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Primitive Neuroectodermal Tumors in the Posterior Fossa: Excluding Medulloblastoma Based on Pathology

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Primitive Neuroectodermal Tumors in the Posterior Fossa: Excluding Medulloblastoma Based on Pathology 대 한 방 사 선 의 학 회 지 1992 ; 28 (1) : 14 암~155 Journal of Korean Radiological Society. January. 1992 Primitive Neuroectodermal Tumors in the Posterior Fossa: Excluding Medulloblastoma Based on Pathology Kyung Jin Suh, M.D., Tae Hun Kirn, M.D., Yong Joo Kirn, M.D., Duk Sik Kang, M.D. Sun Young Kirn, M.D. *, Youn Kyung Shon, M.D. * * Department of Diagnostic Radiology. ColJege o[ Medicine. Kyungpook National UniverSity - Abstract- Four children. with histopathologicaJly confirmed posterior fossa primitive neuroectodermal tumors. were ex­ amined by plain radiography. computed tomography (CT) and cerebral angiography. The homogeneously weJl enhanc­ ed solid mass in the midline of the posterior fossa and hydrocephalus of various degree were seen on a Jl CT scans. One case had calcifications and another case had low density areas in the tumor mass. Three cerebral angiograms showed vascular displacement without tumor vascularities. Unfortunately. these CT and angiography findings are not specific for primitive neuroectodermal tumors. as similar findings are seen in meduJloblastoma. ependymoma and other tumors. Therefore. we think that the primitive neuroectodermal tumors should be included in the differential diagnosis when a weJl enhanced solid mass in the midline posterior fossa is seen on CT scan in children. Index Words: Brain. neoplasm 10.36 CerebeJlar neoplasm 153.36 Primitive neuroectodermal tumor 10.369 retinoblastoma and olfactory neuroblastoma. It INTRODUCTION should be noted they included medulloblastoma in the posterior fossa as primitive neuroectodermal Primitive neuroectodermal tumors were first tumors. Chatty(3) reported these terms have been us­ described by Hart and Earle(l) in 1973. They describ­ ed to describe various tumors. which most often arise ed a group of supratentorial tumors that closely in the cerebellum of children and have traditionally resembled microscopically germinal or matrix cells been called meduUoblastoma. Rubinstein(4). of the primitive neural tube. There are a lot of opi­ however. reported this indiscriminate applicatioin of nions about the primitive neuroectodermal tumor a simplistic concept that would include all the so­ classification. Becker and Hinton(2) categorized and called “ primitive neuroectoderma1 tumors" into a described the pathology of primitive neuroectodermal single neuroepithelial tumor entity is unlikely to tumors. Previous articles classified primitive neuroec­ bring further understanding to the problem. Russel todermal tumors. including medulloblastoma. et al(5) said primitive neuroectodermal tumors and medulloepithelioma. neuroblastomas. polar spong­ medulloblastomas differ histogenetica1ly. We recent1y ioblastoma. pinealoblastoma. ependymoblastoma. reviewed posterior fossa primitive neuroectodermal *영 남대학교 의과대 학 진 단방사선과학교실 *Department o[ Diagnostic Radio1ogy. College o[ Medicine. Youngnam University **경북대학교 의 과 대학 해 부병리학교실 **Department o[ Anatomic Pathology. College o[ Medicine. Kyungpook National University 이 논 문은 1991 년 2 월 23 일 접 수하여 1991 년 10 월 11 일에 채택되었음 Received February 23. Accepted October 11. 1991 - 149- Journal of Korean Radiological Society 1992 ; 28( 1) : 149~ 155 tumors and accumulated 4 cases that fall into a Table 1. Profile ofPatients with Primitive Neuroectoder- category based on pathologic diagnosis. mal Tumors Our goal was to be able to make a resonable Case Age/Sex CT Angiographic Others diagnosis and differnetial diagnosis with radiologic No. Findings Findings studies. so that the clinician can know the tumor 121M Hydrocephalus Vessel displace behavior and it’ s possible rapid course and hence. Mass No tumor vessel proper and more aggressive therapy may be planned Midline Calcification SUBJECTS AND METHODS Well enhance 2 141F Hydrocephalus From December 1988 to September 1989. three Mass cases of primitive neuroectodermal tumors in the Midline Low density portion posterior fossa were diagnosed at Kyungpool‘ Na­ Well enhance tional University Hospital and one case at Youngnam University Hospital. All patients underwent opera­ 3 141M Hydrocephalus Negative study Spinal MRI Mass Metastasis tion. and afterwards histologic proof was obtained. Midline There were two males and two females between 12 Hyperdense portion and 15 years of age. Images were taken from a ll pa Well enhance tients using plain radiography and CT scans with and Meningeal enhancement without contrast enhancement. We performed 4 121F Hydrocephalus Vessel displace cerebral angiography on three pa디 ents. Suspecting Mass No tumor vessel spinal metastasis in one patients. a spinal MR imag­ Paramedian ing was obtained (Table 1). Clinically. all pa tients