Toshihito Shinagawa

PERINEURIAL CELL TUMOR AND THE SIGNIFICANCE OF THE PERINEURIAL CELLS IN NEUROFIBROMA

The authors attempted to clarify the exact cell components of neurofibroma by immunohistochemical and ultrastructural studies. Materials were randomly selected, 40 cases of neurilemoma and neurofibroma (‐tosis) in addition to 2 cases of tumors composed exclusively of perineurial cells and three cases of normal peripheral nerve. The applied markers included antisera of S‐100 protein for Schwann cells, blood coagulation factor XIIIa for endoneurial fibroblasts or perineurial cells, and laminin and collagen type IV for the basement membrane. S‐100 protein was demonstrated only in normal or neoplastic Schwann cells, but not in perineurial cells. On the other hand, factor XIIIa was often recognized in endoneurial fibroblasts and perineurial cells, but not in Schwann cells. Neurofibroma was basically composed of a mixture of Schwann cells, perineurial cells, and endoneurial fibroblasts, the population of each type of cell differing according to the case and area within a given tumor. Perineurial cell tumor exclusively composed of perineurial cells, though rare, appears to be a definite entity, and its characteristic histological and ultrastructural features were described.

ULTRASTUCTURE OF CARTILAGINOUS TUMORS AND S‐100 PROTEIN IN THE TUMORS: With Reference to the Histogenesis of Chondroblastoma, Chondromyxoid Fibroma and Mesenchymal Chondrosarcoma

Twelve cartilaginous tumors were studied by electron microscopy and the presence of S‐100 protein was studied immunohistochemically in order to clarify the cell origin of chondroblastoma, chondromyxoid fibroma, and mesenchymal chondrosarcoma. Three chondroblastomas were characterized by round or ovoid tumor cells with some cytoplasmic processes, well‐developed organelles and thick fibrous laminae in the nuclear membrane, occasional multinucleated giant cells and scanty chondroid matrix. S‐100 protein was demonstrated in the tumor cells and some multinucleated giant cells. Two chondromyxoid fibromas revealed tumor cells of varied shapes with character istic cartilaginous differentiation and abundant chondroid matrix. Spindle tumor cells showed the ultrastructural features of cartilage cells rather than of fibroblasts and S‐100 protein was also demontratrated in their cytoplasm. Chondroblastoma and chondomyxoid fibroma were considered to arise from chondrocytes. Mesenchymal chondrosarcoma ultrastructurally exhibited round tumor cells with cartilaginous nature in cartilage islands. Poorly‐differentiated portions were composed of primitive round or elongated cells with occasionally admixed round cells with ultrastructural features of cartilaginous differentiation. S‐100 protein was demonstrated in the cells in cartilage islands and in single cells admixed in poorly‐differentiated portions. These results support the hypothesis of primitve mesenchymal origin with a tendency to differentiate toward cartilage cells. ACTA PATHOL. JPN. 34: 1285–1300, 1984.

Alpha fetoprotein-producing acinar cell carcinoma of the pancreas showing multiple lines of differentiation

An alpha fetoprotein (AFP)-producing tumour occurring in the head of the pancreas of a 30-year-old woman is reported. Histological examination revealed a markedly solid proliferation of tumour cells with prominent nucleoli and occasional luminal structures, some of which contained mucinous material stained with mucicarmine and alcian blue. No squamoid corpuscles were recognized. Immunohistochemistry showed intense positivity for lipase trypsin, and AFP basically, and single cells were also positive for carcino-embryonic antigen, CA19-9, synaptophysin and neuron-specific enolase. Pancreatic hormone-positive cells were absent. Electron microscopical examination revealed numerous granules of variable sizes in the tumour cells, which were considered to be zymogen. The tumour is an acinar cell carcinoma with multi-directional differentiation including the ability to produce AFP. Among AFP-positive pancreatic tumours, acinar cell carcinoma and pancreatoblastoma seem to be the most frequent.

Disseminated cryptococcal infection with eosinophilia in a healthy person

A 23-year-old man with no recent medical history was hospitalized complaining of high fever and cough. In addition to very marked eosinophilia, chest X-ray revealed extensive bronchovascular bundle thickening. Transbronchial lung biopsy (TBLB) showed moderate eosinophil infiltration. Cryptococcus neoformans infection was diagnosed, based on blood culture, cerebrospinal fluid culture, urine culture, and lung biopsy specimens. The eosinophilia was successfully alleviated by treatment for cryptococcal meningitis. Furthermore, cryptococcal sepsis resolved with amphotericin B and 5-flucytosine treatment. Eosinophilia commonly occurs following chronic Aspergillus infection, but the present case suggests the involvement of Cryptococcus in another mechanism for eosinophilia.

A case report of difficult diagnosis in the patient with advanced laryngeal tuberculosis.

We report a patient with severe laryngeal tuberculosis (LTB) involving thyroid cartilage and combined with whole-bone metastasis. A 57-year-old male had presented only with hoarseness. Radiological findings were indicative of suspected metastasis from a malignant tumor. However, tuberculosis was considered by histopathological findings, and so sputum samples were tested for acid-fast bacilli and purified protein derivatives of tuberculin in order to detect the presence of LTB. A polymerase chain reaction confirmed the diagnosis. Anti-tuberculous medications were effective in resolving the hoarseness, and the removal of the mass in the right wing of thyroid cartilage was confirmed by computed tomography (CT).

