Pineal Dermoid Cyst Developing 18 Years After Gross Total Removal of a Pineal Mature Teratoma

Pineal Dermoid Cyst Developing 18 Years after Gross Total Removal of a Pineal Mature Teratoma

— Case Report—

Tatsuo MORIMURA, Hiroaki KUBO, juji TAKEUCHI, and Ba JII

Department of Neurosurgery, National Utano Hospital, Kyoto

Abstract

A 21-year-old male presented with a pineal dermoid cyst manifesting as headache and diplopia. He had undergone gross total removal of a pineal mature teratoma 18 years before and had done well until re cently. Diffusion-weighted magnetic resonance imaging showed a region of high signal intensity due to a round mass in the pineal region and extending into the trigone of the right lateral ventricle. Subtotal excision of the tumor was achieved. Histological examination showed an epidermoid cyst consisting of keratinized stratified squamous epithelium and connective tissue. Intraoperative observation had de tected black hairs, so the diagnosis was dermoid cyst. The dermoid cyst may have arisen from a microscopic remnant of the cyst wall of the original pineal mature teratoma. The regrowth of a dermoid cyst 18 years after gross total removal of a mature cystic teratoma in the pineal region is exceptional. However, careful follow-up of patients who undergo gross total removal of a pineal teratoma is recom mended for a period more than that of the patient's age at surgery plus 9 months.

Key words: pineal dermoid cyst, diffusion-weighted magnetic resonance imaging, pineal mature teratoma

Introduction ture cystic teratoma.16)

