Biomedical subjects

E E Lack

Publications and source records attributed to E E Lack.

At least 73 records · Page 4Linked to original sources

PubMed1987

Combined modality treatment of adult soft tissue sarcomas of the head and neck.

Between 1975 and 1985, 16 adult patients high grade soft tissue sarcomas arising in the head and neck were treated with combined modality therapy at the National Cancer Institute. Fifteen patients underwent an attempt at gross resection, while only a biopsy was performed in 1 patient. All patients received conventionally fractionated, high-dose, post-operative irradiation. Twelve of 16 patients received adjuvant chemotherapy with cyclophosphamide and adriamycin, given concomitantly with radiation therapy. With a median follow-up of 43 months from diagnosis (range 11-116 months), 10 patients (64%) are disease-free. Of these 10 patients, 1 presented with pulmonary metastases and 2 patients later failed in the lung; all pulmonary disease was resected and these patients are disease-free at 80, 115, and 116 months following presentation. Local control was maintained in 12 patients (75%). An analysis of the iso-effect lines for local control shows a slope of 0.32. The median NSD for local control was 1720 ret. We conclude that aggressive local management of high grade sarcomas which combines gross resection and high-dose post-operative irradiation results in excellent local control. The role of adjuvant chemotherapy is less well defined in this small patient series. The acute and late complications of this combined modality approach are manageable.

Adult↗

PubMed1987

Solid and papillary epithelial neoplasm of the pancreas. An ultrastructural and immunocytochemical study of six cases.

Solid and papillary epithelial neoplasms of the pancreas from six female patients were studied using immunohistochemistry and electron microscopy to define better their histogenesis. The tumors ranged in diameter from 5 to 15 cm (average: 9 cm), and, on cross section, most had areas of hemorrhage and necrosis, sometimes extensive. Microscopically, there was a solid and pseudopapillary pattern, with tumor cells typically having ovoid nuclei with delicate folding and indistinct nucleoli. Of note were the following: a relatively low mitotic rate (range: 0-6/20 hpf), the presence of hyaline globules (four of six cases), and collections of foam cells (three of six cases). Staining for cytoplasmic argyrophil granules was negative in each case. Ultrastructurally, the solid and papillary epithelial neoplasms of the pancreas showed evidence of acinar or ductular differentiation. Two contained zymogen granules, one had intermediate filaments (probably keratin), and three had abundant rough endoplasmic reticulum and mitochondria. Immunostaining was positive for chymotrypsin (six of six cases), trypsin (four of six), and amylase (three of six). None was positive for alpha-1-antitrypsin, neuron-specific enolase, pancreatic polypeptide, gastrin, glucagon, somatostatin, or insulin. The findings support an origin from exocrine pancreas, and follow-up indicates a low rate of malignancy, with local recurrence in two of the six patients.

Adolescent↗

PubMed1987

Prognostic factors and life expectancy of patients with acquired immunodeficiency syndrome and Pneumocystis carinii pneumonia.

To assess determinants of prognosis for 43 patients with the acquired immunodeficiency syndrome (AIDS) and Pneumocystis carinii pneumonia, objective clinical and histopathologic characteristics were analyzed for acute and long-term prognostic significance. Severe abnormalities on initial chest radiographs and alveolar-arterial oxygen differences (AaPO2) greater than 30 mm Hg were associated with higher mortality during the period of treatment for the acute episode (p less than 0.05). Decreased long-term survival after the diagnosis of Pneumocystis pneumonia correlated with the severity of interstitial edema (a component of diffuse alveolar damage) on initial transbronchial biopsy and elevation of AaPO2 at the time of diagnosis (Cox proportional hazards analysis, p less than 0.05). The persistence of Pneumocystis cysts after 3 wk of therapy was associated with significantly decreased long-term survival (p less than 0.05) when follow-up biopsy was performed in 27 of the patients. Patients with a diagnosis of Pneumocystis pneumonia before July 1985 had more advanced disease at the time of diagnosis and a worse prognosis than did those in whom the diagnosis was made after July 1985 (p less than 0.05). This study demonstrates that important prognostic information can be derived from information obtained at initial presentation and follow-up bronchoscopic evaluation in patients with AIDS and Pneumocystis carinii pneumonia, and suggests that early detection and initiation of therapy may improve chances for survival.

