Benjamin H. Landing

Cultivation in vitro of cells derived from a human osteosarcoma.

A cell line was derived from an osteosarcoma. Cells grew to high population density in liquid medium and formed colonies in agar medium. The cell line consisted of polygonal or fusiform cells resembling the cells of the original tumor. No collagen fibers or calcium apatite microcrystals could be demonstrated in the cultured cells by electron microscopy nor were virus particles detected. Isoenzyme studies of the cell line revealed the “B” band of glucose‐6‐phosphate dehydrogenase. Chromosome studies of the cell line revealed a range of stem line number from 58 to 65; chromosome counts ranged from 47 to 183. Some cells had marker chromosome of varied morphology and some had minute chromosomes. Chromosome breaks were found in 30.5% of the cells.

Bronchial anatomy in syndromes with abnormal visceral situs, abnormal spleen and congenital heart disease

Abstract The bronchial anatomy of a series of patients with congenital heart disease plus either or both abnormal visceral situs and abnormal spleen (absent, multiple, bifid or with unusually large accessory spleen or spleens) was studied by dissection and bronchographic methods. The results suggest that 5, and perhaps 6, syndromes can be delineated: (1) asplenia (Ivemark syndrome), characterized by bilateral eparterial bronchi (bilateral right or 3-lobed lung) and abnormal visceral situs; (2) polysplenia, characterized by bilateral hyparterial bronchi (bilateral left or 2-lobed lung) and abnormal visceral situs; (3) M-anisosplenia (third syndrome), characterized by bilateral eparterial bronchi and a relatively normal visceral situs; (4) F-anisosplenia (fourth syndrome), characterized by bilateral hyparterial bronchi with a visceral situs that is less abnormal than that observed in asplenia or polysplenia; (5) situs inversus, characterized by reversed bronchial pattern (eparterial bronchus on left and hyparterial on right) and reversed visceral situs; and (6) a group characterized by total anomalous pulmonary venous return, normal visceral situs, normal bronchial pattern, and 1 or more accessory spleens. Typical cardiac malformations were demonstrated in each of these syndromes. The results suggest that air or contrast bronchography can be of value in assessing patients with congenital heart disease plus abnormal visceral situs.

Sacrococcygeal teratoma: A 33-year experience

During the years 1941 through 1973, 48 patients, 16 males and 32 females, with sacrococcygeal teratoma were seen at the Childrens Hospital of Los Angeles. Forty-four patients have been followed, three are lost to follow-up, and one patient died 2 wk after excision of teratoma. Of the 44 patients with follow-up, 26 had teratoma with mature tissues only, all these patients are living. Six patients had tumor containing mature and embryonic tissues. Of these, five are living and one died with metastases of malignant teratoma 1 yr after excision of the primary tumor. Of the remaining 12 patients, 11 have died during the first 4 yr of life due to malignant teratoma and only one is living without recurrence 15 yr after excision of teratoma containing frankly neoplastic tissues. Recurrence and/or metastasis of malignant sacrococcygeal teratoma was lethal in all instances.

Pancreatic Islets in Older Patients with Cystic Fibrosis with and without Diabetes Mellitus: Morphometric and Immunocytologic Studies

Forty patients with cystic fibrosis (CF), including 34 who died above age 10 years without having developed clinical diabetes mellitus and 6 who died with both cystic fibrosis and diabetes mellitus, were studied. The mean age of the female patients with CF but not diabetes was 15.8 +/- 5.6 years; of males without diabetes, 17.2 +/- 6.4 years; of female patients with CF and diabetes mellitus, 20.2 +/- 6.9 years; and of males with CF and diabetes, 21.3 +/- 6.6 years. The mean number of pancreatic islets in microscopic sections for patients with cystic fibrosis but not diabetes was 4.18 +/- 2.76/mm2, and the value for patients with both cystic fibrosis and diabetes mellitus was 2.61 +/- 2.07/mm2. The lowest density of pancreatic islets (1.69 +/- 0.48/mm2) for cystic fibrosis was found in patients with the latest-stage pathologic lesion. Nesidioblastosis (presence of ductuloinsular complexes) was identified in 14 of 38 cystic fibrosis patients, both with and without diabetes mellitus. The pancreatic islets of both diabetic and nondiabetic patients with CF showed hypertrophy; the mean volume of the three largest pancreatic islets for CF only was 0.0117 +/- 0.00657 mm3 and that for cystic fibrosis and diabetes was 0.00795 +/- 0.00599 mm3, both values being larger than normal. Ratios of the amounts of islet endocrine cells, A cells, B cells, and D cells, were determined by peroxidase--anti-peroxidase labeled antibody staining. The B cells composed 43.0% of endocrine cell mass in cystic fibrosis alone and 30.1% in cystic fibrosis with diabetes mellitus, which were lower than normal proportions. The D cell values, 11.9% in cystic fibrosis and 15.1% in cystic fibrosis with diabetes mellitus, on the other hand, were greater than normal ratios.

Cerebral arachnoid cysts in infants

Nine patients with intracranial supratentorial arachnoid cyst are reviewed. The large cystic lesions compressed and indented the underlying cerebrum, and caused expansion of the overlying cranial vault. Their presence was suggested by physical examination in four children, and all nine were demonstrable radiologically. Cerebral arachnoid cysts have thin translucent walls and clear or pale yellow watery contents resembling cerebrospinal fluid. Clinical manifestations in the younger patients included poor feeding, excessive growth of the skull, and tense fontanels, a picture suggestive of hydrocephalus. Air injection studies showed extracerebral cysts having no demonstrable communication with cerebrospinal fluid pathways. Surgical treatment (resection, cystoventriculostomy, or shunting procedures) resulted in improvement in seven patients.

