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E S Wiener

Publications and source records attributed to E S Wiener.

At least 19 recordsLinked to original sources
PubMed1996

Infantile fibrosarcoma: complete excision is the appropriate treatment.

BACKGROUND: Infantile fibrosarcoma and its pathological variants are unusual childhood tumors. During the past 25 years, 18 children with these lesions were seen at our institution. METHODS: These patients' records were analyzed to determine clinical and pathological findings and outcome. RESULTS: The mean age at diagnosis was 7 months. There were 16 boys and 2 girls. The sites of tumor at presentation were: Extremity, head, trunk, and pelvis. Pathological findings were: fibrosarcoma, myofibroma, and fibromatosis. Histologic characteristics varied from benign hypocellular to more cellular pleomorphism. The initial surgical procedure was a complete excision in 16. Of those patients with complete removal, 6 recurred and all had secondary reexcision. In addition to excision, 3 patients received adjunctive therapy; chemotherapy in all 3 and radiation in 2. Sixteen (89%) children survived, including 4 of 6 with recurrent tumor and 2 of 2 with incomplete initial resection. Two children ages 6 and 9 months at diagnosis died of progressive recurrent tumor at 1 month and 6 years from initial diagnosis, despite operative therapy, chemotherapy, and radiation therapy. Both had paraspinal tumors that were removed completely. Pathological examination revealed spindle cell tumors infiltrating muscle. Neither clinical nor histologic findings distinguished surviving from nonsurviving patients. CONCLUSIONS: Fibrosarcoma occurring in infants has an excellent outcome despite histologic findings. Initial complete excision should be attempted in all children. Recurrence indicates a more aggressive variant and warrants more aggressive complete reexcision wherever possible. Adjunctive therapy probably has no benefit. The occasional death indicates the need for close follow-up.

Female↗
PubMed1996

Hernia survey of the Section on Surgery of the American Academy of Pediatrics.

The members of the Section on Surgery of the American Academy of Pediatrics were surveyed to determine the practice of North American pediatric surgeons in infants with inguinal hernia (IH). Case-scenario multiple-choice-design questionnaires regarding hernias and hydroceles were sent to all members of the Surgical Section, and responses were received from 292 (50%). In healthy full-term infant boys with asymptomatic reducible IH, 82% of responders perform repair electively, no matter what the age or weight. In full-term girls with a reducible ovary, 59% perform surgery at the next available time; if the ovary is nonreducible but asymptomatic, 44% operate emergently or urgently and 42% at the next elective slot. In former preemies, the pattern of repair is as follows. (1) For those recently discharged after 2 months in the neonatal intensive care unit (NICU) with reducible IH, 65% perform the repair when convenient. (2) A general anesthetic is used in 70%; 15% use spinal anesthesia, and 11% use caudal block with sedation. (3) If the repair is done in the hospital outpatient (same-day) unit, 36% wait until 50 weeks postconception (PC) and 33% wait until 60 weeks PC. (4) if the baby's weight is at least 1,000 g. 71% perform the repair before discharge. The pain control choice after childhood IH repair is Tylenol for 30%, local infiltration biquivacaine for 30%, caudal block for 22%, regional block for 11%, and Tylenol/codeine combined for 7%. In 6-week-old full-term infants with communicating hydroceles without definite "hernia," two thirds treat as an IH with elective repair as soon as possible. With respect to contralateral exploration in infants with unilateral IH, 65% perform it in males if they are < or = 2 years of age and 84% use it in females of up to 4 years of age. This approach is not influenced by presenting side, presence of hydrocele, or history of prematurity. Laparoscopic evaluation of the contralateral IH is performed by only 6% of responders, 40% of whom use the open ipsilateral sac for laparoscope introduction.

Age Factors↗
PubMed1996

Laparoscopy through the open ipsilateral sac to evaluate presence of contralateral hernia.

