BEHÇET SYNDROME ASSOCIATED WITH NASAL MALIGNANT LYMPHOMA – REPORT OF AN AUTOPSY CASE –

The present case deals with a patient who was suffering concurrently from malignant lymphoma of the nasal cavities and Behcet disease accompanied with colitis ulcerosa.
A possibility that Behçet syndrome might be accompanied with malignant tumor is discussed. Moreover, it may be possible to point out the presence of such a type of Behçet syndrome as a "tumoral Behcet".     

CEREBRAL FORM OF VON WINIWARTER-BUERGER'S DISEASE – REPORT OF AN AUTOPSY CASE –

A 35-year-old housewife suffered from disorders of the central nervous system since 23 years of age. Autopsy revealed multifocal infarcts distributed widely in the cerebrum and cerebellum. The small pial arteries related to infarctions showed proliferation of loose connective tissue in the intlma and of elastic fibers, and hyperplasia of endothelia, by which the arteries were occasionally occluded. It has been difficult to differentiate this condition from arteriosclerosis or other vascular diseases, but the pathological and clinical features have been found to be a little different in some points.     

A FAMILY OF GARDNER'S SYNDROME WITH REPORT OF AN AUTOPSY CASE

A family with Gardner's syndrome including one autopsy case is reported. A 22 year-old man had multiple polyposis of the colon and the small intestine, epidermal cysts, soft part tumor of the abdominal skin and osteomas in the calvarlum and rib, and died of a carcinoma in the transverse colon and embolism of the brain. Histologically, all polyps were diagnosed as adenomatous polyp. The greater their diameter, the higher the tendency of atypism was observed. However, the polyps around the cancer were few, small, and with low atypism, and we could not obtain evidence showing direct transformation of adenomatous polyps into carcinoma. His two elder brothers had multiple polyposis of the colon, epidermal cysts and osteomas in the calvarlum or mandibular bone, but are still alive.     

HISTOCHEMICAL STUDIES IN WOLMAN'S DISEASE – REPORT OF AN AUTOPSY CASE ACCOMPANIED WITH A LARGE AMOUNT OF MILKY ASCITES –

This is a case of a male infant who suffered from "Primary familial xanthomatosis with involvement and calcification of adrenals" (Wolman's disease). The clinical symptoms were characteristic hepatosplenomega-ly, abdominal distention and a large amount of milky ascites. The patient died at the age of 2 1/2 months.
X-ray revealed enlarged, well-shaped, calcified adrenal glands. Foamy cells in different organs which were observed at autopsy confirmed the diagnosis. On histochemical examination, deposition of a triglyceride-cholesterol mixture was found in mesenchymal and epithelial cells of the liver, adrenals, spleen, lymph nodes and mucosa of the small intestine.     

POST-STEROID PANNICULITIS WITH CEREBRAL VASCULAR and PULMONARY COMPLICATIONS—REPORT OF AN AUTOPSY CASE

An autopsy case report was made on a 28-year-old female who had systemic nodular panniculitis, necrotizing arteritis of the cerebral artery and unusual pulmonary lipogranuloma after massive, long-term administration of corticosteroids for nephrotic syndrome. Panniculitis in this case was fairly consistent with the category of post-steroid panniculitis and was thought to be the first adult case, of post-steroid panniculitis developing in the internal as well as subcutaneous adipose tissue. The other two lesions in the brain and the lung above mentioned might be also regarded as adverse effects of corticosteroid therapy. The subcutaneous nodular lesion was studied chemically and histochemically, and the genesis of nodular panniculitis in this case was discussed as compared with sclerema neonatorum.     

NEURO-BEHCETS SYNDROME: REPORT OF AN AUTOPSY CASE

This report consists of one case who died after a neuropsychological disorder of the central nervous system consistent with Behcet's syndrome clinically and pathologically. Post-mortem examination of the central nervous system revealed disseminated meningo-encephalitis with multiple foci of softening, demyelinization and glial accumulation throughout the brain, especially in the thalamus, midbrain and pons. Review of the literature revealed about 30 cases including 17 cases in Japan.     

