Hart Isaacs

Perinatal (congenital and neonatal) neoplasms: a report of 110 cases.

One hundred ten congenital and neonatal tumors encompassing a 25-year period are described and compared with similar published cases. Forty percent are classified as histologically malignant, and 65% of neonates with malignancies died. The types, frequency, and clinical features of neoplasms encountered in the perinatal period are markedly different from those observed in older children and adolescents. Their biological behavior and response to therapy are also dissimilar. Leukemia was responsible for the largest number of deaths followed by neuroblastoma and brain tumors.

Ovarian cysts and tumors in infancy and childhood

Abstract Cysts and tumors of the ovary are not common in children. At the large childrens hospitals only one to four patients are seen annually with ovarian enlargement.1–11 This paper summarizes our experience with 99 patients with ovarian cysts or tumors seen at the Childrens Hospital of Los Angeles during the years 1951 through 1973.

Lobar emphysema, cystic adenomaloid malformation, pulmonary sequestration, and bronchogenic cyst in infancy and childhood: A clinical group☆

Abstract Sixty-four cases of congenital cystic lesions of the lung fit comfortably into the following four distinct categories: lobar emphysema—16 cases; cystic adenomatoid malformation—19 cases; pulmonary sequestration—15 cases; and bronchogenic cysts—14 cases. In the symptomatic patient, aggressive diagnostic studies are recommended, with excision of the offending lesion. Surgical treatment, if not unduly delayed, is effective and safe (97% survival in this series) although the postoperative complication rate is high.

Benign testicular tumors in children with congenital adrenal hyperplasia

The association between testicular tumors/nodules and congenital adrenal hyperplasia (CAH) has been previously reported. From 1960 to 1989, three patients (13 to 18 years old) with long-standing CAH developed testicular masses. Two patients with 21-hydroxylase deficiency were diagnosed in the neonatal period while one other with 11-hydroxylase deficiency was diagnosed at 3 years of age when he presented with sexual precocity. In all three patients, medical compliance was poor. The testicular masses were bilateral in two patients and unilateral in one, measured 1 to 2 cm, and occupied only the upper half of the testicle. Testicular biopsy specimens were obtained after at least 6 months of evidence of compliance with the adrenocorticotrophic hormone (ACTH) suppressive medication and failure of the nodules to regress. On gross examination the masses appeared to be firm yellow brown nodules. Light microscopy showed interlacing strands, cords, and rests of cells resembling interstitial (Leydig) cells but with no Reinke crystalloids. Electronmicroscopy in all patients showed variable amounts of both smooth and rough endoplasmic reticulum, the later with occasional dilated cisternae. Follow-up ranged from 6 months to 6 years. No further surgical treatment has been necessary. There has been no evidence of recurrence, distant metastases, or secondary malignancies during the time of follow-up. These findings suggest that testicular tumors may develop from chronic excessive ACTH stimulation of a putative pluripotential testicular cell, a Leydig cell, or an adrenal cortical rest. Unlike other testicular tumors these do not require orchiectomy as the initial form of therapy.

Benign and malignant ovarian tumors in children and adolescents: A review of 63 cases

Sixty-three patients (15 months to 17 years of age) with ovarian tumors were seen. Fifty-six patients had germ cell tumors and 7 epithelial tumors, 6 patients had bilateral tumors, 47 patients had teratomas (41 benign, 3 with embryonic tissue, and 3 malignant), 6 germinomas, 1 endodermal sinus tumor, and 2 mixed germ cell tumors. Of the patients with epithelial tumors, six had cystadenomas and one cystadenocarcinoma. Abdominal pain was the most common symptom, and an abdominal mass the most common sign. A calcification on abdominal films was seen in 29 patients with benign teratoma. At surgery, the opposite ovary was bivalved or biopsied in 31 patients with benign tumors. Two patients had teratomas burried in the ovarian tissue. Among 47 patients with benign tumors, 37 have remained well after the operation and 10 were lost to follow-up. Two girls with embryonic teratoma are well 71 and 19 months after diagnosis but the third died with embryonal carcinoma. Of the 13 patients with malignant tumors, 8 are alive disease free 4 1/2 to 17 years after diagnosis, 4 are dead, and 1 is lost to follow-up (disease free 15 months after diagnosis).

