[Heterotopic pancreas tissue in the stomach--clinicopathological studies of 29 cases].
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Biomedical subjects
Publications and source records attributed to M Enjoji.
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This clinicopathologic study concerns 63 instances of glomus tumor of the soft tissues. The tumors occurred at different ages but were more common in earlier adult life and most commonly occurred on the fingers (35 cases), frequently as subungual nodules (26 cases). Other sites of occurrence were the forearm in seven, the knee in seven, and the leg in six. All patients but one complained of pain. Histologically, the tumors, showing endothelium-lined vascular spaces surrounded by masses of epithelioid cells, could be divided into three varieties: vascular (29 cases), myxoid (23 cases), and solid (11 cases). Under electron microscope the tumor cells in four cases proved to be smooth-muscle cells. The clinicopathologic evidence presented supports the hypothesis that the glomus tumor is a tumor-like lesion of mesodermal disorder rather than a true neoplasm.
Cell cultures were carried out from fresh tumor tissues obtained from seven cases of histiocytic tumors of the soft tissues including four of malignant fibrous histiocytoma, two of benign fibrous histiocytoma, and one of giant cell tumor of tendon sheath. The cultured cells were studied by light and electron microscopy, rosette formation for surface receptors, immune phagocytosis, and enzyme cytochemistry. The culture of each tumor revealed a mixture of histiocyte-like cells, fibroblast-like cells, amd intermediate forms. Bizarre giant cells were numerous in the early cultures of malignant fibrous histiocytoma, but they were absent in the cultures of benign fibrous histiocytoma and giant cell tumor of tendon sheath. In all the seven cases the cultured cells exhibited several features characteristic of histiocytes: (1) surface receptors for the Fc-portion of immunoglobulin G; (2) surface receptors for the third component of complement; (3) immune phagocytosis of opsonized erythrocytes; and (4) lysosomal enzymes including alpha-naphthyl butyrate esterase, and acid phosphatase. These findings suggest that the histiocytic tumors of the soft tissues in discussion actually comprise the cells that have the functional property of histiocyte. The tumor cells in the surgical specimens presented essentially the same ultrastructural and enzyme histochemical characteristics as those of the cultured cells.
A one-year-five-month-old boy with hepatoblastoma producing both human chorionic gonadotropin (hCG) and alpha-fetoprotein (AFP) is presented. Histologically, the primary tumor was mainly composed of well differentiated hepatoblastoma cells, with minor areas of poorly differentiated cells. Immunoperoxidase staining of the tumor for hCG and AFP showed that a few well differentiated, fetal type cells and multinucleated giant cells were positive for hCG, and AFP was never stained in the same cells. In areas where cells were poorly differentiated, positive reactions for either hCG or AFP were not observed. Electron microscopic studies revealed focal aggregates of cytoplasmic cored vesicles in some tumor cells, similar to secretory granules.
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Fifty-two cases of liposarcoma were analyzed clinicopathologically, and were grouped into the following five types: myxoid (28 cases), well-differentiated (14 cases), pleomorphic (4 cases), round-cell (3 cases), and mixed (3 cases). Three of the 28 myxoid tumors showed a pleomorphic pattern resembling that of the myxoid variant of malignant fibrous histiocytoma, and one of the 3 round-cell tumors was considered to be a malignant counterpart of hibernoma. Ages ranged from 22 to 86 years with the average age of 49 years, the average patients with myxoid or round-cell liposarcoma being over 10 years younger than those with well-differentiated or pleomorphic liposarcoma. There was also a variation in the site of predilection of the cases: the great majority of myxoid type tumors occurred in the thigh, popliteal fossa and buttock (23 cases), while the majority of well-differentiated type tumors were seen in the retroperitoneum (10 cases). The rate of local recurrence of the tumor was 46%. Four of the 5 well-differentiated liposarcomas which recurred exhibited dedifferentiated pleomorphic components resembling those in malignant fibrous histiocytoma. The prognosis was more favorable in patients with ordinary myxoid tumors than in those with well-differentiated tumors. The overall relative five-year survival rate was 63%.
The ultrastructural findings in 17 cases of malignant fibrous histiocytoma (MFH) are described. The tumors consisted of fibroblast-like cells and histiocyte-like cells in different proportions in different cases. Intermediate, undifferentiated, xanthomatous and multinucleated giant cells were also identified. In 12 of 17 cases myofibroblasts were evident. Acid phosphatase activity was detected cytochemically in the Golgi zone, endoplasmic reticulum and lysosomes (GERL) mainly within histiocyte-like cells, in three cases. These observations indicate that the GERL of the tumor cells are engaged in th formation of lysosomes. The polymorphic cellular composition, including undifferentiated cells, lends support to the concept that the MFH originates from a primitive multipotent undifferentiated mesenchymal cell.
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This clinicopathologic study deals with 84 patients who had recurrent malignant fibrous histiocytoma (MFH), and is primarily concerned with 50 cases in which specimens of both the initial and recurrent tumors were available for histopathologic examination. Quantitative and qualitative differences in the histologic patterns characterizing MFH, such as storiform and myxoid, between the initial and recurrent tumors, were clear-cut in nearly two-thirds of the cases. Conversion in histologic subtypes of MFH occurred in nine cases, including six cases which converted from myxoid to nonmyxoid pleomorphic. The superficially located tumors recurred somewhat more frequently, but in such cases, there was a significantly better prognosis than was seen with the deeply located tumors. Changes in the degree of mitotic rate, nuclear atypism, and cellularity from the initial to the recurrent tumor correlated better with the biological behavior of the tumor than did the degree in the initial tumor itself. The increased proportion of histiocyte-like tumor cells in the recurrent tumor was another morpholic feature which indicated a much poorer prognosis than did the increased proportion of fibroblast-like tumor cells.