Primary squamous cell carcinoma of the liver: a case report.

BACKGROUND Bile cytology from percutaneous transhepatic cholangio-drainage (PTCD) is a useful procedure in case of obstructive jaundice. CASE Repeated cytology via PTCD from a cystically dilated duct in a 56-year-old female with a long clinical history permitted a diagnosis of squamous cell carcinoma (SCC). The smears showed excessive benign-appearing squamous cells with or without a nucleus and a few keratinized- and nonkeratinized-type SCC cells. No adenocarcinoma-derived cells were identified. No focus of SCC was detected clinically. The resected hepatic specimen disclosed a keratinized-type SCC simultaneous with benign metaplastic squamous epithelium. CONCLUSION This case of cytology of primary SCC of the liver seems to be the first reported. Even when showing a great number of benign-appearing squamous cells, the possibility of malignancy should not be ruled out. Repeating the cytology from PTCD is effective for a diagnosis.

Myxoid Variant of Epithelioid Leiomyosarcoma of the Uterus

A rare case of myxoid variant of epithelioid leiomyosarcoma of the uterus in a 76‐year‐old woman is reported. Palpation and computed tomography revealed an enlarged uterus. Total hysterectomy and bilateral salpingo‐oophorectomy were performed, and a hemispheric tumor, measuring 2.7 X 2.5 X 1.8 cm, was found protruding into the uterine cavity of the upper uterine segment. The tumor contained a prominent myxoid stroma and epithelioid tumor cells, which were round and polygonal in shape and showed positive immunoreactivity for desmin and vimentin. High mitotic activity was observed in the tumor cells. Electron microscopic examination revealed bundles of filaments in the cytoplasm and fine reticular material in the extracellular matrix. Histochemically, the myxoid stroma contained abundant acid mucopolysaccharide. The tumor cells were considered to originate from smooth muscle cells, while the myxoid stroma expressed varying differentiation of uterine mesenchymal cells. The patient is currently well with no evidence of recurrence or metastasis one year after the operation. Acta Pathol Jpn 41: 778‐783, 1991.

Increased occurrence of caspase-dependent apoptosis in unfavorable neuroblastomas

Neuroblastoma frequently shows spontaneous regression in which two distinct types of programmed cell death, ie, caspase-dependent apoptosis and H-Ras-mediated autophagic degeneration, have been suggested to play a key role. The current study was conducted to determine which of these cell suicide pathways predominated in this tumor regression. Periodic acid-Schiff (PAS) staining and immunostaining for H-Ras and for the full-length and cleaved forms of caspase-3, poly (ADP-ribose) polymerase (PARP), and lamin A were carried out on 55 archival tumor specimens. The incidence of caspase-dependent apoptosis in each tumor was quantified by cleaved lamin A staining and compared with clinicopathologic prognostic factors. Although a recent report has shown that neuroblastic cells undergoing autophagic degeneration were readily detectable by PAS and H-Ras staining, we could not confirm this result in any of our samples with the exception of one tumor. Instead, many of our neuroblastoma samples showed nonspecific PAS and Ras staining in areas of necrosis, suggesting that autophagic “degeneration” indeed corresponds to coagulation necrosis or oncosis. Unexpectedly, the incidence of caspase-dependent apoptosis was significantly correlated with indicators of a poor prognosis in these tumors, including Shimadas unfavorable histology, MYCN amplification, and a higher mitosis-karyorrhexis index, but not with factors related to tumor regression such as clinical stage and mass screening. These results indicate that neither caspase-dependent apoptosis nor autophagic “degeneration” may be involved in spontaneous neuroblastoma regression. This suggests that other mechanisms, perhaps such as tumor maturation, may be responsible for this phenomenon.

Papillary urothelial carcinoma of the urinary bladder demonstrating prominent signet-ring cells in a smear. A case report.

BACKGROUND The appearance of numerous signetring cells (SRCs) without any other type of adenocarcinoma cells originating in papillary transitional cell carcinoma (TCC) in a urine smear is rare. CASE The cytology from mucus-urine that was initially obtained by washing from a 69-year-old female revealed three different types of cells: (1) numerous single SRC carcinoma-type cells, (2) low grade TCC-type cells arranged in sheets, and (3) intermediate (transitional)-type cells with both aspects of TCC and adenocarcinoma (SRC carcinoma) and mucus in the background. The latter two cell populations were retrospectively confirmed after histologic diagnosis of a primary papillary TCC with glandular differentiation. CONCLUSION One should keep in mind that even a low grade papillary TCC with glandular differentiation of the bladder can exhibit excessive SRC-type cells in urine.

CHONDROMYXOID FIBROMA OF BONE

A resected case of chondromyxoid fibroma of the right fibula in a 31‐year‐old male is presented. The histology was composed of characteristic lobular features of this tumor with abundant cartilaginous matrix. The ultrastructural study exhibited stellate, ovoid or elongated tumor cells with features of cartilage cells and abundant loose matrix with many fine filamentous structures. The nuclei were often of peculiarly indented contour and revealed thick fibrous lamina. The villous cell processes and intracytoplasmic fine filaments were prominent. No cells suggesting a fibroblast were recognized in this study. From the light microscopical and ultrastructural findings of this case, it is supported that this tumor might be derived from adult cartilage cells most likely related to the epiphyseal cartilage rather than fibroblasts.