Louis B. Thomas

Mycosis fungoides: The pathology of extracutaneous involvement

A survey of the autopsy material of 45 clinically and histologically typical cases of mycosis fungoides (M.F.) revealed extracutaneous involvement in 32, or 71%. The lymph nodes showed diagnosable M.F. in 24 of these 32 cases. Viscera most commonly involved were, in order of decreasing frequency: lung, spleen, liver, kidney, thyroid gland, pancreas, bone marrow, and heart. Practically all organs and tissues of the body were found to be involved. Lesions of the lymph nodes and viscera have a characteristic, if not a specific cellular composition which closely resembles that of cutaneous lesions. The neoplastic cells are of lymphocytic origin. Among them, the hyperchromatic mycosis cells and the cells with convoluted, deeply indented, and cerebriform nuclei have considerable diagnostic value. They are usually essential for the differentiation of the cellular proliferation of M.F. from those of other malignant lymphomas. Mycosis fungoides is a pathologic as well as a clinical entity; the neoplastic cellular proliferation in both cutaneous and extracutaneous tissue is distinct and different from those of other lymphoid and histiocytic neoplasms which usually arise in extracutaneous sites, but occasionally also in the skin.

Vinyl-Chloride-Induced Liver Disease from Idiopathic Portal Hypertension (Banti's Syndrome) to Angiosarcomas

Histologic examination of liver tissue (eight autopsy and 18 biopsy specimens) and five spleens from 20 workers with vinyl chloride polymerization showed hepatic angiosarcomas in 15. In addition, a peculiar pattern of progressive portal-tract, inconspicuous intralobular and conspicuous capsular fibrosis was observed in the five workers without angiosarconma, in all the seven patients with angiosarcoma from whom tumor-free portions of the liver were available, and in two tumor-free biopsies from patients subsequently found to have angiosarcoma. The fibrosis was accompanied by splenomegaly. Hypertrophy and hyperplasia of both hepatocytes and hepatic and splenic mesenchymal cells were also seen. The histologic similarity to chronic inorganic arsenical poisoning, in which angiosarcomas also occur, and to idiopathic portal hypertension (Bantis syndrome) suggests that the latter syndrome at times results from unknown toxic, possible environmental, chemicals.

Leukopenia, Bone Pain, and Bone Necrosis in Patients with Acute Leukemia: A Clinicopathologic Complex

Excerpt Several recent studies have demonstrated the occurrence of bone necrosis in acute leukemia (1-4). Kundel, Brecher, Bodey, and Brittin (1) recently have studied a group of patients with acut...

Preliminary Experience with Total Nodal Irradiation in Hodgkin's Disease

Total nodal irradiation (TNI) of all major lymph-node areas was evaluated in 163 consecutive previously untreated patients with Hodgkins disease and compared to extended-field therapy in Stages I and II. The decreased relapse rate following TNI is reflected in improved survival rates. TNI has also been effective for Stage III-A, less satisfactory for Stage III-B. Extension of disease to extranodal sites has correlated with clinical staging and histopathology, defining certain groups as especially suitable for adjuvant chemotherapy.

Review of four cases of childhood hepatic angiosarcoma--elevated environmental arsenic exposure in one case.

Four cases of childhood hepatic angiosarcoma (HAS), representing the malignant form of infantile hemangioendothelioma, are described. The morphologic appearance of childhood HAS differs from the adult form in the following features: the associated presence of benign infantile hemangioendothelioma; the presence of dysontogenetic features; and an altered appearance of the angiosarcoma itself. It is postulated for these cases that the benign infantile hemangioendothelioma progressed to the malignant angiosarcoma. One of the four cases had exposure to elevated levels of arsenic in the environment that may have contributed to this progression. This latter case adds to published reports associating arsenic exposure with increased risk for hepatic angiosarcoma.

Pelvic exenteration for carcinoma of the uterine cervix. A 15-year experience.