Acquired Immunodeficiency Syndrome↗

PubMed1987

Nonspecific interstitial pneumonitis in patients with AIDS: radiologic features.

Chest radiographic abnormalities in patients with AIDS usually are associated with opportunistic infection or neoplasm. One hundred five patients with AIDS and clinical evidence of pneumonitis had chest radiographs and underwent bronchoscopy with bronchoalveolar lavage, transbronchial biopsy, or open-lung biopsy. Chest radiographs were abnormal in 73 (70%). Pneumocystis carinii pneumonia was identified at bronchoscopy or open-lung biopsy in 52 (50%). Nonspecific interstitial pneumonitis occurring in the absence of an identifiable infection was documented histologically in 36 (34%). Twenty (56%) of these 36 patients had abnormal chest radiographs. No currently recognized infectious agents were recovered from lavage fluid or were seen histopathologically in patients with nonspecific interstitial pneumonitis. The results show that nonspecific interstitial pneumonitis is clinically and radiologically indistinguishable from Pneumocystis carinii pneumonitis and should be considered in the differential diagnosis of AIDS patients with interstitial infiltrates.

Acquired Immunodeficiency Syndrome↗

PubMed1987

Nonspecific interstitial pneumonitis: a common cause of pulmonary disease in the acquired immunodeficiency syndrome.

During a 4.4-year period, nonspecific interstitial pneumonitis was seen in 41 of 110 (38%) patients with the acquired immunodeficiency syndrome and accounted for 32% (48/152) of all episodes of clinical pneumonitis. Diffuse alveolar damage was typically a feature of nonspecific interstitial pneumonitis, but neither lung biopsy nor bronchoalveolar lavage detected a pathogen. Of these 41 patients, 13 had no associated pulmonary tumor and had not been exposed to pulmonary toxins, whereas 28 patients had either concurrent pulmonary Kaposi sarcoma, previous experimental therapies, or a history of pneumocystis pneumonia or drug abuse. Of these 41, 23 had normal chest radiographs. The clinical features of patients with nonspecific interstitial pneumonitis were similar to those of patients with pneumocystis pneumonia, although histologic findings showed less severe alveolar damage in patients with nonspecific interstitial pneumonitis (p less than 0.001). Pathologic evaluation and clinical follow-up suggest that many clinical episodes of pneumonitis in patients with the acquired immunodeficiency syndrome are due to nonspecific interstitial pneumonitis of unknown cause.

Acquired Immunodeficiency Syndrome↗

PubMed1986

High-grade soft tissue sarcomas of the extremities.

From July 1975 to December 1982, 358 patients were referred to the Surgery Branch of the National Cancer Institute (NCI) with the diagnosis of soft tissue sarcoma of the extremities. Two hundred eleven of these patients presented with resectable, localized high-grade soft-tissue sarcomas and have been included in the present analysis of the management and outcome of patients with high-grade soft tissue sarcomas of the extremity treated at the NCI. One hundred forty-seven of these 211 patients have been included in randomized prospective trials. The remaining 64 patients in this analysis have been followed at the NCI, but were not included in randomized trials because of patient refusal or ineligibility. Tumor size was identified as a highly significant prognostic variable for disease-free and overall survival (P2 = 0.00001 and 0.0081, respectively). Tumor site, histologic type, and microscopic margins of resection were not significant prognostic variables. There was no difference between patients undergoing amputation compared to those undergoing limb-sparing procedures plus postoperative radiotherapy in disease-free or overall survival for all 211 patients in this study (P2 = 0.068 and 0.131, respectively). A significantly greater frequency of local failure among patients treated by local excision was noted compared to patients undergoing amputation (12/128 versus 0/83, P2 = 0.004), but this did not result in decreased overall survival in patients undergoing combined modality limb-sparing procedures. Adjuvant chemotherapy significantly prolonged disease-free survival (P2 = 0.005) for the 124/211 patients treated with adjuvant chemotherapy, although analysis of overall survival did not reveal a significant increase (P2 = 0.10). In a subset of 65 patients included in a prospective randomized trial evaluating the efficacy of adjuvant chemotherapy, a significant improvement in both disease-free (P2 = 0.033) and overall (P2 = 0.055) survival was seen in patients receiving chemotherapy. Sixty-five patients developed recurrent disease (65/211, 31%) and 42 of these patients were rendered disease-free surgically. Survival from the time of first recurrence was significantly prolonged among the 42 patients who were rendered disease-free (median survival, 31 months) compared to those who were not (median survival, 9 months, P2 less than 0.001).

