Primary intracranial choriocarcinoma in a very youngest child

Abstract

Primary Intracranial Choriocarcinoma (PICC) is a rare and very malignant tumor constituting < 2% of cases. 1 Varied clinical manifestations, challenges in establishing diagnosis especially if unresectable, along with absence of specific management algorithm make it unique among germ cell tumors.A three year old female child presented with history of headache, vomiting and fever for a month. Apart from these clinical symptoms, no other abnormality was detected; there was no pressure focal neurological deficit. Her hematological and biochemistry profile was normal. Magnetic resonance imaging (MRI) showed a heterogeneous mass lesion (25×26×17mm) in the posterior part of the third ventricle. Post contrast images showed moderate heterogenous enhancement with hemorrhage [Figure 1] causing dilatation of the supratentorial ventricular system with periventricular ooze noted. The tumor was partially excised through a transtentorial route. Further procedures deferred due to active bleeding. A vascular tumor composed of sheets of cells with highly pleomorphic nuclei and multi-nucleated giant cells (supplemental Fig S1) indicated choriocarcinoma. Immunohistochemistry was positive for cytokeratin and negative for placental alkaline phosphatase (supplemental figure S1). Immunoperoxidase using HCG antibodies showed the presence of brown granules in the cytoplasm, confirming the diagnosis. Her beta HCG ( -HCG) levels were raised (14087 MIu/ml) andfetoprotein (AFP) was normal. Whole body positron emission tomography scan showed no other metastatic or gonadal lesions. Cerebrospinal fluid (CSF) was negative for malignant cells. Following an incomplete surgical resection, the child was started on chemotherapy. Two cycles of chemotherapy with Cycle A (Cisplatin, Etoposide, Cyclophosphamide and Bleomycin) and cycle B (Carboplatin, Etoposide and Bleomycin) were administered. 2 Repeat MRI showed significant reduction in tumor size (13×10mm) with heterogeneous enhancement (supplemental figure S2). Her Beta HCG level was 350MIU/ml. Post chemotherapy, external beam radiotherapy (RT) to ventricle (local tumor site) with image guided radiation therapy at a dose of 4000 cGY in 20 fractions over 4 weeks given due to the local residual tumor.Following RT she received an additional four cycles of chemotherapy.An MRI after completion of eight cycles of chemotherapy and radiotherapy showed a subcentimeter fibrotic band (supplemental figure S3). Her -HCG level was < 2MIU/ml. She was treated successfully with combination chemotherapy, radiotherapy and partial surgery. During 2 years of follow-up, there has been no recurrence nor residual morbidity.There are no definitive guidelines for the management of this tumor. A combination of surgery, chemotherapy, and radiation therapy are known to improve survival. [3][4][5] When complete resection is a possibility, surgery should always be considered as the first line of management. 5-7 However, unresectable tumors as in our case can be started on chemotherapy followed by radiotherapy as this is chemosensi...