The pineal region is the most common site for the oc Case Report currence of intracranial germ cell tumors. The most frequent types are germinomas (55.6%) followed by A 21-year-old right-handed male student presented teratomas (16.3%),3) but others include gliomas, complaining of headache and diplopia persisting for pineoblastomas, pineocytomas, choriocarcinomas, 3 months. He had suffered from acute intussuscep epidermoids, and dermoids. Intracranial teratomas tion at the age of 7 months. He underwent surgery at are rare and comprise only 0.5% of all intracranial age 3 years for a pineal cystic teratoma at a univer tumors. The frequency is higher in Japan, like those sity hospital, after transfer by ambulance when semi of germ cell tumors in general. Intracranial terato comatose with left hemiparesis. Gross total excision mas account for 2.6% of intracranial tumors in was carried out after emergent implantation of a ven Japan in children younger than 14 years of age.3) triculoperitoneal shunt. Postoperative computed There is a male preponderance during the first two tomography confirmed gross total removal of the decades of life. The prognosis for patients with ma tumor.") The tumor was fairly large (5 cm in di ture teratomas is good after surgical excision 2) but ameter) and the cyst contained a central solid malignant transformations of included teratomatous portion, which was pearly white. Histological exami tissue do occur.7,14,15) However, long-term follow-up nation revealed a mature teratoma consisting of of these patients after total removal of mature pineal epidermis, hair follicles, sebaceous and sweat teratomas is very rare. glands, columnar gland, bone, cartilage, muscle, adi We describe a case of pineal dermoid cyst develop pose tissue, nervous tissue, and connective tissue. ing 18 years after gross total removal of a pineal ma No immature components or malignant features were noted. The histology was typical of mature cys Received November 5, 1997; Accepted February 24, tic teratoma. Postoperatively, the patient showed left 1998 abducens nerve palsy, positive Parinaud's sign, and Fig. 1 T1-weighted magnetic resonance images, Fig. 3 Operative photograph through the right oc showing a low intensity mass in the pineal cipital transtentorial approach showing a region extending into the right trigone (left: pearly tumor. axial view), and no apparent contrast en hancement (right: coronal view). no deficit except for symptoms of increased in tracranial pressure. His extraocular movement was full and there was no Parinaud's sign. The valve of a ventriculoperitoneal shunt, which was implanted 18 years ago, was dented after repeated flushing. Diffu sion-weighted MR imaging revealed a high signal in tensity mass (5 X 5 X 3 cm) compatible with epider moid cyst (Fig. 2 left). A right occipital craniotomy using the previous oc cipital, transtentorial approach with the patient in the three-quarter prone position was used to per form subtotal excision of the tumor. The tumor, which was well demarcated and had a shiny "moth er of pearl" appearance, was removed piecemeal (Fig. 3). Tumor content was granular, grumose, and flaky but there were also black hairs. The right occip Fig. 2 Preoperative (left) and postoperative (right) diffusion-weighted magnetic resonance im ital horn was opened and the capsule, which was ages, showing a high signal intensity mass tightly adherent to the pial surface, was left partially compatible with epidermoid cyst. intact. The right posterior choroidal artery, which was embedded in the tumor, was spared. After sub total removal, thick arachnoid membrane of the left spastic hemiparesis but these symptoms im quadrigeminal plate was recognized. It was thought proved within 2 weeks after the operation. No rou that the tumor had grown up from the pineal gland tine follow-up radiological examination was per compressing right occipital lobe and trigone. Histo formed after a few years postoperatively because his logical examination revealed an epidermoid tumor parents reported yearly that he was developing nor consisting of keratinized stratified squamous mally. Eighteen years after gross total removal, the epithelium and connective tissue (Fig. 4). There was patient consulted a local physician complaining of no other teratomatous or germinomatous compo headache and diplopia. Magnetic resonance (MR) nent. Based on the operative finding of black hairs in imaging revealed a round mass in the pineal region the tumor, the diagnosis was dermoid cyst. associated with hydrocephalus. The tumor was low The patient had transient left hemiparesis after the intensity on the T1-weighted image, high intensity operation but recovered. Occasional fever due to on the T2-weighted image, but with no apparent con chemical meningitis occurred during the postopera trast enhancement (Fig. 1). The patient was admitted tive period. He now visits the outpatient clinic to our hospital for further treatment. without help and receives steroid and anticonvul Neurological examination at admission revealed sant medication. Postoperative diffusion-weighted mature teratoma. Suprasellar germinomas have de veloped several years after total removal of pineal teratomas.11.17) The potential for phenotypic differentiation and cellular maturation within immature teratomas of the ovary') and testis') is well known. Differentiation of experimental tumors was studied by injecting sin gle cells of mouse malignant teratoma into the peritoneal cavity of other mice.') The successfully im planted tumors had both embryonal cancer cells and many well-differentiated cells of various types. Therefore, maturation of malignant teratomas is pos sible and the embryonal cell is a multipotent cell capable of producing various well-differentiated Fig. 4 Photomicrograph of a section of the re daughter cells. A case of primary intracranial germ moved tumor capsule showing keratinized cell tumor