Inflammatory bowel disease in glycogen storage disease type Ib

We have observed the development of chronic inflammatory bowel disease, indistinguishable from Crohn disease, in two boys with glycogen storage disease type Ib (GSD-Ib). A chance association of these diseases in two patients is unlikely. Studies of their neutrophils showed severe chronic neutropenia (mean absolute granulocyte counts of less than 500 cells/microliter) and markedly deficient chemotactic response (less than 5% of reference values) in the patients with GSD-Ib and normal neutrophil values in four patients with glycogen storage disease type Ia (GSD-Ia). Monocyte counts and responses to chemotactic stimulation were normal in both GSD-Ia and GSD-Ib. Chronic inflammatory bowel disease appears to be associated with GSD-Ib, and neutrophil abnormalities may be involved in the pathogenesis of the bowel inflammation.

Pulmonary blastoma associated with cystic lung disease

A 2 ½‐year‐old child with chronic cystic lung disease also had a large pulmonary tumor develop. A diagnosis of pulmonary blastoma, a rare primary malignant tumor of the lung, was made on pathologic examination. A review of the literature yielded reports of several patients with pulmonary blastoma who had either preexisting or concurrent cystic lung disease. Patients with chronic cystic pulmonary disease may have a predisposition for this malignant tumor.

Hemangiopericytoma in infants and children: A report of six patients

The case histories of six infants and children (five male and two female) with hemangiopericytoma have been reviewed (two patients were born with the tumor). The tumor originated on an extremity in three patients, in the intranasal fossa in one, in the neck and upper mediastinum in one, and in the abdomen in another. In the patients with the tumor on the extremity, the lesion was removed with a margin of normal tissue. These patients remained well for 2,8, and 1.5 years after diagnosis. In the patient with an intranasal mass, the tumor was removed, but local recurrence developed 3 months later. The recurrent tumor was removed, and the patient remained well for 10 years. The patient with the neck and upper mediastinal tumor was born with the lesion and presented with airway obstruction at 2 months of age. This patient received radiotherapy and cyclophosphamide with no apparent response. He underwent partial excision of the lesion 1 month after diagnosis but died 1 month later from massive hemorrhage into the airway. The patient with the abdominal mass had extensive retroperitoneal tumor, and excision was not possible. He received chemotherapy and radiotherapy, and although the tumor decreased in size, osseous metastases developed 4 months after diagnosis. The metastatic lesions in this child disappeared with further chemotherapy, and at second-look operation 9 months after diagnosis, the tumor was removed. Postoperatively, new bone metastasis developed but again responded to chemotherapy with complete disappearance of the metastatic lesion. The patient had no evidence of disease 16 years after diagnosis.

Benign liver tumors in infancy and childhood. Report of 48 cases.

This is a clinicopathologic study of 48 patients with benign liver tumors seen during a 32 year period; 2 adenomas, 3 focal nodular hyperplasias, 14 mesenchymal hamartomas, and 29 hemangiomas were reviewed. All patients except those with adenomas were younger than 5 years. Thirty-four patients were symptomatic, 24 of whom were diagnosed by open liver biopsy, whereas 10 with hemangioma had their diagnoses based on clinical and radiologic findings. Death due to tumor occurred in four patients with hemangioma. Infants and children with liver hemangioma and congestive heart failure should receive steroids, digitalis, diuretics, and radiation, alone or in combination. If medical treatment is unsuccessful and the hemangioma is localized to one lobe, resection of the tumor can be performed, but if the lesion is massive, ligation or embolization of the hepatic artery should be considered.

Rectal suction biopsy for the diagnosis of Hirschsprung's disease

The diagnosis of Hirschsprungs disease is at times difficult, particularly in the young patient. Since 1972 we have used rectal suction biopsy as a screening technique in neonates and infants with failure to pass meconium or evidence of obstruction. In addition, it is used to confirm the diagnosis of Hirschsprungs disease when suspected by barium enema study. This technique has been used in 444 patients, 302 of whom were less than one year of age. No anesthesia is necessary, and there have been no associated complications. Only one patient early in the study had an initial misdiagnosis. There have been no false-positive or false-negative specimens since this initial problem, and no patients have undergone inappropriate pull-through procedures for suspected Hirschsprungs disease. It is recommended that all neonates who do not pass meconium in the first 48 hours of life undergo rectal suction biopsy to establish the diagnosis of congenital megacolon.