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A case of hepatocellular carcinoma with intracytoplasmic hyaline globules is reported. By serological, histochemical, immunofluorescent and electron microscopic studies the authors maintain that in this case intracytoplasmic hyaline globules represent lysosomal structures. According to Von Ardenne's theory, these lysosomal globules appear related to neoplastic cells necrosis.
Intracytoplasmic hyaline globules (IHG) were found in 16 out of 105 hepatocellular carcinomas (15.2%). In all cases, there was a male preponderance, most of the patients were Black, and the mean age was in the fifth decade. IHG were mostly noted in the better-differentiated tumors. Bile production was present in 12% of all tumors. Over 80% of tumors with globules showed necrosis as compared with 40% of the tumors without globules. Cirrhosis was present in almost half the cases, with liver cell dysplasia in 15%. These globules are possibly alpha-fetoprotein. Some of them may be giant lysosomes. Their role in carcinogenesis and prognosis is uncertain.
A case of primary sarcoma that arose in the right temporal lobe of a 59-year-old man is reported. The patient had a history of resection of a "pituitary tumor" and postoperative radiotherapy about 40 years earlier. The cerebral tumor consisted of loose and diffuse proliferation of large pleomorphic cells in a matrix showing a markedly myxoid change and containing a variable amount of delicate collagen fibers. Some tumor cells had a large intracytoplasmic vacuole containing acid mucopolysaccharide, thus featuring "pseudolipoblasts." Differentiation along other specific mesenchymal lineages was not found. Many tumor cells contained densely eosinophilic hyaline globules in the cytoplasm. Tumor cells showed a loss of the nuclear expression of H3K27me3. Although cerebral sarcomas showing similar histopathological features have not been previously reported, a markedly myxoid change of the stroma admixed with delicate collagen fibers and "pseudolipoblasts" suggests a close pathological kinship to myxofibrosarcoma. Another notable finding was the appearance of many hyaline globules, which has been considered a characteristic finding of primary intracranial sarcoma, DICER1-mutant. In the cytogenetic study of the present case, however, no DICER1 gene mutations were demonstrated.
Intracytoplasmic hyaline globules were found frequently in chromaffin cells of the adrenal medulla from several species of laboratory animals that died from various causes. The globules stained intensely red with PAS, were weakly acid-fast, did not stain with oil-red O and showed bright yellow autofluorescence. Ultrastructurally, they were finely granular, round to oval, intensely osmiophilic and were surrounded by an intimately bound trilaminar membrane. The globules were interpreted as aggregated secretory granules that occur in degenerating cells.
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All seven pure yolk sac tumors of gonadal and extragonadal origin tested showed a bright positive fluorescence for alpha-fetoprotein in the tumor tissue. A positive reaction was seen in both the tumor cells and the hyaline globules. In all cases, however, the positive fluorescence was distributed in some focal areas of the tumor tissue. Certain tumor cells showed a strong granular intracytoplasmic fluorescence, whereas others showed a weak or a negative fluorescence. The fluorescence-positive tumor cells were located mainly in the areas rich in fluorescence-positive hyaline globules. Besides alpha-fetoprotein, certain plasma proteins--albumin, alpha-1 antitrypsin, and transferrin--were also demonstrated in all five yolk sac tumors tested. The pattern of the distribution of positive fluorescence was basically similar to that of alpha-fetroprotein. Other plasma proteins--orosomucoid, haptoglobin, Gc-globulin, alpha-2 macroglobulin, hemopexin, and ceruloplasmin--were present in certain tumors, and were distributed mainly in a limited number of hyaline globules. Both IgG and IgA were present in two tumors of ovarian origin. The immunoglobulins were for the most part present in extracellular hyaline globules, suggesting that these are taken up from the circulation. Test for fibrinogen, beta-lipoprotein, IgM, IgE, beta-1C/beta-1A and beta-1E globulins were negative or questionable. In a hepatoblastoma, tests for alpha-fetoprotein were positive, but those for other plasma proteins were negative. Fine granular fluorescence was seen in each hepatocellular tumor cell. Mesenchymal elements were virtually unstained.