This clinicopathologic study concerns 14 cases of extraskeletal myxoid chondrosarcoma from among 603 cases of soft tissue sarcomas. The ages of the patients at the time of initial biopsy ranged from 16 to 69 years. The tumors mainly arose in the lower extremities including buttocks (10 cases), and consisted histologically in cords and strands of small acidophilic cells with occasional vacuolated cells in an abundant myxoid matrix composed of chondroitin sulfate. Electron microscopy in four revealed a continuum of cell differentiation of the tumor cells from chondroblastic cell to undifferentiated mesenchymal cell. Morphological studies indicated that the extraskeletal myxoid chondrosarcoma included so-called chordoid sarcoma, as a variant.
Three cases of infantile digital fibromatosis were studied by electron microscopy, enzyme histochemistry, and tissue culture. The tumors were made up equally of myofibroblasts containing electron-dense inclusions which were composed chiefly of microfilaments measuring about 5 to 7 nm. Dense bodies usually observable in the smooth muscle cells were found in the bundles of these microfilaments and in the process of the inclusions, suggesting that these inclusions may represent an abnormal accumulation of contractile protein in the cytoplasm of tumor cells. Two cell lines were established from culture of the tumor cells, and the cultured cells also contained inclusion bodies showing the same morphologic characteristics as those of the original tumor cells. Lysosomal enzymes were abundant in the cultured cells, but they were scant in the cells of the fresh tissue specimens. Cocultivation of the cultured cells with human embryonic lung cells yielded no cytopathic effect.
Sarcoma of the chest wall following postoperative radiation therapy (2500 rads) for breast carcinoma is presented; it appeared 16 years after the initial radiation, and had light and electron microscopic features of malignant fibrous histiocytoma (MFH). Unique ultrastructural finding was the presence of Langerham cell granules in some of the tumor cells. This appears to be the first documented instance of a soft tissue MFH containing such granules in the tumor cells.
Two hundred and fifty-six tumors of carcinoma of the colon and rectum which had been surgically removed five or more years previously, and preserved in paraffin blocks, were examined for venous invasion using both routine hematoxylin and eosin (H-E) and by special stain for elastic fibers. In all these samples, 156 (61%) showed histological evidence of venous invasion and such was most frequently seen in the submucosa, followed by the subserosa or the perirectal fat. Since most of the involved veins showed marked destruction of the wall structure and were associated with total obstruction of the lumen, identification was difficult using the routine H-E strain. The frequency of venous invasion varied with the gross type of the tumor, the depth of penetration and the degree of differentiation, although there was no relation to size or to the site of the tumor. The incidence increased in cases of evident lymphatic permeation or lymph node involvement. The number of patients with venous invasion and a 5-year survival rate was significantly decreased, particularly in the Dukes' C cases. Thus, the determination of the extent of venous invasion using the elastic tissue stain is of definite prognostic value.
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This clinicopathologic study concerns four cases of epithelioid sarcoma, which were seen among a group of 603 cases of soft tissue sarcomas in our file. The patients were of young age between 8 and 21 years at the time of initial treatment. The tumors arose in the upper (2 cases) or lower extremities (2 cases), and consisted histologically of irregular nodular masses of Polygonal epithelioid cells merging with spindle cells. Electron microscopy in two cases revealed that the tumor cells were an admixture of synovioblast-like cells and fibroblast-like cells, the former cells being characterized by pseudoacini and filopodia. In three of the four patients, the tumors recurred one to three times, and one patient with primary tumor had metastases to the skin of the more proximal portions of the same extremity and to the regional lymph nodes. Follow-up ranging from two to four years, however, revealed that all patients were living and well after single or multiple operations.
A 10-year-old girl with undifferentiated (embryonal) sarcoma of the liver reported here had abdominal pain, nausea, vomiting and weakness when she was 8 years old. Chemical analyses of the blood and urine were normal. Serum alpha-fetoprotein was within normal limits. She died of cachexia 1 year and 8 months after the onset of symptoms. Autopsy showed a huge tumor mass in the liver and a few metastatic nodules in the lungs, which were consistent histologically with undifferenitated sarcoma of the liver. To our knowledge, this is the second case report of hepatic undifferentiated sarcoma of children in Japan, the feature being compatible with the description of Stocker and Ishaka.
A clinicopathologic study of 130 cases of malignant fibrous histiocytoma (MFH) of the soft tissues is reported. This malignant neoplasm principally of middle and late adults occurred most often in the proximal portions of the extremities (48%) including the thigh and buttocks (35%). MFH may be subclassified into common (storiform and pleomorphic), myxoid, xanthogranulomatous, and giant cell types, the common type being accounted for 100 cases (77%) of the series. The prognosis was more favorable in patients with storiform and myxoid tumors than in patients with pleomorphic or other type tumors, the overall relative five-year survival rate being 48%. The depth of the tumor also affected prognosis with a significantly lower survival rate in deeply situated tumors. The rate of local recurrence of the tumor was 48%. Because of incomplete informations, metastasis was confirmed in only 26 patients and was most frequently to the lung (73%). In addition, electron microscopic, histochemical and tissue culture findings in limited cases are presented, concerning the histogenesis of the MFH.
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