Actuarial Analysis↗

PubMed1986

Focal nodular hyperplasia and hepatic adenoma: a review of eight cases in the pediatric age group.

A review of all primary hepatic tumors seen at Children's Hospital, Boston over a 57-year period disclosed six cases of focal nodular hyperplasia (FNH) and two hepatic adenomas (HA). The children with FNH (four females, two males) ranged in age from 6 months to 15 years (average age: 7 years). Three patients had "hepatomegaly" noted on physical examination 2 months to 2 or more years prior to diagnosis. The average diameter of FNH was 7.3 cm (range 2.5-10 cm). The lesion was confined to the right lobe in four cases and showed bilobar involvement in two. The HA's were diagnosed in a newborn male and a 2-year-old girl, both of whom were symptomatic because of large tumor size (10 cm each). There was no maternal history of exposure to exogenous steroids during pregnancy. Three children with FNH underwent hepatic lobectomy and were alive and well 4 to 17 years later. One child who died of acute leukemia had an incidental FNH discovered at autopsy. Two patients were treated conservatively with biopsy only and were alive and symptom-free 13 and 15 years later. Both children with HA underwent hepatic lobectomy. One died postoperatively because of intra-abdominal hemorrhage and the other was alive and well 10 1/2 years later. Complete surgical resection is recommended for most children with HA when technically feasible because of the lingering suspicion of possible (albeit remote) malignant transformation and diagnostic difficulties in distinguishing HA from a well-differentiated hepatocellular carcinoma.

Adenoma↗

PubMed1986

Polymicrobial cholangitis and Kaposi's sarcoma in blood product transfusion-related acquired immune deficiency syndrome.

Before presenting to the Mayo Clinic, a 24-year-old white woman had received 35 transfusions of blood products over a 72-hour period in February 1981. Two and one half years later, the diagnosis of polymicrobial cholangitis (Cryptosporidium, Candida albicans, and Klebsiella pneumoniae) was established. Further evaluation demonstrated profound helper T lymphocyte suppression, disseminated Mycobacterium avium-intracellular infection with mycobacteremia, and Kaposi's sarcoma of lymphoid tissue, confirming a diagnosis of acquired immune deficiency syndrome (AIDS). This case represents an unusual infectious complication of AIDS. Additionally, this is believed to be the first report of Kaposi's sarcoma occurring in a patient with AIDS associated with blood product transfusion.

Acquired Immunodeficiency Syndrome↗

PubMed1986

Danazol-induced cystitis: an undescribed source of hematuria in patients with hereditary angioneurotic edema.

From 1975 through 1983, 69 patients with hereditary angioneurotic edema were treated with danazol, a semisynthetic anabolic steroid. Hematuria developed in 13 of these patients (19 per cent). Careful evaluation of the genitourinary tract showed the presence of a distinct form of hemorrhagic cystitis in 10 patients. Clinically, 9 patients presented with microscopic and 1 with intermittent gross hematuria. Irritative bladder symptoms were reported by 2 patients. Neither the dose nor duration of danazol therapy correlated with the severity of the cystoscopic or pathological findings. Cystoscopically, a rather nonspecific pattern of erythema, submucosal telangiectasia and neovascularity was observed. Histopathologically, bladder biopsy typically showed numerous dilated submucosal vessels with hemorrhage, mucosal ulceration and occasional inflammatory cells. These changes regressed in all but 1 patient when danazol therapy was discontinued. Further studies are needed to elucidate the role of danazol itself or of the danazol-hereditary angioneurotic edema interaction in the pathogenesis of these abnormalities.