Well enhance showed signs of increased intracranial pressure. evi­ dent by headache and vomiting. A 12 year old boy was a dmitted with a persistent vomiting of 10 day's duration. The skull series were CASE REPORTS n egative. Cranial CT scan revealed mode rate Case 1 hydrocephalus. In the midline of the posterior fossa. a b c Fig. 1. Case 1. a. Nonenhanced CT scan shows a slightly hyperdense midline mass with hydrocephalus and intratumoral calcification b. Contrast enhanced CT scan shows marked tumoral enhancement with relatively little adjacent edema c. Lateral projection of vertebral arteriogram shows vascular displacement without tumor neovascularity - 150- Kyung Jin Suh, et al Primitive Neuroectodermal Tumors in the Posterior Fossa distributed chromatin. However, the cells had scan­ ty cytoplasm and no nucleoli. The boundary of the cytoplasm was not distinct. A small number of the multinucleated giant cells were observed. The tumor cells were arranged in a compact sheet without rosette or psedorosette formation. There were a lot of mitotic figures. Marked vascular proliferation and prominant collagen deposite were encountered in the stromal tissue. Immunohistochemical study for glial fibrillary acidic protein (GFAP) and neurofillament (NF) protein revealed negative findings (Fig. ld). ld Fig. l.d. Photomicroscopic feature shows small round Case 2 tumor cells without organoid pattern. Several giant cells are scattered. (H&E x 100) This patient was a 14 year old girl who had h eadaches and vomitings for 10 months. Bilateral there was a mass measured as 4.5 x 4cm in size. It papilledema with optic atrophy and nystagmus were had nodualr calcifiations and was well enhanced. No presen t. Three different times CSF cytology studies peritumoral edema was evident. Cerebral revealed a Clas3 1 result. Plain skull showed diastatic angiography showed vessel displacement without suture lines and dorsum sella widening. A cranial CT tumor vascularity (Fig. 1). After total removeal ofthe scan revealed severe hydrocephalus and a midline tumor the patient developed stress ulcer bleeding, mass in the posterior fossa. The mass, 4.8 x 3.6cm in hence we performed gastroduodenal artery emboliza­ size, had no calcification. After a contrast injection , tion with Gelfoam and surgical intervention follow- the mass became wel1 enhanced and showed some ed. Brain radiotherapy was also done. low density portions which were be tumor necrosis. Pathologic examination proved the the tumor was The peritumoral edema was scanty (Fig. 2). After total of a primitive neuroectodermal origin. The tumor removal of the tumor, radiation therapy was given cells showed small round nucleus and relatively even a b c Fig 2. Case 2. a and b. Nonenhanced and enhanced CT scans show a hyperdense midline mass with low density portions. c. A higher cut shows moderate hydrocephalus. - 151- Journal of Korean Radi 이 ogical Society 1992 ; 28( 1) : 149~ 155 before admission. a brain CT scan showed subarachnoid enhancement. and subdural effusion but no hydrocephalus. After being admitted because of aggravation of his symptoms. a cranial CT scan revealed mild hydrocephalus. meningeal enhance­ ment and a mass in the midline at the posterior fossa. The well enhanced mass. 1.8x 2cm in size. showed questionable areas of calcification. and little peritumoral edema. Cerebral angiogram revealed no tumor vascularities or displacement. Since we suspected spinal metastasis. spin머 MR imaging was done. The MR imaging revealed high signal intensi. ty on T2 WI in the spinal cord at the level of the 6th and 7th thoracic vertebrae. Both posterior fossa and spinal cord lesions proved to be primitive neuroec. todermal tumors (Fig. 3). The tumor cells showed two different kinds of cell type. One was undifferentated neuronal cells with small round nuc1ei. The other was spindle cells with long cytoplasmic process. Undif. ferentiated neuronal cells aggregated without rosette. At the interstitial portion. deposition of collagen fibers and ground substance was noted. Spindle ce\ls reveal­ 2d Fig. 2.d. Uniform small round cells are noted. Fine ed cellular atypia with occasional mitosis. Both fibrillary are seen in the background with vascular pro. cerebral and spinal tumors revealed the same liferations. (H&E x 100) features. The immunohistochemical study for GAP Case 3 revealed weak positive findings and the study for NF This patient was a 14-year.old boy a ff1 icted with revealed posivie features in the cytopolasms of tumor three days of headaches and vomiting. One month cells. Most of the positive features of NF were noted a b C Fig 3. Case 3. a. Nonenhanced CT scan shows a hyperdense small midline mass with hydrocephalus. b. Contrast enhanced CT scan shows tumoral and meningeal enhancement. c. Lateral projection of vertebral arteriogram shows no abnormality - 152- Kyung Jin Suh. et al Primitive Neuroectodermal Tumors
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