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Biomedical subjects

E E Lack

Publications and source records attributed to E E Lack.

At least 109 records · Page 6Linked to original sources

Sacrococcygeal germ cell tumors in childhood: an updated experience with 118 patients.

The histopathology of 118 sacrococcygeal germ cell tumors (SGCT) was correlated with clinical presentation, therapeutic management, and prognosis. There were 97 teratomas (78 mature, 19 immature), 19 embryonal, and 2 anaplastic carcinomas. Mature and immature teratomas usually presented externally (AAP type I) permitting relatively early detection and surgical removal. The immature group was significantly larger (11.6 cm vs. 7.5 cm) with a greater incidence of subsequent embryonal carcinoma (16%). The embryonal and anaplastic carcinomas were diagnosed at a later age (average 21 mo) and had a substantial presacral or endopelvic component (AAP types II-IV); this group had the worst prognosis with no survivors. Our data suggest that combined analysis of clinical findings and histopathology may help to identify some of the children at risk for early recurrence.

Carcinoma↗

Congenital mesoblastic nephroma: relationship to other renal tumors of infancy.

Although nephroblastomas (Wilms tumor) do occur in the newborn most of the solid renal tumors in this age group constitute a distinctly different pathologic entity, congenital mesoblastic nephroma. While a more cellular histologic variant of congenital mesoblastic nephroma has been recognized recently, which occasionally may follow a malignant course, the tumor with conventional histology has never been reported to metastasize and follows a benign course when treated by adequate local excision alone. We report our 50-year experience with 11 cases of this tumor (9 cases with conventional histology and 2 with cellular variant histology). Typically, the tumor presented in the newborn as a large asymptomatic mass. Local invasion was never seen and the tumors were resected without difficulty. Grossly, the tumors usually resembled a uterine "fibroid" and, unlike nephroblastomas, rarely exhibited hemorrhage or necrosis. Microscopically, the conventional histologic congenital mesoblastic nephroma was composed of sheets of spindle-shaped cells. The cellular variant of congenital mesoblastic nephroma exhibited increased cellularity and a higher mitotic index. The relationship of congenital mesoblastic nephromas to other neonatal renal tumors is discussed and a unifying schema of neoplasia in infantile renal tumors is proposed.

Diagnosis, Differential↗

Gingival granula cell tumors of the newborn (congenital "epulis"): a clinical and pathologic study of 21 patients.

The clinical and pathologic features of 24 gingival granular cell tumors in newborns are reviewed. These tumors occurred exclusively in females and were localized on the anterior alveolar ridge (maxilla: 14; mandible: 9); the gingiva overlying future canine and lateral incisor teeth was most frequently involved. Follow-up data for 15 patients (average duration 15 years) indicated a lack of tumor recurrence despite incomplete resection in 11 instances. Tumors resected early in the newborn period were larger (maximum diameter 2.0 cm) and showed confluent to nodular submucosal growth with relatively less collagen. Some showed features suggesting involution. The histology in most cases supported a mesenchymal origin rather than one from odontogenic epithelium. Immunohistochemical staining for selected oncofetal and other antigens was negative. Electron microscopy did not entirely resolve the controversy regarding histogenesis.

Cell Transformation, Neoplastic↗

Botryoid rhabdomyosarcoma of the biliary tract.

We report the clinical and pathologic features of five children wih a botyroid rhabdomyosarcoma of the biliary tract. There were three boys and two girls ranging in age from 11/2 to 51/2 years at diagnosis. Obstructive jaundice was the usual presenting sign, often with fever and hepatomegaly. Of four tumors involving the common bile ducts, two extended into porta hepatis and adjacent liver parenchyma; the fifth tumor arose in the wall of the cystic duct. Each had a typical botryoid (grape-like) configuration with a cambium layer of tumor cells beneath biliary epithelium. Rhabdomyoblasts were identified in all cases and confirmed by ultrastructure in one. Our data, along with review of other cases reported in the English literature, indicate a rather guarded prognosis. Although metastases have developed in 40% of cases, death was usually due to the effects of local invasion by tumor. Aggressive adjuvant therapy currently in use for childhood rhabdomyosarcomas may lessen the high mortality associated with this tumor.

