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.
We herein report a rare case of malignant mixed germ cell tumor comprising both an embryonal carcinoma (60%) and a yolk sac tumor (40%). A 7-year-old girl presented to the emergency department with persistent vaginal bleeding, abdominal pain, and vomiting. A CT scan of the pelvis and abdomen revealed a huge pelvic mass, 16 × 16 × 8 cm, arising from the left ovary and extending into the upper abdomen and displacing bowel loops to the sides. The mass was lobulated and heterogeneously enhancing. Initial investigations revealed raised values of beta-human chorionic gonadotrophin (B-hCG) and alpha-fetoprotein (AFP). She underwent a laparotomy and a left salphingo-oophorectomy. Histopathology of the excised mass revealed a malignant mixed germ cell tumor comprising both an embryonal carcinoma (60%) and a yolk sac tumor (40%). Following surgery, she was started on adjuvant chemotherapy based on the MAKEI 96 protocol and was given 4 cycles of PEI chemotherapy (cisplatin, etoposide, and ifosfamide). Multidisciplinary team management consisting of careful initial surgery with adequate staging biopsies followed by combination chemotherapy and endocrinological evaluation and treatment can greatly improve the prognosis of these patients.
Hepatocellular carcinoma (HCC) is the fifth most common malignancy found in men and ninth most common in women, out of which 72.5% reported cases are from Asia. In children, it accounts for <2% cases worldwide with even rarer incidence of 1.2% involving intracardial extension. However, it presents with a high mortality rate with mean survival ranging from 1 to 4 months. The present case is an extremely rare case of intracardiac extension of HCC in a 3.5-year-old Asian girl with no history of hepatitis B infection presented at an advanced stage of HCC who succumbed within one month of presentation to the hospital.
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