PEMBERTON - DR. LINO

Summary

Dr. Leno presents a clinical case of a 36-year-old driver with recurrent fever, non-productive cough, facial swelling, and significant weight loss over several months. The patient was diagnosed with Superior Vena Cava (SVC) syndrome due to a mediastinal mass, likely a germ cell tumor, based on imaging and elevated tumor markers. The diagnosis was confirmed through biopsy, and the patient is undergoing chemotherapy. Dr. Leno also highlights the grading system for SVC syndrome and discusses the challenges in diagnosing mediastinal masses, emphasizing the importance of tumor markers and clinical history.

Takeaways

• 🩺 Dr. Leno presents a clinical case of a 36-year-old male, Mr. Y, with recurrent fever for 7 months.
• 🌡️ Mr. Y's symptoms include low-grade intermittent fever, evening temperature rise, and mild expectoration with a persistent cough.
• 📉 He has experienced significant weight loss (10 kg in 6 months) and swelling of the face, especially in the morning.
• 🔍 Physical examination revealed prominent dilated veins on both sides of the neck, suggesting Superior Vena Cava (SVC) syndrome.
• 🦠 Differential diagnosis points towards a malignant cause, with possibilities including lung cancers, lymphomas, and germ cell tumors.
• 📊 The grading of SVC syndrome ranges from asymptomatic (Grade 0) to life-threatening (Grade 4), based on symptoms and complications.
• 🧪 Basic investigations showed normal blood counts, liver function, and electrolytes, but chest X-ray revealed mediastinal widening and a probable mass.
• 🧠 Differential diagnosis for the mediastinal mass includes thymoma, lymphoma, or germ cell tumors, depending on the tumor's location and characteristics.
• 🧬 Tumor markers such as Alpha-fetoprotein, Beta HCG, and LDH were elevated, suggesting a germ cell tumor as the likely diagnosis.
• 💉 The patient began chemotherapy, and further evaluation confirmed the diagnosis of a germ cell tumor with seminomatous components.

Q & A

What were the main symptoms presented by Mr. Y?

Mr. Y, a 36-year-old driver, presented with recurrent low-grade fever with chills, evening rise of temperature, a cough with mild expectoration for 7 months, facial swelling worsening in the morning, and a significant loss of weight (10 kg in 6 months).

What physical examination findings were noted in Mr. Y?

Physical examination revealed prominent dilated veins on both sides of the neck, facial swelling, and pleura. Other system examinations were normal, and the Pemberton sign was negative.

What syndrome was Mr. Y diagnosed with?

Mr. Y was diagnosed with Superior Vena Cava (SVC) syndrome, characterized by symptoms such as dilated neck veins and facial swelling.

What are the potential causes of SVC syndrome?

SVC syndrome can be caused by both malignant and non-malignant conditions. Malignant causes include non-small cell carcinoma of the lung, small cell lung carcinoma, lymphoma, metastatic cancers, germ cell tumors, and thymoma.

How is SVC syndrome classified based on severity?

SVC syndrome is classified into five grades: Grade 0 (asymptomatic), Grade 1 (head and neck edema), Grade 2 (edema with functional impairment), Grade 3 (cerebral and lingual edema), Grade 4 (life-threatening with hemodynamic compromise), and Grade 5 (death).

What were the key findings in Mr. Y’s chest x-ray?

Mr. Y’s chest x-ray showed mediastinal widening with a probable mediastinal mass.

What are the differential diagnoses for a mediastinal mass?

The differential diagnoses for a mediastinal mass depend on the compartment involved. In the anterior compartment, it could be thymoma, germ cell tumors, thyroid carcinoma, or lymphoma. In the middle compartment, bronchogenic cysts, lymphadenopathy, or esophageal tumors are considered. For the posterior compartment, neurogenic tumors or spinal lesions are possibilities.

What tumor markers were elevated in Mr. Y, and what diagnosis was considered?

Mr. Y had elevated tumor markers, including alpha-fetoprotein (328), beta-HCG (142), and LDH (762), leading to a diagnosis of germ cell tumor.

What is the prognosis for germ cell tumors?

Germ cell tumors have a high survival rate, with about 90% of patients surviving 5 years post-diagnosis, even without an initial biopsy.

What treatment was initiated for Mr. Y’s condition?

Mr. Y was started on chemotherapy (Cytin Plate B), and a fine needle aspiration cytology (FNAC) confirmed the diagnosis of a germ cell tumor with seminoma components.