Bile Duct Neoplasms↗

Thymic hyperplasia with massive enlargement: report of two cases with review of diagnostic criteria.

Two cases of massive thymic hyperplasia (MTH) are reported in boys 11 and 14 years of age. Each was detected on routine chest roentgenogram as a large anterior mediastinal mass. Total thymectomy was successfully performed and the two patients were alive and well 9 and 17 years later. The thymectomy specimens weighed 324 and 490 gm, respectively, and had uniform histologic anatomy without lymphoid nodules. Morphometric study documented the massive increase in thymic parenchyma (i.e., true hyperplasia) with retention of a normal proportion of connective tissue expected for age. Since thymic size and structure normally become modified with age, it is essential to consult standard reference data when evaluating hypertrophy and hyperplasia. Gross and histologic features distinguishing MTH from childhood thymoma are discussed.

Adolescent↗

Infiltrating (intramuscular) lipomas and angiolipomas. A clinicopathologic study of six cases.

Infiltrating (intramuscular) lipomas and angiolipomas are benign mesenchymal tumors that usually appear as a deep, nontender mass within soft tissue, particularly in the extremities. The average tumor size in six cases studied was 11.2 cm (range, 2.0 to 22.0 cm). On gross examination, these tumors are circumscribed but unencapsulated, with infiltration of adjacent skeletal muscle. The correct preoperative diagnosis is seldom made, and the characteristic infiltrating pattern seen microscopically can lead to a mistaken diagnosis of sarcoma. Soft-tissue roentgenograms can be helpful in diagnosis and localization. The recommended mode of therapy is complete local excision with tumor-free soft-tissue margins. None of the six patients described here have experienced recurrence of tumor an average of two years after surgical resection. Prolonged follow-up is recommended, however, since inadequate resection can result in late tumor recurrence.

Adult↗

Granular cell tumor: a clinicopathologic study of 110 patients.

The clinicopathologic features of 118 granular cell tumors (GCT) encountered at two affiliated hospitals were reviewed. A total of 110 patients were affected over this 32-year period of study (71 men, 39 women), and in 5% GCT were multiple. Patients ranged in age from 16 to 58 years (average 32 years) and were symptomatic for an average duration of 11 months prior to diagnosis. There was a greater than expected frequency of GCT among black patients (29%). Although tongue was the single most common anatomic site involved, relatively more GCT (44%) occurred in skin or subcutaneous tissue. Less common locations were breast parenchyma (10 cases), rectal mucosa and anus (6), vulva (4), esophagus and larynx (2 cases each). The correct preoperative diagnosis of this protean tumor was made in only three patients. GCT were surgically treated with the average diameter of resected tumor being 1.2 cm (range 0.2--3.5 cm). Pseudoepitheliomatous hyperplasia was noted in 11 tumors and in one vulvar GCT there was overlying in situ squamous cell carcinoma. Tumors were incompletely excised in 24 of 56 patients having adequate followup; only five of these 24 patients experienced a local recurrence of tumor. Malignant behavior was not observed. Results of histochemical and ultrastructural study are briefly discussed. The precise histogenesis of GCT is uncertain but Schwann cell origin is favored in most cases.

Adenofibroma↗

Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors.

The clinical and pathologic features of 12 extra-adrenal paragangliomas of the retroperitoneum are presented. The patients (eight men, four women) had an average age of 43 years at the time of diagnosis; three had symptoms related to excess catecholamine secretion. Complete surgical resection was attempted in each case but was possible for only four patients. Five tumors were malignant; four patients died from regional and/or distant metastases 17 months to 10 years after surgery. One patient was alive with metastatic paraganglioma 25 years after initial treatment. Each patient with metastatic or locally recurrent paraganglioma had incomplete resection of the primary tumor. An important microscopic feature of each paraganglioma was the presence of cytoplasmic argyrophilia. Three of the five metastasizing paragangliomas showed readily identifiable mitoses or evidence of vascular invastion; the two other cases and all of the benign tumors lacked these features. Our findings suggest that in some cases histology may prove helpful in evaluating the malignant potential of these tumors.