Infantile Hepatic Hemangiomas Clinical Features, Radiologic Investigations, and Treatment of 20 Patients

The clinical features, radiologic investigation, and treatment of 20 infants with hepatic hemangiomas are presented. Palpable abdominal mass (n = 18) and cardiac failure (n = 11) were the common presenting features. Nine patients had hyperconsumptive coagulopathy. Seven patients had other hemangiomas. Ultrasound (n = 15) showed the number and distribution of the hemangiomas within the liver. Hypoechoic and hyperechoic elements were present in addition to prominent vascular channels and diminished caliber of the distal aorta. Radionuclide sulfur colloid (n = 12) and labeled red blood cell (n = 7) studies showed the distribution and vascularity of the hemangiomas. Computed tomography (n = 8) revealed central hypointensity with marked peripheral enhancement after contrast. Arteriography now performed only as a prelude to therapeutic embolization demonstrated hypervascularity in each patient, contrast pooling in six and early draining veins in five. Magnetic resonance scanning (n = 3) showed decreased signal intensity on T1 images and high intensity signal on T2. In two patients, there was resolution or improvement of the hemangiomas without therapy. Four patients had surgery (lobectomy [2], trisegmentectomy [1], and surgical evacuation of a central hematoma [1]). Steroids and radiation were given to seven patients, and one patient also required therapeutic embolization. Steroids were the initial therapy in five patients, one of whom later required therapeutic embolization and another cyclophosphamide. Two patients were treated initially with radiation therapy, one of whom also needed emergency hepatic artery ligation. Seventeen of the 20 patients are alive and well from 6 months to 14 years after diagnosis.

Thrombosis, nephrosis, and corticosteroid therapy

Thromboemboli occurred in 5 children receiving corticosteroid therapy for idiopathic nephrosis; 3 had fatal pulmonary thromboemboli and 2 survived peripheral involvement. The factors related to thromboemboli in children with nephrosis are considered and discussed in relationship to the known and possible effects of corticosteroid therapy.

Cockayne Syndrome: Clinical Study of Two Patients and Neuropathologic Findings in One:

‡ Department of Dermatology, University of California School of Medicine, Irvine (deceased 1976). I N 1936, COCKAYNE DESCRIBED two English siblings with dwarhsm, retinal atrophy, and deafness.’ Ten years later he published a review of their progressive degeneration.2 2 In 1950, Neill and Dingwall reported two brothers with the same disorder. To date, over 30 such patients have been describedmostly of British origin .3-1 ’ The major and minor findings in this syndrome are summarized in Table 1. The syndrome has been described six times in siblings and perhaps once in a pair of twins. 12 Parental consanguinity was noted in the family described by Paddison and Moossy13 and in three Japanese families described by (Jhno and Ht>rooka.l‘~’The disorder thus shows the features of autosomal reces-

Renal Lesions and Clinical Findings in Thalassemia Major and other Chronic Anemias with Hemosiderosis

Renal lesions found in 21 autopsied patients with hemosiderosis, 18 with beta-thalassemia, two with Blackfan-Diamond anemia, and one with aplastic anemia included: cellular glomeruli with increased mesangial matrix; hemosiderin deposit in visceral and parietal glomerular epithelial cells; greater hemosiderin deposit in terminal straight portions of proximal convoluted tubules and distal convoluted tubules than in connecting segments, or collecting tubules, connective tissue ferrugination; lipofuscin in tubular epithelium and vascular smooth muscle; infrequently, intimal or medial arterial thickening, and, in one patient with thalassemia, an infarct resulting from arterial thrombus. The progression of these lesions over the course of disease, and possible effects on the various lesions of high transfusion regimen, oral pancreatin, vitamin E supplementation, or treatment with intramuscular, subcutaneous, or intravenous desferrioxamine were evaluated. The results of urine and renal function studies of 4 of the autopsied patients (3 thalassemia, 1 Blackfan-Diamond anemia), and 14 patients with thalassemia and 4 with Blackfan-Diamond anemia who were not autopsied, are presented. Rarely significant until preterminal stages, the renal functional changes reflect distal more than proximal tubule dysfunction.

Fibrosarcomas in Infants and Children

Abstract Of ten patients with fibrosarcoma treated during the past 11 years, two died as a result of tumor dissemination within five months, two are alive and tumor-free 10 years after the last recurrence, one is alive and well five years after the last recurrence and five are tumor-free nine months to three years after resection. Four of the tumors were congenital; none have metastasized. Rapid tumor growth and prompt local recurrence following incomplete excision characterized congenital fibrosarcoma in this series. Control of the tumor in the cases reported followed radical surgical resection. Radiation therapy and chemotherapy have, at times, striking temporary effects and may be effectively employed in preparation for wide excision. Prognosis is dependent almost entirely on the anatomic site of the lesion. If radical extirpation is feasible, the prognosis is excellent. When fibrosarcomas are located deep in body cavities, detection of the tumor may be delayed and adequate excision made difficult or impossible.