cells positive for calretinin, WT-1, CK5/6, CK7(patchy), Ber-EP 4(patchy), p16(patchy). The patient was not an ideal candidate for cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) given thoracic and retroperitoneal involvement therefore, he was initiated on carboplatin, pemetrexed and bevacizumab. Discussion: Risk factors for MPeM include asbestos exposure, non-occupational (i.e., household, or residential) exposure, radiation exposure and BAP1 mutation. As MPeM is an extremely aggressive
disease with a vague clinical presentation, patients typically present in a more advanced stage. Cytoreductive surgery with HIPEC remains the standard therapy for operable MPeM. The mainstay of care for advanced pleural mesothelioma has been systemic chemotherapy using pemetrexed with cisplatin. Bevacizumab added to the pemetrexed- cisplatin combination improved overall survival and progression-free when compared to pemetrexed plus cisplatin. There is limited data for safety and efficacy of immune checkpoint (PDL1) inhibitors.
EMERGENCY! REALLY, IS IT? - SUPERIOR VENA CAVA SYNDROME IN T-CELL LYMPHOBLASTIC LYMPHOMA
Maithri Vallabhaneni MD, Nadjel Opamen MD, Sneha Puvvada MD, Sowbharnika Arivazhagan MD, Sanjay Juneja MD; Department of Medicine, Baton Rouge General Medical Center, Baton Rouge, LA.
Introduction: Superior vena cava syndrome (SVCS) develops in 2-4% of cases in non-Hodgkins's lymphoma. Patients with life-threatening symptoms of SVCS represent a true medical emergency. Accurate histologic diagnosis is essential before initiating tumor-directed therapy. Case: A 21-year-old female was admitted due to one month history of progressive facial, neck, and arm swelling associated with pain and dysphagia. She also complained of having heavy eyelids, particularly at the end of the day. Computed Tomography (CT) Angiogram of the chest revealed an anterior and superior mediastinal mass encasing the aortic branch vessels and descending aorta near the level of the arch. Magnetic Resonance Imaging revealed a large infiltrative anterior superior mediastinal mass (12.4 x 6.2 x 7.7 cm) extending to the lower neck and along the upper anterior pericardium. Differential diagnoses at that time included thymoma, lymphoma, and myasthenia gravis. A decision was made to empirically treat this patient with pyridostigmine per neurology recommendations but without improvement of symptoms. Upon obtaining a core biopsy, T-cell acute lymphoblastic lymphoma was discovered, with early T-cell precursor phenotype with <20% blasts and +CD3, CD34, CD7, and +/- TdT (~30% positive) negative for myeloperoxidase. Urgent
cytoreductive/decompressive radiation therapy was done to prevent critical SVCS. Abdomen and pelvis CT demonstrated no concern for metastatic disease. The patient was initiated on hyperCVAD (cyclophosphamide, vincristine, adriamycin, decadron), venetoclax and intrathecal methotrexate. On follow-up, patients reported vast improvement of symptoms with repeat CT chest revealing near complete resolution of mediastinal mass. Discussion: Historically, SVCS was considered a medical emergency necessitating acute intervention with irradiation even before determining the underlying pathology. While this is a reasonable approach in life-threatening manifestations of SVCS, such as cerebral edema or acute respiratory failure, recent studies have emphasized the need for histopathological identification taking precedence over the conventional radiotherapeutic approach in non-emergent cases. This is especially true in cases of chemo-sensitive sensitive cancers, such as NHL (as with our patient) and small cell cancer, which have shown complete regression of symptoms with chemotherapy alone, obliterating the need for unwarranted radiation. Endovascular interventions can be considered for refractory cases. A multidisciplinary approach and timely diagnosis is the most pressing need.
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