From 1954 to 1969, 162 patients at the National Cancer Institute were treated with pelvic exenterations for carcinoma of the uterine cervix. A total cumulative 5‐year survival of 38% was obtained. Sixty‐eight of these patients presented with large, previously untreated lesions not amenable to lesser curative therapy. Their actuarial 5‐year survival was 48%. The remaining 94 patients were treated for radiation recurrent cancer with a 5‐year actuarial survival of 28%. Positive pelvic lymph nodes did not affect prognosis in patients treated for primary cancer, but survival decreased to 11% in those patients with recurrent disease and positive pelvic nodes. The 30‐day mortality was 7%, and the total intrahospital mortality was 17% (1 to 108 days). This mortality correlated with previous radiotherapy, patient age, preoperative medical status, operative time, and intraoperative transfusion requirements. Operative time and intraoperative transfusion requirements appeared to be related to the relative experience of the 17 surgeons involved in this study. Postoperative complications were similarly related to the above factors and have increased over the years in proportion to the degree that preoperative selection criteria have been liberalized. Over the 15 years encompassed in this study, there has been, however, a steady significant increase in cumulative 5‐year survival. Criteria for patient selection for pelvic exenteration are outlined, and salient suggestions are made for operative and postoperative management.

Diagnosis of liver involvement by lymphoma: Results in 96 consecutive peritoneoscopies

Percutaneous liver biopsy and multiple needle biopsies at peritoneoscopy were done in 47 patients with untreated Hodgkins disease and 46 patients with untreated non‐Hodgkins lymphoma to determine whether hepatic involvement was present. In patients with Hodgkins disease, grouped homogenously by extent of nodal disease, hepatic involvement was proven by these biopsy methods with the same frequency as has been reported for wedge biopsy at laparotomy. With lymphocytic lymphomas, percutaneous biopsy was positive in 40% of cases, but peritoneoscopy gave frequent false‐negative results in the remaining cases. The presence of hepatomegaly was valuable in predicting positive biopsies with lymphocytic lymphoma but was of no use with Hodgkins disease or other lymphomas. Liver function tests were not of value. With Hodgkins disease, the presence of symptoms and the classification in the Lukes‐Butler categories did not help in predicting positive biopsies. The gross appearance of early lymphomatous involvement of the liver is described. Peritoneoscopy is useful in the staging of Hodgkins disease and in the post‐therapy follow‐up of patients with Hodgkins disease and non‐Hodgkins lymphomas. There is minimal morbidity, even with multiple liver biopsies, and peritoneoscopy may be done repeatedly on the same patient, if necessary.

Vinyl chloride-associated liver disease.

Although polyvinyl chloride has been produced from vinyl chlride monomer for more than 40 years, recognition of toxicity among vinyl chloride polymerization workers is more recent. In the mid 1960s, workers involved in cleaning polymerization tanks were found to have acro-osteolysis. In 1974, the same population of workers was found to be at risk for an unusual type of hepatic fibrosis and angiosarcoma of the liver. We describe two cases of vinyl chloride-associated liver injury, one of hepatic fibrosis and one of angiosarcoma. Histologic features of these lesions are similar to the hepatic fibrosis and angiosarcomas resulting from chronic exposure to inorganic arsenicals. Preliminary studies suggest that the toxicity of vinyl chloride may result from formation, during high-dose exposure, of active metabolites by mixed function oxidases of the liver. Epidemiologic studies indicate an increased incidence not only of liver disease, but also of cancers of the brain, lung, and possibly other organs.

Pathology of angiosarcoma of the liver among vinyl chloride-polyvinyl chloride workers.

We described the histologic features of 13 hepatic angiosarcomas which developed in workers engaged in the polymerization of vinyl chloride to polyvinyl chloride. Although the histologic features varied considerably in different portions of the angiosarcoma in the same liver and in the angiosarcomas of the liver from different patients, many features were similar such as sinusoidal, papillary, and cavernous growth patterns coincident with the precursor lesions of proliferation and atypia of sinusoidal lining cells.

Epidemiology of Thorotrast-induced hepatic angiosarcoma in the United States

Abstract An epidemiologic investigation of hepatic angiosarcoma (HAS) in the United States, directed primarily at the years 1964–1974, has identified 26 cases of Thorotrast-induced HAS. This study indicates that the occurrence of Thorotrast-induced HAS was still increasing in the early 1970s and that a larger proportion of the more recent cases had relatively low-dose Thorotrast procedures and a prolonged latent period. A number of cases were identified who had a history of preexisting liver disease, associated vascular malformations, or exposure to arsenic or iron; the possible role of such additional risk factors is uncertain.