Atypical Teratoid/Rhabdoid Tumor: Surgery



Fig. 40.1
Intraoperative view (prone position) of an AT/RT of the left cerebellar hemisphere. The huge tumor inflates the left cerebellar hemisphere (the normal architecture of its folia disappeared) and compresses the right one. The midline cannot be visualized. Note the dishomogeneous appearance of the mass, with the hemorrhagic area along its lower aspect



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Fig. 40.2
Typical appearance of a midline AT/RT during a piecemeal excision (prone position). The tonsils are divaricated and the vermis upward displaced. The tumor appears as a reddish, soft, and bleeding mass. These features make AT/RT similar to medulloblastoma


Tumor resection is usually carried out under magnification. The first step consists of a careful microdissection of the tumor away from the surrounding cerebellar tissue. Although AT/RT is easily distinguished from the normal tissue, appearing as a reddish pink (or gray), richly vascularized mass, an obvious interface with the surrounding neural structures may be lacking because of its infiltrative growth. The peripheral supplying vessels are progressively coagulated to reduce the bleeding from the tumor. Since such a bleeding is often profuse, the visual intraoperative control of the major arteries providing the vascular supply (namely, PICA) is recommended. Therefore, both PICAs should be identified at the beginning of the procedure prior to attempt the tumor excision. When a demarcation plane from normal tissue is completely missing, the mass removal has to start from inside the neoplasm. The tumor excision is carried out in a piecemeal fashion as the tumor size and infiltration rarely allow the surgeon to perform an “en bloc” resection (Fig. 40.2). The piecemeal removal, however, favors the tumor bleeding, and, considering that this bleeding usually stops only when the resection is completed, the removal should be realized as quickly as possible using both microsurgical instruments and ultrasonic aspirator. At the end of the procedure, the walls of the surgical field are carefully checked looking for possible tumor remnants or sources of bleeding. During this step, self-retaining retractors can be used to improve the visualization of the residual surgical cavity. We actually utilize retractors only during the late phases of cytoreduction in order to avoid unnecessary retraction and stretching of the cerebellar structures which may result in further edema when applied on the normal tissues already compressed by the tumor itself at the beginning of the operation.

The involvement of brainstem and cranial nerves is one of the most critical steps of AT/RT excision. When dealing with AT/RT located within the fourth ventricle, it is recommended to dissect the tumor up to visualize the ventricular floor before starting the tumor reduction; such a maneuver allows the surgeon to use the ventricular floor as a marker to avoid injury to the brainstem. When the superficial portion of the mass has been removed, the tumor is progressively excised starting from the inferior portion (close to the cervical region) and proceeding cephalad until the Sylvius’ aqueduct is visualized, opened (if needed), and protected with Gelfoam; finally, the tumor can be gently detached from the fourth ventricle floor trying to reduce its manipulation as well as bipolar cautery on the ventricular surface as much as possible. In case of infiltration of the floor (which is not infrequent), attempts to follow the tumor inside the brainstem should be avoided to decrease the risk of perioperative and postoperative damages (namely, cardiorespiratory impairment, VI and VII cranial nerve palsy, vomiting syndrome) (Fig. 40.3). Similarly, a cautious dissection of the tumor from the lower cranial nerves is recommended unless a clear infiltration of nerve sheath or a severe encasement of the structures of the CP angle is present (Fig. 40.4). Such a dissection may result very hard as the anatomy is often completely distorted. For this reason, it is advisable to separate the tumor from the cranial nerve proceeding from laterally to medially, trying to individuate as many marker points as possible (e.g., internal acoustic foramen to preserve VII and VIII cranial nerves, jugular foramen to preserve IX and X cranial nerves). The dissection of the tumor from the cranial nerve carried out from the medial surface of the brainstem, indeed, is more likely to result in cranial nerve loss because of the usually severe encasement at this level.

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Fig. 40.3
Intraoperative view of a brainstem infiltration (prone position). After removal of the tumor, which involved the IV ventricle extending into the right CPA, an extensive infiltration of the right lateral surface of the brainstem is evident (arrows)


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Fig. 40.4
AT/RT of the left CPA. Intraoperative view (prone position) of the tumor dissection and detachment from the lower cranial nerves. The medial portion of the tumor is still adherent to the brainstem



40.4.4 Closure


After irrigation of the surgical field with saline solution to reduce the inflammatory reaction and to counterbalance the cerebellar collapse, the dura is watertight closed. Dural substitutes or muscular fascia is utilized for a duraplasty in case of shrinking of the autologous dura mater. Afterwards, the bone flap is replaced and secured with reabsorbable plates and screws or silk sutures. The bone replacement prevents postoperative cerebellar ptosis and ensures protection to the PCF content. Cervical and nuchal muscles are then reconstructed and carefully closed to reduce the risk of CSF leakage. Such a complication and the following risk of CSF infection, indeed, prolong the postoperative course delaying the adjuvant treatments that are mandatory for children with such a dismal prognosis like those with AT/RT. Finally, subcutaneous sheet and skin are sutured in separate layers.

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Jun 22, 2017 | Posted by in NEUROSURGERY | Comments Off on Atypical Teratoid/Rhabdoid Tumor: Surgery

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