[Pediatric bone and soft tissue neoplasms].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M Enjoji.
Explore the source record for details and available documents.
A case of gastric leiomyoblastoma with liver and lymph node metastases occurring in a 31-year-old man, who has been alive with the tumor but without symptoms for more than 18 years after only an exploratory laparotomy without resection, is reported. On laparotomy, the liver metastasis and paragastric and para-aortic lymphadenopathies made it impossible to resect the stomach with the tumor. A diagnosis of malignant leiomyoblastoma was confirmed by incision of the liver and paragastric lymph nodes. Postoperative chemotherapy with mitomycin C and cyclophosphamide was performed for 6 months. As demonstrated by roentgenography, echography and computed tomography, the size of the tumor has remained almost identical for an 18-year period. Histologically, the low mitotic rate of the tumor in this case is considered primarily responsible for the long survival of the patient. A review of the records has revealed that our patient is the longest survivor to have unresectable gastric leiomyoblastoma with liver and lymph nodes metastases.
We report herein the clinicopathological, immunohistochemical and ultrastructural studies on 13 female patients and one male patient with papillary cystic tumors of the pancreas. Their ages ranged from 12 to 60 (mean 25) years. Most patients complained of abdominal mass or abdominal pain. Following complete resection of the tumor, all have remained well for between 3 months and 19 years (maen 5 years). In one patient the tumor was malignant and, 10 years after the initial partial resection, there was a recurrence with involvement of the colon, metastasis to the lymph nodes and venous invasion. Immunohistochemically, most tumor cells were positive for neuron specific enolase, synaptophysin, alpha-1-antitrypsin and vimentin and sometimes for the estrogen receptor related antigen, ER-D5, and the oncogen product of c-Ha-ras, c-Ha-ras P21. Ultrastructurally there were zymogen-like intracytoplasmic granules, intercellular junctions and intercellular spaces. These results support the hypothesis that the tumor originates from undifferentiated cells capable of differentiation toward acinar, endocrine or ductular cells. Estrogen and the c-Ha-ras oncogene presumably are linked to the development.
Thirty-four minute (less than 5 mm in the largest diameter) and 60 small (6 to 10 mm in the largest diameter) early gastric carcinomas (EGCs) were studied to assess the correlation between gross and histologic features. The characteristic macroscopic feature of many of the histologically differentiated, minute and small EGCs was a depression accompanied by a circumferential elevation of the adjacent noncancerous mucosa. The circumferential elevation was carefully studied histologically. On the other hand, most of the undifferentiated-type minute EGCs included a proliferation of the malignant cells affecting the lamina propria in the middle layer of the mucosa, leaving noncancerous glands and foveolae, and therefore presenting no specific macroscopic change. Most of the undifferentiated-type small EGCs, however, manifested distinct depressions, with or without ulceration; these EGCs were histologically made up of a compact proliferation of carcinoma cells that destroyed both the foveolae and glands. These findings clearly showed the difficulty in detecting the undifferentiated type of minute EGCs and explained the increase in the frequency of the differentiated type in EGCs with a decrease of the size of clinically detectable EGCs. Topographic mapping of the EGCs revealed that the undifferentiated-type minute EGCs that occurred in the middle third of the stomach may well be overlooked.
Twenty-six cases of neurogenic tumors consisting of 2 ganglioneuromas (GN), 8 ganglioneuroblastomas (GNB), and 16 neuroblastomas (NB) were studied to evaluate their cytodifferentiation. Ultrastructurally, a moderate to large number of neuritic processes and high density neurosecretory granules (NSG) were found in all cases of GN and well-differentiated GNB, in two-thirds of poorly differentiated GNB, in about a half of rosette-fibrillary NB, and in no case of round-cell NB. All GN and GNB had tumor cells which were positive for both chromogranin and neurofilaments. Of the 16 cases of NB, 12 were positive for chromogranin, and 13 and 15 were positive for Mr 200,000 and 68,000 neurofilament polypeptides, respectively. However, both markers appeared mainly in tumor cells maturing toward neuroblasts. Electron microscopy was helpful for the diagnosis of undifferentiated NB in those cases immunohistochemically negative for chromogranin or neurofilaments. We conclude that ultrastructural and immunohistochemical examinations are useful for the morphologic assessment of the degree of maturation of neuroblastic tumors.
