Managing Life-Threatening Malignant Superior Mediastinal Syndrome in Pregnancy: When Benefits of Radiation and Chemotherapy Outweigh the Risks—A Case Report and Review of Literature

There is scarce literature on managing superior mediastinal syndrome during pregnancy. We report a case of 26-year-old primigravida who presented with life-threatening superior mediastinal syndrome at 32 weeks of gestation. The diagnosis was significantly delayed and, as a result, she reached the emergency with stridor and impending respiratory failure. She was diagnosed with primary mediastinal B cell lymphoma Lugano Stage II with a bulky mediastinal mass. She was treated with chemoimmunotherapy and underwent a preterm vaginal delivery after a week. She delivered a 1.6 kg healthy child with no malformations. Later, she completed three cycles of rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisolone and five cycles of dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, rituximab, followed by radiotherapy. She continues to be in remission at 18 months of follow-up. Delaying diagnostic imaging that involves ionizing radiation exposure and chemotherapy to avoid teratogenic and obstetric complications during pregnancy can adversely affect the prognosis in certain patients with high-grade malignancies. On the contrary, prompt multidisciplinary management can lead to a gratifying outcome.

Langerhans Cell Histiocytosis in an Infant Mimicking a Lymphoma at Presentation

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation and accumulation of clonal dendritic cells with varied clinical presentation and an unpredictable course. We report a 5-month-old infant with LCH who presented with severe respiratory distress, a large mediastinal mass, significant generalized lymphadenopathy, and hepatosplenomegaly. Lymphoma, especially T cell lymphoblastic lymphoma, can present with superior mediastinal syndrome needing urgent empirical therapy without biopsy. However, lack of response prompted a biopsy which confirmed it to be a case of LCH and that leads to appropriate therapy and survival. There have been reports of LCH presenting with isolated mediastinal mass or with generalized lymphadenopathy, but the combined presentation of generalized lymphadenopathy with large mediastinal mass, hepatosplenomegaly, and fever in an infant has rarely been reported.Conclusion. LCH should also be considered in the differential diagnosis of an infant presenting with generalized lymphadenopathy, mediastinal mass, hepatosplenomegaly, and fever.