Childs Nerv Syst (2003) 19:769–772 DOI 10.1007/s00381-003-0800-7

Tai-Tong Wong Sang-Hue Yen Donald M. Ho Feng-Chi Chang Kai-Ping Chang

Received: 19 April 2003 Published online: 29 July 2003 © Springer-Verlag 2003 T.-T. Wong (✉) Division of Pediatric Neurosurgery, Neurological Institute, Taipei Veterans General Hospital and National Yang Ming University School of Medicine, VACRS No. 210 Sec 2, 11217 Shih-Pai, Taiwan, Republic of China e-mail: [email protected] Tel.: +886-2-28757587 Fax: +886-2-28757587 S.-H. Yen Cancer Center, Taipei Veterans General Hospital and National Yang Ming University School of Medicine, VACRS No. 210 Sec 2, 11217 Shih-Pai, Taiwan, Republic of China D. M. Ho Department of Pathology, Taipei Veterans General Hospital and National Yang Ming University School of Medicine, VACRS No. 210 Sec 2, 11217 Shih-Pai, Taiwan, Republic of China

C A S E R E P O RT

Pineal germinoma with intratumoral hemorrhage after neuroendoscopic tumor biopsy

F.-C. Chang Division of Neuroradiology, Department of Radiology, Taipei Veterans General Hospital and National Yang Ming University School of Medicine, VACRS No. 210 Sec 2, 11217 Shih-Pai, Taiwan, Republic of China

pressure to zero during the endoscopic procedure are discussed. Keywords Pineal tumor · Germinoma · Hydrocephalus · Neuroendoscopy · Intratumoral hemorrhage · Long-tract EVD

K.-P. Chang Department of Pediatrics, Taipei Veterans General Hospital and National Yang Ming University School of Medicine, VACRS No. 210 Sec 2, 11217 Shih-Pai, Taiwan, Republic of China

Abstract Case report: We report an intratumoral hemorrhage immediately after a ventricular endoscopic procedure in an 18-year-old man who had a pineal germinoma with symptomatic hydrocephalus. The patient was successfully treated using long tract external ventricular drainage and urgent radiation therapy. Discussion: The contributing factors for the acute reduction of intracranial

Introduction Tumoral hemorrhage after ventricular shunting has been reported and is believed to be the result of a sudden decrease in the elevated intracranial pressure (ICP) during the shunting procedure [4, 23]. Reported histological features of tumoral bleeding include tumor necrosis, vesselwall hyalinization, degeneration or necrosis of vessel walls, thrombosis, and the presence of many thin-walled vessels and ruptured vessels [11]. Pineal tumors are more common in children and juveniles, especially in Northeast

Asia [2, 3, 16]. Most of these tumors obliterate aqueduct causing obstructive hydrocephalus and presenting clinical symptoms of increased intracranial pressure (IICP) [3]. The majority of tumors in the pineal region are radiosensitive and chemosensitive [8, 15, 16]. A minimally invasive approach along with adjuvant treatment rather than extensive surgical excision is preferred. Although controversial, endoscopic third ventriculostomy and tumor biopsy is one of the preferential modalities for their initial management [17, 18]. Acute reduction of ICP to zero is inevitable during the ventricular endoscopic approach and this may ini-

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Fig. 1 a Preoperative unenhanced CT scan showing the hyperdense pineal tumor with calcifications. b Unenhanced CT scan 1.5 h after endoscopic tumor biopsy showing intratumoral hemorrhage and acute expansion of the lesion

tiate tumoral bleeding in the pineal region, similar to the mechanism of ventricular shunting [14]. We report a case of a pineal germinoma in which intratumoral hemorrhage was found during and immediately after a neuroendoscopic approach. The patient was successfully treated using long subcutaneous tract external ventricular drainage (EVD) and urgent radiation on the tumor.

Case report An 18-year-old young man was admitted because he had been suffering with headache for 2 months. He had also had intermittent vomiting, blurring of vision, diplopia, and unsteady gait for 2 weeks. Neurological examination revealed clear consciousness with papilledema, upward gaze paralysis (Parinaud’s sign), left abducent nerve palsy, and unsteady gait (shifting to left). Brain CT showed a pineal mass with obstructive hydrocephalus and freckles of calcification (Fig. 1a). MRI showed a heterogeneous mass with multiple tiny cysts occupying the pineal region with infiltration into the midbrain. Serum tumor marker studies for alpha-fetoprotein (AFP) and beta subunit of human chorionic gonadotropin (b-HCG) were within reference ranges. Clinically, germinoma of the pineal region was highly considered. We tried to perform endoscopic third ventriculostomy along with tumor biopsy on 27 December 2002. After ventricular puncture and insertion of a peel away introducer to the frontal horn of the right lateral ventricle, endoscopically, we could see fresh blood coating the tumor in the posterior aspect of the third ventricle. We stopped the third ventriculostomy procedure. Endoscopic biopsy of the tumor was performed using a 1-mm microbiopsy forceps. A long-tract EVD was placed for the drainage of cerebral spinal fluid (CSF). The drainage catheter was exteriorized through the lateral aspect of the right side abdominal wall and then connected to a drainage system. Intracranial pressure was measured and monitored after the operation. His consciousness deteriorated in the postoperating recovery room (POR) about 1.5 h after surgery. Emergency brain CT showed intratumoral hemorrhage with acute expansion of the tumor (Fig. 1b). Through an emergency transoccipital and transtentorial approach, we tried to remove the tumor. As the dura was opened, we observed diffused subarachnoid hemorrhage. Loose and soft tumor tissue was seen as the tentorium was split for 2 cm. However, the attempt at extensive tumor removal failed because of profuse tumor bleeding. Only a tumor biopsy was performed. Urgent radiation to the tumor site

