Nozomu Aoki

Gastric lesion of familial polyposis coli

Gastric polyps in familial polyposis coli are a unique type of adenomatous (neoplastic) polyp characterized by multiple occurrences. This paper reports 15 cases of familial polyposis coli in 6 different unselected families. Clinical studies such as roentgenogram and endoscopic examinations were carried out in 12 cases, the resected stomach was examined in 2, and autopsy material was used in 2. Elevated lesions were found in the stomachs of 10 (66.7%), which was a much higher incidence than in the results of a nationwide survey of this disease in Japan (10%). We believe that this disease is not necessarily localized to the large intestine, and that the absence of gastric polyps in other case reports is probably due to insufficient examination of the stomach. We also believe that our findings could be influenced by an environmental factor in Japan.

MUCOEPIDERMOID CARCINOMA OF THE THYMIC REGION

A case of mucoepidermold carcinoma in thymus in a 59‐year‐old Japanese female is presented. She died of cardiac tamponade due to tumor invasion after a 5 years clinical course. At autopsy the main tumor was found in the thymic region with metastases to the sternum, regional lymph nodes, pericardial, and left pleural cavity. The mucoepidermold carcinoma might be probably originated from a hens egg‐sized cyst which was located in the upper posterior aspect of the tumor‐Involved thymus. No teratomatous components were present. The cyst was most likely to be of thymic or bronchogenic cyst origin, though it was not determined, in view of the lining with pseudo‐stratified ciliated columnar epithelium of the cystic wall and the surrounding with the thymic tissue outside. Moreover, there was thymic hyperplasia with germinal center that was compatible with SLE‐like symptoms in her past history and autoimmune nature of the autopsy findings of pulmonary fibrosis.

Radiological features of familial polyposis coli: grouping by polyp profusion

The radiological features of familial polyposis coli (FPC) were studied in 60 patients by double-contrast barium enema examinations. When classified by profusion of polyps, cases fell into two groups: the profuse type and the sparse type. The radiologically determined boundary range distinguishing these types was 6-9 polyps/cm2 in adult patients and 3-6 in children. But on grouping the FPC patients by size of the polyps, one group of 34 cases had one or more polyps of more than 1 cm diameter, and in 16 of this group, one or more advanced cancers were histologically demonstrated; whereas in the group of 20 patients, in which the lesions were 5 mm or less, no malignancy was detected. In contrast to cases of the sparse type, in which the size of the polyps bore no relation to age, all cases of the profuse type who were 15 years old or more had polyps more than 1 cm in diameter.

A case of rectal carcinoma concomitant with pagetoid lesion in the perianal region

SummaryA case of extramammary Paget’s disease of the perianal area adjacent to a mucinous adenocarcinoma of the rectum is presented.The histopathological examination of the case revealed that within the epidermis adjacent to the invading rectal cancer nest, the formation of glandular structures can be observed occasionally, around which so-called Paget’s cells are noted to be scattered and further many points of similarity between cancer cell and Paget’s cell are proved by electron microscope. Perianal skin lesion looking like Paget’s disease is proved to be due to intraepidermal spread of the rectal carcinoma.Referring to the literature, this is the first report to our knowledge, to elucidate the histogenesis of perianal extramammary Paget’s disease associated with a rectal carcinoma using the electron microscopy.

The Peutz-Jeghers Syndrome and Malignant Tumor

In our 19 cases of the Peutz-Jeghers (P-J) syndrome, we experienced two cases of carcinoma of the small intestine and two cases of uterine carcinoma. In 420 reported cases the of the P-J syndrome in Japanese literature there were 70 cases of gastrointestinal carcinoma ( mean age of 34.4 years) and 21 cases of extra gastrointestinal cancer (mean age of 41.2 years). Mucus producing adenocarcinoma was common patho-histological subdivision of gastrointestinal carcinoma (39%). Twelve of 20 cases of extra gastrointestinal cancer were uterine (9 cases) or ovarial (2 cases) carcinoma. Cervical adenocarcinoma (6 cases) was thought to be a specific cancer associated with the P-J syndrome.

MALIGNANT PHEOCHROMOCYTOMA ARISING IN THE ORGAN OF ZUCKERKANDL

An autopsy case of malignant pheochromocytoma arising in the organ of Zuckerkandl is reported, of which distant metastases were found in the liver and lymph nodes. The biochemical and electron microscopic findings from the tumor tissue showed nor adrenaline‐ secreting type. It was revealed that sex ratio in malignant pheochromocytoma was more prominent in the female, and the frequency of extra‐adrenal origin was higher in malignant pheo chromocytoma than hi benign one from comparative study of reported cases of malignant pheochromocytoma. ACTA PATH. JAP.28: 731–740, 1978.