Yasuhiro Nakamura

Spontaneous regression of primary malignant intracranial lymphoma

A rare case of intracranial multiple tumor, which disappeared spontaneously on serial cranial computed tomography (CT) scans, is described. The initial CT scan showed multiple, well-enhanced lesions in the right frontal and parietal lobes. The lesions disappeared spontaneously without any treatment during the 2 following months of observation. One month later, however, a newly formed tumor was found on serial CT-scanning. Surgical intervention confirmed the histologic diagnosis of a non-Hodgkins, large, and diffuse-type malignant lymphoma. The possible mechanism of temporal disappearance of tumors on CT scanning is discussed.

Nonimmunologic Hydrops Fetalis

Fifty cases of nonimmunologic hydrops fetalis found in Japanese infants are reported. Nonimmunologic hydrops fetalis is associated with various pathological conditions, twin transfusion syndrome including acardiac monsters, fetal heart diseases, congenital cystic adenomatoid malformation, pulmonary sequestration, pulmonary lymphangiectasia, intrauterine infections such as cytomegalovirus infection and neonatal hepatitis, congenital neuroblastoma, Kasabach-Merritt syndrome, cystic hygroma, and chromosomal aberrations. The mechanism of hydrops fetalis found in these conditions is discussed from various viewpoints. Despite a careful examination, no causative conditions were found in 14 cases. The placenta showed a proliferation of Hofbauer cells that were strongly positive for immunoreactive alpha 1-antichymotrypsin and there were other common findings such as edema of terminal villi and fibrin thrombi.

Cornelia de Lange syndrome associated with Wilms' tumour and infantile haemangioendothelioma of the liver: report of two autopsy cases

Two cases of Cornelia de Lange syndrome associated with infantile haemangioendothelioma of the liver and Wilms tumour are reported. The patients showed the characteristic facies of the Cornelia de Lange syndrome, with synophrys, long curly eyelashes and small upturned nose, and physical features, including generalized hirsutism, monodactyly, syndactyly and clinodactyly. Post-mortem examination revealed annular pancreas, patency of the foramen ovale, duodenal atresia and evidence of cytomegalic infection. The cases are reported to document a possible association between malformations and neoplasms in this syndrome.

HYPOXIC‐ISCHEMIC BRAIN LESIONS FOUND IN ASPHYXIATING NEONATES

Early hypoxic‐ischemic brain lesions were examined regarding 26 autopsy cases which had severe asphyxia at birth and died within the first week. All cases were divided into three groups according to the birth weight: group A of less than 1,000 grams, group B of 1,001–2,500 grams, and group C of more than 2,501 grams. Neocortical and deep gray matter revealed pyknotic and karyorrhectic neuronal changes; however, in group A, these changes tended to be obscure. The hippocampus was the predictive site of the neuronal changes such as pyknotic neurons in Sommers sector and karyorrhectic neurons in subiculum. In 12 cases, pontosubicular‐type necrosis was found. White matter lesions were relatively characteristic and there was an early appearance of pathological astrocytes such as gemistocytic, Alzheimer‐type 2 and stellate astrocytes, periventricular leukomalacia with or without hemorrhage. The brain stem and cerebellar lesions were also found occasionally revealing neuronal or glial changes. We applied the immunoperoxidase method using antisera to glial fibrillary acidic protein (GFAP) and S‐100 protein for determination of pathological astrocytes. GFAP was a useful marker for pathological astrocytes in the subpial region and in the white matter. S‐100 protein was present in Bergmanns glia and satellite glia as well as pathological astrocytes.

