Edgar G. Harrison

Renal angiomyolipoma. A clinicopathologic study of 32 cases

The data from 32 cases of angiomyolipoma (renal hamartoma) are presented: 23 of the tumors were removed at surgery and nine at autopsy. The histopathologic findings are reviewed and the spectrum of gross renal involvement is emphasized. On the basis of the duration of symptoms and findings and the subsequent course of patients subjected to surgical resection, the clinical behavior of the tumor is assessed. Of particular interest is the relationship of this tumor to the tuberous sclerosis complex. Data presented indicate that many of the patients have various components of the syndrome, which in some patients have familial manifestations. It is inferred from this study that the tuberous sclerosis complex may vary in expression from a solitary renal tumor to the fully developed clinical syndrome.

Lymphoma of the spinal extradural space

Ninety‐four patients with lymphoma involving the extradural space with spinal cord compression proven at the time of laminectomy were reviewed. There were about three times as many patients with non‐Hodgkins lymphoma than with Hodgkins disease. The majority of those with Hodgkins disease had a proven histologic diagnosis before the onset of the spinal cord compression syndrome, whereas only 15% of those with non‐Hodgkins lymphoma had previously been so diagnosed. Plain roentgenograms of the spine were suggestive of tumor involvement in less than one‐third of the patients, whereas myelograms were invariably abnormal. As noted by others, the outlook for functional recovery and extended life expectancy is relatively good for patients with this type of cancer, in contrast to reports in the literature regarding prognosis for patients who have metastatic carcinoma with extradural spinal cord compression.

Familial erythrophagocytic lymphohistiocytosis. report of two cases and clinicopathologic review

An unusual syndrome of hepatosplenomegaly and fever followed by rapid deterioration and death has been described in 38 children from 21 families Pancytopenia, liver dysfunction, and bleeding developed prior to death from hemorrhage, sepsis, or lymphocytic meningitis. This report reviews the literature and adds a set of twins to the reported cases.

Pleural effusion in lymphoma.

Pleural effusion in lymphoma is usually, but not invariably, a poor prognostic sign. The presence of underlying parenchymal lung involvement or a chylous type of effusion does not further alter the prognosis, but the presence of malignant cells in the fluid probably shortens survival. Obstruction of lymphatic drainage of the lung and pleura by enlarged mediastinal nodes, with resulting lymphedema of the pleural space, is probably the most common cause of nonchylous pleural effusion. In contrast to patients with metastatic carcinoma, pleural involvement by lymphoma is uncommonly the major factor in fluid formation. Radiation therapy to the mediastinum or to the affected hemithorax is more likely to relieve lymphomatous effusions than is intrapleural therapy or systemic chemotherapy alone.

Lobular carcinoma arising in fibroadenoma of the breast

Lobular carcinoma may arise within the epithelial component of fibroadenoma of the breast, as evidenced by 5 cases reported from the files of the Mayo Clinic and 21 cases cited in the literature. The 5 cases reported here occurred in a series of 4000 cases of fibroadenoma during a 43‐year period. Lobular carcinoma is the more frequent type to be seen in fibroadenoma (in 22 of 26 cases); it usually develops in situ. Extra‐adenomatous carcinoma of the ipsilateral breast was identified in 11 of 26 cases, and 3 cases of contralateral carcinoma were noted in the entire series. Thus, lobular carcinoma arising within the epithelial component of a fibroadenoma has biological features similar to the behavior of lobular carcinoma of the breast in general. The prognosis has been favorable; lesions are usually encountered early; in only 2 of 26 cases were there axillary metastases.

Lymphadenopathy due to infectious mononucleosis: Its confusion with malignant lymphoma

Infectious mononucleosis (IM) may be diagnosed clinically, or, since lymphadenopathy is a major feature, lymph node biopsy may be performed to establish the diagnosis. However, in 5 of 11 cases in which lymph node biopsies were obtained, the initial histopathologic examination suggested lymphoma or Hodgkins disease. The histopathologic or clinical features eventually led to consideration of IM in all cases, and the diagnosis was confirmed by the usual laboratory findings and by follow‐up information. Histopathologic findings of IM include, 1. variable incomplete effacement of lymph node architecture, 2. “mixed” lymphoid proliferation, often with a prominent component of reticular lymphoblasts, producing a “mottled” appearance, 3. cellular filling of sinuses, and 4. moderate trabecular, capsular, and perinodal infiltration. Recognition of the reticular lymphoblast, which may simulate a Sternberg‐Reed cell, is important to prevent an erroneous diagnosis of Hodgkins disease in these cases. The histopathology resembles that of postvaccinial and related lymphad‐enopathies which may reflect its probable viral pathogenesis.

