Mediastinal Germ-cell Tumors Relapse in a Male With Klinefelter Syndrome. Is Longer Surveillance Needed?

Germ cell tumors (GCTs) are a heterogeneous group of pediatric cancers. In up to one-third of male patients, a primary mediastinal location is associated with the presence of Klinefelter syndrome (KS). We describe a case of mediastinal GCT in a patient, with unacknowledged KS, that presented a relapse 7 years from diagnosis, that is, 2 years after the end of the follow-up program usually recommended for patients with GCT. There are no recommendations for screening for KS in patients with mediastinal GCT and there are no specific guidelines for surveillance of GCT in KS patients. Our experience suggests that KS should be suspected in patients with mediastinal GCT, and a longer follow-up plan should be implemented when GCT occurs in patients with KS.

Huge mediastinal ancient schwannoma causing acute respiratory failure: a case report.

Benign mediastinal tumor is usually asymptomatic and exhibits uncomplicated clinical course. Posterior mediastinal schwannoma is common, but a huge benign tumor causing acute respiratory failure due to mass effect is unusual. We present a patient who suffered from acute respiratory failure due to huge mediastinal mass effect and improved after en bloc surgical resection. A 56-year-old woman had no history of systemic disease, but experienced general discomfort and malaise for several months. She was referred to our emergency department after developing sudden respiratory failure. Intubation was performed with ventilator support and she was admitted to the intensive care unit. Chest radiograph and computed tomography showed a huge mass over the left pleural cavity causing left lung, heart, and mediastinal compression. After en bloc resection, she was weaned off the ventilator successfully and was discharged at 24 days after the operation. Postoperative outpatient follow-up showed no symptoms. Mediastinal ancient schwannoma is a rare posterior mediastinal benign tumor. However, mass effect might lead to lethal complications. En bloc resection is necessary for curative treatment.

Primary cardiac rhabdomyosarcoma in a mitral valve involved with rheumatic disease.

A 51-year-old female underwent emergency mitral valve replacement for mitral stenosis with an undetermined mass which was attached to the anterior mitral leaflet. Histopathological testing of the excised specimen confirmed the diagnosis of rheumatic mitral disease in combination with a primary rhabdomyosarcoma. Postoperative adjuvant chemotherapy with pazopanib hydrochloride was given. At 10 months of follow-up, repeated computed tomographic screening has not shown any signs of local recurrence or secondary metastases. The potential for the existence of primary rhabdomyosarcomas should be borne in mind when faced with undetermined masses on mitral leaflets, even in the presence of rheumatic disease.

Massive anterior mediastinal lipoma causing cardiac arrest in a middle-aged male: a case report and literature review.

Lipoma is a common benign soft tissue tumor, but its size and location can lead to serious issues. We report a case of a 48 year-old male patient who experienced sudden cardiac arrest outside the hospital. After resuscitation and examination, we determined that this was due to a massive mediastinal lipoma compressing the lungs, leading to respiratory failure and pulmonary encephalopathy, ultimately resulting in cardiac arrest. This case serves as a reminder to promptly identify and manage chest lipomas to avoid compression and functional impairment of the respiratory system. Early evaluation and treatment of massive lipomas are crucial for preventing complications.

Conservative rehabilitation therapy for respiratory dysfunction due to phrenic nerve sacrifice during resection of massive mediastinal tumor: A case series study.