MALIGNANT FIBROUS HISTIOCYTOMA WITH GRANULAR CELLS, MIMICKING A GRANULAR CELL TUMOR: Light, Electron Microscopic and Immunohistochemical Observation of a Case

The tumor that occurred subcutaneously in the left hypochondriac region of an 87‐year‐old female is reported. Light microscopically, this tumor was composed of two kinds of cells, spindle cells and granular cells. The spindle cells showed various pictures such as fascicular arrangement, pleomorphic giant cells in the myxoid stroma and areas with abundant blood vessels. The granular cells contained PAS positive and diastase‐resistant granules, and these granules, being electron microscopically phagocytic lysosomes, showed the same findings as the granules seen in the granular cell tumor. The immunohistochemical studies for S‐100 protein, neuron specific enolase, CEA, myoglobin, and lysosome were negative, but α‐antichymotrypsin was positive. From the microscopic and immunohistochemical findings, this tumor was diagnosed as a malignant fibrous histiocytoma with granular cell changes of tumor cells. ACTA PATHOL. JPN. 35 : 1555–1560, 1985.

MYOEPITHELIOMA OF THE PAROTID GLAND

Two cases of myoepithelioma occurring in the parotid gland were reported. These tumors consisted of spindle cells that exhibited an arrangement similar to leiomyoma or fibroma. The diagnosis was made on the basis of characteristic ultrastructural as well as immunohistochemical findings : The tumor cells of the two cases likewise contained myofilaments and gave a positive result for the S‐100 protein immunohistochemical technique. ACTA PATHOL. JPN. 35 : 409–417, 1985.

Elastase digestion and biochemical analysis of the elastin from an elastofibroma

Elastin from both elastofibroma and control skin samples was analyzed by means of pancreatic elastase digestion and subsequent biochemical studies, such as for elastin content and amino acid composition. Elastin of elastofibroma was more resistant to elastase digestion than that of controls. Elastin content was increased almost twice (wet weight) or three times (dry weight) in elastofibroma. The amino acid composition had the characteristics of elastin; however, the cross‐linking amino acids such as desmosine, isodesmosine, and lysinonorleucine were increased in elastofibroma when compared with controls. An electron microscopic study showed that the interspersed cells had prominent intermediate filaments without any periodicity, pinocytotic vesicles, rough endoplasmic reticulum, and other organelles. These cells were considered to be fibroblasts not myofibroblasts. Therefore, it could be supposed that fibroblasts newly form an elastin that has a slightly different amino acid composition from that of controls. Cancer 58:1070‐1075, 1986.

Multiple ring enhancement in a case of acute reversible demyelinating disease in childhood suggestive of acute multiple sclerosis

A 5-year-old Japanese boy was admitted to our hospital because of progressive visual disturbance and quadriplegia. Computed tomography (CT) revealed multiple low density areas in the cerebral white matter, and intravenous injection of contrast medium resulted in ring enhancement around these areas. Exploratory aspiration of one of the low density areas gave a xanthochromic, viscous fluid. Pathologic examination of the drained fluid demonstrated small pieces of brain tissue with necrotizing lesions surrounded by demyelinating areas. The patient has been followed up for 3 years with corticosteroid treatment, and marked improvement has been achieved both in neurological examination and CT findings. From these clinicopathological findings an acute form of multiple sclerosis (MS) in childhood was suspected.

Bilateral cystic nephroblastomas and botryoid sarcoma involving vagina and urinary bladder in a child with microcephaly, arhinencephaly, and bilateral cataracts

The case of a 1‐year‐old infant with bilateral cystic nephroblastomas, a botryoid sarcoma involving the vagina and urinary bladder, microcephaly, arhinencephaly and bilateral cataracts is reported. The unusual association of these lesions suggests a syndrome due to genetic abnormalities.

Malignant teratoma in the brain. An immunohistochemical study.

A case of intracranial malignant teratoma found in a 27‐year‐old man was reported. This unique tumor was found in the right frontal lobe separated from the pineal region and revealed various tissue components such as stratified squamous epithelium, glandular tissues, neuron, glia, ependyma, fibro‐muscular tissue, cartilage, bone, hemangiomatous lesion, malanin‐laden cells, and some germ cell components. An immunohistochemical study demonstrated the presence of S‐100 protein, glial fibrillary acidic protein (GFAP), neuron‐specific enolase (NSE), carcinoembryonic antigen (CEA), and Factor VIII in some tumorous components. In particular, the distribution of S‐100 protein in some germ cells suggested the possibility of the neuroectodermal origin of the germ cells or, alternatively, differentiation to the neuroectoderm.