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Biomedical subjects

E E Lack

Publications and source records attributed to E E Lack.

At least 91 records · Page 5Linked to original sources
PubMed1985

Mediastinal germ cell tumors in childhood. A clinical and pathological study of 21 cases.

Twenty-one patients aged 16 years or less had been treated for a primary mediastinal germ cell tumor at the Children's Hospital, Boston Massachusetts, during the last 54 years. There were 13 boys and eight girls with the average age at diagnosis being 7 years (range 2 weeks to 16 years). Twelve mediastinal germ cell tumors were classified as pure teratoma, five contained embryonal carcinoma admixed with other germ cell components, and four were pure embryonal carcinoma. Of 12 patients with pure teratoma, 10 underwent complete surgical resection and were alive and well 1 to 13 years later; two children left untreated died of complications related to local tumor growth. Complete surgical resection was possible for only two of nine patients with embryonal carcinoma; both received adjuvant therapy and were alive and well 3 and 20 years later. Seven patients received radiation and/or chemotherapy but died of residual or metastatic disease. Successful treatment for children with embryonal carcinoma requires an operation aimed at either debulking or complete resection (if possible) coupled with early and aggressive combination chemotherapy. The role of radiation in primary therapy remains undefined with regard to curative intent.

Adolescent↗
PubMed1985

Carotid body hyperplasia in cystic fibrosis and cyanotic heart disease. A combined morphometric, ultrastructural, and biochemical study.

A combined morphometric, ultrastructural, and biochemical study was done on carotid bodies (CBs) obtained at autopsy from 213 patients in a pediatric and young adult population. The objective was to determine whether this group had statistically significant differences in sudden infant death syndrome (SIDS, n = 38), cystic fibrosis (CF, n = 30) and cyanotic heart disease (CHD, n = 17), compared with an age-matched control population (n = 128). Average combined weights of CBs in CF and CHD were significantly greater than those of controls in most age intervals (Student t test, P less than 0.05), and computerized planimetry showed an increase in both total surface area and area of "functional" parenchyma. There was diminished chief cell argyrophilia in 72% of CF CBs, and in 8 cases studied ultrastructurally there was moderate to marked depletion of dense-core neurosecretory granules. Most CBs from patients with CHD showed intense cytoplasmic argyrophilia similar to that of controls. Quantitative analysis for tissue catecholamines showed that dopamine was present in greatest concentration in each group of patients but was significantly elevated in CHD. There were no significant differences in morphometry, ultrastructure, or catecholamine content of CBs from SIDS victims, compared with age-related controls. These data add further support to CBs having a chemoreceptor role in humans with compensatory hypertrophy and hyperplasia occurring in most patients with chronic hypoxia due to CF and CHD. There were no significant findings to indicate that CBs play a direct role in the etiopathogenesis of SIDS.

Adolescent↗
PubMed1984

Ganglioneuroma presenting with differentiated skeletal metastases. Report of a case.

The authors report the case of a child with retroperitoneal ganglioneuroma and cytodifferentiated skeletal metastases. The primary tumor was surgically resected, and the child is alive and well 2 years later without additional therapy. This rarely documented phenomenon can be explained by spontaneous cytomaturation within both primary and metastatic tumor.

Bone Neoplasms↗
PubMed1984

Prognostic significance of complete surgical resection of pulmonary metastases in patients with osteogenic sarcoma: analysis of 32 patients.

Between 1972 and 1981, 93 patients with extremity osteogenic sarcoma without detectable metastatic disease were treated with surgery and adjuvant chemotherapy. Fifty-two patients remain continuously free of disease. Thirty-two of the 41 patients who relapsed had pulmonary metastases only and 26 underwent thoracotomy to remove all metastatic disease. Complete resection was possible in 11 of 26 patients as defined by the removal of all macroscopic disease, no microscopic disease at resection margins, and no histologic evidence of pleural disruption by tumor. Nine of 11 patients are currently free of disease with a median duration of most recent remission of 42 months (range, 3-72 months). Four of these nine patients have had only one relapse. Only two of 15 patients with incomplete resection of metastatic disease defined by the above criteria are currently free of disease for 57 and 101 months. A significant difference in survival from initial relapse for patients made surgically free of disease using this stringent criteria was observed even when the result is stratified for time to first relapse and number of pulmonary nodules (p = 0.005). A subgroup of patients within the group undergoing thoracotomies who can be expected to have an improved survival has been defined.

