ABSTRACT
Background: Cholangiocarcinoma (CCC), a rare tumor arising from the viscera, has a poor prognosis. Although CCC is prone to metastasis, spread to the cranium and spine is exceedingly rare. Treatment for metastatic disease is palliative, with total resection of the primary lesion the only cure. We describe a case of metastatic CCC to the spine and cranium treated with surgical resection. Case Report: A 61-year-old male with a history of hepatitis C with liver transplant and incidental discovery of CCC presented with gradually increasing back pain. Physical examination revealed a palpable nontender mass in the parieto-occipital area. Computed tomography survey of the spine and head revealed mixed sclerotic and lytic lesions of the T9, T11, L2, and L5 vertebral bodies, a lytic lesion on the T6 vertebral body, and a 1.4-cm lesion in the right occipital calvarium. The patient underwent right occipital craniotomy for excisional biopsy of the calvarial mass with gross total resection and immunohistochemical confirmation of CCC. The patient was started on gemcitabine chemotherapy and radiation therapy for spinal metastases. Three months later, the patient died from metastatic disease complications. Conclusion: To our knowledge, only 6 cases of cranial CCC have been reported, and only 2 reported mixed cranial/spinal involvement. We report a rare case of CCC metastasis to the spine and cranium that was treated with surgery, chemotherapy, and radiotherapy. CCC should be considered an exceedingly rare etiology with treatment options aimed solely at palliation. This case supplements the existing literature to inform medical and surgical decision-making.
ABSTRACT
Background: Pleomorphic xanthoastrocytoma (PXA) is a rare and often focal glioma that most commonly affects children and young adults. Lesions are preferentially supratentorial and superficial, although infratentorial masses have been described, along with occasional involvement of the leptomeninges. The World Health Organization (WHO) categorizes these tumors as grade II, with surgical resection carrying a favorable prognosis. However, these tumors may undergo malignant degeneration and must be identified for appropriate treatment and prognosis. PXA has been associated with neurofibromatosis type 1 (NF1), although it is not the classic phenotype of NF1. We present a novel report of PXA, atypically located in the pineal region of a patient with a history of NF1. Case Report: A 17-year-old male with a history of NF1 presented with 1 month of bifrontal headaches. Magnetic resonance imaging was significant for a heterogeneous tectal mass, suspicious for a glioma extending to the fourth ventricle and causing displacement of the cerebral aqueduct without obstructive hydrocephalus. Following an infratentorial-supracerebellar approach for tumor resection, histopathology confirmed a low-grade variable neoplasm consistent with PXA. Postoperative imaging confirmed gross total resection with no evidence of recurrence at 9 months postoperatively. Conclusion: To our knowledge, this case is the fifth report of pineal PXA and the first associated with NF1. Because PXA presents similarly to other NF1-related intracranial tumors, careful diagnosis via immunohistochemistry is imperative. Gross tumor resection is usually curative; however, PXA has the propensity to undergo malignant degeneration and may require adjuvant treatment.
