Katsuo Ogawa

Familial juvenile Polyposis of the Stomach

Innumerable polyps of the stomach were recognized in a 13-yr old girl. She had no extragastric polyps on roentgenographic and endoscopic studies. Her elder brother received a subtotal gastrectomy because of gastric polyposis at 14 yr of age. Their mother died of gastric cancer at 37 yr of age. Only these three subjects in this family had unusual brown hair and were low in normal intelligence. Polyps produced chronic and severe loss of both blood and protein, which resolved after a subtotal gastrectomy at 18 yr of age. Macroscopic and histologic observations of polyps in the resected stomach confirmed the diagnosis of juvenile polyposis. Both siblings are now in good health. Classification of this hereditary syndrome as a newly recognized entity, familial juvenile polyposis of the stomach, is proposed.

Prevention of Mammary Tumorigenesis in Acatalasemic Mice by Vitamin E Supplementation

Adult male and female acatalasemic (C3H/AnLCsbCsb), hypocatalasemic (C3H/AnLCscCsc) and normal mice of C3H strain fed on regular laboratory chow for 15 months showed an increased incidence of spontaneous mammary tumor in the decreasing order of female acatalasemic, male acatalasemic, female hypocatalasemic and male hypocatalasemic mice. Normal mice did not develop mammary tumor. We conducted a prospective study with female acatalasemic mice, which showed the highest incidence of mammary tumor, to examine the preventive effect of vitamin E on mammary tumor. Female acatalasemic mice were fed on vitamin E‐deficient (28 animals) and vitamin E‐supplemented diet (25 animals) for 29 months. The incidence of mammary tumor in mice given the vitamin E‐supplemented diet was 47%, while that in mice given vitamin E‐deficient diet was 82% (P<0.002). Mammary tumors were apparent after 9 months of vitamin E deprivation and after 14 months of vitamin E supplementation. Female normal mice did not develop mammary tumor during a comparable period of time. The mean catalase activity of mammary gland in acatalasemic mice was 18.8% of that in normal mice. The results indicate that vitamin E protects acatalasemic mice against the development of mammary tumor.

Immunohistochemical studies on human brain tumors using anti-Leu 7 monoclonal antibody in paraffin-embedded specimens

SummaryUsing the four-step peroxidase-antiper-oxidase (PAP) method, the presence of the antigen recognized with anti-Leu 7 monoclonal antibody was investigated in paraffin-embedded human brain tissue and tumors. The antigen was demonstrated in the myelin sheaths, oligodendrocytes, and some choroid plexus cells in normal brain and in oligodendrogliomas, some astrocytomas and choroid plexus papillomas. The technique can be used to identify hormal and neoplastic oligodendrocytes.

INDUCTION OF UNDIFFERENTIATED TUMORS BY JC VIRUS IN THE CEREBRUM OF RATS

Newborn Sprague‐Dawley rats were inoculated intracranially with JC virus (Tokyo‐1), a human polyomavirus, which had been isolated by Nagashimaet al. from the autopsied brain of a patient with progressive multifocal leukoencephalopathy in Japan. Twenty‐one to 70 weeks later, 21 of 27 rats developed brain tumors in the cerebrum, but not in the cerebellum. Most of the tumor cells were of an undifferentiated neuroectodermal nature and showed nuclear palisades and pseudorosettes. In some tumor cells glial flbrillary acidic protein was positive immunohistochemically, and many glial filaments were demonstrated ultrastructurally. Neuronal differentiation was not proved. Two continuous lines of cultured tumor cells were established, and T antigen of JCV (Tokyo‐1) was present in both cell lines. Glial differentiation was confirmed also in the tumors produced by subcutaneous transplantation of cultured tumor cells.

Immunohistochemical studies on cellular character of microtumors induced by ethylnitrosourea in the rat brain utilizing anti-Leu 7 and anti-glial fibrillary acidic protein antibodies.

SummaryTo clarify the chronologic changes in the cellular morphology of ENU-induced rat brain tumors, microtumors in the early stage were examined ummunohistochemically in comparison with macrotumors in the advanced stage. The tumor cells composing microtumors were negative for glial fibrillary acidic protein (GFAP), a specific marker of astrocylic cells, and Leu 7, a marker of oligodendrocytes, while cells of macrotumors were positive for either GFAP or Leu 7, showing characteristics of mature glial cells. The results suggested that the small round cells in the early devolopmental stage, generally thought to resemble mature oligodendrocytes, are not differentiated oligodendrocytes or astrocytes.