RATIONALE: Cases of respiratory dysfunction due to phrenic nerve sacrifice during resection of massive mediastinal tumor have rarely been studied in detail. Diaphragmatic dysfunction in such cases can lead to potentially fatal respiratory and circulatory disturbances. Therefore, timely diagnosis and intervention are important. Conservative rehabilitation therapy is the first choice for respiratory dysfunction due to diaphragmatic dysfunction. PATIENT CONCERNS, DIAGNOSES AND INTERVENTIONS: We present 3 patients with respiratory dysfunction due to phrenic nerve sacrifice during resection of massive mediastinal tumor. The diagnostic methods and therapeutic procedures for diaphragmatic dysfunction for each patient are described in detail. This study highlights the role of ventilator support combined with physical therapy in the treatment of respiratory dysfunction in such cases. The diagnosis of diaphragmatic dysfunction as well as the risk assessment of phrenic nerve involvement are also discussed. The modalities of ventilator support, including modes and parameters, are listed. OUTCOMES AND LESSONS: This study provides experiences of diagnosis and treatment of respiratory dysfunction due to phrenic nerve sacrifice during resection of massive mediastinal tumor. Timely diagnosis of diaphragmatic dysfunction primarily relies on clinical manifestations and radiography. Conservative rehabilitation therapy can improve or restore diaphragmatic function in majority of patients, and avert or delay the need for surgical intervention. Preoperative assessment of the risk of phrenic nerve involvement is important in such cases.

Child Neurology: Anti-Hu Encephalitis in an Adolescent With a Mediastinal Seminoma.

Anti-Hu antibodies are associated with autoimmune syndromes, mainly limbic encephalitis, encephalomyelitis, and painful sensory polyneuropathy (Denny-Brown). We report the case of a 15-year-old boy presenting with epilepsia partialis continua (EPC) found to have a right middle frontal gyrus brain lesion without atrophy or contralateral involvement. After partial resection, neuropathology revealed neuronal loss, reactive gliosis and astrocytosis, and perivascular mononuclear inflammatory infiltrate and features of neuronophagia resembling Rasmussen encephalitis. Suboptimal response to antiseizure drugs and surgery prompted further workup with identification of positive serum anti-Hu antibodies and a mediastinal seminoma. The patient was treated with immunotherapy including steroids, IV immunoglobulin, azathioprine, rituximab, plasmapheresis, and mediastinal lesion resection. However, he continued to experience EPC and psychomotor impairment along with left hemiparesis and dysarthria. Given clinical progression with failure to respond to immunotherapy and antiseizure polytherapy, hemispherotomy was attempted and seizure freedom achieved. A review of the literature found only 16 cases of neurologic presentations associated with anti-Hu antibodies in children, confirming the rarity of EPC in these cases. Thus, this report provides a new observation of germ cell mediastinal tumor associated with anti-Hu antibodies in children, broadening the spectrum of anti-Hu-associated neurologic disorders in children and highlighting the importance of considering antineuronal antibody testing in children presenting with EPC and brain lesions suggestive of Rasmussen encephalitis.

A case of suspected primary mediastinal large B-cell lymphoma: An uncommon cause of sudden death.

Primary mediastinal large B-cell lymphoma (PMLBCL) is an aggressive tumor originating from thymic B-cells. Clinically, it presents with general signs such as cough, chest pain and dyspnea. Although these symptoms are not specific, they are severe enough to reveal the disease. We report an autopsy case of a 25-year-old man, with a recent past history of cough and dyspnea, for which he consulted twice the emergency department and no diagnosis was made. He presented to the Emergency Unit, with a sudden onset of a dyspnea followed by a loss of consciousness. He was shortly declared dead after, a medico-legal autopsy was requested. On external examination, no traumatic lesions on the body were found, an important cyanosis of the face and ears, was, however, found. On autopsy, a mediastinal mass was found, measuring 19 cm × 25 cm and weighing 600 g, extending to the infra-hyoid region and to the thoracic cage and infiltrating the pericardium. Trachea had a necrotic mucosa with a partially obstructive lymph node mass. The diagnosis of a primary mediastinal large B-cell lymphoma was suspected based on pathological and immunohistochemical findings. The cause of death was finally attributed to respiratory failure due to this tumor.

Mediastinal Hemangioma Masquerading as a Simple Cyst.

ABSTRACT: This report presents imaging from a mediastinal mass in a patient with colon cancer. At baseline and surveillance chest computed tomography examinations, it was characterized as a pericardial cyst. However, during chemotherapy, complications arose and this mass was further characterized with a chest MRI. It was then decided to be removed, and histopathology confirmed the diagnosis of a hemangioma.

Anesthetic management of mini sternotomy and excision of mediastinal neurogenic tumor: Brain-Heart crosstalk.