A "depressed adenoma" was detected in surgically removed stomachs. There were 40 such lesions (11%) of a total of 357 lesions of gastric adenomas. Macroscopically, these lesions often occurred along the lesser curvature of the stomach, had shallow depressions of a light-brown color, a round or oval shape with irregular margins, and could hardly be distinguished from a depressed early carcinoma. Although 14 of the lesions were detected preoperatively with confirmation by endoscopic biopsy, the other 26 remained unrecognized until the gross examination of the formalin-fixed specimens. Histologically, all were tubular adenomas of the intestinal type with varying degrees of epithelial atypia. Carcinoma in adenoma was present in 5% of these depressed lesions, in contrast to 2.5% of the conventional protruded adenoma. Thus, depressed adenoma seems to be a variant of gastric adenoma which has a somewhat higher malignant potential.
Tissue samples from 103 patients with gallbladder carcinoma were examined, using 5-mm stepwise tissue sections. Three pathologic stages were used: Stage I; carcinoma invading not further than the muscle coat of the gallbladder, with or without extension along Rokitansky-Aschoff sinuses in the subserosa (11 cases); Stage II, carcinoma extending to the subserosal fibroadipose tissue of the gallbladder (73 cases); and Stage III: carcinoma invading the adjacent organs such as the duodenum, liver, and colon (19 cases). In the 11 patients with Stage I carcinoma, there was no apparent lymph node metastasis and all remained well for 3 months to 14 years after the initial operation. The cumulative 3-year survival rate of Stage I (100%) was significantly higher than of Stage II (40%) and Stage III (10%) (P less than 0.01, P less than 0.001). The Stage I tumors, therefore, can be defined as early carcinoma of the gallbladder. Dysplastic epithelium was seen in the mucosa adjacent to the malignant lesion in 57% of those with gallbladder carcinoma: 73% in Stage I, 59% in Stage II, and 42% in Stage III. Nine gallbladders had multiple foci of adenocarcinoma in a background of a diffuse dysplasia. Immunohistochemical study for carcinoembryonic antigen and carbohydrate antigen 19-9 resulted in positive staining of the dysplastic epithelial element adjacent to the invasive carcinoma and, in a similar fashion, in the carcinoma itself, thereby indicating a close relation between the epithelial dysplasia and adenocarcinoma of the gallbladder.
Gastric tissues from 85 patients with advanced carcinoma were studied immunohistochemically for localization of carcinoembryonic antigen (CEA), secretory component (SC) and alpha-fetoprotein (AFP), and for S-100 protein-positive cells. Our objective was to determine if these factors could serve as prognostic indicators. Forty patients died of recurrence or metastases within 2 years (Group I), and the remaining 45 survived 10 years or longer (Group II) after resection. CEA-positive cells were present in 93% and 89% of Groups I and II, respectively. In case of staining for CEA, positive results were seen not only in the malignant cells but also in the stroma in 35% of Group I but none in Group II. The immunohistochemical status of SC was not essentially different between Groups I and II; the positive staining was recognized in 50% and in 57%, respectively. Gastric carcinomas rarely reacted to AFP. S-100 protein-positive cells, which play an important role in local immunity, were often present in Group II (47%) but rarely in Group I (10%). Thus, in addition to the various prognostic factors available to the surgical pathologist, the presence of CEA and the appearance of S-100 protein-positive cells in tumor tissue will aid in predicting the prognosis of patients with advanced gastric carcinoma.
