Malignant lymphoma of the central nervous system in Japan: histologic and immunohistologic studies.

Ninety-seven Japanese patients with so-called primary non-Hodgkin's lymphoma of the central nervous system (CNS-NHL), unrelated to the acquired immunodeficiency syndrome (AIDS) or organ transplantation, were reviewed. The patients' ages ranged from 1 to 87 years (median: 58 years) with a male to female ratio of 1.77:1. The most frequent past histories were acute appendicitis (appendectomy), head injury, uveitis or iritis, and gastritis or gastric ulcer. These patients presented with symptoms suggesting an expanding intracranial lesion with no signs of extracranial lymphomatous disease. Combined computed tomographic scans, angiography, and findings at surgery or autopsy showed that the cerebrum was the commonest site of involvement, 87% of all cases, with the frontal to temporal region being the most commonly involved. Histologically, the diffuse large-cell type was most frequent and 26% of lymphomas were of high-grade malignancy as defined by the Working Formulation. The reported frequency of high-grade CNS-NHLs in AIDS patients in the United States is much higher (over 60%). Immunohistochemistry on paraffin-embedded sections revealed a B-cell nature of the present series of tumors. In 16% of the cases, large numbers of small lymphoid cells with a positive reaction predominantly for anti-T lymphocyte antibodies surrounded the tumors or aggregated around the capillaries. The tumors which were infiltrated by small lymphoid cells showed more favorable prognosis than those which were not, suggesting a host reaction to tumor growth in these patients.

[Metastatic malignant lymphoma of the skull with extradural and extracranial extension. Case report].

A 72-year-old male was hospitalized because of a steadily growing mass (4 x 4 x 2 cm) in the frontal region, noticed following head injury. Six months earlier, he had declined treatment for malignant lymphoma (small non-cleaved cell type), discovered upon evaluation of a subcutaneous mass in his forearm. Neurological examination on admission was negative. Plain skull films revealed a large, osteolytic lesion with an irregular margin. Bone scintigraphy disclosed no uptake other than in the frontal bone. Computed tomography (CT) showed a large, homogeneously enhanced mass with both extradural and extracranial extension, which had destroyed the left frontal bone. The skull tumor was totally resected, and the diagnosis was malignant lymphoma, large cell, immunoblastic and plasmacytoid type. Postoperative combined chemotherapy with vincristine, cyclophosphamide, prednisolone, and adriamycin resulted in the complete disappearance of liver metastases. The patient was readmitted 2 months later, however, because of headache and gradually progressive impairment of consciousness. A CT scan disclosed brain metastasis in the paraventricular region. Radiation therapy (20 Gy) was administered but no further treatment was given. Skull metastasis is not uncommon in cases of malignant lymphoma, but a large mass with both extradural and extracranial extension is rare. Although aggressive chemotherapy was effective, prophylactic irradiation of the brain should have been considered in this case.

Ovarian involvement as an initial manifestation of malignant lymphoma.

An immunohistochemical study of a rare initial manifestation of non-Hodgkin's lymphoma (NHL) in the ovaries is presented. There have been very few reports to date on immunohistochemical studies of lymphoma involving the ovaries. A 53-year-old woman suffering from lower abdominal pain and abnormal genital bleeding was diagnosed as having a tumor in her left ovary by ultrasonic echograms and CT scanning. The patient underwent a simple total hysterectomy and bilateral salpingo-oophorectomies. The tumor, measuring 14 x 10 x 10 cm, was located in the left ovary and extended to the major omentum, mesocolon and left ureter. The histology of the tumor was that of NHL showing diffuse proliferation of small cleaved cells. Immunohistochemical studies of the ovarian tumor showed that the tumor cells were of a B-cell lymphoma nature with LCA+, MB-1+, lambda+, keratin-, IgG-, IgM-, IgA-, kappa-, and MT-1-. Although the main lesion involved the ovary, the case could not be identified definitely as primary lymphoma of the ovary.

[A comparative study of the treatment of vulvar carcinomas].

The results of treatment in our department of 24 patients with various vulvar carcinomas between 1969 and 1987 are described. There were 8 patients in stage I, 3 patients in stage II, 10 patients in stage III, 2 patients in stage IV and one patient in recurrence. Eighteen patients could be followed for more than 5 years. Particular attention was given to stage and treatments. The 5-year survival rate was 87% for stage I patients, 28% for stage III patients, 0% for stage IV patient and 0% for a recurrent patient. The 5-year survival rate was 80% (4/5) for operation group, 60% (3/5) for radiation group and 20% (1/5) for radiation and chemotherapy group.

[A case of primary intrasellar malignant germ cell tumor].

A case of a 13-year-old boy with a primary, intrasellar malignant germ cell tumor is reported. An immunohistochemical study revealed that the serum HCG and AFP levels were highly elevated and that the tumor contained elements of seminoma, choriocarcinoma, a yolk sac tumor, and an embryonal carcinoma. After operation, combined chemotherapy (cisplatin, vinblastine and bleomycin, i.e. PVB therapy) and irradiation were performed in hope of achieving the radiosensitizing effect of cisplatin. As a result, partial remission was obtained. Although PVB therapy is considered effective, combined radio-chemotherapy, as was done in this case, is thought to be better method for treating an intracranial, malignant germ cell tumor.

[A case of leiomyosarcoma of the duodenum--histologic diagnostic criteria of borderline malignancy].