occurred in a 5-year-old Chinese boy.") stratified squamous epithelium and connec tive tissue. HE stain, x 200. The initial diagnosis was pineal immature teratoma because of elevated serum and cerebrospinal fluid levels of a-fetoprotein and $ human chorionic gonadotropin in addition to the histological exami MR imaging showed no apparent mass (Fig. 2 right). nation of a biopsy specimen. After chemotherapy consisting of cisplatin, etoposide, and bleomycin, Discussion total excision revealed only a mature teratoma con taining components of all three germ line elements. The dermoid cyst in our patient must have arisen Therefore, multifocality in intracranial germinomas from a microscopic remnant of the cyst wall of the and teratomas cannot be dismissed.") Recently, dif mature teratoma 18 years after the solid portion of fusion-weighted MR imaging, equivalent to a physio the tumor was removed en bloc, although some of logical MR cisternogram, has been helpful to charac the cyst wall was possibly left intact. The period of terize and distinguish between arachnoid and epi regrowth is unknown but may have been equivalent dermoid cysts and is the only method that can dis to the patient's age plus 9 months.') The patient tinguish between epidermoid cyst recurrence and presented with symptoms of increased intracranial postoperative encephalomalacia.'$) The preoperative pressure due to the slowly expanding tumor and diagnosis of this patient was an epidermoid cyst in shunt malfunction. Epidermoid and dermoid cysts the pineal region based on the diffusion-weighted of the pineal region are usually part of a well MR image. Careful follow-up observation of patients differentiated teratoma. Mature teratomas are typi who undergo gross total removal of pineal mature cally benign, firm in texture, and clearly demarcat teratoma should continue for a period exceeding ed, so complete resection is easily achieved.') the age at the time of surgery plus 9 months. However, half of the patients in one series died within the 1st postoperative year, and three pa References tients survived for 8, 12, and 13 years, with the lat ter patient developing a symptomatic recurrence at 1) Alvord EC: Growth rates of epidermoid tumors. Ann 8 years, which was again resected.19) Neurol 2: 367-370, 1977 The World Health Organization classification of 2) Burger PC, Scheithauer BW: Germ cell tumors, in: intracranial teratoma includes three histological Tumors of the Central Nervous System. Washington variants: mature, immature, and malignant.') The DC, AFIP, 1994, p 251 correct diagnosis of mature teratoma requires exten 3) The Committee of Brain Tumor Registry of Japan: In cidence of tumors in pineal region (1969-1983). Neu sive sectioning of the surgical specimen to exclude rol Med Chir (Tokyo) 32 (Special Issue): 438, 1992 mixed and/or malignant elements.',") Some ger 4) Dara P, Rich WM, Hodel K, Disaia PJ: Long-term dis minomatous components may be present in mature ease free survival in immature teratoma of the ovary. teratomas.12.11) In fact, malignant transformation or Cancer 50: 159-162, 1982 selection of malignant tumors has occurred in ter 5) Freilich RJ, Thompson SJ, Walker RW, Rosenblum atocarcinoma,14) and leiomyosarcoma15) in a terato MK: Adenocarcinomatous transformation of in ma of the pineal area or ectopic (septum pellucidum) tracranial germ cell tumors. Am J Surg Pathol 19: teratocarcinoma7) after the total removal of pineal 537-544,1995 6) Hong WK, Wittes RE, Hajdu ST, Cvitkovic E, ma developing after gross total excision of a pineal Whitmore WF, Golbey RB: The evolution of mature teratoma in a patient with systemic lupus erythemato teratoma from malignant testicular tumors. Cancer sus. Neural Res 17: 229-232, 1995 40:2987-2992,1977 15) Skullerud K, Stenwig AE, Brandtzaeg P, Nesland JM, 7) Kamiya K, Inagawa T, Ogasawara H: A case of malig Kerty E, Langmoen I, Saeter G: Intracranial primary nant teratoma developing from the septum pelluci leiomyosarcoma arising in a teratoma of the pineal dum 7 years after removal of a mature teratoma in area. Clin Neuropathol 14: 245-248, 1995 the pineal region. Surg Neural 30: 462-467, 1988 16) Tani E, Morimura T, Ametani T: [Pineal teratoma. 8) Kleihues P, Burger PC, Scheithauer BW (eds): Histo Case report]. No Shinkei Geka 7: 211-218, 1979 (Jpn, logical Typing of Tumours of the Central Nervous Sys with Eng abstract) tem, ed 2. Berlin, Springer-Verlag, 1993, 46 pp 17) Tsuchida T, Tanaka R, Kobayashi K, Ueki K, Koizumi 9) Kleinsmith LJ, Pierce GB Jr: Multipotenciality of sin K, Oyake Y: [A case of two cell pattern pinealoma, de gle embryonal carcinoma cells. Cancer Res 24: 1544 veloped 15 years after total removal of pineal terato 1548, 1964 - ma]. No To Shinkei 28: 893-899, 1976 (Jpn, with Eng 10) Lee ACW, Chan GCF, Fung CF, Leung SY, Lau YL: abstract) Paradoxical response of a pineal immature teratoma 18) Tsuruda JS, Chew WM, Moseley ME, Norman D: to combination chemotherapy. Med Pediatr Oncol 24: Diffusion-weighted MR imaging of the brain: value of 53-57, 1995 differentiating between extraaxial cysts and epider 11) Nagasawa S, Handa H, Yamashita J, Yamamura K: moid tumors. AJR Am J Roentgenol 155: 1059-1065, Suprasellar germinoma occurring two years after 1990 total removal of pineal teratoma. Surg Neural 22: 19) Yasargil MG: Teratoma, in: Microneurosurgery of 481-485,1984 CNS Tumors. Stuttgart, New York, Georg Thieme Ver 12) Rubinstein LJ: Tumors of the pineal region, in: lag, 1996, p 381 Tumors of the Central Nervous System. Washington, DC, AFIP, 1972, p 269 13) Shaffrey ME, Lanzino G, Lopes MBS, Hessler RB, Kassell NF, Vanderberg SR: Maturation of intracrani Address reprint requests to: T. Morimura, M.D., Depart al immature teratomas. Report of two cases. J Neu ment of Neurosurgery, National Utano Hospital, 8 rosurg 85: 672-676, 1996 Ondoama-cho, Narutaki, Ukyo-ku, Kyoto 616 14) Shah A, Sadasivan B, Leng BM, Ho J: A teratocarcino 8255, Japan.