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Home , Craniopharyngioma, Pinealoma

d. Neurosurg. / Volume 30 / February, 1969

Statistical Reevaluation of and Related Tumors in Japan

CHISATO ARAKI, M.D.,* AND SATOSHI MATSUMOTO, M.D.']" Department of , Kyoto University Medical School, Kyoto, Japan, and Max-Planck-lnstitut fii Hirnforschung Abteilung ]iir Allgemeine Neurologie, Cologne, Germany

ATSURA, et al., 9 in 1959 and Ueki 1~ in ified tumors. These were located as follows: 1963 concluded that the pinealoma 1) 81 in the pineal region; 2) 16 K was far more common in Japan than ectopic pinealomas; and 3) 39 in elsewhere. Although their conclusions have the pineal region. Approximately 10% of not been universally accepted because their the pinealomas in the pineal region were series were based mainly on surgical speci- diagnosed as pineoblastomas. A large num- mens, observations do indicate that surgery ber of ectopic pinealomas were located is preferred by Japanese surgeons, whereas around the third and lateral ventricles. radiation or other conservative therapy is Table 1 shows the predilection of pinealo- preferred in other countries. 1~ Our purpose mas for male children (1-15 years old). in this paper is to re-evaluate the statistics of However, this is also true of teratomas in the cases of pinealoma in Japan. pineal region, and in the posterior Our material consists of autopsied cases part of the diencephalon or the tectum of the alone, cited from Annual Reports of the . Pathological A utopsied Cases in Japan, The incidence of specific primary intracra- edited by the Japanese Pathological So- nial tumors in surgical and autopsy series is ciety, s in which cases from all the univer- constant regardless of the national origin, sity clinics and main large hospitals in Japan except for pinealomas and craniopharyn- are summarized. Between 1958 and 1964 giomas which are much more common in there were 87,395 such autopsies; 1,977 of this survey of Japanese cases (Table these cases showed primary intracranial tu- 2) ol-5,7,13,15-17 mors. Thus, the percentage of primary intra- cranial in all autopsied cases was Discussion 2.2%, which is comparable with that in se- Comparisons of "surgical" series are par- ries outside Japan. 14 ticularly subject to the hazards of nonunifor- Most reports dealing with statistics of mity since, for the most part, they are se- brain tumors have been based on surgical lected for surgical interest and weighted in specimens plus autopsy specimens ("mixed favor of tumors believed to be operable. On series"). As our material was based on au- the other hand, autopsy series are weighted topsied cases alone, we have compared it in favor of diagnostic problems and incur- with similar foreign series. able diseases, that is, against operable tu- mors. Variation in incidence in different General Results populations as suggested by surgical or au- We found that 1,802 cases out of 1,977 topsy statistics must therefore be interpreted (91.2%) had been histologically verified. very carefully. All histologically unverified tumors were ex- Although autopsy series do not reveal the cluded from statistical evaluation. Pineal tu- absolute incidence of tumors, variation in in- mors (136) constituted 8% of the total ver- cidence in comparable series may demon- strate the geographical or racial variation re- Received for publication February 28, 1968. garding a particular tumor. It is clear that * Retired Professor Emeritus. Present address: the incidence of the majority of intracranial 36-71 Izumikawa-cho, Shimogamo, Sakyo-ku, Kyo- to, Japan. tumors is quite constant in autopsy series 5" Present address: Division of Neurosurgery, from varying countries. Only pinealomas, Kitano Hospital, Kita-ku, Osaka, Japan. teratomas, and are 146 Pineal Tumors in Japan 147

TABLE 1 Age and sex of patients with tumors in the pineal region

Sex Age (yrs) Total Tumor Type . M F ? 1-15 16-20 21+ Cases Pinealoma and Pineoblastoma (97) pineal region 69 12 0 34 25 22 81 ectopic 10 5 1 8 1 7 16 in pineal region 34 5 0 25 8 6 39 Total 113 22 1 67 34 35 136 more common in our series than in foreign from countries other than Japan. However, countries (Table 3). 3,6,11,12 The percentage there were 120 tumors in the pineal region. of pinealomas in autopsy series is larger than This relatively high incidence included 81 that in mixed series. pinealomas or 4.5% of the total tumors; the series from outside Japan showed an average Sulnlll~lty of 0.7% pinealomas. There were also 39 We have analyzed 1,802 Japanese au- teratomas in the pineal region, a percentage topsy cases showing verified primary intra- of 2.1% in our series as compared to 0.2% cranial tumors. The proportion of common in non-Japanese series. Thus, as in the case tumor types (, , neuri- of craniopharyngiomas, there is an unusually noma) in this autopsy series was almost high incidence of tumors of the pineal region identical to that reported in autopsy series in the Japanese population.

TABLE 2 Incidence of pinealoma, ("mixed series" from surgical and autopsy verification)

Total Eetopie Pinealoma Cases of Pinealoma +Teratoma in Craniopharyngioma Author Primary Pineal Region Intracranial Tumor Cases % Cases % Cases ~o Cushing (1935) 1872 14 0.7 15 0.8 92 4.9 Gage1 (1937) 411 11 2.7 0 0.0 17 4.1 Henschen (1955) 2009 13 0.6 15 0.7 21 1.0 Zimmerman (1956) 547 6 1.1 0 0.0 16 2.9 Grant (1956) 2107 13 0.6 0 0.0 70 3.3 Zfilch (1956) 3799 20 0.5 27 0.7 107 2.8 Earle (1957) 513 2 0.4 0 0.0 9 1.7 Ringertz (1962) 569 2 0.3 0 0.0 15 2.6

Arendt (1964) 1368 9 0.7 0 0.0 38 2.8 rotal & Average % 13195 90 0.7 57 0.7 385 2.9 Ueki (all Japan) 5794 231 3.9 ? ? 500 8.6 (1963)