Adolescent↗
PubMed1984

APO therapy for malignant lymphoma of large cell "histiocytic" type of childhood: analysis of treatment results for 29 patients.

Twenty-nine patients with biopsy-proven malignant lymphoma of large-cell "histiocytic" type were treated with the APO protocol (vincristine, adriamycin, and prednisone). Treatment consisted of two years of therapy with a modified adriamycin-containing acute lymphoblastic leukemia regimen with preventive cranial irradiation and regional radiotherapy (for patients with clinically localized lymphoma). The median age was 13 years (range, two to 20 years). Thirteen patients had localized disease (stage I, II), and 16 had disseminated disease (stage III, IV). The median follow-up is four years (range, seven months to nine years), and Kaplan-Meier estimates of overall and relapse-free survival are 83% and 76%, respectively. No recurrences have been observed in primary or bulk sites of lymphoma in the group of children treated with chemotherapy only. We conclude that the APO protocol, which was modeled after an acute lymphoblastic leukemia regimen, combined with regional radiotherapy can produce long-term remissions for children with malignant lymphoma of large cell "histiocytic" type.

Adolescent↗
PubMed1983

Hepatocellular carcinoma. Review of 32 cases in childhood and adolescence.

The clinical and pathologic features of 32 children and adolescents with hepatocellular carcinoma (HCC) are reviewed. Their average age at diagnosis was 9.7 years (range, 5 months to 21 years) and there was a slight predilection for males in a ratio of 1.7 to one. Of eight patients with associated or underlying abnormalities, five had cirrhosis, two had an antecedent (or coexisting) tumor fulfilling pathologic criteria for hepatic adenoma, and one developed HCC ten years after nephrectomy and radiation therapy for a Wilms' tumor. Our data reaffirm the high mortality associated with HCC (91%). Three of five tumors classified as fibrolamellar type were amenable to surgical resection while only 15% of the remaining HCC were operable. The average duration of disease for patients with conventional HCC was 4.2 months, while those with the fibrolamellar variant had a more lingering course (average, 28.5 months). Available data indicate that the fibrolamellar variant should be distinguished from HCC with more conventional histology because of greater resectability and improved overall survival.

Adolescent↗
PubMed1983

Primary bronchial tumors in childhood. A clinicopathologic study of six cases.

A primary bronchial tumor was diagnosed in six children younger than age 12 years. These cases include four bronchial carcinoids, a low-grade mucoepidermoid carcinoma, and a granular cell tumor ("myoblastoma"). Lobar or segmental resection was performed in each case and remains the treatment of choice. The most common bronchial tumors in childhood are carcinoids with 17 other evaluable cases in the English Literature. This series includes the youngest child to be reported with bilateral choroidal metastases leading to blindness; in addition, there were clinical manifestations of gigantism and acromegaly possibly related to ectopic hormone production. Low-grade mucoepidermoid carcinomas rank second in frequency to carcinoids and have an excellent prognosis. Judging from the childhood bronchial tumors reported to date, proved examples of bronchogenic carcinoma are vanishingly rare.

Bronchial Neoplasms↗
PubMed1983

Heterochromia iridis and Horner's syndrome due to paravertebral neurilemmoma.

A case of heterochromia iridis and Horner's syndrome is reported in a 7-year old girl with paravertebral neurilemmoma. These clinical findings can be useful in the early diagnosis of mediastinal tumors in the paravertebral axis. While typically associated with neuroblastoma, these findings can be due to tumors which are inately benign--in this case neurilemmoma. The mechanism for heterochromia is briefly discussed.

Child↗
PubMed1983

Postmortem manifestations of echovirus 11 sepsis in five newborn infants.