PURPOSE: Laparoscopic evaluation of the contralateral side (LECS) in children with unilateral inguinal hernia (UIH) has been criticized because of the abdominal trocar risk and costs. LECS was modified to avoid abdominal trocar insertion by using the open hernia sac for instrumentation (OHLECS). This study was performed to determine the utility, safety, and effectiveness of this technique. METHODS: During a 15-month period, 80 children with unilateral hernia underwent attempted OHLECS. All ordinarily would have undergone open contralateral exploration. The indications were UIH in boys < or = 2 years of age and girls < or = 4 years of age (n = 53) or high clinical suspicion (but not certainty) of contralateral hernia in older children with UIH (n = 27). Endotracheal intubation was not used unless otherwise indicated. Reusable 3-mm blunt trocars and 3-mm 30 degrees or 70 degrees laparoscopes were employed, with and 6 to 8 mm of insufflation pressure. No urethral catheter was used. The ipsilateral hernia sac was dissected, opened, and instrumented, and the contralateral side was evaluated for patency. Concurrent external palpation of the contralateral inguinal canal is an important diagnostic adjunct. Positive results were visible patency of processus vaginalis or bubbles or fluid and/or gas expressed from the processus by palpation. Only if the evaluation was positive was contralateral incision and repair performed. OHLECS added no more than 2 minutes of operating time. The operating room cost is similar to that of opening the contralateral side. Only reusable laparoscopic instruments are used, and less operating time, anesthetic time, suture material, and dressings are required if the contralateral side is not opened. RESULTS: In 10 patients (all < 6 months old) OHLECS was aborted because the hernia sac was smaller than 3 mm at the internal ring. OHLECS was successful in 70 (88%) patients-56 boys and 14 girls, aged 2 mo to 12 years (mean, 2.6 years). The presenting hernia was right-sided in 46 (66%) and left-sided in 24 (34%). Overall, 43 (61%) OHLECS results were negative and 27 (39%) were positive. The OHLECS results were positive for 22 (39%) boys and 5 (36%) girls. Their mean age was 1.9 years (range, 2 months to 10 years). There were no false-positives and one false-negative. There have been no complications during follow-up (mean, 1.14 years; range, 6 months to 2 years). No additional costs were incurred because nondisposable equipment was used. CONCLUSION: Laparoscopic evaluation of the contralateral side via the open ipsilateral hernia sac is feasible, quick, safe, cost effective, and requires no additional incisions. The rate of positive findings is comparable with that of open exploration. Long-term follow-up is required to determine the ultimate effectiveness of the technique.

Child↗
PubMed1996

Extremity sarcomas: an analysis of prognostic factors from the Intergroup Rhabdomyosarcoma Study III.

PURPOSE: Prognostic factors for extremity sarcomas have been reported previously, after analysis of Intergroup Rhabdomyosarcomas Studies (IRS) I and II. This report reviews the experience of IRS III (1984-1992), in light of these reported factors, and the pretreatment factors used in the staging system currently being evaluated in IRS IV. The results of treatment of extremity sarcomas in IRS III are reported. METHODS: The charts of all patients entered in IRS III with an extremity-site tumor were reviewed. This group included patients with shoulder girdle and buttock sites. All patients were treated according to IRS III protocols. Survival rates were estimated by the method of Kaplan and Meier, and comparisons among groups of patients were made using a log-rank test. A multivariate analysis was performed to analyze all pretreatment factors that were significant by univariate analysis. RESULTS: Of the 189 patients entered in IRS III with extremity sites, 88 (47%) had the most common alveolar histology. Fifty-nine patients were in group I at the time of presentation (completely resected disease), 48 in group II (microscopic residual disease), 36 in group III (gross residual disease), and 46 in group IV (metastatic disease). By univariate analysis, the significant prognostic factors affecting survival were clinical group, age at time of diagnosis, tumor size, distant metastases, nodal metastases, and local and distant recurrence. By multivariate analysis of pretreatment factors, age at time of diagnosis, nodal metastases, and distant metastases were significant prognostic factors affecting survival; tumor size approached significance. Both the clinical group system and pretreatment staging system (Lawrence/Gehan) predicted significant differences in survival between groups of patients. The lower survival rate among group II and III node-negative patients with a distal tumor (who, with more extensive surgery, could have been in group I) in comparison to group I patients with a distal lesion, approached significance. In patients without distant metastases, survival was significantly different in those patients that had negative nodes from those in whom nodes were not biopsied. CONCLUSION: This review confirms that both clinical grouping and the new pretreatment staging system used in IRS IV can predict the likelihood of survival of children with extremity sarcomas. By multivariate analysis of the elements included in the staging system, nodal metastases, distant metastases, and tumor size were useful in predicting survival. In addition, age was a significant predictor. This study confirms previous suggestions that complete excision with gross and microscopically negative margins is preferable in the treatment of children with extremity rhabdomyosarcomas.

Adolescent↗
PubMed1995

Minimally invasive surgery in children with cancer.