RETICULUM CELL SARCOMA OF THE BREAST – AN AUTOPSY CASE WITH TERMINAL LEUKEMIC MANIFESTATION –

An autopsy case of rapidly growing reticulum cell sarcoma of the breast with terminal leukemic manifestation and wide disseminations, including bilateral ovarian involvement, is reported. Metastatic tumors in the lymph nodes and in the right chest wall were sensitive for bleomycin, the combined chemotherapies with Mitomycin C, 5-FU, and Cytosine Arabinoside, and with Vincristine, Endoxan, 6MP, and prednisolone, and radiation therapy by Lineac, but only a short survival was finally obtained. The histology of the tumors is characterized by single cell pattern with medium sized polygonal cells. Investment of reticular fibrils by the cytoplasm of the tumor cells is revealed by electron microscopy.     

GAMMA HEAVY CHAIN DISEASE; REPORT OF AN AUTOPSY CASE WITH A REVIEW OF THE LITERATURES

An autopsy report of a 76 years old Japanese male, who had suffered from gamma-HCD, was presented. This Is the fifth case of gamma-HCD In Japan and this communication is the first autopsy report made on gamma-HCD in our country. The primary pathological alteration of this disorder is a neoplastic proliferation of atypical lympho-plasmacytes extending throughout the entire hematopoietic and parenchymatous organs. Pathological characteristics of our case were compared with the same disorders reported in the world literatures.     

AN AUTOPSY CASE OF CHOLANGIOCARCINOMA WITH HYPERCALCEMIA

Au autopsy case of cholangiocarcinoma which showed clinically hypercalcemia and hypophosphatemia without bone metastases is presented in this report. Although parathyroid hormone (PTH)-like substance of 520 ng/g. dry weight was measured in neoplastic tissue by the radioimmunoassay, membrane-limited secretory granules as those of parathyroid gland were not found in the fine structure. The significance of an existence of secretory granules in ectopic PTH producing tumor is discussed.     

AN AUTOPSY CASE OF ALKAPTONURIA WITH OCHRONOSIS

An autopsy case of alkaptonuria in a 47-year-old man is reported. Since childhood, he had noticed black color of his excreted urine. Autopsy revealed characteristic pathologic changes due to alkaptonuria such as ochronosis and ochronotic spondylosis, etc. The primary cause of death was pontine hemorrhage. Histologically, two types of ochronotic pigment were observed. Enzyme activity of homogentisate oxygenase in the liver and kidney homogenate was extremely decreased.     

GENERALIZED TUBERCULOSIS AFTER BCG VACCINATION –REPORT OF AN AUTOPSY CASE–

An autopsy case of generalized tuberculosis after BCG vaccination was reported. The patient, a boy BCG-vaccinated at the age of three, developed regional lymphadenitis, which was followed by subcutaneous abscesses in the chest wall. At the age of eleven, chest X-ray examination revealed an infiltrative shadow in the upper field. He died at thirteen years of age in poor condition with diarrhea and severe attacks of general convulsions. Autopsy disclosed generalized tuberculosis, especially in the skin, lymph nodes, lung, small intestine and brain. Microscopically, there was little tendency to develop tuberculous granulation tissue. Myriads of acid-fast bacilli were found in these ill-defined foci. The acid-fast bacilli were indistinguishable from BCG by bacteriological investigations. No definite causative factor could be traced. Complications induced by BCG vaccination were briefly discussed. ACTA PATH JAP. 19: 395˜407 1969.     

