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Download PDF File ORIGINAL PAPER/ARTYKU£ ORYGINALNY Rare primary tumours of the hypothalamus in adults: clinical course and surgical treatment Rzadkie pierwotne guzy podwzgórza u osób doros³ych: przebieg kliniczny i leczenie chirurgiczne Krzysztof Majchrzak1, Gra¿yna Bierzyñska-Macyszyn2, Barbara Bobek-Billewicz3, Henryk Majchrzak1, Piotr £adziñski1 1Department of Neurosurgery in Sosnowiec, Medical University of Silesia in Katowice, Poland 2Section of Pathomorphology, Medical University of Silesia in Katowice, Poland 3Radiodiagnostics Department, Comprehensive Cancer Centre, Maria Sklodowska-Curie, Memorial Institute Branch, Gliwice, Poland Neurologia i Neurochirurgia Polska 2010; 44, 6: 546–553 Abstract Streszczenie Background and purpose: The paper presents the operative Wstêp i cel pracy: W artykule przedstawiono technikê chirur- technique and the results of treatment of adult patients with giczn¹ oraz wyniki leczenia operacyjnego doros³ych chorych primary tumours of the hypothalamus, including rare ones. z pierwotnymi, w niektórych przypadkach rzadkimi, guzami The aim of the study was to show the possibility of safe sur- podwzgórza. Celem pracy jest pokazanie mo¿liwoœci bez- gical treatment of rare tumours of the hypothalamus through piecznego leczenia chirurgicznego pierwotnych, rzadko spo- a bifrontal basal interhemispheric trans-lamina terminalis strzeganych guzów podwzgórza przez blaszkê graniczn¹ approach. z dostêpu dwuczo³owo-podstawnego, miêdzypó³kulowego. Material and methods: Five patients with tumours of the hypo- Materia³ i metody: W latach 1990–2008 w Klinice Neuro- thalamus were operated on in the Neurosurgical Clinic in chirurgii w Sosnowcu operowano 5 pacjentów z guzami Sosnowiec between 1990 and 2008. There were 2 patients with zlokalizowanymi w przedniej czêœci podwzgórza, w tym craniopharyngiomas located exclusively in the third ventricle, 2 pacjentów zczaszkogardlakiem ograniczonym tylko do and single patients with gemistocytic astrocytoma, Langerhans komory III mózgu i po jednym pacjencie z gwiaŸdziakiem cell histiocytosis X and hamartoma of the hypothalamus each. tucznokomórkowym, histiocytoz¹ X oraz odpryskowcem pod- The patients were treated surgically with a bi frontal basal inter- wzgórza. Chorzy byli operowani z dostêpu dwuczo³owo- hemispheric trans-lamina terminalis approach. In two cases, -podstawnego, miêdzypó³kulowego przez blaszkê graniczn¹. the neuronavigation system with the use of tractography (DTI) W 2 przy padkach zastosowano system neuronawigacji was used to determine the location of the lamina terminalis, the z wykorzystaniem traktografii (DTI), co umo¿liwi³o zlokali- posterior surface of the optic chiasm and the optic tracts. zowanie blaszki granicznej, tylnej powierzchni skrzy¿owania Results: All lesions were resected totally, except for partially nerwów wzrokowych oraz obustronnie pasma wzrokowego. resected hamartoma of the hypothalamus. The most common Wyniki: Guz o charakterze odpryskowca podwzgórza usu- postoperative complication was diabetes insipidus, which was niêto czêœciowo, pozosta³e guzy ca³kowicie. Najczêstszym transient in two cases. A long-lasting follow-up of all the powik³aniem obserwowanym po operacji guzów podwzgórza patients operated on did not reveal regrowth of the lesion. by³a moczówka prosta (w 2 przypadkach przemijaj¹ca). Correspondence address: Krzysztof Majchrzak, plac Medyków 1, 41-200 Sosnowiec, Poland, phone +48 32 368 20 24, fax +48 32 368 25 50, e-mail: [email protected] Received: 28.03.2010; accepted: 24.09.2010 546 Neurologia i Neurochirurgia Polska 2010; 44, 6 Rare primary tumours of the hypothalamus in adults Conclusions: The bifrontal basal interhemispheric trans-lami- D³ugotrwa³a obserwacja chorych nie wykaza³a wznowy pro- na terminalis approach allows for radical resection of prima- cesu ekspansywnego. ry tumours of the hypothalamus while avoiding serious post - Wnioski: Dostêp dwuczo³owo-podstawny, miêdzypó³kulowy operative deficits. This approach enabled the preservation of przez blaszkê graniczn¹ umo¿liwi³ radykaln¹ resekcjê pier- the olfactory bulb and tract and prevented damage of the fron- wotnych guzów podwzgórza bez powodowania powa¿nych tal lobes. The use of DTI helped to establish the location and powik³añ pooperacyjnych. U³atwi³ tak¿e zachowanie opusz- borders of the lamina terminalis, to establish the posterior ki i pasma wêchowego oraz zapobieg³ uszkodzeniu p³atów surface of the optic chiasm and the optic tracts, and to save czo³owych w trakcie operacji. Dziêki wykorzystaniu neuro- the anterior and lateral wall of the hypothalamus. nawigacji wraz z DTI ³atwiej by³o ustaliæ granice blaszki gra- nicznej i tyln¹ powierzchniê skrzy¿owania nerwów wzroko- Key words: primary hypothalamic tumours, clinical signs, wych, po³o¿enie pasm