BACKGROUND: The safety and efficacy of minimally invasive oncologic procedures in children have not been well defined and only limited anecdotal experience has been published. METHODS: A retrospective review of all patients undergoing either a laparoscopic or thoracoscopic procedure at Childrens Cancer Group institutions between December 1, 1991, and October 1, 1993, was performed. RESULTS: Eighty-five children underwent 88 minimally invasive surgical procedures as part of the evaluation or treatment for cancer at 15 participating centers. In 25 patients, laparoscopy was performed and 60 patients underwent 63 thoracoscopic operations. Tissue biopsies were taken in 67 cases and diagnostic material was obtained in 99% of the biopsies. Seven complications occurred, all within the thoracoscopic group. These included conversion of six operations to an open procedure. One patient developed atelectasis postoperatively. CONCLUSIONS: In pediatric patients with suspected cancer, laparoscopy was highly accurate with minimal morbidity; thoracoscopy was nearly as efficient with slightly higher morbidity. Both modalities are useful for assessment of resectability, for staging purposes, and for evaluation of recurrent or metastatic disease.

Adolescent↗
PubMed1995

Conservative management of uterine pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III and IV pilot.

Previous studies have suggested that women with uterine rhabdomyosarcomas (RMS) represent a distinct group of patients who present at an older age, are less responsive to treatment, and have a poorer prognosis than patients with vaginal RMS. During the Intergroup Rhabdomyosarcoma Study (IRS) III and the IRS IV pilot study, 14 patients were registered with uterine primary RMS. Three patients presented with cervical tumors that were completely removed (group 1). Eight patients had initial biopsies with gross residual disease (group 3), and 3 had metastatic disease at presentation (group 4). Of the 5 patients treated with primary chemotherapy or chemotherapy and radiation, 2 had delayed hysterectomy and vaginectomy, 1 had no further surgery, and 2 had exploratory laparotomy with no evidence of disease. There were no relapses or deaths in this group. One patient underwent initial resection of a broad ligament mass, experienced an early (3-week) recurrence of the mass while on chemotherapy, and progressed to developing distant metastases and death. Four patients died of chemotherapy toxicity or sepsis, one after achieving a complete response from chemotherapy and hysterectomy. This primary chemotherapy or chemotherapy and radiotherapy regimen resulted in 8 of 9 (89%) patients (not including those who died of chemotoxicity) surviving between 1.5 and 6 years without evidence of disease. Of the surviving patients, 2 had hysterectomy and vaginectomy, but pathological specimens showed only localized microscopic residual tumor. This report suggests that less vigorous operative resection may be possible in combination with primary chemotherapy when treating uterine rhabdomyosarcomas.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1995

Conservative surgical management of vaginal and vulvar pediatric rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study III.

The current management of pediatric vaginal and vulvar rhabdomyosarcoma (RMS) uses cytoreductive chemotherapy before surgical intervention. During the Intergroup Rhabdomyosarcoma Study (IRS)-III (1984 to 1988), 27 evaluable patients were entered on a preoperative chemotherapy protocol. Among 24 patients with vaginal primaries, 20 had an initial biopsy with gross residual (group III), 3 had resection with positive margins (group IIA) and 1 had metastatic disease (group IV). At subsequent surgery, 7 patients underwent partial or complete vaginectomy and 6 of them had no viable tumor identified in the specimen. Only 1 of these 7 patients underwent a cystectomy, whereas 5 underwent hysterectomy. Seventeen patients in this group have no evidence of disease 66 to 108 months after diagnosis; 2 died of chemotoxicity and 1 of unknown causes after achieving a complete response. Ten of these 17 patients were treated with biopsy and chemotherapy only. Four of these 10 had radiotherapy as well. All group IIA patients have no evidence of disease. The 1 group IV patient had rapidly progressive disease with early death. This primary chemotherapy protocol resulted in less need for operative intervention or irradiation compared with previous experience. There was no local recurrence, and 20 of 24 patients remain continuously relapse-free with no evidence of disease. All 3 patients with vulvar primaries were treated by wide local excision and chemotherapy and have no evidence of disease. Conservative surgical intervention for vaginal RMS with primary chemotherapy and adjunctive radiation when necessary appears to result in excellent disease-free survival.

Adolescent↗
PubMed1994

Neonatal rhabdomyosarcoma: the IRS experience.