Regional Proteus syndrome: Report of an autopsy case

An uncommon autopsy case involving Proteus syndrome with multiple hamartomatous lesions in a 52 year old woman is reported. At birth, hemihypertrophy was noted on the right side of the face. Leiomyoma of the urinary bladder was extirpated at 37 years of age. She died of sepsis due to a brain abscess. At autopsy, diffuse asymmetrical lipomatosis was noted on the right side of the face, scalp, lip, oral mucosa, tongue and on the left side of the cerebellar peduncle. Muitiple meningiomas, cavernous hemangiomas and osseous hypertrophy overlapped in the intracranial regions. The present case is considered as a regional type of Proteus syndrome displaying a somatic mosaicism.

Histological pancreatitis in end-stage renal disease

SummaryTo clarify a possible cause of hyperainylasemia in end-stage renal disease (ESRD), histological studies were performed on the pancreatic glands of twenty-seven autopsied patients with ESRD who had received long-term hemodialysis. The findings were compared with those in a similar number of age-matched control subiects. Histological evidence of pancreatitis was found in 51.9% of the ESRD patients as compared with 14.8% in the controls (p < 0.005). The pancreatitis was chronic in nature in 85.7% of the ESRD patients showing changes of pancreatitis. Secretin administration to an additional group of twelve patients with ESRD induced an elevation in the activities of both total and P-type serum amylase in only one patient. These findings suggest that although histological pancreatic alterations are common in patients with ESRD, they are probably not responsible for the P-type hyperamylasemia frequently found in such patients.

MALIGNANT FIBROUS HISTIOCYTOMA OF THE MAXILLARY SINUS

A case of malignant fibrous histiocytoma arising primarily in the left maxillary sinus is described. The patient, a 39‐year‐old male, who had suffered from sinusitis for 20 years, began to have paresthesia or sharp pain of the left side of the face and toothaches of the left maxilla. At operation a white fibrous tumor developing extensively from the lateral wall to the upper and medial walls of the left maxillary sinus and into the ethmoidal sinus was noted. Following gradual progression of dyspnea, he died approximately one year after the onset in spite of radiation therapy and anticancer chemotherapy. An autopsy revealed recurrence of the tumor in the left maxillary sinus with wide‐spread metastases to the lungs, pleurae, pancreas, kidneys and bone marrows. The direct cause of death was respiratory failure due to extensive growths of the pulmonary and pleural metastases.

AN IMMUNOHISTOCHEMICAL STUDY ON THE DISTRIBUTION OF GLIAL FIBRILLARY ACIDIC PROTEIN, S-100 PROTEIN, NEURON-SPECIFIC ENOLASE, AND NEUROFILAMENT IN MEDULLOBLASTOMAS

In order to clarify the differentiation of medulloblastomas, the authors studied on the morphological features and immunohistochemical expression of glial flbrillary acidic protein (GFAP), S‐100 protein, neuron‐specific enolase (NSE), and neuroftlament (NF) in 31 medulloblastomas. GFAP was detected only in a small number of tumor cells of 5 medulloblastomas; S‐100 protein in both small tumor cells and some so‐called spongloblastic cells in 16 medulloblastomas; NSE in the more abundant tumor cells and the matrix in 28 medulloblastomas; NF in a few tumor cells of 12 medulloblastomas; GFAP and NF in 2 medulloblastomas, but each of them in different tumor cells. These results suggest that medulloblastomas have a capacity of differentiation along neuronal and/or glial lines. The conventional morphological markers of differentiation in medulloblastomas such as spongioblastic cells and Homer Wright rosettes were not necessarily compatible with expression of immunohistochemical markers such as GFAP or NF. NSE and S‐100 protein seem less valuable markers of differentiation because they were detected in both neuronal and glial elements. But NSE, which was observed in most medulloblastomas, might have a value as a marker for medulloblastomas.

Spontaneous Rupture of the Spleen in Acute Myeloid Leukemia

The results of an autopsy of an 80‐year‐old Japanese male with acute myeloid leukemia who died of spontaneous rupture of the spleen are reported. The patient was admitted because of anorexia, fatigue, weight loss, and multiple skin eruptions. Hematological examinations indicated a rapid increase in myeloblasts. The patient collapsed on the 28th hospital day, immediately after complaining of severe epigastralgia and vomiting. He died ten hours later. The autopsy revealed extensive leukemic infiltration of the bone marrows, spleen, lymph nodes, skin, and other internal organs. The spleen was enlarged and was ruptured in places at the hilar portion. Massive intraperitoneal hemorrhage from the rupture was the direct cause of death. The mechanisms of splenic rupture are discussed.