Adult↗

Immunoperoxidase localization of human papillomavirus in laryngeal papillomas.

Five antigenically distinct serotypes of human papillomavirus (HPV) cause various cutaneous verrucae with distinct clinical features. Laryngeal and other mucosal papillomas, also suspected to have a viral etiology, were examined for the presence of a genus-specific (common) antigen of HPV using a peroxidase-antiperoxidase (PAP) technique. The primary antiserum used was reactive against laryngeal papillomas from 26 of 35 randomly selected patients (74%); overall, 49 of the 102 squamous papillomas stained were positive for HPV (48%). HPV antigen was not demonstrated within frankly dysplastic squamous cells. Nuclear localization of virus was confirmed by electron microscopy in 3 cases positive for HPV. PAP localization of HPV is rapid and more efficient than routine electron microscopy. Our data indicate a common pathogenesis for some papillomas and cutaneous verrucae and suggest a future role for immunohistochemistry in evaluating the epidemiology and pathobiology of laryngeal papillomatosis.

Antigens, Viral↗

Fatal-cat trasmitted tularemia: demonstration of the organism in tissue.

The case reported represents an unusual mode of transmission of tularemia from cat to man, with fatal outcome. The use of the modified Dieterle spirochete stain demonstrated F tularensis in tissue when all other routine stains failed. Direct immunofluorescent staining confirmed the presence of the organism in tissue sections. F tularensis and the etiologic agent of Legionnaires' disease are similar only in their ability to stain with the modified Dieterle stain and their inability to stain with conventional histochemical technics.

Aged↗

Intrathoracic paravertebral malignant paraganglioma.

This article reviews the clinical and pathologic features of intrathoracic paravertebral paragangliomas. Including the present case, there have been 31 tumors reported in the English literature; the average age of patients was 29 years, with a sex distribution of 20 men and 11 women. Fifteen patients (48%) had symptoms related to excess secretion of catecholamines; the remaining 16 tumors were clinically nonfunctional. Seven patients (22%) had multiple paragangliomas. Complete surgical resection was attempted in 25 patients, 13 were alive with no evidence of tumor an average of 2.2 years later. Tumor was locally invasive in five patients, with involvement of the vertebral canal and symptoms of spinal cord compression. Malignant behavior with distant metastases was observed in two patients. As shown by the present case, the Grimelius stain is a useful diagnostic technique for demonstrating cytoplasmic argyrophilia of neoplastic chief cells. Electron microscopy demonstrated neurosecretory granules (average core diameter, 100 nm). "Light" and "dark" chief cell types were inconspicuous. Due to important clinical and pathologic differences, paravertebral paragangliomas should be distinguished from similar tumors occurring in the anterosuperior mediastinum (aorticopulmonary paragangliomas).

Adult↗

Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature.

The light microscopic and ultrastructural features of an aortico-pulmonary paraganglioma (A-PP) are presented. The tumor was characterized by organoid clustering of neoplastic chief cells to form Zellballen. Argyrophilic granules were demonstrated within chief cell cytoplasm using a modified Grimelus technique. Ultrastructurally, three distinct cell types were present within the tumor: endothelial cells, pericytes and neoplastic chief cells. Membrane-bound neurosecretory granules were present and measured 100 to 2000 nm in diameter. "Light" and "dark" chief cells were less distinct than previously reported in other head and neck paragangliomas. Analysis of the 36 documented A-PP reported in the English literature reveals that the tumor has been either incompletely excised or has been considered unresectable in one-third of the cases. The reported surgical mortality is 9%, or approximately equal to the incidence of malignant behavior. The treatment of choice is surgical resection but when this is not possible, radiation may be a useful adjunct in therapy.

Adolescent↗

Adrenal cortical carcinoma: a clinical and pathologic study of 49 cases.