Brachial plexus tumors are rare and pose challenges for neurosurgeons due to their anatomical complexity. Retrosternal extension of a tumor makes it more difficult for the surgeons as well as for the anesthesiologists to secure a definitive airway. A cardiopulmonary bypass would be lifesaving in the event of acute cardiorespiratory decompensation. Multidisciplinary collaboration and cooperation between the neurosurgeon, oncosurgeon, cardiothoracic surgeon, and anesthesiologist are imperative to ensure good patient outcomes. Meticulous preoperative evaluation and operative planning are essentially the key factors in anesthetic management. Here we report a successful management of a 49-year-old male patient presented with a large painless mass arising from his right supraclavicular region and compressing the roots of the brachial plexus, trachea, and esophagus and extending up to the apex of the lungs, posted for mini sternotomy and excision of the mass.

[Pulmonary vein stenosis with pulmonary infarction secondary to primary mediastinal seminoma: a case report].

Pulmonary vein stenosis is a rare condition that is often underdiagnosed and misdiagnosed. The clinical and radiologic manifestations are unspecific such as cough, hemoptysis and pulmonary lesions and are therefore difficult to distinguished with pneumonia and tuberculosis. The present study is a successful case report of pulmonary vein stenosis and pulmonary infraction secondary to mediastinal seminoma. This case suggested that pulmonary vein stenosis should be considered when a mediastinal mass is accompanied by pulmonary opacites that cannot be explained by common causes such as infection.

A Case Report of Mediastinal Teratoma in a Pediatric Patient With Nephrotic Syndrome: Paraneoplastic Syndrome or Incidental Finding?

A 12-year-old female presented with weight gain, edema, and shortness of breath. Laboratory and urine studies confirmed nephrotic syndrome and presence of a mediastinal mass, identified as a mature teratoma after resection. Nephrotic syndrome persisted despite resection and renal biopsy confirmed minimal change disease, which ultimately responded to steroid treatment. She had two relapses of nephrotic syndrome after vaccination administration, both of which occurred within eight months of tumor resection and were responsive to steroids. Autoimmune and infectious workup for other causes of nephrotic syndrome was negative. This is the first reported case of nephrotic syndrome associated with mediastinal teratoma.

[Solitary mediastinal fibrous tumor complicated by hypoglycemic crises (Doege-Potter syndrome)]. / Solitarnaya fibroznaya opukhol' sredosteniya, oslozhnennaya gipoglikemicheskimi krizami (sindrom Doge­Pottera).

Diagnostic and treatment algorithms for large mediastinal tumors are clear. However, long-term results are not always good. They largely depend on early diagnosis and morphological structure of tumor. Neoplasms may be asymptomatic for a long time, especially in case of slow growth. These tumors are usually diagnosed as soon as complications occur (for example, compression syndrome). Routine X-ray screening is rarer situation. Paraneoplastic syndromes are rare, and some ones are casuistic and unknown to surgical community. We describe the diagnosis and treatment of a patient with giant solitary mediastinal tumor complicated by hypoglycemic crises (Doege-Potter syndrome). This complication was life-threatening and required a multidisciplinary approach. Aggressive surgical approach cured the patient and returned her to normal lifestyle. The proposed algorithm for perioperative drug therapy was effective and deserves attention. This report will be useful for surgeons, oncologists, anesthesiologists, intensive care specialists and endocrinologists.

[Risky confirmation of a diagnosis: case series of three female patients with mediastinal mass syndrome]. / Riskante Diagnosesicherung: Fallserie über drei Patientinnen mit Mediastinal-mass-Syndrom.

Mediastinal mass syndrome (MMS) is a life-threatening complication of anesthesia for which prevention and treatment are a complication-prone interdisciplinary task. Clinical symptoms vary from asymptomatic patients up to life-threatening cardiorespiratory impairments, depending on the extent and size of a mediastinal tumor as well as the involvement of corresponding anatomical structures. Especially in the context of sedation or general anesthesia, there is a considerable risk of acute cardiopulmonary or respiratory decompensation related to tumor-induced compression of central blood vessels or even the large airways, which may result in severe complications, including death. In this case series three female patients are presented, who were each referred to this hospital with a mediastinal tumor for interventional or surgical confirmation of the diagnosis. Based on the case histories, characteristic complications are demonstrated and strategies to avoid possible adverse events of MMS are discussed. The specific anesthesiological requirements for MMS, the safety aspects of the choice of surgical and anesthesia procedures, circulatory and airway management for the required single-lung ventilation, and various aspects of the selection of the anesthetic agents are discussed in this case series.