Adult↗

PubMed1986

High-grade epithelioid angiosarcoma of the scalp. An immunohistochemical and ultrastructural study.

The ultrastructural and immunohistochemical characteristics of a high-grade epithelioid angiosarcoma of the scalp are reported. By light microscopy, the neoplasm simulated a poorly differentiated carcinoma, either primary or metastatic from some occult site. Immunohistochemical study, using a specific antibody against factor VIII-related antigen, was positive when direct immunofluorescence was applied to frozen specimens of the neoplasm. Application of immunoperoxidase (avidin-biotin complex method) on paraffin-embedded tissue using the same antibody was negative. Ultrastructually, the neoplasm had a prominent cytoskeleton of intermediate filaments, numerous pinocytotic vesicles, small intercellular lumina with microvilli on their surfaces, and characteristic intracytoplasmic vacuoles. A brief discussion of the differential diagnostic features is made.

Aged↗

PubMed1986

Pulmonary and extrapulmonary lymphangioleiomyomatosis. Report of a case with bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis, and a glial polyp of the endocervix.

The authors report an extraordinary case of a 33-year-old female who had pulmonary and extrapulmonary lymphangioleiomyomatosis, bilateral renal angiomyolipomas, multifocal lymphangioleiomyomatosis involving the uterus, ovaries, periadrenal vessels, and liver. The patient also had a glial polyp of the endocervix which represented retained fetal parts from an abortion 15 years earlier. Both ovaries showed multiple follicle cysts which may have been a source of endogenous estrogen. The classical stigmata of the tuberous sclerosis complex were absent. The multifocal lymphangioleiomyomatosis of the various organs described presents an extremely rare manifestation of this disorder.

Adult↗

PubMed1986

Leiomyosarcomas in childhood: a clinical and pathologic study of 10 cases.

Ten children (5 males, 5 females) with leiomyosarcomas were treated at the Children's Hospital, Boston, during the 20-year period from 1963 through 1983. The patients ranged in age from 2 1/2 to 15 years, the average age at diagnosis being 10 1/2 years. The tumors originated in the following sites: stomach (4 cases), jejunum, cecum, endocervix, retroperitoneum, urinary bladder, and base of tongue (1 case each). In each case the primary tumor was surgically resectable, with the average diameter being 6.7 cm (range 2-11 cm). The tumor proved fatal for 3 of 6 children with regional or distant metastases. Three others were alive with metastatic leiomyosarcoma 6-18 years following diagnosis. A fourth death was attributed to a massive chondrosarcoma of the lumbosacral area, which developed 7 years after hysterectomy, chemotherapy, and radiation therapy for an endocervical leiomyosarcoma. While leiomyosarcomas are extremely rare in the pediatric age group (less than 2% of all soft tissue sarcomas), diagnostic features and biologic behavior appear similar to those in adults. Tumor size and mitotic counts seem to be the most important features in assessing malignant potential, but absolute minimum criteria for malignancy are not well defined.

Adolescent↗

PubMed1986

Carotid bodies in sudden infant death syndrome: a combined light microscopic, ultrastructural, and biochemical study.

Carotid bodies (CBs) are peripheral chemoreceptors that can undergo compensatory hypertrophy and hyperplasia in some patients with chronic hypoxemia. At the Children's Hospital morphometric and biochemical analyses were done on CBs obtained at autopsy from 89 infants in the first year of life to determine if there were statistically significant differences in sudden infant death syndrome (SIDS, n = 38) compared with an age-matched control population (n = 51). Mean combined weights of CBs from SIDS victims were greater than controls in the 1- to 4-month age interval (mean = 3.4 mg, p less than 0.075) and the 4- to 8-month interval (mean = 5.0 mg, p less than 0.098) but the differences were not statistically significant (t-test, p less than 0.05). Computerized planimetry of total surface area and area occupied by "functional" parenchyma revealed no statistically significant differences. Both groups showed an equally intense degree of cytoplasmic argyrophilia of chief cells, and electron microscopy of CBs from 5 SIDS victims was indistinguishable from controls, including overall content of dense-core neurosecretory granules. There were also no significant differences in catecholamine content using liquid chromatography with electrochemical detection (SIDS n = 29, controls n = 21). These morphometric, ultrastructural, and biochemical data are not supportive for CBs having a significant role in the pathogenesis or etiology of SIDS.

