Yoshiro Sasaki

Hepatitis B surface antigen positive hepatocellular carcinoma in children: Report of a case and review of the literature

This report describes a case of adult type hepatocellular carcinoma (HCC) of a ten‐year‐old boy. The liver showed no cirrhosis but hepatitis B surface antigen (HBs‐antigen) was demonstrated in the nonneoplastic liver cells. Serological examination of his family revealed positive HBs‐antigen of the patients mother suggesting familial transmission to the patient in his early life. Histologic demonstration of HBs‐antigen was performed on 36 adult HCC cases and 14 childhood cases including three HCC and eleven hepatoblastoma. HBs‐antigen was demonstrated in 12 adult cases, one of which showed no cirrhosis. All childhood cases except for the one presented here showed no HBs‐antigen. Review of the literature disclosed four other cases of HBs‐antigen positive HCC in children. The present case is unique in that the non‐neoplastic portion of the liver showed neither hepatitic nor cirrhotic manifestations. These HBs‐antigen positive HCC in childhood may imply the significant relationship between HB virus and HCC not only in adult but also in childhood.

BANNAYAN SYNDROME—GENERALIZED LIPOMATOSIS ASSOCIATED WITH MEGALENCEPHALY AND MACRODACTYLY

This paper reports an autopsy case of a 5‐year and 11‐month‐old girl with generalized subcutaneous lipomatosis, megalencephaly, and macrodactyly. Marked emaciation and poor prognosis were the characteristic findings of this case. We thought that this case was identical with a rare syndrome which was initially reported by Bannayan in 1971. Autopsy disclosed diffuse lipomatosis in the thoracic and abdominal cavity, and infiltration of fat tissue to the pancreas. Hyperplasia of the small intestinal mucosa and Peyers patch, and a pedunculated polyp of the sigmoid colon were the unique findings which could not be seen in the previously reported cases.

Carcinoma of the prostate in childhood and adolescence: Report of a case and review of the literature

This paper reports a case of carcinoma of the prostate in an 11‐year‐old boy. The clinical findings were characterized by a mass in the prostatic region, extensive osteoblastic bone metastasis, and normal serum acid phosphatase. Autopsy demonstrated an undifferentiated tumor, which probably originated from the outer gland of the prostate. Metastases to the bones, liver, lungs, and the lymph nodes were present. Light and electron microscopic studies revealed undifferentiated neoplastic cell, which is in contrast to the usual adenocarcinoma in older individuals. Histochemical examination failed to demonstrate acid phosphatase activity within the tumor cells. The authors considered that this tumor probably originated from immature basal cells of the prostatic gland. Review of the literature disclosed 15 cases of carcinoma of the prostate in individuals under 21 years of age. These cases were also characterized by an undifferentiated appearance of tumor cells and normal serum acid phosphatase level.

Immunohistochemical Study of Neuroblastoma and Related Tumors with Anti-S-100 Protein Antibody

Histological sections of 36 cases of neuroblastoma and related tumors were studied with anti-S-100 protein antibody (PAP method). Schwann cells in the ganglioneuromas and ganglioneuroblastomas always strongly stained. In addition, varying numbers of spindle-shaped or elongated positively staining cells, which were probably Schwann cells and their precursor cells, were demonstrated in ganglioneuroblastoma and differentiating neuroblastoma. Undifferentiated round cell neuroblastoma showed no reaction. Immunohistochemical findings of these cases were classified into four groups (+ +, +, +/-, -) according to the number of the positive cells and compared with prognosis, histological typing, location of the tumors, stage, and age at surgery. The cases with many positive cells, group (+ +) showed excellent prognosis, and group (-) showed very poor prognosis. The results of this study indicate that S-100 protein staining provides a reliable objective method for evaluation of differentiation of the neuroblastoma cells toward Schwann cells, which appears to be an important factor to predict prognosis.

Kawasaki Disease Complicated With Mitral Insufficiency Autopsy Findings With Special Reference To Valvular Lesion

A case of mucocutaneous lymph node syndrome (Kawasaki disease, MCLS) complicated with mitral insufficiency is reported. This patient showed severe valvulitis, which was thought to be the main cause of mitral regurgitation. Two other patients with MCLS who did not present clinical signs of mitral insufficiency revealed the presence of mild valvulitis. The morphological alterations of cardiac valves were non‐specific and mainly consisted of inflammatory infiltration, increment of fibrous connective tissue, and proliferation of small capillaries. In addition to the coronary aneurysms, the involvement of cardiac valves should receive attention as the sequelae of MCLS. ACTA PATHOL. JPN. 34: 605–616, 1984.