The correct classification of carcinoma of the lung is not only of therapeutic and prognostic importance but is also considered to have epidemiological and aetiological significance. Histological tests for mucin are essential in the classification of lung tumours but there is little information available about the influence of the method of detection used on the results of classification. Five established staining techniques were tested using paraffin blocks from surgical specimens of 81 human lung tumours diagnosed as adenocarcinoma, i.e. tumours of WHO Type III. Mowry's alcian blue-periodic acid-Schiff (AB-PAS) technique gave the highest proportion of positives (93%) slightly fewer (90%) being obtained by the PAS technique alone. Both these methods were influenced by the presence of cytoplasmic hyaline globules, structures which cannot be regarded as mucin. The stain recommended by the World Health Organization was also influenced by the presence of hyaline globules, was less frequently positive than the PAS techniques and was considered to have no special advantages. The aldehyde fuchsin-alcian blue sequence was positive in only 83% of cases but provided some information about the type of mucin present. Southgate's mucicarmine also detected mucin in only 83% of cases. It was concluded that the apparent incidence of adenocarcinomas may be influenced by staining methods used. Some standardization of technique is desirable and the AB-PAS combination appears to be the most satisfactory.
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An autopsy case of 20-year-old male with primary intracranial yolk sac tumor (endodermal sinus tumor) is reported. Whereas the biopsy specimen obtained from the pineal region showed diffuse proliferation of atypical tumor cells, the metastatic subdural tumor removed from lumbar spinal region had the characteristic histologic appearance of yolk sac tumor. The histologic diagnosis was intracranial yolk sac tumor originating in the pineal gland. The elevated amount of alpha-fetoprotein in the cerebrospinal fluid and in the serum further supported the diagnosis. At autopsy, only metastatic tumor was present in the posterior fossa. The immunofluorescence study demonstrated the presence of intra- and extracellular alpha-fetoprotein globules in the tumor tissue. The intra- and extracellular distribution of alpha-fetoprotein, in general, appeared to coincide with that of the PAS-positive hyaline globules in the tumor.
Inherited antithrombin III (AT-III, heparin cofactor) deficiency is a rare condition, presenting with thrombotic disease in adult life. This paper reports an 8 months old South African Black male infant with multiple large vessel venous and arterial thromboses, and E. coli septicaemia. This was associated with an extremely low plasma AT-III level. Micronodular cirrhosis and intracytoplasmic hyaline globules in the liver cells were present. These globules were eosinophilic, and PAS-positive after diastase. They measured approximately 5 mu to 30 muin diameter, occurred singly in the liver cells and were located mainly in the periportal areas. The histological findings in the liver are similar to those observed in alpha 1-antitrypsin (AAT) deficiency in which the intracytoplasmic globules represent accumulation of altered AAT. Immunochemical studies carried out on formalin fixed tissue failed to detect cross reaction material with anti-alpha 1 antitrypsin or anti-AT III antiserum. This is the first case report of AT-III deficiency presenting in infancy. It is also the first case associated with distinctive liver pathology. The available data presented are insufficient to distinguish between an inborn defect and acquired caused of the severely depressed AT-III plasma level and the distinctive liver pathology.
Human yolk sac carcinomas have been studied only twice with the electron microscope, and have never been compared at this level with normal human yolk sac. In the present study, the ultrastructural features of three primary ovarian yolk sac carcinomas, omental metastases from one of these, and a primary retroperitoneal yolk sac carcinoma in a male are reported, as are the ultrastructural findings in human yolk sac from normal 7- and 12-week gestations. The most prominent feature of the tumors is the presence of voluminous basement membrane material (the nature of which is confirmed by indirect enzyme-labeled antibody technique in one case) in both intra-and extracellular location, corresponding to the PAS-positive hyaline globules seen in these tumors by light microscopy. The tumor cells are also demonstrated to produce this material in tussue culture. Although basement membrane has not been described previously in normal human yolk sac at 8 and 10 weeks' gestation, it was present in the 7-week specimen which we studied, suggesting that its production may be a feature of only very young sac. Other ultrastructural findings are also similar in human yolk sac carcinoma, normal human yolk sac, and rodent yolk sac and yolk sac carcinomas. Thus, these studies confirm the suggested germ cell-derived yolk sac origin of the human tumor.
