCLUSIONS
Besides carcinoid, other tumors, including thymoma, need to be identified for thymic space-occupying lesions in MEN1 patients. The impact of thymoma on the long-term prognosis of MEN1 patients needs further investigation.
Topics: Humans; Multiple Endocrine Neoplasia Type 1; Thymoma; Thymus Neoplasms; Carcinoid Tumor; Pancreatic Neoplasms
PubMed: 37668926
DOI: 10.1007/s12020-023-03440-5 -
Cancers May 2023For decades, lung surgery in thoracic cancer has evolved in two ways: saving more parenchyma and being minimally invasive. Saving parenchyma is a fundamental principle... (Review)
Review
For decades, lung surgery in thoracic cancer has evolved in two ways: saving more parenchyma and being minimally invasive. Saving parenchyma is a fundamental principle of surgery. However, minimally invasive surgery (MIS) is a matter of approach, so it has to do with advances in surgical techniques and tools. For example, MIS has become possible with the introduction of VATS (video-assisted thoracic surgery), and the development of tools has extended the indication of MIS. Especially, RATS (robot-assisted thoracic surgery) improved the quality of life for patients and the ergonomics of doctors. However, the dichotomous idea that the MIS is new and right but the open thoracotomy is old and useless may be inappropriate. In fact, MIS is exactly the same as a classic thoracotomy in that it removes the mass/parenchyma containing cancer and mediastinal lymph nodes. Therefore, in this study, we compare randomized-controlled trials about open thoracotomy and MIS to find out which surgical method is more helpful.
PubMed: 37174096
DOI: 10.3390/cancers15092630 -
Annals of Cardiothoracic Surgery Mar 2023Early results have illustrated the multiportal robotic approach to be safe and oncologically efficacious in the treatment of thoracic malignancies. Industry leaders have...
BACKGROUND
Early results have illustrated the multiportal robotic approach to be safe and oncologically efficacious in the treatment of thoracic malignancies. Industry leaders have improved upon the lessons learned during the early multiportal studies and have now come to establish the feasibility of the biportal, and subsequently the uniportal robotic-assisted approach, all in an effort to offer patients equivalent or better outcomes with less surgical trauma. No current, coherent body of evidence currently exists outlining the early-term outcomes of patients undergoing uniportal robotic-assisted thoracic surgery. This systematic review and meta-analysis sought to clarify the early-phase outcomes of these patients.
METHODS
An electronic search of four databases was performed to identify relevant studies outlining the immediate post-operative outcomes of patients undergoing uniportal robotic-assisted thoracic surgeries. The primary endpoint was defined as technical success (i.e., no conversion to secondary robotic, video-assisted thoracoscopic, or open approaches). Secondary endpoints of interest included post-operative outcomes and complication rates. A meta-analysis using a random effects model of proportions or means was applied, as appropriate.
RESULTS
The search strategy ultimately yielded 12 relevant studies for inclusion. A total of 240 patients (52% male) split across cohort studies and case reports were identified. The mean age of the two groups was 59.7±3.0 and 58.1±6.8 years, respectively. The mean operative time was 133.8±38.2 and 150.0±52.2 minutes, respectively. Length of hospital stay was 4.4±1.6 and 4.3±1.1 days, respectively. The mean blood loss was 80.0±25.1 mL The majority of identified procedures were lobectomies, segmentectomies, and wedge resections, though complex sleeve resections and anterior mediastinal mass resections were also completed. Cumulative technical success was 99.9%.
CONCLUSIONS
The uniportal robotic-assisted approach, when completed in expert hands, has been illustrated to have exceedingly low rates of conversion to secondary procedures, along with short length of stay (LOS), minimal blood loss, and short procedural times (variable depending on operation type). Current evidence on the feasibility of this approach will be bolstered by upcoming multi-institutional series.
PubMed: 37035647
DOI: 10.21037/acs-2023-urats-37 -
Frontiers in Oncology 2023Castleman´s disease is an extremely rare heterogenous lymphoproliferative pathology with a mostly benign behavior. It is a localized or generalized lymph node...
BACKGROUND
Castleman´s disease is an extremely rare heterogenous lymphoproliferative pathology with a mostly benign behavior. It is a localized or generalized lymph node enlargement of an unknown aetiology. Unicentric form is typically a slow-growing solitary mass occurring mostly in the mediastinum, abdominal cavity, retroperitoneum, pelvis and neck. Aetiology and pathogenesis of CD is probably diverse, varying in different types of this heterogeneous disease.
MATERIALS AND METHODS
Authors present a review of this issue based on their extensive experience. The aim is to summarize the crucial factors in the management of diagnostics and a surgical treatment of the unicentric form of Castleman´s disease. One of the key issues in the unicentric form is precise preoperative diagnostics and thus choosing the right surgical treatment strategy. Authors highlight pitfalls of the diagnosis and surgical treatment.
RESULTS
All histological types such as a hyaline vascular type, plasmacytic type and a mixed type are presented as well as options of surgical and conservative treatment. Differential diagnosis and malignant potential is discussed.
