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A previously undescribed form of a congenital neural hamartoma composed entirely of Schwann cells in a fascicular pattern was found on the leg of a male infant. The lesion was thought to be an unusual variant of plexiform Schwannoma or a newly recognized unencapsulated form of Schwannoma. On light microscopic examination, the lesion, which measured 5 x 4 cm when it was surgically removed when the infant was 7 months old, showed an unencapsulated dermal mass composed of fascicles of spindle cells with frequent Verocay body-like structures. The intervening stroma was collagenous and contained an increased number of mast cells. Special stains did not demonstrate any axons in the tumor. There was a strongly positive immunohistochemical reaction for S-100 protein and collagen type IV in the spindle cells. These cells were weakly or focally positive for Leu-7 and vimentin, and completely negative for neural filaments, neuron-specific enolase, glial fibrillary acidic protein, epithelial membrane antigen, desmin, and muscle-specific actin. On electron microscopic examination, the spindle cells were found to be surrounded by basal lamina and showed frequent cytoplasmic invagination filled with collagen bundles. No unmyelinated nerve fibers were identified.

The case of a 61-year-old woman with a surgically resected solitary cholangiocarcinoma of the liver is reported, where many discrete multiple bile duct hamartoma (MBDH) were also seen. The latter is a congenital lesion of the liver that potentially may be confused with widespread metastatic disease. The relationship between cholangiocarcinoma and MBDH was studied histologically by the use of an immunoperoxidase technique for cytokeratin. MBDH was strongly positive for cytokeratin, while the neoplasm showed this to a lesser extent, but a clear continuity between the MBDH epithelial cells and those of the neoplasm was demonstrated by the use of this technic. The potential use for the various cytokeratins in the differentiation of primary from secondary liver tumors, is discussed. This differentiation is a significant problem to the pathologist. Although cholangiocarcinoma may, on occasion, be associated with various congenital lesions of the bile ducts, the association with MBDH is extremely rare, this being only the third reported case.

An unusual case of solid hepatic hamartoma, which could not be correctly categorized under the WHO classification, is reported. The well defined, solid nodular lesion, arising in the anterior edge of the left hepatic lobe, was found in a 15-month-old female infant. The lesion was composed of irregularly shaped yellow islands of parenchyma and white-gray nodules of fibrous stroma of various sizes, which were intermingled revealing a mosaic pattern. Prominent bile ductal proliferation was observed histologically in the fibrous stroma, associated in parts with dense or loose periductal bundles and vascular proliferation. The stromal proliferation seemed to result in erosion of the islands of differentiated parenchymal cells with no regeneration. Alpha-fetoprotein was demonstrated immunohistologically in the parenchymal cells of the lesion. From these findings, the essential component of this solid hamartoma was thought to be markedly proliferating bile ducts associated with periductal fibrosis, resulting in a fibroadenomatous pattern.

Fibrous hamartoma of infancy is an uncommon lesion, the histogenesis and biological nature of which are uncertain. Ten cases have been studied by light microscopy, mucin histochemistry and immunohistochemistry. The typical histological features are presented. The presence of hyaluronic acid and of chondroitin-4- and -6-sulphate and keratan sulphate has been demonstrated in different components of the tumour. Vimentin positivity was noted in the undifferentiated and fibroblastic components. The implications of these findings are discussed. Whether the lesion merits the designation of hamartoma or is, in fact, a benign neoplasm remains obscure.

An unusual type of benign vascular hamartoma, which shows a curious papillary organization of thrombus and abundant haemosiderin deposition, is liable to be misdiagnosed histologically as pigmented villonodular synovitis. Nine examples of this type of lesion are briefly presented and the differential diagnosis is discussed.

A series of 29 pulmonary sclerosing haemangiomas is analysed. Included is one case with multiple tumours and another with metastatic growth in a hilar lymph node. Histochemical and electron microscopic studies show that type 2 pneumocytes are an important constituent cell type though bronchial structures, tumorlets and angiomas also occur within these tumours. They are considered to be pulmonary hamartomas formed from distal lung structures. They are of slow growth and have been confused in the past with pulmonary histiocytomas and plasma cell granulomas.

Content of cAMP was studied in fatty tissue of 33 patients with tumor of mammary gland, of 27 patients with tumor of lung tissue and of 27 persons (15 men and 12 women), which constituted the control group. A procedure involving competitive binding of the nucleotide with protein was used. The data obtained were related to the size and amount of adipocytes in fatty tissue. The size of adipocytes was distinctly larger and content of cAMP in fatty tissue was slightly lower in patients with tumor of mammary gland as compared with the corresponding controls. With ageing content of fatty tissue cAMP tended to increase in men and to decrease in women. Negative correlation between the content of cAMP in fatty tissue (pM per kg of body mass) and the size of adipocytes, characteristic for the control group, was not found in the patients with the both forms of tumor. Significance of impairments in synthesis of cAMP in adipocytes for development of specific metabolic patterns of adipose tissue in oncological patients is discussed.

We examined 4 cases of sclerosing epithelial hamartoma for the presence of carcinoembryonic antigen (CEA) by the unlabelled antibody peroxidase-antiperoxidase method but could not detect CEA in these lesions. Because syringomas contain CEA, the detection of this antigen is a useful marker for differentiating sclerosing epithelial hamartomas from syringomas.