Pseudoendocrine sarcoma (PS) is a recently described neoplasm of uncertain differentiation, characterized by recurrent CTNNB1 mutations, frequent paravertebral location, and a neuroendocrine-like histomorphology. In this study, we report the clinicopathologic, immunohistochemical, transcriptomic, and epigenetic findings of 12 PS cases. The tumors affected 7 men and 5 women with a median age of 66 years and were located in the paraspinal/paravertebral region (n = 11) and the thigh (n = 1). Median tumor size was 82 mm (range, 32-170 mm). Histologically, the tumors comprised sheets and nests of epithelioid-to-ovoid cells with uniform nuclei and speckled chromatin, frequently associated with extracellular hyaline globules and fibrovascular cords/septa. Uncommon findings included microcalcifications, myxoid stroma, pseudopapillary, pseudoglandular, microcystic or corded architecture, and lumen and rosette-like structures. Necrosis was absent, and mitotic activity was low. On immunohistochemistry, the tumors showed aberrant nuclear staining for beta-catenin (8/8) and expression of CD56 (7/7), S100 (8/8), desmin (2/6), and androgen receptor (1/4). Pankeratin (AE1/AE3), progesterone receptor, synaptophysin, chromogranin, and INSM1 were negative. All tested cases harbored CTNNB1 mutations. Using a customized cohort, methylation profiling revealed that PS formed a common cluster with solid pseudopapillary neoplasm of the pancreas (SPNP), distinct from all methylation classes from the Heidelberg sarcoma classifier and a subset of paragangliomas. Transcriptomic analysis showed that PS formed an independent cluster from a control group of tumors (including SPNP). Differential gene expression analysis showed enrichment in genes of the Wnt signaling pathway (HALLMARK gene sets) and biological processes related to sensory perception, among others (gene ontology - biological process [GO-BP]). Additionally, upregulated genes were related to various fetal cell types from the cell type signature data set (MSigDB), particularly of neuronal and epithelial lineage. Immunohistochemical assessment of potential markers identified through gene expression analysis revealed focal-to-diffuse expression of GLUT1 (6/6) and focal/multifocal expression of Brachyury (4/9) and HuD (3/7). Follow-up information, available for 10 cases (median duration of 18 months; range, 7-69 months), showed local recurrences and metastatic spread in 2 patients each. Evidence of response to radiotherapy was documented in one tumor. Altogether, this study expands knowledge on PS and suggests biological links with SPNP, including a potential shared cell of origin.
A combined immunocytochemical and quantitative serum and tissue study was performed on a group of endodermal sinus (yolk sac) tumors, localizing and measuring both alpha1-antitrypsin (AAT) and alpha-fetoprotein (AFP) in tumor tissue and patient sera. Utilizing indirect immunofluorescent and triple-sandwich immunoperoxidase methods, both proteins were demonstrated within intra- and extracellular periodic acid-Schiff-positive hyaline globules characteristic of the tumor, as well as within the cytoplasm of tumor epithelial cells lining endodermal sinuses, where AAT deposition predominated. Tumor tissue extracts confirmed the presence of significant quantities of both proteins, and pretreatment serum elevations of both showed a parallel decline during therapy. In this study, AAT is characterized as a tumor protein marker for the first time, and a parallelism between AAT and AFP is demonstrated in both serum and tumor tissue. These findings represent additional supportive evidence for the yolk sac origin of endodermal sinus tumors in man.