Neonatal rhabdomyosarcoma is rare, and the characteristics and optimal therapy for patients with this condition have not been well described. Of 3,217 eligible patients entered in the Intergroup Rhabdomyosarcoma Study (IRS), 14 were less than 30 days old at the time of diagnosis. Among these patients, male gender, Caucasian race, caudal tumors, and embryonal/botryoid and undifferentiated histology predominate. Although half the neonates survived, neither histology, tumor size, nor type of surgery were predictive of outcome. The presence of necrosis and small round cell configuration coincides with a poor prognosis, regardless of histological diagnosis. Neonatal rhabdomyosarcoma of caudal origin has a favorable prognosis.

Age Factors↗
PubMed1994

Retroperitoneal node biopsy in paratesticular rhabdomyosarcoma.

UNLABELLED: Retroperitoneal lymph node dissection (RPLND) was used in 121 Intergroup Rhabdomyosarcoma Study (IRS) III patients with nonmetastatic paratesticular (PT) rhabdomyosarcoma (RMS) to assess retroperitoneal lymph node (RPLN) involvement so as to determine the need for x-ray therapy (XRT). Clinical node evaluation (CNE) was accomplished by a computed tomography (CT) scan in 105 and a sonogram in six. Pathological node evaluation (PNE) was performed in 113: lymphadenectomy (9 bilateral, 85 unilateral) and biopsy in 19. Vincristine and actinomycin D were used for 1 year postoperatively in 89%; all patients who had positive PNE received RPLN XRT. This study compares CNE with PNE and evaluates predictors of relapse and survival. FINDINGS: There were clinically negative nodes (cN0) in 81% of the 121 patients. Among cN0 patients, 14% had positive nodes (pN1). Of the clinically positive (cN1) patients, 94% had pN1. RPLN relapse occurred in only two of the 121 patients. Initially both had cN0 and one had PNE that was negative. For all 121 patients, the 5-year survival was 91%. For cN0 patients, the 5-year survival was 96% compared with 69% for cN1 patients (P < .001). Among the children in whom treatment failed, nodes were cN1 in 5 of 11 (45%) compared with 15 of 107 (14%) in those whose treatment did not fail (P < .008). CONCLUSIONS: (1) Results of RPLN imaging studies were negative in 81% of patients with PT RMS (specificity 99%, sensitivity 57%). (2) RPLN recurrence is uncommon (even when RPLN are initially involved) if regional XRT and appropriate chemotherapy are used.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Improved sternal fixation in the correction of pediatric pectus excavatum.

Pectus excavatum is the most common congenital chest wall deformity; it is manifest by abnormal costal cartilage segments causing sternal depression and rotation. The most popular repair involves resection of the abnormal costal cartilage segments, followed by sternal osteotomy and fixation to the adjacent chest wall. Methods of sternal fixation have included metal bars and struts, K-wires, pins, sutures, synthetic mesh, vascularized rib struts, and large AO plates, but they have not included more recent rigid fixation technology. Current rigid fixation techniques allow incorporation of malleable, low-profile reconstruction plates that can be contoured to correct the three-dimensional aspect of these defects. Three pediatric patients are presented in whom plate fixation was utilized for sternal fixation. Current reconstruction plate technology provides an improved fixation alternative for the correction of congenital chest wall anomalies, as well as offering similar potential advantages for traumatic, oncologic, and reconstructive surgery of the thorax.

Adolescent↗
PubMed1994

Head and neck rhabdomyosarcoma.

Rhabdomyosarcoma (RMS) accounts for approximately half of all pediatric soft tissue sarcomas and 15% of all pediatric solid tumors. The head and neck site accounts for 35% of all RMS. The head and neck site can be divided into three broad groups: cranial paramengingeal, orbital, and nonorbital-nonparameningeal. The predominant specific sites are orbit and nasopharynx, each accounting for 25%, with paranasal sinuses, ear, intraoral, neck, and parotid each accounting for about 10%. RMS in the orbit has the best outlook; parameningeal involvement is associated with the poorest outcome. Treatment is determined by the extent of disease, ease of resectability, and precise site. Superficially placed tumors can be managed either with excision and relatively nontoxic adjuvant chemotherapy or by biopsy, radiation, and similar chemotherapy, with equally good results (survival rate, 90%). More deeply placed lesions, especially those with parameningeal locations or extension, generally are not removable and require radiation therapy, including radiation to the entire cranial neuraxis, intrathecal chemotherapy, and very aggressive chemotherapy. The more intensified therapy resulted in increased survival rates of 57%. Non-RMS soft tissue sarcomas occur infrequently in the head and neck. Resection is the most effective treatment for this diverse group of tumors. Wide resection is best, if feasible. The role of chemotherapy is unclear because most of these tumors are radiosensitive. These tumors are often managed by protocols used for the treatment of RMS or extraosseous Ewing's sarcoma.