Ki-I LYMPHOMA PRODUCING G-CSF

Ki-1 lymphoma is a large-cell anaplastic non-Hodgkin’s lymphoma (NHL) that expresses the Ki-1 antigen (CD30), and various clinical and histologic features have been described (Stein et al, 1985: Kadin et al, 1986: Greer et al, 1991). We report here that a patient with Ki-1 lymphoma had marked neutrophilia and extremely high titre of G-CSF (granulocyte colony-stimulating factor) in the serum and that the lymphoma cells produced a large amount of G-CSF. A previously healthy 10-year-old Japanese girl was admitted to our hospital for evaluation of right inguinal pain and high fever. On admission, X-ray photographs showed an osteolytic lesion of the right pubic bone. Laboratory studies revealed normal values for peripheral blood counts and routine serum chemistry. The possibility of malignant disease was considered and open biopsy of the right pubic bone lesion was performed. A diagnosis of NHL. large cell type, was made and the patient was treated with multi-drug combination chemotherapy and dramatic clinical responses were obtained. Following induction therapy, the patient received maintenance chemotherapy every month. After 4 months the peripheral blood showed marked leucocytosis and neutrophilia: WBC 1 3 7 x 10y/l, the differential count showed 94% mature neutrophils (absolute neutrophil count, ANC. 128 x 109/1). 3% lymphocytes. 3% monocytes: haemoglobin 13.5 g/dl. RBC 4.55 x lOl2/1, and platelets 207x lOy/1. There was n o evidence of infection on physical examination and laboratory studies. A CT scan showed enlargement of the inguinal, iliac and para-aortic lymph nodes. A biopsy of the right inguinal lymph node was performed: its histology was similar to the primary pubic bone lesion. The patient was treated with intensive chemotherapy but only a minimal response was obtained. The clinical course and WBC and ANC of the patient is shown in Fig 1. The relationship between tumour progression and ANC is seen. Although the neutrophil count decreased after the commencement of chemotherapy, neutrophilia developed soon after cessation of chemotherapy. The tumour became resistant to intensive chemo-radiotherapy and disseminated to the thorax: the patient died 6 months after diagnosis. A routine histologic examination was made of the patient’s biopsied lymph node. A portion of the tissue was used for immunohistochemical analysis. Immunoperoxidase staining was carried out by a n indirect method described previously (Fujimoto et al. 1988). Monoclonal and polyclonal antibodies used in this study were Ki-1 antigen (CD30). anti-epithelial membrane antigen (EMA). anti-leucocyte common antigen (LCA). alpha 1 antitrypsin, OKT9, OKTlO, HLADR. ILZR, etc. Histologically, the tumour cells were large in size with abundant cytoplasm, round to oval nuclei and prominent nucleoli, and were positive for Ki-1 antigen, EMA, LCA and

PANCREATIC CARCINOMA IN CHILDHOOD: Report of an Autopsy Case and a Review of the Literature

Clinical and histological findings of pancreatic carcinoma in a 6‐year‐old boy are reported. Gradual change of histological appearance of the tumor during his course of 3 years and elevation of serum alpha‐1‐fetoprotein (AFP) are documented. Two biopsy specimens showed immature histological appearance compatible with pancreatoblastoma, and autopsy material showed well‐differentiated adenocarcinoma with distinct ductal and acinar differentiation. Electron microscopy demonstrated zymogen‐like granules in the apical portion of the neoplastic cells. Immunoperoxidase method demonstrated AFP in the neoplastic cells in addition to alpha‐1‐antitrypsin. Literature of pancreatic tumor in the young was reviewed, and characteristics of this case were discussed. ACTA PATHOL. JPN. 35 : 1543–1554, 1985.

Cloverleaf Skull Syndrome--An Autopsy Case and Review of Literatures

An autopsy case of cloverleaf skull deformity associated with hydrocephalus, systemic skeletal malformation including facial dysostosis, fused elbow, syndactyly of the toes, odd digits and striking anomaly of tracheal cartilage is presented. Extra‐skeletal abnormalities included covered anus, dermal sinus and the absence of corpus callosum and the septum pellucidum. Severe basal skull deformity appeared to be a primary morphologic alteration associated with premature closure of the specific sutures, which terminally resulted in life‐threatening hydrocephalus. Histological investigation showed the abnormalities of endochondral ossification in the cartilage at the epi‐ and meta‐physis of the fused elbows. In addition, electron microscopical study revealed unusual fat droplet‐containing chondrocytes even in the resting and multiplicative phase. Association of the tracheal anomaly with this syndrome was disclosed in this case. Generalized bone and cartilage abnormalities not only in the skeletal system but also in the internal organs strongly suggest that this disorder involves generalized osteocartilagenous system. The present case makes a total of 14 cases of the cloverleaf skull syndrome reported in the Japanese literatures to date. Major clinical and pathologic findings of these cases were summerized.

Hunter's syndrome. An ultrastructural study of an autopsy case.

An autopsy case of a 10‐year, 8‐month‐old boy with Hunters syndrome Is reported with emphasis on the ultrastructural findings of almost all the organs, except the brain. Intracytoplasmic inclusion bodies were observed in various organs as follows: nerve cells and glia in the spinal cord, hepatocytes and Kupffer cells in the liver, sinusoidal endothelium of the spleen, proximal tubules, podocytes and epithelium of Bowmans capsule of the kidney, interstitial fibroblast‐like cells among cardiac muscle bundles, cardiac valves and aorta, exocrine and endocrine cells of the pancreas, adrenocortical cells, follicular epithelial cells of the thyroid, Leydig cells of the testis, chondrocytes, fibroblasts and endothelium of capillaries throughout the body. Three types of inclusion bodies were morphologically distinguishable. Type 1: clear vacuole, Type 2: zebra body, Type 3 : clear vacuole with a lipid‐like lamellar structure. The clear vacuole (Type 1) was thought to represent an accumulation of glycosaminoglycans, and the zebra body (Type 2), probably ganglioside. The type 3 inclusion body might be an intermediate and mixed form of the type 1 and type 2 inclusions. Histochemical study also suggested that the type 3 inclusion body contained glycosaminoglycan and a type of lipid. ACTA PATHOL JPN 38: 1175 ∼ 1190, 1988.

Syphilitic placentitis: Demonstration ofTreponema pallidum by immunoperoxidase staining

We report a case of early congenital syphilis in which the placenta showed diffuse proliferative villitis andTreponema pallidum was identified by indirect immunoperoxidase stain in formalin-fixed paraffin-embedded placental tissue. This is the first report demonstratingT. pallidum in placental tissue using an immunohistochemical method.