Two extraskeletal myxoid chondrosarcomas with a solid soft tissue mass occurred on the right upper arm of a 4-year-old boy and on the chest wall of a 1-year-old boy. Microscopically, both tumors were characterized by lobular configuration and were sparsely cellular with a background of myxoid matrix. The cells were small and round, and appeared undifferentiated, sometimes with a narrow eosinophilic cytoplasm. They grew in nests or strands and sometimes in a single file. They were strongly positive for S-100 protein and vimentin. Ultrastructural features suggested that the cells had a poorly differentiated mesenchymal nature with chondrocytic differentiation. These are the sixth and seventh reported cases of extraskeletal myxoid chondrosarcoma occurring in children. There are definite differences between this tumor with immature features and the extraskeletal myxoid chondrosarcoma in adults. Problems of differential diagnoses from other small round cell sarcomas also are discussed.
Among 284 cases of carcinoma of the gallbladder, 21 were identified as undifferentiated carcinoma (UC), with little glandular or other specific epithelial differentiation. These tumors were classified into three histologic types according to the components: (1) small cell type (eight cases); (2) pleomorphic cell type (eight cases); and (3) spindle cell or pseudosarcomatous type (five cases). Histochemical and immunohistochemical study by the immunoperoxidase technique revealed that most of the tumors (13/21) contained mucosubstances, and that all examples of the UC were immunoreactive for epithelial membrane antigen (EMA), keratin, and carcinoembryonic antigen (CEA), thereby indicating the epithelial nature of the neoplastic cells. Vimentin immunoreactivity was found in nine tumors. In 19, the tumor contained various neoplastic endocrine cells, including somatostatin-immunoreactive (14/19), gastrin-immunoreactive (14/19), human chorionic gonadotropin (HCG)-immunoreactive (9/19), pancreatic polypeptide-immunoreactive (4/19), and serotonin-immunoreactive cells (4/19). The prognosis of patients with UC of the gallbladder was poorer than that of patients with differentiated adenocarcinoma.
A clinicopathologic and immunohistochemical study of fifty-two primary carcinomas of the small intestine (20 duodenal, 18 jejunal, and 14 ileal) was performed. Most of these neoplasms were located in the proximal duodenum, proximal jejunum, and distal ileum. Most of these tumors produced both sialomucin and sulfomucin, although the adjacent mucosa showed hyperplastic changes with increased sialomucin secretion. Argyrophil cells were recognized in seven duodenal (35%), 13 jejunal (72%), and nine ileal (69%) carcinomas. Eighteen of the 29 tumors showing positive argyrophil reactions also had argentaffin cells. The common features of mucins and endocrine cells in these tumors suggest the multipotency of small intestinal carcinoma. The prognosis correlated with the histologic type, carcinoembryonic antigen (CEA) grading, invading pattern of tumor margins, and vascular permeation and regional lymph node metastasis.
Explore the source record for details and available documents.
The nuclear deoxyribonucleic acid (DNA) content of tumor cells and the exudative stromal reaction were compared in materials obtained from the same patients both at the time of surgery and autopsy. There were nine cases of gastric carcinoma and six of colorectal carcinoma. All patients had undergone surgical resection of their primary tumors, survived for over three years and died following a recurrence. The nuclear DNA content of the recurrent or metastatic tumors obtained at autopsy did not appreciably increase, although the exudative stromal reaction was invariably less or even absent. The results of this study suggest that the DNA distribution pattern in gastric and colorectal carcinomas does not change significantly throughout the entire course of the disease, which thus reflects an unchanging biological behavior in these tumors. Furthermore, the exudative stromal reaction decreases at the end stage, implying extensive diminution in host defense mechanisms.