Carotid Body↗

PubMed1986

Mesenchymal hamartoma of the liver. A clinical and pathologic study of nine cases.

Mesenchymal hamartoma (MH) is a rare but distinctive tumor-like malformation of the liver with predilection for infants in the first 2 years of life. MH accounted for only 5% of all primary hepatic tumors seen at Children's Hospital, Boston, over a 57-year-period from 1924 through 1981. There were nine children (five females, four males) with a median age of 11 months at the time of diagnosis (range: 5 months to 7 years). The most common presenting manifestation was abdominal enlargement with a palpable nontender mass. Exploratory laparotomy was performed in each case. The MHs were located in the right lobe and varied in size from 3 to 21 cm (average diameter, 16 cm). One MH also involved a portion of the left lobe. Four of seven children who underwent hepatic lobectomy were alive and symptom-free for 1 to 18 years later. There were three intraoperative deaths due to hemorrhage and cardiac arrest. One child underwent biopsy only with drainage of cyst fluid but died in the postoperative period because of pneumonia. The last child who had wedge resection of an incidental MH died of extrahepatic biliary atresia. Although complete surgical resection is curative, past experience indicates a substantial risk for operative mortality. In some cases, a more conservative approach to therapy is suggested with partial excision or drainage of cysts and possible resection at a future date.

Child↗

PubMed1985

Retroperitoneal germ cell tumors in childhood. A clinical and pathologic study of 11 cases.

During the last 54 years at the Children's Hospital 11 children (10 female, 1 male) had been treated for an extragonadal germ cell tumor arising in the retroperitoneum. There were eight teratomas (five mature, three immature), two endodermal sinus tumors and one tumor with a mixture of both components. Abdominal pain or discomfort was the usual presenting complaint, with the average age at diagnosis being 18 months. On physical examination, each child had a palpable abdominal mass usually localized in the upper quadrants. The finding of calcification, bone or teeth, on radiologic study was most helpful in establishing a preoperative diagnosis of teratoma. The preferred treatment for children with mature and immature teratomas is complete surgical resection; decisions regarding adjuvant therapy for patients with immature tumors must be evaluated on an individual basis. The prognosis remains guarded for children with unresectable primaries or those with endodermal sinus tumor. Of three tumor-related deaths, two were due to metastatic endodermal sinus tumor and one resulted from extensive local growth by immature teratoma. Successful management of children with endodermal sinus tumor requires surgery aimed at debulking or complete resection in combination with aggressive adjuvant chemotherapy.

Child, Preschool↗

PubMed1985

Renal cell carcinoma in childhood and adolescence: a clinical and pathological study of 17 cases.

From 1955 through 1984, 17 patients aged 17 years or younger were treated or seen in consultation for renal cell carcinoma at the Children's Hospital and Dana Farber Cancer Institute. There were 11 girls and 6 boys, with an average age at diagnosis of 11 years (range 4 to 17 years). Gross hematuria and abdominal or flank pain were the most common presenting complaints, and in 5 cases there was a history of antecedent trauma. The tumors ranged in size from 2 to 15 cm. (average diameter 7.5 cm.). There were 10 tumors in the left kidney and 7 in the right kidney. Childhood renal cell carcinoma displayed similar gross and microscopic features compared with counterparts in adults. The smallest renal cell carcinoma to metastasize measured 3 cm. in diameter and the pathological features suggested an origin from distal collecting tubules (ducts of Bellini). There were 6 tumor-related deaths (35 per cent over-all mortality) occurring 2 months to 2 years after diagnosis. Eleven children were well, with an average duration of followup of 12 years (range 4 to 27 years). The most important prognostic factor was clinical stage at the time of diagnosis. The presence of renal vascular invasion per se did not appear to be an adverse finding. The most optimal treatment for children with stage I renal cell carcinoma is radical nephrectomy with regional lymphadenectomy. While adjuvant radiation and/or chemotherapy may be used in patients with more advanced stage disease, their efficacy in achieving long-term survival free of relapse or cure is not well defined.