CONCLUSION
Patients with Castleman´s disease should be treated in the high- volume centers, with a great experience in major surgical procedures as well as with preoperative imaging diagnostic techniques. Specialized pathologists and oncologists focusing on this issue are also absolutely necessary to avoid misdiagnosis. Only this complex approach can lead to excellent outcomes in patients with UCD.
PubMed: 36793610
DOI: 10.3389/fonc.2023.1057683 -
Cureus Dec 2022Epithelioid sarcoma is a rare entity that shows a predilection for the young and middle-aged population. There are two subtypes, i.e., the distal or conventional type,...
Epithelioid sarcoma is a rare entity that shows a predilection for the young and middle-aged population. There are two subtypes, i.e., the distal or conventional type, which is located in distal extremities, and the proximal type, located in proximal areas of the extremities, pelvis, perineal, and genital region. The latter is characterized by more aggressive behavior, a higher recurrence rate, and poor prognosis. Histopathological and immunohistochemical diagnoses are key to correct and timely treatment and a higher survival rate. We report a case of a 41-year-old man who presented a palpable progressive growth mass in the hypogastrium. The disease time was of nine months, and the tumor was resected, but it recurred a few months later in the same location. Computed tomography (CT) scans showed images suggestive of lung metastasis and the patient had to undergo a second surgery. He received eight cycles of chemotherapy and a subsequent CT scan control showed the progression of the disease, so a new chemotherapy regimen was established. The patient received three cycles of chemotherapy without improvement, so he decided to discontinue treatment. His last outpatient medical consultation was in January 2022. A systematic review of the studies published in PubMed and Google Scholar was performed. We identified 291 articles, but only 41 reports and case series were included, with a total of 55 patients. It is important to include this type of tumor in the differential diagnosis of epithelial tumors due to its aggressive behavior. Correct and timely diagnosis is crucial to obtain lower recurrence rates, lower mortality, and higher survival rates in these patients.
PubMed: 36712724
DOI: 10.7759/cureus.32962 -
Langenbeck's Archives of Surgery Mar 2022Giant parathyroid adenoma (GPA) can present with severe biochemical derangement similar to the clinical presentation of parathyroid carcinoma (PC). This study aims to...
PURPOSE
Giant parathyroid adenoma (GPA) can present with severe biochemical derangement similar to the clinical presentation of parathyroid carcinoma (PC). This study aims to present the current evidence on surgical management of GPAs in primary hyperparathyroidism.
METHODS
A systematic review of the literature on GPAs was conducted following the PRISMA guidelines. Data on clinical, biochemical, preoperative diagnostic, and surgical methods were analysed.
RESULTS
Sixty-one eligible studies were included reporting on 65 GPAs in eutopic, ectopic mediastinal, and intrathyroidal locations (61.5%, 30.8%, and 7.7%, respectively). A palpable neck mass was present in 58% of GPAs. A total of 90% of patients had symptoms including fatigue, skeletal pain, pathological fracture, nausea, and abdominal pain. Ninety percent of patients had significant hypercalcaemia (mean 3.51 mmol/L; range: 2.59-5.74 mmol/L) and hyperparathyroidism with PTH levels on average 14 times above the upper limit of the normal reference. There was no correlation between the reported GPA size and PTH nor between GPA weight and PTH (p = 0.892 and p = 0.363, respectively). Twenty-four percent had a concurrent thyroidectomy for suspicious features, intrathyroidal location of GPA, or large goitre. Immunohistochemistry such as Ki-67, parafibromin, and galectin-3 was used in 18.5% of cases with equivocal histology. Ninety-five percent of GPAs were benign with 5% reported as atypical adenomas.
CONCLUSION
The reported data on GPAs are sparse and heterogeneous. In GPAs with suspicious features for malignancy, en bloc resection with concurrent thyroidectomy may be considered. In the presence of equivocal histological features, ancillary immunohistochemistry is advocated to differentiate GPAs from atypical adenomas and PCs.
Topics: Adenoma; Humans; Hypercalcemia; Hyperparathyroidism, Primary; Parathyroid Hormone; Parathyroid Neoplasms
PubMed: 35039921
DOI: 10.1007/s00423-021-02406-3 -
Cancer Imaging : the Official... Jan 2022To demonstrate and analyze the relatively common imaging findings in this rare primary pleural angiosarcoma (PPA).
BACKGROUND
To demonstrate and analyze the relatively common imaging findings in this rare primary pleural angiosarcoma (PPA).