AN AUTOPSY CASE OF MENINGIOMA WITH EXTRACRANIAL REMOTE METASTASES

A case of meningioma with marked extracranial remote metastases is described.
The patient was a 33-year-old female and was operated according to a diagnosis of parasagittal meningioma in both frontal lobes. Nine years later, however, she died of local recurrence and extracranial metastases in the lungs, liver, pancreas and vertebrae. Histologically, the tumor was diagnosed as angioblastic meningioma with high cellularity, some mitotic figures and abundant intersecting network of reticulin fibers frequently outlining vascular architectures.     

AN AUTOPSY CASE OF TOXOPLASMOSIS ASSOCIATED WITH MYELOCYTIC LEUKEMIA

The case of a 44-year-old male with subacute myelocytic leukemia complicated by clinically unsuspected toxoplasmosis was reported on the basis of previously autopsied cases. Many pseudocysts were found in the brain, heart and mucous membrane of the stomach by using H.E. staining and studied by the aid of special stains and electronmicroscope.     

AN AUTOPSY CASE OF LIPOSARCOMA WITH GRANULOCYTIC LEUKEMOID REACTION

A 57-year-old female with a large retroperitoneal tumor was atutopsied. She showed through the entire course of illness marked leukocytosis with the appearance of immature marrow cells, and this finding was hematologically interpreted as granulocytic leukemoid reaction. An intermittent pyrexia was also seen without infective etiology. The histologic diagnosis of the tumor was liposarcoma of the pleomorphic type. The tumor showed extensive necrosis, but no metastatic invasion was found. So-called leukemoid reaction appears sometimes together with malignant neoplasms. The majority of these tumors show widespread bone marrow metastases, and without bone marrow invasion they only rarely induce this reaction. Besides it is not common for leukemoid reaction to be evoked by the development of malignant mesenchymal tumors except for malignant lymphomas. The pathophysiologic process of the present case is thought to be a rare occurrence.     

AN AUTOPSY CASE OF SO-CALLED DIFFUSE MYELOMA WITH PYROGLOBULINEMIA

An autopsy case of a 62 year-old Japanese woman who showed marked hyperglobulinemia and anemia with two kinds of M-components (IgG(L), Bence-Jones protein(L)) and pyroglobulin was presented.
There was no tumor formation detectable by repeated roentogeno-graphic examinations in the skeletal system during the clinical course of one year and two months or on the autopsy table.
Histologically, however, infiltration of plasma cells showing only mild atypia was noted diffusely in the bone marrow, spleen and lymph nodes. There was also a considerable infiltration of the same in the liver, kidney, ovary and lung. While, throughout the clinical course, leukemic increase of plasma cells in the peripheral blood was not found. No significant underlying chronic disorders were disclosed.
Fairly rapid down-hill course, a myeloma pattern of the serology and the pathological findings of this case denoted primarily a neoplastic proliferative disorder of the plasma cell, and thus this was diagnosed as so-called diffuse myeloma.
A short review was made on the literatures of diffuse myeloma and pyroglobulinemia.     

AN AUTOPSY CASE OF DIPROSOPUS

A case of diprosopus, a very rare malformation belonging to dupli-citas, of a female newborn is reported. This diprosopus monauchenos tetrophthalmus triotus dioris was delivered from a 33 years old multipara. Autopsy revealed duplication of cerebral hemispheres, 1st, 2nd and 3rd cranial nerves, hypophysis, intracranial internal carotid arteries, basilar artery and tip of tongue. This diprosopus had also congenital cardiac and great vessel anomalies consisting of double outlet right ventricle without pulmonary stenosis, coarctation of aorta (infantile type), ventricular septal defect, patent ductus arteriosus, patent foramen ovale and thick right ventricular wall. Except for mesenterium commune, the organs in the chest and abdomen, and the extremities were normal in position, shape and number. ACTA PATH. JAP. 20: 239–249, 1970.     

AN AUTOPSY CASE OF ATAXIA-TELANGIECTASIA

A case of a 10-year-old girl with ataxia-telangiectasia was described. While the cause of this disease is still unknown, possible etiologies and relevant clinical implications were briefly discussed.