A clinicopathologic and immunohistochemical review was made of 24 cases of distinctive nerve sheath tumors located in the gastrointestinal tract. The tumors were microscopically evident in the presence of peripheral lymphoid cuffing and benign nuclear atypia. The tumors arose in the muscularis propria of the stomach in 23 cases and in the ascending colon in 1 case. The patients included 9 men and 15 women whose ages ranged from 36 to 78 years (average, 58). No recurrence has developed in any patient who underwent resection. Although positive immunostaining for S-100 protein, Leu 7 antigen, and laminin might support the schwannian nature of these tumors, the positive immunoreactivity for glial fibrillary acidic protein (GFAP) indicated the possibility of a myenteric plexus origin. The pattern of S-100 protein immunostaining differed from that seen in cases of gastrointestinal stromal tumors associated with von Recklinghausen's neurofibromatosis or that noted in cases of conventional leiomyomas. We propose that these tumors be designated as benign schwannoma of the gastrointestinal tract.
A primary osteosarcoma occurred in the left ovary of a 47-year-old Japanese woman. The preoperative diagnosis, based on computerized tomography, was cystic teratoma. The excised tumor was composed of large multilocular cysts containing blood and associated with an area of solid tissue. Histologically, the tumor was a "pure" osteosarcoma that showed prominent cellular anaplasia and blood-filled spaces lined with tumor cells. The lesion resembled a telangiectatic osteosarcoma of bone. Serum alkaline phosphatase levels reflected progression of the disease. Despite aggressive adjuvant chemotherapy, the patient died 8 months later of a local recurrence and intra-abdominal spread of the tumor.
We studied a series of 585 patients with non-invasive or invasive ductal carcinoma in an attempt to assess the significance of elastosis. Elastosis in the neoplasm was recognized in 60% of the 549 patients with invasive ductal carcinoma. The grade of elastosis was correlated with both the histologic grade of differentiation and the 5-year survival rate. The incidence of elastosis, however, was 17% in the 36 patients with non-invasive ductal carcinoma and 38% in the 21 with invasive ductal carcinoma with a predominant intraductal component. The increased elastic tissue may therefore be influenced by the stromal infiltration of cancer cells. Mastectomy specimens from another series of 100 patients with mammary carcinoma were examined with regard to the volume of elastic tissue. Increased periductal elastic fibers were also identified in the non-neoplastic tissue, but the volume density was far less than in the neoplasm. A significant correlation was found between the increased amount of periductal elastic fibers in the non-neoplastic tissue, periductal elastosis of the neoplasm and an increase in parity. We propose that cancer cells in mammary carcinoma exert an inductive effect on mesenchymal cells for the synthesis of elastic material, under the basic condition of an increased amount of elastic fibers with an increase in parity.
A rare case of adenomatoid tumor arising in the ovary is presented. At autopsy on a 61-year-old woman, a soft, solid and cystic tumor, measuring 0.8 X 0.7 cm, was detected in the hilus of the left ovary. Light microscopic study showed characteristic features of adenomatoid tumor. Alcian blue stain, with and without hyaluronidase pretreatment, revealed the presence of hyaluronic acid on the luminal surface and in the vacuoles of the tumor cells. Immunohistochemical stains of tumor cells were positive for low-molecular-weight cytokeratin (PKKL), vimentin, and carbohydrate antigen (CA) 125, whereas they were focally positive for high-molecular-weight cytokeratin (34 beta E12). They were negative for factor VIII-related antigen (FVIII-RAG), Ulex europaeus I lectin (UEA I), carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). Ultrastructural studies disclosed surface microvilli and bundles of tonofilaments. These observations strongly support the idea of this tumor being of mesothelial origin.
We treated a 62-year-old woman with adenomyoepithelioma of the breast, an extremely rare tumor, which showed a bicellular pattern of ductal and myoepithelial elements bearing some histologic resemblance to pleomorphic adenoma of the salivary gland. The tumor was made up of cells positive for actin and S-100 protein, and some positive for epithelial membrane antigen (EMA). Thus, this tumor showed biphasic differentiation towards myoepithelial cells and to duct epithelial cells. Compared with findings in related tumors reported in the literature, the myoepithelial cells in the present tumor were less frequently spindle-shaped and had abundant eosinophilic or clear cytoplasm. A simple mastectomy was done and at the time of writing 20 months later there has been no recurrence. Adjuvant chemotherapy was not prescribed.