The correlation between serum alpha-foetoprotein (AFP) and the clinical pathological finding of 24 germ cell tumours arising from the testes (14 cases), the ovaries (3 cases), the mediastinum (3 cases), the retroperitoneal region (2 cases), and the sacrococcygeal region (2 cases) are presented. Irrespective of marked differences in age and sex of the patients, primary site of the tumours and clinical outcome, the 24 cases constituted a homogeneous group in fundamental histological patterns and in AFP synthesis. In all cases of endodermal sinus tumour or teratocarcinomas with a distinct vitelline component an increased serum AFP concentrations was found in the pre-operative serum samples. AFP was also demonstrated in the tumour tissue by quantitative determination of AFP in tumour homogenate (5 cases) and, by immunofluorescence technique, positive staining of the cells lining the endodermal sinuses and of the hyaline globules was found (3 cases). In 12 germ cell tumours without vitelline components in the tumour tissue sections, a normal AFP concentration below 20 mug/1 was found in preoperative serum samples.
An ultrastructural study of 10 liposarcomas is reported. Four of the liposarcomas were wholly or predominantly of well-differentiated, lipoma-like or fibrosing type, 3 of myxoid type, 2 of round cell type and 1 pleomorphic type. The well-differentiated, lipoma-like liposarcomas showed cells with a few, large lipid droplets, few organelles and a peripherally located, fairly large nucleus, The well-differentiated liposarcomas of fibrosing type revealed mostly spindle-shaped, fibroblast-like cells, with abundant rough endoplasmic reticulum and inconspicuous lipid inclusions, surrounded by collagen. One well-differentiated liposarcoma contained an area which was similar to brown adipose tissue and hibernoma. The spindle and stellate shaped cells of the myxoid liposarcomas showed abundant rough endoplasmic reticulum and large smooth-membraned vacuoles filled with moderately dense amorphous material, which appeared to be extruded extracellularly by rupture of the vacuoles. Cytoplasmic lipid droplets were seen in most cells but were much less prominent than in the well-differentiated lipoma-like liposarcomas. Ultrastructurally there were many similarities between the myxoid and round cell liposarcoma, indicating a close relationship between the two types. The pleomorphic liposarcoma revealed cells with one or more large, irregular nuclei, numerous large vacuoles after dissolved lipids, abundant dilated cisternae of rough endoplasmic reticulum and rounded, electron-dense bodies corresponding to PAS-positive hyalin globules seen in the light microscope. The ultrastructural study suggests that the variegated cellular appearance of the different subtypes of liposarcoma reflects the wide cellular spectrum seen during the differentiation of adipose tissue and supports the view that all liposarcomas histogenetically represent a single entity.
6 cases of endodermal sinus tumor of the ovary are presented. In 4 patients pure endodermal sinus tumor was found microscopically. 1 patient had endodermal sinus tumor in one ovary and gonadoblastoma in the contralateral one. In another case endodermal sinus tumor was accompanied by an embryonal teratoma. Histologically, the tumor had characteristic features with meshwork of spaces and channels lined by embryonal cells, glomerulus-like structures known as Schiller-Duval bodies, solid aggregates of epithelial cells, hyaline basement membranes and round, PAS-positive small globules found both intra- and extracellulary. In 1 patient the elevated serum alpha-fetoprotein was stated. All patients were treated surgically with adjunctive radiation and/or with chemical agents. None of them were cured. The median duration of survival amounted to 8.5 months. Discussing the value of the more recent approach to diagnostic and therapeutic methods found in the literature, it must be emphasized that the demonstration of elevated serum alpha-fetoprotein in patient with that tumor lend not only further support to its yolk sac origin but also might be useful to monitor response to the therapy applied. It is also of prognostic significance by indicating the presence of residual or recurrent disease, even in its subclinical stage. Combined postoperative irradiation and triple chemotherapy according to the VAC regimen of patients can prevent recurrence and in some cases even may cause permanent remission of the neoplasm.