Fig. 2 a Photomicrograph shows germinoma with large, thin-walled blood vessel and extravasation of blood cells. b In the vicinity of the bleeding area, there are fibrin and platelet depositions as well as degeneration of tumor cells (H&E stain, original magnification, ¥200)

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with the help of anesthesiologist was given during the morning after surgery and then daily. The patient regained consciousness on the 4th postoperative day and was weaned off controlled ventilation. We continued the radiation for 2 weeks. After local radiation of 2,000 cGy/10 fractions/2 weeks, the follow-up brain MRI showed a marked decrease in tumor size but there was still evidence of hemorrhage within the tumor. Spinal MRI demonstrated a teardrop metastasis in the lumbosacral region. Histopathologic examination showed a germinoma consisting of sheets of large and round neoplastic cells interspersed with scattered lymphocytes. There were many large, thin-walled blood vessels in the tumor. Evidence of recent hemorrhage with extravasation of blood cells, fibrin, and platelet depositions, as well as degeneration of tumor cells, were noted (Fig. 2). No other germ cell tumor components were identified. The treatment was then changed to craniospinal axis irradiation (CSI) with a boost to the primary tumor site and spinal metastasis area. Six courses of chemotherapy with the vinblastine, bleomycin, cisplatin, and etoposide (VPBE) regimen will be given after radiation treatment.

Discussion Brain tumors with spontaneous hemorrhage have been reported in adults and children [11, 12, 13, 24]. Astrocytic tumors are the most common primary brain tumors with bleeding reported in the literature [11, 13]. Hemorrhage into medulloblastomas [12] and pineal tumors including germ cell tumors and pineal parenchymal tumors (pineocytomas/pineoblastomas) have also been reported [6, 9, 20]. Common histological features of tumors that bleed include tumor necrosis, increased vascularization, the presence of many thin-walled vessels, ruptured vessels, thrombosis, vessel-wall hyalinization, and degeneration or necrosis of vessel walls [11, 13]. In this report, the patient showed almost immediate acute apoplectic deterioration after the ventricular endoscopic procedure. Gross intratumoral hemorrhage with acute tumor expansion was seen on the emergency CT scan. Microscopically, large, thin-walled blood vessels were seen in the germinoma, which also showed changes related to recent hemorrhage, i.e. extravasation of blood cells, fibrin and platelet deposition, and degeneration of tumor cells. The above findings were similar to those reported previously in cases of intratumoral hemorrhage [11, 13]. However, there was no trace finding of recent or old tumor hemorrhage on the preoperative CT and MRI. Tumoral hemorrhage after ventricular shunting has been reported and is believed to be the result of a sudden decrease in the elevated ICP. The subsequent increase in the transmural pressure of the intratumoral vessels may be responsible for the hemorrhage [4, 23]. We believe that the immediate spontaneous intratumoral hemorrhage during and after ventricular endoscopic procedure in our patient was due to these factors: 1. Loose tumor tissue 2. Hypervascularization of the tumor with large, thinwalled vessels

3. Abrupt decrease in intracranial pressure to zero after ventricular puncture and the insertion of the endoscopic peel away introducer Histologically, the majority of pineal region tumors are germ cell tumors and pineal parenchymal tumors [8, 16]. For germ cell tumors in the pineal region with elevate serum titer of AFP and/or bHCG, the treatment is less controversial. We can first apply a combination of chemo- and radiotherapy and lastly resect the residue teratoma [5, 22]. For pineal region tumors with normal serum titer of AFP and b-HCG, it is important to differentiate between germinoma and pineal parenchymal tumors, especially pineoblastomas. The clinical malignancy and hence the adjuvant treatment for pineal germinomas and pineal pineoblastomas are different [10, 15, 19, 21]. For pineal germinomas without perioperative CSF metastasis, radiation therapy will be limited to the tumor area [15]. For pineoblastomas, radiation therapy should be to the craniospinal axis with a boost to the tumor site [10, 19]. Although it is controversial due to the possibility of initiating tumor spreading [7] or tumor bleeding, neuroendoscopic third ventriculostomy along with tumor biopsy has been a reported clinical practice for pineal region tumors with symptomatic hydrocephalus [17, 18]. Intratumoral hemorrhage after ventricular shunting for pineal tumors has been reported [6, 14, 23], but it has not been a concern in ventricular endoscopic procedures (third ventriculostomy and/or tumor biopsy). In this paper, we reported an infiltrating tumor in the pineal region with obstructive hydrocephalus. Serum titers for AFP and bHCG were within the reference range. The reasonable management should be endoscopic third ventriculostomy along with tumor biopsy [17, 18] or endoscopic tumor biopsy along with long subcutaneous tract external ventricular drainage [9]. Long tract EVD is beneficial for prolonged CSF drainage without catheter change and limits the problem of infection [1, 25]. However, one of the inevitable results of the ventricular endoscopic approach is the abrupt decrease in intracranial pressure to zero, a condition even worse than shunting of a tumor with hydrocephalus. We should be aware that in cases of pineal tumors with symptomatic hydrocephalus, perioperative tumoral hemorrhage after shunting or ventricular endoscopic procedure may occur. Acute apoplectic intratumoral bleeding after shunting is catastrophic [9, 14, 23]. Because the pineal germinoma is very radiosensitive, we succeeded in saving this particular patient by using long subcutaneous tract EVD and emergency radiation therapy.

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