Extremely rare presentation of Frantz's tumour: synchronous localisation in the pancreatic head and tail.

INTRODUCTION: Solid pseudopapillary tumour of the pancreas is a rare lesion with low malignant potential occurring predominantly in young women. This is a report of an extremely rare occurrence of synchronous presentation of pseudopapillary tumour in the pancreatic head and tail of a 16-year-old female patient. CASE PRESENTATION: The patient presented with a three-month intermittent upper abdominal pain and swelling. Computed tomography scan showed two separate masses, involving the pancreatic head and tail. The patient underwent surgery, where successful tumour enucleation of both tumours was performed. Histological report confirmed solid pseudopapillary tumour of the pancreas with the low malignant potential. CONCLUSION: To the best of our knowledge, this is the first report of synchronous presentation of pseudopapillary tumour of the pancreas.

Anti-CD20 monoclonal antibody (rituximab) for therapy of CD20-positive nodular lymphocyte-predominant Hodgkin lymphoma in an 10-year-old girl.

Biologic treatments including antibody-based therapies are still in early-phase development in Hodgkin lymphoma. The authors present the case of a 10-year-old girl with massive, solid, unilateral cervical, nodular lymphocyte-predominant Hodgkin lymphoma. Chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine [ABVD]) and radiotherapy were given, according to the Italian Association of Pediatric Hematology and Oncology (AIEOP) MH-96 study protocol, but the patient failed to enter complete remission. Soon after, 6 intravenous infusions of the chimeric anti-CD20 monoclonal antibody rituximab 375 mg/m2 were administered, resulting in complete remission. The patients is still in continuous complete remission for 2 years. Novel therapies, such as rituximab, may be useful for children with CD20+ nodular lymphocyte-predominant Hodgkin lymphoma. To the authors' knowledge, this is the first report of CD20+ nodular lymphocyte-predominant Hodgkin lymphoma treated with rituximab in children. Further controlled trials and long-term outcome studies are warranted to define its clinical application and to improve the care of patients.

Disseminated BCG infection resembling langerhans cell histiocytosis in an infant with severe combined immunodeficiency: a case report.

We present a very rare congenital immunologic disease, severe combined immunodeficiency syndrome (SCID) in 6-months-old-boy with prolonged mucocutaneous candidiasis, severe anaemia, skin rash similar to the infiltrative eczema of Langerhans cell histiocytosis (LCH) and subcutaneous nodules with histiocytic infiltration. Laboratory findings show profound absence of humoral and cell-mediated immunity. Pathology specimens analysis of subcutaneous nodule revealed numerous S-100 protein and Cd1a negative histiocytes, occupied by BCG intracellular growth. Histopathology and immunohistochemistry confirmed the diagnosis of BCG dissemination. BCG vaccination in infants with SCID can lead to life threatening dissemination, resembling to the infiltrative eczema of LCH and may mislead the clinician.