Five cases of fatal neonatal echovirus type 11 infection were observed in the Boston area during the summer and fall of 1979. Four of the mothers experienced gastroenteritis with fever and abdominal cramps late in the third trimester of pregnancy. The clinical course of each case was characterized by jaundice, hepatosplenomegaly, and progressive hepatic failure; all five infants were severely hypotonic. At autopsy massive hepatic and adrenal hemorrhage and necrosis, with evidence of consumption coagulopathy, were found. Echovirus type 11 was isolated from various sites before and after death. The histopathologic features and epidemiologic aspects of these cases are briefly discussed.

Echovirus Infections↗
PubMed1983

Tumors of the exocrine pancreas in children and adolescents. A clinical and pathologic study of eight cases.

Eight patients with a tumor of the exocrine pancreas were treated or seen in consultation at the Children's Hospital Medical Center over the last 30 years. There were five girls and three boys ranging in age from 15 months to 18 years (average age: 8 years). Tumors were surgically resectable in six of eight cases--three by modified Whipple procedure and three by local resection. The average diameter of tumor was 13.6 cm (range: 3.8-20 cm). There were three adenocarcinomas of duct cell type, two acinar cell carcinomas, two acinar cell adenomas, and a papillary-cystic tumor of low-grade malignancy. One of the adenocarcinomas arose in a field of orthovoltage radiation and another occurred in a patient with tuberous sclerosis. Five of eight patients died, four with regional or distant metastases documented at autopsy. The patient with the papillary-cystic tumor experienced several local recurrences but was alive 14 years after initial surgery. Data from the literature indicate that acinar cell tumors are proportionately more common in children than in adults and have a somewhat better prognosis than tumors of duct cell type. Further reporting of cases with long-term follow-up is encouraged in order to gain clearer perspective of nonendocrine tumors of the pancreas in childhood.

Adenocarcinoma↗
PubMed1983

Teratomas in infancy and childhood. A 54-year experience at the Children's Hospital Medical Center.

The clinical and pathologic features of 254 teratomas from 245 patients are reviewed. All patients were 21 years of age or younger and were treated at the Children's Hospital Medical Center from 1928 to 1982. Tumors arose in the following anatomic sites: sacrococcygeal (102), ovary (94), head and neck (14), retroperitoneum (12), mediastinum (11), testes (eight), central nervous system (nine), liver (two), abdominal wall, and back (one each). One hundred twenty-four tumors (49%) were detected in the newborn period. Teratomas characteristically contained elements derived from all three embryonic germ layers. Tumors with any recognizable component of embryonal carcinoma or other malignant germ cell elements at the time of initial surgery were excluded. Immature teratomas were significantly larger than mature tumors in nearly all sites where statistical analysis was possible. The single most important factor affecting prognosis was whether the tumor could be resected successfully at initial surgery. No patient who did not undergo surgery, or in whom only partial resection was possible, survived the disease--regardless of other treatments used. Based upon the experience reported here the authors conclude: 1) complete surgical resection is the treatment of choice for all childhood teratomas; and 2) this is one of the few childhood tumors where decisions regarding adjuvant therapy must be individualized, particularly with regard to site of origin and age of the patient.

Adolescent↗
PubMed1982

Gingival granular cell tumor of the newborn (congenital "epulis"): ultrastructural observations relating to histogenesis.

The authors report the electron microscopic features of gingival granular cell tumors (GGCT) resected from two newborn girls. Although origin from odontogenic epithelium has been proposed, the ultrastructural findings strongly support histogenesis from stroma (mesenchymal) cells. Tissue assay for estrogen receptors was negative, but considering the marked predilection of GGCT for females, a hormonal factor (or factors) may still be important in the development of GGCT. Clinical and morphologic features distinguishing GGCT from granular cell "myoblastomas" are briefly emphasized.

Female↗
PubMed1982

Benign vascular tumors of the liver in infants: sonographic appearance.

The distinctive sonographic appearance of benign vascular tumors of the liver (one hemangioendothelioma and two mesenchymal hamartomas) in three infants is presented. Each child had hepatomegaly and congestive heart failure, and all had confirmatory arteriography. The sonographic pattern of a complex mass, dilated proximal aorta, and enlarged nutritive and draining vascular structures should alert the radiologist and clinician to this diagnosis.