wzrokowych oraz zaoszczêdziæ surgical treatment, bifrontal basal interhemispheric trans- przednie i boczne czêœci podwzgórza. lamina terminalis approach, DTI, outcome. S³owa kluczowe: pierwotne guzy podwzgórza, objawy klinicz- ne, leczenie chirurgiczne, dostêp dwuczo³owo-podstawny, miê - dzypó³kulowy przez blaszkê graniczn¹, DTI, wyniki leczenia. Introduction Below, we present the operative technique we applied in the surgical treatment of tumours of the hypothala- Tumours of the hypothalamus are relatively rare, and mus, the clinical course of the condition in brief, as well half of the cases occur in childhood. The most common as a diagnosis for five adult patients with primary primary lesions of this area include gliomas (68.6%), tumours of the hypothalamus who were treated in the germinomas, and – less frequently – teratomas (alto- Department of Neurosurgery in Sosnowiec. gether 22.9%) [1]. The third ventricle is secondarily penetrated by craniopharyngiomas which compress the Material and methods hypothalamus [2-5]. Only rarely do craniopharyn- giomas develop exclusively in the third ventricle [6]. Operative technique Primary tumours of the hypothalamus cause endocrinological and thermoregulation disorders, The patient is placed in a supine position. The apex dysorexia and visual field defects. Sometimes they lead of the head is then lowered by 15° to 20° in relation to to non-communicating hydrocephalus, obstructing both the horizontal body. A skin incision is made in a binau- foramina of Monro [1]. ricular line, starting at 1 cm in front of the tragus and In this paper we present five patients with primary along the hair line. After raising the skin flap we dissect tumours of the hypothalamus who were treated surgi- the pericranial flap, which is sandwiched between two cally by means of the bifrontal basal interhemispheric sponges and kept moist with saline during the proce- trans-lamina terminalis approach. dure. The bifrontal craniotomy should be performed The interhemispheric approach allowing access to as close to the roof of the orbit as possible. The bone lesions penetrating the third ventricle, the region around flap is removed in one piece. The posterior wall of the septum pellucidum or lateral ventricles from the the deroofed frontal sinus is removed with a rongeur. suprasellar region has been described previously by The entire mucosa of the frontal sinus should be removed. other authors [2,7,8]. Modifications to this approach The dura of the brain should be incised as close to the which make it possible not to traumatize the frontal base of the anterior fossa as possible. The superior sagit- lobes, maintain olfactory tracts and expose the suprasel- tal sinus is ligated and incised. The falx cerebri is incised lar region have already been described by several neu- lengthways. Then we dissect both olfactory tracts using rosurgeons, of whom Dehdashti and de Tribolet made the existing subarachnoid space. The olfactory bulbs are a particularly thorough description in 2005 [4-6,9]. also dissected. Afterwards, we expose both optic nerves, However, the majority of descriptions found in the lite - the optic chiasm, both anterior cerebral arteries in their rature of the approach and related to the surgical treat- A1 and A2 sections, and the anterior communicating ment of tumours of the hypothalamus have been con- artery (ACoA). Above the optic chiasm we identify the fined to resections of craniopharyngiomas [4-6,8,9]. lamina terminalis, which constitutes the anterior-infe- Neurologia i Neurochirurgia Polska 2010; 44, 6 547 Krzysztof Majchrzak, Gra¿yna Bierzyñska-Macyszyn, Barbara Bobek-Billewicz, Henryk Majchrzak, Piotr £adziñski rior limitation of the third ventricle, extending from the rior surface of the optic chiasm, we try to separate anterior commissure to the posterior surface of the optic sharply these two formations. Sometimes the lesion chiasm, and laterally bordering with the optic tract on reaches as far as to the floor of the third ventricle. In this the left and right side. It sometimes happens that access case, it is essential to remove the lesion very carefully, as to the lamina terminalis is hindered by the ACoA com- any damage done to the hypothalamus, particularly to plex. In such a case, we try to carefully dissect free this the mammillary bodies, may result in a cerebral coma area and change the position of the ACoA. In autopsy or temporary consciousness disorders. After the removal studies, Italian authors did not find it necessary to of the tumour, we tightly close the dura mater so that mobilise the anterior cerebral artery-ACoA complex in the cerebrospinal fluid cannot leak out. The wall of the order to achieve free access to the lamina terminalis frontal sinus is covered with the periosteal
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