CASE PRESENTATION
Three cases of PPA, proven by video-assisted thoracic surgery biopsies are retrospectively reviewed. Patients were all male. Age ranges from 65 to 75 years old age (mean; 69). Major chief complaints were dyspnea and chest pain. One has a history of colon cancer, the other has a tuberculosis history and the other has no known history. Multidetector chest CT and PET CT were all done. Immunohistochemical studies were performed including CD31, CD34, or factor VIII-related antigen, vimentin, and cytokeratin. We also review the literatures on recently published PPA. All masses were from 1 to 10 cm. All three patients had multiple pleural based masses, which were ovoid in shape with relatively sharp margin in unilateral hemithorax. Multiple small circumscribed pleural masses are limited in the pleural space in two patients, whereas two, huge lobulated masses about up to 10 cm were present with pleural and extrapleural involvement in one patient. In two patients with pleural mass only, multiple pleural masses were only seen in parietal pleura in one patient and were in both visceral and parietal pleura in one patient. Pleural effusion were found in one side in one patient and in both sides in one patient. One angiosarcoma was arised from chronic tuberculotic pleurisy sequelae. All pleural masses are heterogenous with irregular internal low densities in all patients. Hematogenous metastases were found in liver, vertebra, rib in one patient, and were in lungs with mediastinal lymph node metastases in the other patient. Three patients survived for longer than 3months after diagnosis, but continued to deteriorate rapidly. Two patients underwent chemotherapy after surgical excision, and the other one with multiple metastases treated chemotherapy after CT-guided biopsy, but eventually all died. As a result of comparative analysis of a total of 13 patients' images including 10 cases previously published, there was pleural effusion in all except 2 cases.
CONCLUSIONS
PPA were all necrotic without any vascularized enhancing nature, and manifested as unilateral circumscribed or localized pleural-based masses.
Topics: Aged; Hemangiosarcoma; Humans; Male; Pleura; Pleural Effusion; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 35022068
DOI: 10.1186/s40644-021-00435-1 -
The Journal of International Medical... May 2021Fibrosing mediastinitis (FM) is a progressive, life-threatening disease characterized by extrinsic compression of mediastinal bronchovascular structures, and the...
Fibrosing mediastinitis (FM) is a progressive, life-threatening disease characterized by extrinsic compression of mediastinal bronchovascular structures, and the clinical manifestations largely depend upon the affected structures. Pleural effusion is rarely reported in patients with FM. We herein describe a 70-year-old man who presented with recurrent breathlessness and refractory left pleural effusion. He was misdiagnosed with and treated for tuberculous pleurisy for several months. Thoracentesis revealed a transudative pleural effusion, and a contrast-enhanced computed tomography scan of the thorax showed an extensive mediastinal soft tissue mass consistent with FM. Pulmonary angiography demonstrated pulmonary artery stenosis on the right side and pulmonary vein stenosis mainly on the left side. After measurement of the pulmonary arterial pressure by right heart catheterization, the patient was diagnosed with pulmonary hypertension associated with FM. He underwent balloon angioplasty and stent implantation of the stenosed pulmonary vessels, which led to long-term improvement in his breathlessness and pleural effusion. Our systematic review of the literature highlights that pleural effusion can be an uncommon complication of FM and requires careful etiological differentiation.
Topics: Aged; Constriction, Pathologic; Humans; Male; Mediastinitis; Pleural Effusion; Sclerosis; Vascular Diseases
PubMed: 33947262
DOI: 10.1177/03000605211010073 -
Journal of Pediatric Surgery Nov 2021Ectopic cervical thymus (ECT) is a rare cause of neck swelling in children. Our aim was to evaluate the diagnostic and management challenges of this condition. (Review)
Review
BACKGROUND/PURPOSE
Ectopic cervical thymus (ECT) is a rare cause of neck swelling in children. Our aim was to evaluate the diagnostic and management challenges of this condition.
METHODS
Systematic review of the literature (1997-2020) using PRISMA guidelines.
RESULTS
A total of 192 cases of ECT were identified. Forty-two cases (4 studies) were excluded because of insufficient data resulting in 150 cases (7 bilateral) in a total of 143 patients. ECT presented as a solid mass in 71 cases (10 incidentally found at autopsy, 33 conservatively managed and 28 excised) and as a cystic mass in 79 cases (all excised). A total of 165 imaging studies in 123 patients were performed (65 ultrasound scans (USS), 51 magnetic resonance imaging (MRI) scans and 49 computer tomography (CT) scans). ECT was diagnosed by imaging in 18.7% (23/123) of patients. Fine needle aspiration cytology or incisional/needle biopsy were performed in 16% (23/140) cases; a correct diagnosis of ECT was obtained in 52% (12/23) of those cases. There were no reports of malignancy in 143 specimens analysed. A normal mediastinal thymus was absent in 2.5% (1/40) of patients investigated.
CONCLUSIONS
ECT is a benign and relatively rare condition in children that is seldom diagnosed on imaging before tissue sampling or surgical excision. ECT might be the only active thymus in 2.5% of cases and careful consideration should be taken prior to its excision in young children. We recommend that ECT should be considered in the differential diagnosis of neck masses in children. Conservative management might be appropriate in selected cases and the presence of a normal mediastinal thymus in the thorax should be investigated before planning surgical excision.
Topics: Biopsy, Fine-Needle; Child; Child, Preschool; Choristoma; Diagnosis, Differential; Humans; Neck; Thymus Gland; Ultrasonography
PubMed: 33789804
DOI: 10.1016/j.jpedsurg.2021.03.003