A case of a leiomyosarcoma of the duodenum is reported. A 52-year-old man with a duodenal submucosal tumor underwent a pancreatico-duodenectomy. Histologically, the resected tumor revealed moderate cellular atypism and moderate pleomorphism, but revealed no mitotic figures. The histological diagnosis was leiomyoma. Nine years after operation, the tumor showed a recurrence and metastasis in the liver. Thus, the patient underwent a lobectomy of the liver. The resected liver tumor showed similar histological features seen in the primary duodenal tumor but with high mitotic activity. Histologically, the patient was diagnosed as having a metastatic leiomyosarcoma. This history suggests that a smooth muscle tumor of potential malignancy, as seen in this case, should be followed continuously, even though mitoses may not be recognized.

[Clinico-pathological study of uterine papillary serous carcinoma].

Eight cases of uterine papillary serous carcinoma were studied clinicopathologically. Postoperatively, four were stage I and four were stage III. Operations combined with radiation and chemotherapy were performed on the stage I cases. Three of them died within one year, and the other one died 3 years and 7 months after treatment. The prognosis was extremely poor even in stage. I. UPSC is histologically similar to ovarian serous carcinoma and is characterized by a papillary architecture supported by a fibrovascular stalk and a lining epithelium of single or stratified atypical cuboidal epithelium. Psammoma bodies were found in 3 out of the 8 cases.

Accumulation of neurofilaments in a sporadic case of amyotrophic lateral sclerosis.

We report a case of sporadic amyotrophic lateral sclerosis (ALS) characterized by a marked accumulation of neurofilaments in the cytoplasm of neurons. The neurofilament was identified by immunohistochemical and electron microscopic studies. The distribution of the accumulation in this case was unique, not only in the motoneurons of the anterior horn but also in the neurons of the other areas of the spinal gray matter, some nuclei in the brain stem, pontine reticular formation, substantia nigra, dentate nucleus in the cerebellum and pyramidal cells in the motor cortex. These observations shed light on the pathogenesis of ALS.

[Nonfunctioning adrenocortical carcinoma revealed by metastasis to the base of the tongue].

A 60-year-old man visited our clinic with the complaint of a tumor of the tongue base. Clinical and histological examination revealed that the tumor was metastasis from left adrenal carcinoma. Metastatic lesions were present in the tongue base, cervical lymph node and lung. There were no endocrinological abnormalities in the laboratory examination. The patient died about three months after admission. The autopsy disclosed nonfunctioning carcinoma of the adrenal cortex. Ninety-two cases of nonfunctioning adrenocortical carcinoma in Japan including the present patient are briefly reviewed.

Hepatoid adenocarcinomas of the stomach. An analysis of seven cases.

Hepatoid adenocarcinomas of the stomach are gastric carcinomas with both adenocarcinomatous and hepatocellular differentiations. They usually produce large amounts of alpha-fetoprotein (AFP) with a Concanavalin A-binding property of hepatic type. In this study, these carcinomas occurred in older persons, with the antrum being a common site. Observed grossly, growth of the tumors was nodular and massive. Prognosis was poor because of frequent liver metastases. In the cytoplasms of tumor cells, various serum proteins were identified, including AFP, alpha-1 antitrypsin (AAT), alpha-1 antichymotrypsin (ACT), albumin, and prealbumin. Localizations of ferritin, prothrombin, and transferrin were demonstrated with less frequency. Adenocarcinomatous foci were composed of well-differentiated, intestinal-type epithelial cells and often contained carcinoembryonic antigen. These adenocarcinomatous and hepatoid areas were often intermingled with each other. There were extensive venous involvements by tumor cells. The poor prognosis of the tumors may be attributed to these involvements as well as to production of AFP and presence of AAT/ACT, which have immunosuppressive and protease-inhibitory properties, respectively.

Long-dormant invasive mole associated with multiple malignancies.

A 65-year-old previously healthy housewife, gravida 3, para 3, was first diagnosed as Stage Ib carcinoma of the uterine cervix (poorly differentiated squamous cell carcinoma) and admitted. The external radiation of 5400 rad by telecobalt source was performed. No intracavitary radiation was added. After about 7 1/2 years the patient noticed a tumor of fist size on her buttocks, but she did not present in our clinic regularly. Because of enlarging tumor and general malaise she was readmitted a year later. On the fifth hospital day she died with ileus. Autopsy revealed osteosarcoma of buttocks in the radiation field, stomach cancer (tubular adenocarcinoma) with perforated peritonitis, and invasive mole of the uterine corpus. The patient's last pregnancy terminated as a full-term delivery at 26 years of age and she was 43 years at her menopause. The dormant period of invasive mole was 47 years after her last pregnancy, 30 years after her menopause, and at least 8 years after pelvic radiation.

[Transformation of ovarian yolk sac tumor to immature teratoma following chemotherapy].

The patient, a 28-year-old woman, in her ninth week of pregnancy, was operated on for stage Ia, mixed germ cell tumor (grade 3 immature teratoma + yolk sac tumor) of AFP decreased to the normal level. Eight months later, an intrapelvic mass and raised AFP were found. The extirpated recurrent tumor in the pouch of Douglas was a grade 2 immature teratoma with no yolk sac element. FAM chemotherapy was given again, and the patient is alive and well after taking oral UFT. As in testicular germ cell tumors, ovarian germ cell tumors can be converted to a more differentiated tumor following chemotherapy.

[Skull base metastasis of prostatic cancer presenting as exophthalmos--a case report].

A 72-year-old man was admitted to our hospital complaining of diplopia and right exophthalmos. Craniography and CT scan showed thickening of the right orbital roof and no intracranial lesion. Total acid phosphatase and prostatic acid phosphatase were high. Bone scintigraphy revealed high uptake at that lesion and the right pelvis. Unroofing of the right orbit and opening of the optic canal were performed in order to reduce intraorbital pressure. Microscopic examination revealed a metastatic lesion of prostatic cancer. Postoperatively, the patient was treated with "Honvan" and the course has been good.