Katsumi Asanuma

Intraductal papillary neoplasms of the pancreas. A clinicopathologic study of six patients.

A clinicopathologic study was conducted on six patients with intraductal papillary neoplasms of the pancreas. The patients were of both sexes and their ages ranged from 64 to 79 years. Three patients had a long history of symptoms mimicking chronic pancreatitis. The tumors involved the main pancreatic duct in the head‐‐body region either diffusely or focally. Histologic examination showed papillary proliferations of well‐differentiated, mucus‐secreting cells that occasionally stained for carcinoembryonic antigen (CEA) and carbohydrate antigen (CA 19‐9). The proliferations filled the main pancreatic duct, and extended into smaller ducts in some cases. In three patients, the lesions contained foci of pronounced to severe cellular atypia and carcinoma in situ. None of our series or any similar cases reported in the literature has shown invasion into peripancreatic tissue, metastasis, or tumor recurrence after pancreatectomy. Because of their favorable prognosis, intraductal papillary neoplasms should be considered low‐grade malignancies that must not be confused with the common ductal adenocarcinoma.

Three siblings of fatal infantile encephalopathy with olivopontocerebellar hypoplasia and microcephaly

We report three male siblings born with fatal encephalopathy comprising microcephaly, myoclonus and muscle hypertonia. All three patients died during infancy. Postmortem examination on the brain revealed that all infants had neuronal loss in the cerebellar cortex, inferior olivary and pontine nuclei, which were more pronounced in the older subject than the younger ones. In addition, they were associated with polymicrogyria in the cerebral cortex of the insula, olivary and dentate nuclear dysplasia, and a hypoplastic corticospinal tract. The clinical and neuropathological findings in our cases were identical to those in fatal infantile encephalopathy with olivopontocerebellar hypoplasia and microencephaly [Albrecht et al., Acta Neuropathol 1993;85:394-399], but an association of malformations suggests a new genetic factor in pathogenesis of olivopontocerebellar hypoplasia.

A cystic adenomatoid tumor of the uterus simulating lymphangioma grossly.

We are reporting a rare case of an adenomatoid tumor of the uterus having multicystic gross appearance. A 32‐year‐old woman complaining of dysmenorrhea had multicystic mass lesions on the posterior wall of the right cornual region of the uterus. The specimen showed a honeycomb appearance with mucoid content. Microscopically, numerous gland‐like spaces lined with low cuboidal cells were observed beneath the serosa, and mucopolysaccharide material accumulated in the cystic spaces forming honeycomb‐like lesions surrounded by myometrial tissue.

Siblings with carbamyl phosphate synthetase i deficiency

This paper concerns with two autopsied cases of siblings who died from cerebral disturbances. In these patients hyperammonemia developed in the neonatal phase due to carbamyl phosphate synthetase I (CPS I) deficiency. The patient in Case 1 was admitted 2 days after birth because of oliguria and vomiting. Hyperammonemia developed and she died on the 43rd day. In Case 2 hyperammonemia developed from the 2nd day after birth and she expired on the 42nd day. In both cases the diagnosis of CPS I deficiency was established from autopsy findings of the liver. Acta pathol. jpn. 34: 901∼910, 1984.

Malignant clear cell cystadenoma of the ovary.

A 50‐year‐old female had suffered from abdominal distension and a lower abdominal mass for 3 months. Ultrasonographic examination demonstrated a myoma uteri pattern with a large cystic ovarian tumor. Laparotomy was done on August 30, 1983 and simple total hysterectomy with bilateral salpingo‐oophorectomy was carried out FIGO stage IIC ovarian cancer was stated clinically. Histologically, clear cell cystadenoma of right ovary having benign, prollferative and malignant epithelial elements without dense ovarian stroma was found and diagnosed as malignant clear cell cystadenoma. In spite of Cisplatin therapy, the patient expired on April 6, 1984.