Angiography↗
PubMed1982

Hepatoblastoma. A clinical and pathologic study of 54 cases.

The clinical and pathologic features of 54 children with hepatoblastoma are reviewed. Their average age at diagnosis was 17 months (range: 4 months-4 1/2 years) and there was a predilection for males in a ratio of nearly 2:1. Unusual manifestations included isosexual precocity (two cases), hemihypertrophy and Budd-Chiari syndrome (one case), and synchronous Wilms' tumor (one case). Thirty-two of 49 conventional hepatoblastomas were subclassified as epithelial type (19 predominantly embryonal, 13 predominantly fetal), and 12 had mixed epithelial-mesenchymal histology. Pathologic material in five cases was too limited for reliable subclassification. Hepatoblastomas classified as anaplastic type had more primitive histology with a predominance of small- to medium-sized cells in diffuse sheets. The overall mortality was 76% and the average duration of disease from diagnosis to death was 8 months (range: 1 month-27 months). Complete surgical resection remains the key treatment for achieving long-term survival and was attempted in 33 of the 54 children (61%). There were 13 long-term survivors followed for an average of 9 years. The most common type of hepatoblastoma in these children was epithelial with a predominantly fetal pattern. None of the children with anaplastic tumors survived.

Carcinoma, Hepatocellular↗
PubMed1981

Granulosa theca cell tumors in premenarchal girls: a clinical and pathologic study of ten cases.

Granulosa theca cell tumor (GTCT) in the premenarche accounted for 7% of all ovarian tumors treated at the Children's Hospital Medical Center from 1928 through 1979. The average age of the ten girls at the time of diagnosis was 5 years (range 11 months-121/2 years) with precocious "pseudopuberty" and an abdominal mass being the most common presentation. GTCTs were solitary (five right, five left) with an average diameter of 12.1 cm. Histologic examination showed a predominantly diffuse or solid pattern with prominent luteinization; Call-Exner bodies and folded ("coffee-bean") nuclei were inconspicuous. Ultrastructural examination of one GTCT confirmed the presence of both granulosa and theca components with the latter showing extensive luteinization; estradiol, testosterone and prolactin were demonstrated in the same tumor using immunologic techniques. The average follow-up time was 21 years with nine of the ten patients being followed for more than ten years. Salpingo-oophorectomy resulted in cures despite the occurrence of tumor spillage in two patients. The prognosis for GTCTs in the premenarche appears more favorable than for those occurring in adulthood, but further study is needed to completely define their full biologic potential; the significance of subsequent breast cancer in two long-term survivors merits further investigation as well.

Basement Membrane↗
PubMed1981

Solitary lung nodule due to Dirofilaria immitis (dog "heartworm").

The filarial parasite Dirofilaria immitis (dog "heartworm") is the cause of a progressive zoonosis among canine species in the United States; it is rarely transmitted to man, in whom it can present as a solitary lung nodule. There are currently no reliable clinical or radiologic features to distinguish it from other solitary necrotizing granulomas, and thoracotomy is currently the only means of establishing the diagnosis. D immitis can be identified and properly classified by routine light microscopy despite being immature and partially necrotic. Canine Dirofilariasis may well assume even greater medical importance because of its expanding geographic range and the concomitant increase in the number of cases observed among humans.

Aged↗
PubMed1981

Angiomyolipomas and polycystic renal disease in tuberous sclerosis. Ultrastructural observations.

We report the ultrastructure of bilateral renal angiomyolipomas in a case of tuberous sclerosis. The patient also had adult-type polycystic renal disease (Potter type 3) with systemic hypertension. Smooth muscle differentiation was supported by immunofluorescence localization of contractile protein using both smooth muscle and platelet-specific antibodies against myosin. There were cells with ultrastructural features intermediate between mature smooth muscle and fat suggesting origin from progenitor pericytic cells closely related to vascular endothelium. There was also evidence of secretory or synthetic capacity by some mesenchymal cells raising the possibility that hypertension in part may have been hormonally mediated by tumor. A unifying theory of histogenesis is proposed with special reference to the study of Wassermann regarding embryonal lipogenesis.

Adolescent↗