Adolescent↗

PubMed1985

Extragonadal germ cell tumors of the head and neck region: review of 16 cases.

During the period from 1928 to 1982, 16 children were treated or seen in consultation at the Children's Hospital Medical Center for extragonadal germ cell tumors of the head and neck region. Collectively, these tumors accounted for only 5 per cent of all benign and malignant germ cell tumors. Fourteen tumors were diagnosed in newborns and were classified as pure teratomas, either mature or immature. Six of these tumors were located in the cervical region, three presented within superficial facial structures, two were retro-orbital, and three arose within oropharyngeal or nasopharyngeal tissues. There were three endodermal sinus tumors, one of which appeared in an 11-month-old child who had undergone incomplete resection of an oropharyngeal teratoma as a newborn. Endodermal sinus tumors presented within the oropharynx, nasopharynx, and floor of the mouth and affected children at older ages (6 to 11 months). The overall prognosis for infantile teratomas of the head and neck region (exclusive of brain and spinal cord) is excellent, despite the presence of immature elements; however, tumor-related deaths can result from large unresectable primary tumors. The prognosis for children with endodermal sinus tumors remains guarded, with successful management depending on early diagnosis and aggressive adjuvant therapy.

Facial Neoplasms↗

PubMed1985

Patterns of recurrence in patients with high-grade soft-tissue sarcomas.

From July 1975 to December 1982, 563 patients were referred to the Surgery Branch of the National Cancer Institute with the diagnosis of soft-tissue sarcoma. Three hundred and seven of these patients had fully resectable, localized high-grade soft-tissue sarcomas and were treated at the National Cancer Institute using standard protocols with surgery alone, or in combination with chemotherapy and/or radiotherapy. An aggressive surgical approach was undertaken in the management of patients who subsequently developed recurrent disease. These 307 cases have been reviewed, with a median duration of follow-up of 30 months, to determine the frequency of recurrent disease, the patterns of recurrence, and the impact of surgery on the survival of patients who developed recurrent disease. Disease recurred in one hundred seven patients (107/307, 35%), with a median disease-free interval of 18 months (range, 0.5 to 72.0 months). The frequency of recurrence by site of primary sarcoma was extremity, 31% (65/211); head and neck, 33% (4/12); trunk, 40% (17/42); retroperitoneum, 47% (17/36); and breast, 67% (4/6). Isolated pulmonary metastatic disease was the most common pattern of initial recurrence (56/107, 52%) followed by isolated local recurrence (21/107, 20%). Single other sites of recurrence and multiple concurrent sites of recurrence each accounted for 14% (15/107) of all initial recurrences. The relative frequency of each of these four patterns of recurrence varied with the site of the primary sarcoma. The outcome for patients with recurrent disease depended on the site of recurrence, rather than on the site of the primary sarcoma. Sixty-six patients (66/107, 62%) with recurrent disease were rendered surgically disease-free with the first recurrence, including 40 (40/56, 72%) patients with isolated pulmonary metastases, 20 patients (20/21, 96%) with isolated local recurrences, five patients (5/15, 33%), with isolated other sites of recurrence and one patient (1/15, 7%) with multiple sites of initial recurrence. Following surgical resection, the actuarial three-year survival for the 66 patients rendered disease-free was 51%. The median survival for the 41 patients not rendered surgically disease-free with the first recurrence was only 7.4 months. Thirty of the sixty-six patients (30/66, 45%) rendered disease-free with the first recurrence remained disease-free at follow-up, with a median follow-up of 28 months from the time of resection of the first recurrence. The remaining 36 patients (36/66, 55%) subsequently recurred, with a median disease-free interval of 7.3 months.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