The clinical and pathologic features of 49 patients with adrenal cortical carcinoma are presented. The series included 26 men and 23 women with a mean age of 34 years. The average duration of symptoms prior to diagnosis was 6.6 months. Eighteen patients (37%) presented with recognizable endocrine syndromes secondary to excess tumor steroid production. Surgical excision was the only mode of therapy to offer any opportunity for prolonged survival. Nine of the 21 patients (43%) with a completely resectable tumor were alive with no evidence of disease an average of 7.2 years postoperatively. Death due to metastatic tumor occurred in 36 of the 49 patients (73%) an average of 8.7 months following diagnosis. Chemotherapy had no apparent effect on outcome while radiotherapy produced an objective palliative response in 50% of treated patients. The average size and weight of the tumors were 12.4 cm and 875.6 g, respectively. The difficulty of precise pathologic classification of certain large adrenal tumors is discussed and the importance of histopathologic features relative to gross morphologic findings is emphasized.

Adolescent↗

Paragangliomas of the head and neck region. A pathologic study of tumors from 71 patients.

The histopathology of 72 paragangliomas originating in the head and neck region of 71 patients is presented. There were 45 carotid body, 13 vagal body, eight jugulotympanic, and three nasal paragangliomas. In addition, two arose in the larynx and one in the area of the aortic arch. Tumors occurred in four unrelated families. The two most important histologic features leading to a diagnosis of paraganglioma were zellballen and the presence of cytoplasmic argyrophil granules in all cases in which staining with the Grimelius technique was carried out. Ultrastructural study of three carotid body and two vagal body paragangliomas revealed both light and dark chief cells. Tumor cells contained membrane bound, electron dense neurosecretory types of granules, which usually ranged in diameter from 120 to 200 nm. Follow-up information was available for 67 patients (94 per cent). Two of the three nasal paragangliomas, 50 per cent of the jugulotympanic paragangliomas, 17 per cent of the vagal body paragangliomas, and 10 per cent of those of the carotid body recurred locally following attempted surgical resection. All patients treated with radiation had persistent tumor. Four (9 per cent) of the carotid body paragangliomas were malignant, all four patients dying with widespread metastases. One vagal body paragangliomas metastasized to regional lymph nodes (the patient was alive and well at five years), and another caused death by direct intracranial extension. In contrast to the benign tumors, malignant paragangliomas tended to show foci of necrosis and vascular invasion. Mitotic figures, which usually were not identified in the benign cases, were seen in all malignant tumors.

Adult↗

Laryngeal paraganglioma. Case report with ultrastructural analysis and literature review.

Laryngeal paraganglioma is an infrequently reported tumor; only 16 examples have been recorded in the English literature. All but one laryngeal paraganglioma originated superiorly in the larynx; involvement of the ipsilateral aryepiglottic fold is common. Male patients predominate (11:5). The average age of patients at the time of diagnosis was 47 years, and symptoms had been present for an average duration of 5.8 years (range 6 months to 27 years). Attempted biopsy has resulted in significant hemorrhage in three cases. As illustrated by the present case, the Grimelius argyrophil stain is a useful diagnostic procedure. Electron microscopy confirmed the presence of neurosecretory granules with core diameters ranging from 110 to 140 nm. Surgical resection is the preferred treatment and has been possible in 14 cases; nine patients are alive and free of tumor for an average of 3 years. Compared to other head and neck paragangliomas, these have a more malignant course with a 25% mortality; tender subcutaneous metastases are commonly observed in these patients.

Adult↗

Radiological evaluation of paragangliomas of the head and neck.

Paragangliomas can be differentiated angiographically from most other tumors in the head and neck by their profuse vascularity; specific classification is based on their position and displacement of adjacent vessels. Subtraction usually aids in diagnosis. In most cases, bilateral carotid angiography should be performed, since the ipsilateral carotid artery may have to be ligated and an asymptomatic contralateral tumor may also be found during surgery. Studies of multiple vessels will often show an additional blood supply. Computed tomography can demonstrate the tumor's vascular nature and position and is valuable in follow-up after radiation therapy.

Aged↗