[Giant Mature Cystic Teratoma Causing Loss of Consciousness before Surgery].

A 30-year-old woman who presented loss of consciousness was diagnosed as having large anterior mediastinal tumor. Computed tomography (CT) showed a 17.0×13.0×7.3 cm cystic mass with internal calcification in the anterior mediastinum that was markedly compressing the heart, great vessels, trachea and bronchi. A mature cystic teratoma was suspected, and the mediastinal tumor was resected through a median sternotomy. At the induction of anesthesia to prevent the development of the respiratory and circulatory collapse, the patient was consciously intubated under the right lateral decubitus position while preparing for percutaneous cardiopulmonary support by cardiac surgeons, and the surgery was safely performed. The tumor was pathologically diagnosed as a mature cystic teratoma, and symptoms such as loss of consciousness have disappeared.

Pericardial rupture of mediastinal mature cystic teratoma: An unusual cause of pediatric acute chest pain.

A previously healthy 12-year-old girl presented to the emergency department with severe chest pain and dyspnea that woke her from sleep. She had short-term syncope just before the emergency admission. On physical examination, respiratory rate was 26 breaths per minute while resting, and blood pressure was 92/56 mmHg. Other physical examination findings were insignificant. She had no past medical history, and her family history was unremarkable. Laboratory test results showed elevated C-reactive protein (27 mg/L; reference range: <5 mg/L) and white blood cell count (13.7 K/µL; reference range: 4-12 K/µL). Other laboratory test results were within normal limits, including troponin T value (3 ng/L; reference range: 3-14 ng/L). An electrocardiogram showed 1 mm ST-segment elevation in bipolar (D1 and D2) limb leads, and augmented vector foot leads, and echocardiography revealed a complicated pericardial effusion and a suspicious mass adjacent to the left ventricle.

Cavernous mediastinal hemangioma presenting with persistent cough: a rare case report and review of literature.

BACKGROUND: Cavernous hemangioma is a rare benign tumor which can sometimes mimic the clinical presentation and radiological findings of malignant tumors. Here we present a rare presentation of cavernous hemangioma in the mediastinum (CHM), along with a literature review among the main databases. CASE PRESENTATION: We present a 48-year-old male who had suffered from persistent cough as the sole symptom of an anterior CHM. Computed tomography scan demonstrated a 12.5 × 10.8 cm mass in the anterior mediastinum. The mass was surgically resected, and histopathological evaluation established the diagnosis of CHM. The patient was discharged in good condition, in which during his four-month follow-up period, no recurrence of the tumor has been observed. CONCLUSION: Although cavernous hemangioma rarely present in the mediastinum, it should be considered in the differential diagnosis of mediastinal tumors. However, our review of literature demonstrated a female dominance and average age of 40 years, with a 52% mortality rate based on previous reports.

A rare coexistence: Poland's syndrome and cardiac angiosarcoma.

Poland's syndrome, a rare genetic disorder that accompanies malignancies, musculoskeletal disorders, cardiac and genitourinary syndromes. There is no study that represents the association between cardiac angiosarcoma and Poland's syndrome. A 24-year-old female patient previously diagnosed with Poland's syndrome was admitted to our hospital complaining of dyspnea. Diagnostic imaging showed an irregular mass in the right atrial cavity. After successful surgery, she was discharged uneventfully and the 3rd month oncologic follow-up reveals none of residual mass. The coexistence has not been diagnosed and treated in a cardiac surgery department before. With this presentation, we aimed to contribute to the literature with this presentation, for the right and early diagnosis and management of possible new cases in the future can be diagnosed and treated correctly and early.