Histochemical and Immunohistochemical Study of Diffuse Large-cell Lymphomas

Ninety diffuse large-cell lymphomas (diffuse histiocytic lymphoma) were subclassified into B-cell, T-cell, and histiocytic types according to their enzyme histochemical and immunohistochemical characteristics. The B-cell type was characterized by presence of intracellular monoclonal immunoglobulin; negative or weakly positive diffuse acid phosphatase activity; and an occasional focal nodular pattern or preceding nodular lymphoma. The T-cell type was characterized by moderate, focal acid phosphatase activity; convoluted nuclear structure; and frequent preceding cutaneous manifestations. The histiocytic type was characterized by strong nonspecific esterases and diffuse acid phosphatase activity and presence of lysozyme and phagocytic activity. Most of the lesions (74 cases) were of the B-cell type. This group was further subdivided into follicular center cell type and B-cell immunoblastic sarcoma, according to the stage of cellular transformation. Preliminary clinical correlation suggests that the histiocytic type is most resistant to treatment. B-cell immunoblastic sarcomas were much more aggressive than neoplasms of the follicular center cell type.

Myelomonocytic leukemia in Hodgkin's disease

The records of 1,500 patients with Hodgkins disease seen at the Mayo Clinic from 1950 to 1967 were reviewed in search of any later‐developing myelo‐proliferative disorders. No cases of polycythemia vera, agnogenic myeloid metaplasia, or primary thrombocythemia were found in patients with pre‐existent Hodgkins disease. Three case reports of myelomonocytic leukemia (2 manifesting Auer bodies) developing 1 1/2, 5 1/2, and 18 years after the diagnosis of Hodgkins disease are presented. Myelomonocytic leukemia does not appear to represent a transitional stage of Hodgkins disease and is more likely related to therapy of the lymphomatous disease.

Management of hypertension of renal origin

Abstract Studies as clinically warranted were accomplished in more than 2,000 patients seen from January 1964 through December 1968 because of hypertension and suspected or proved renal disease. Data from the clinical history, physical examination, laboratory investigation and results of surgical or medical treatment are reported for 100 cases each of 5 entities—glomerulonephritis, pyelonephritis, renal cyst, renal tumor and apparent primary hypertension with renal disease. Comparison is made with data included from 100 cases of renal artery stenosis and hypertension. Female patients numbered 286, and predominated in the groups with renal artery stenosis and with pyelonephritis. Ages ranged from 6 to 81 years. Hypertension was less severe in the groups with renal tumor or cyst. Hypertensive changes of the retinal arterioles were more severe in the group with renal artery stenosis and in the group with primary hypertension, and renal function was more severely impaired with glomerulonephritis and primary hypertension. Surgical treatment was given 234 patients, 71 of whom later had normal blood pressures without medical treatment. For 366 patients medical treatment was primary; 125 of these usually maintained diastolic blood pressure of less than 90 mm Hg. Sodium restriction proved a valuable part of conservative management in most cases. Dialysis or transplantation, or both, were required for 49 patients. In the 600 cases, 507 patients are alive 2 to 13 years after initial examination, and 93 have died.

Mammography after mastectomy

Bilateral mammary cancer occurs with surprising frequency and is of greater clinical importance than is com monly recognized. Twenty-five patho logically-confirmed carcinomas were found in the remaining breast when 397 patients, postoperative from uni lateral mastectomy for cancer, were studied by a combination of thorough physical examination and mammog raphy. Physical examination alone de tected 15 tumors, of which 9 were thought to be malignant and 6 benign on the basis of their clinical character istics. Mammography demonstrated carcinoma in 10 additional cases when it was not suspected at physical exami nation. In 2 cases carcinomas which were clinically palpable could not be demonstrated in the mammograms. Whether a carcinoma appearing in the remaining breast after unilateral mas tectomy for carcinoma is a metastasis from the preceding malignant tumor or the opposite breast, or an independent, primary lesion may be impossible to distinguish by mammography alone. Stippled calcification in the second carcinoma may be evidence of a new primary growth, since it is usually in dicative of comedocarcinoma. However, comedo-like formations rarely may be