Recurrent pineal parenchymal tumor of intermediate differentiation with intratumoral hemorrhage: A case report and review of the literature

Abstract

Pineal apoplexy is a rare clinical condition. Its common symptoms include headaches, nausea, vomiting, ataxia, and gaze paralysis. These symptoms are mainly caused by obstructive hydrocephalus or direct compression of the cerebellum or midbrain. There have been no previous reports on the development of a recurrent pineal parenchymal tumor of intermediate differentiation (PPTID) with intratumoral hemorrhage. We report a case of PPTID with intratumoral hemorrhage. A 44-year-old woman developed recurrent PPTID following tumor removal and ventriculoperitoneal shunting in 2010. She visited the emergency department in April 2021 for sudden-onset dizziness and generalized weakness. Blurring of vision occurred and progressed over the previous month. Neurological examination revealed upward conjugate gaze paralysis. Brain computed tomography revealed a hyperdense lesion in the pineal region, and a recurrent tumor with hemorrhage was suspected. Magnetic resonance imaging of the brain confirmed a pineal tumor with intratumoral hemorrhage. The pineal tumor and hematoma were surgically removed via the suboccipital transtentorial approach. The patient was discharged from the hospital 2 weeks after the surgery. The pathological findings were consistent with the diagnosis of recurrent PPTID. PPTID is a rare tumor, accounting for less than 0.1% of primary central nervous system tumors. Pineal apoplexy is rare, and its incidence and clinical significance remain unclear. There have only been nine reported cases of pineal apoplexy, associated with pineal parenchymal tumors. The recurrence of PPTID with apoplectic hemorrhage after 10 years has not been reported. Despite its rarity, PPTID with apoplexy should be considered in patients with PPTID who develop sudden-onset neurological symptoms.

Optimal therapeutic strategies for pineal region lesions

“…Moreover, as previously reported, VPS can lead to a sudden decrease in intracranial hypertension (ICP) which may be the cause of intratumoral hemorrhages. Disturbance of the dynamic balance caused by ventricular tapping could lead to the increase in cerebral blood flow and vascular congestion and which could trigger intratumoral hemorrhages, particularly in highly vascularized tumors (26,27). We agree with this view.…”

“…Only one patient survived for more than 1 year. Some researchers believe that irradiation, tumor growth characteristics, hypertension, craniocerebral trauma, anticoagulant therapy, and idiopathic causes are the causes of intratumoral hemorrhage (23,26). Moreover, as previously reported, VPS can lead to a sudden decrease in intracranial hypertension (ICP) which may be the cause of intratumoral hemorrhages.…”