Described here are the clinical features and results of treatment in a 10-year-old Saudi Arabian girl with primary T-cell lymphoma of the central nervous system. At presentation the patient had nystagmus and ataxia. The diagnosis was established by tissue biopsy obtained from the cerebellum. Therapy included cranio-spinal irradiation and combination chemotherapy of a systemic high dose of methotrexate, cytosine, arabinoside, and L-asparaginase. Remission was obtained easily but was interrupted by a local intracranial relapse 57 months after diagnosis (37 months after cessation of therapy; at present the patient is still alive and receiving palliative treatment). This report is warranted because of the rarity of this condition in children.
Introduction
Primary hepatic neuroendocrine tumours (PHNETs) are a rare form of hepatic neoplasms, and it is difficult to differentiate them from common hepatic malignancies in routine imaging studies.
Presentation of the case
We describe the case of a 60-year-old Indian male patient with a tentative preoperative diagnosis of hepatocellular carcinoma (HCC). Nevertheless, the definitive post-operative diagnosis was made by Histopathological and immunohistochemical assessment, which revealed a grade II neuroendocrine tumour (NET) of moderate differentiation. Surgical resection was performed through a minimally invasive approach with a favourable postoperative course and a short hospital stay. One-month Post-operative Octreotide scan showed no extrahepatic primary origin of the tumour.
Discussion
PHNET is a rare entity, and multi modalities investigations, including imaging, serology, endoscopy series, and histopathology findings, aside from long-term follow-up to rule out another primary origin, are essential for the final diagnosis of PHNET. Surgical resection stands as the mainstay of treatment of PHNETs.
Conclusion
The absence of primary liver diseases should expand our possible differential diagnosis. Laparoscopic surgical resection of PHNETs carries a favourable outcome.
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