Previous investigations on polysialic acid of the neural cell adhesion molecule NCAM in human kidney have demonstrated its presence during nephrogenesis in embryonic kidney, absence in normal adult kidney, and reexpression in Wilms' tumor. These data showed that polysialic acid of NCAM is an onco-developmental antigen in human kidney and provided more direct evidence for the metanephric origin of Wilms' tumor. In the present study, five cases of Wilms' tumor associated with nephroblastomatosis complexes were immunohistochemically investigated with a monoclonal antibody for the presence of polysialic acid. Regardless of the type of nephroblastomatosis complex, ie, renal nodular blastema, simple tubular metanephric hamartoma, sclerosing metanephric hamartoma with adenoma, or incipient Wilms' tumor, immunoreactivity for polysialic acid was found in the blastemal cells, but was undetectable in all other structural elements. Because only blastemal cells exhibited a characteristic feature of embryonal differentiating metanephric derivatives, it appears that Wilms' tumor has its origin not exclusively in nodular renal blastema but rather in blastemal cells present in the various forms of nephroblastomatosis complex. The presence of polysialic acid of NCAM in blastemal cells in such lesions indicates that further events in addition to the expression of the embryonic form of this cell adhesion molecule may be involved in the pathogenesis of Wilms' tumor.

Synaptophysin is an acidic, integral membrane glycoprotein (Mr 38,000) of presynaptic vesicles in various neurons and neuroendocrine cells, and in tumours derived from such cells. By indirect immunofluorescence microscopy of cryostat sections, using the monoclonal antibody SY 38 to synaptophysin, a consistent positive immunoreactivity was observed in all medulloblastomas (n = 6) and neuroblastomas (n = 3) as well as a ganglioneuroma and a glioneuronal hamartoma. The presence of synaptophysin in medulloblastomas was confirmed biochemically by immunoblotting experiments. For purpose of comparison, the expression of intermediate-sized filament (IF) proteins was also examined. While neurofilament proteins were consistently expressed in the neuroblastomas (3/3), the ganglioneuroma and the glioneuronal hamartoma, IF distribution in medulloblastomas was variable. A neurofilament-positive type of tumour (1/6) could be distinguished from vimentin-expressing neoplasms (4/6) by immunocytochemistry. These data indicate that synaptophysin is a reliable marker for medulloblastomas as well as other differentiated and undifferentiated neuronal tumours and in this respect is superior to the more heterogeneous expression patterns of IF proteins in these tumours.

An autopsy case of cardiac rhabdomyoma in a male infant is reported. Many nodules of rhabdomyoma were present in all four cardiac chambers and were microscopically composed of ovoid, glycogen-laden cells and typical "spider cells". Atrial natriuretic peptide (ANP) was immunohistochemically demonstrated in both normal myocytes and rhabdomyoma cells of both atria, but not in normal myocytes and rhabdomyoma cells of both ventricles. Ultrastructurally, atrial specific granules were present in atrial rhabdomyoma cells and normal atrial cardiocytes, and these showed ANP immunoreactivity with protein A-gold technique. It could be said that the localization and intracellular distribution of ANP in this cardiac rhabdomyoma were closely similar to those of normal human heart. With regard to the presence of ANP, cardiac rhabdomyoma cells arising in atria seemed to differ from those in ventricles, although many tumor nodules occurred in both atria and ventricles. Furthermore, it seemed that cardiac rhabdomyomas could also be divided into two parts: 1) an atrial part with ANP, and 2) a ventricular part without ANP. Therefore, this study confirms the hypothesis that cardiac rhabdomyoma is a hamartoma rather than a true neoplasm.

An 8-month-old boy was found to have a solitary hepatic mesenchymal hamartoma. Histologically, the lesion appeared as a large island of loose mesenchymal tissue with few cystic bile ducts and liver cells. Electronmicroscopy showed microvilli on the surface of tumor cells and desmosomes between the cells. Immunohistochemical studies showed that alpha-fetoprotein was localized in the proliferating liver cells and bile ductal epithelium of this neoplasm. This case is the 17th case of hepatic mesenchymal hamartoma reported in Japan.

A solitary pedunculated polyp of the oxyntic mucosa removed from a 66-year-old patient with atrophic gastritis and achlorhydria exhibited distinctive histological features consisting of submucosal, mostly oxyntic-type glands with a smooth muscular framework. Histochemical and immunohistochemical studies demonstrated that the glands were composed of well differentiated chief, parietal, and endocrine cells. Moreover, less frequent glands of the antro-pyloric type were also seen. The lesion was regarded as a previously unrecognized variety of gastric hamartoma.

Clinical and neuropathologic observations are reported of a 16-year-old girl with a right temporoparietal tumor. The histologic picture revealed cortical areas with a peculiar growth of neurons, glia or tumor cells, and strands of collagen following the pattern of the cortical architecture. In this area of hamartoma-like cortex a large tumor nodule was found with anaplastic cells in fibrous stroma exhibiting partially positive GFAP reaction. The picture closely resembles the features of a tumor described in 1978 by Friede in the medulla oblongata of a 3-year and 9-month-old girl. A comparison of histology and ultrastructure of both tumors is followed by discussion of the origin of this peculiar type of tumor, focussing on the existence of unusually pluripotent cells that may develop into glial and mesenchymal cells.