Child↗
PubMed1993

Routine total bilateral adrenalectomy is not warranted in childhood familial pheochromocytoma.

The surgical management of familial childhood pheochromocytoma is based on data derived from clinical series that have focused primarily on the multiple endocrine neoplasia (MEN)-associated cases. Prophylactic bilateral total adrenalectomy has been advocated for this group due to a high incidence of bilateral adrenal involvement and the propensity for tumor recurrence after partial adrenal resections. We retrospectively reviewed our experience with a cohort of children with nonsyndromatic familial pheochromocytoma to see if their surgical management should parallel that advocated for patients with the MEN syndrome. From 1977 through 1991, 17 children from 9 families with a strong family history of pheochromocytoma were treated at the Children's Hospital of Pittsburgh. None of the patients or their families displayed an association with the MEN syndrome. There were 13 males and 4 females with a mean age of 10.1 years. Follow-up was obtained on all patients and averaged 6 years. All tumors were located within the abdominal cavity. Seven patients presented with unilateral adrenal tumors while four patients had synchronous bilateral tumors. Eight patients had tumors in extra-adrenal locations; 2 with concomitant adrenal tumors. Ten patients underwent total unilateral adrenalectomy with or without resection of extra-adrenal tumor(s). All patients with synchronous bilateral adrenal tumors underwent unilateral adrenalectomy in combination with a partial contralateral adrenal resection without a recurrence after an average of 7.3 years. Three patients developed metachronous adrenal tumors an average of 4.2 years after their initial surgery. All required complete removal of the affected adrenal, resulting in two patients with bilateral adrenalectomies.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Pediatric surgical oncology.

This article highlights the surgeon's role in childhood cancer especially as it relates to local control of solid tumors, research, and surgical supportive care. There is a trend toward preresection chemotherapy, and rarely, radiotherapy. This may allow safer, less extensive, and function-preserving delayed resection in neuroblastoma, hepatoblastoma, bone tumors, and nephroblastoma in selected patients without negatively affecting outcome. Ultimately, complete resection for most tumors, even advanced neuroblastomas, significantly improves survival. Organ transplantation allows complete resection with good survival in children with otherwise nonresectable liver tumors. In non-Hodgkin's lymphoma, resection should not be attempted, except for localized disease and if complete resection is possible. Second-look procedures have limited value in lymphoma, but have an important role in germ cell tumors. The differentiation of typhlitis from appendicitis is critical in the neutropenic patient to avoid life-threatening complications. Studies of venous access devices show a clear benefit of totally implantable devices in preventing dislodgment and decreasing the rate of infection. Neuroblastoma models are fertile soil for research into tumor biology and novel treatment modalities. Surgeons continue to play crucial roles in childhood oncology.

Bone Neoplasms↗
PubMed1993

Rhabdomyosarcoma: new dimensions in management.

Thus, RMS is a common childhood malignancy with a myriad of presentations, treatments and outcomes depending on the primary site and stage. The outlook for children with these tumors has steadily improved with the multidisciplinary approach exemplified by the Intergroup Rhabdomyosarcoma Studies. The surgeon plays a pivotal role on this multidisciplinary team in the initial tumor staging and diagnosis and in complete initial resections, when judicious, or in delayed secondary resections after chemotherapy and radiotherapy. Operations on these tumors should be performed by surgeons who possess a full knowledge of the biology and treatment options of this complex tumor.

Adolescent↗
PubMed1992

The CCSG prospective study of venous access devices: an analysis of insertions and causes for removal.

This is an interval analysis of the 2-year prospective multicenter Childrens Cancer Study Group study of 1,141 chronic venous access devices in 1,019 children with cancer. Device type was external catheter (EC) 72%, totally implantable (TID) 28%, and did not differ for diagnosis or age except more double-lumen devices in bone marrow transplant protocols (77%) and more TIDs in children less than 1 year old (17.7%). Insertion characteristics evaluated in 1,078 (95%) were: operating room placement 99%; general anesthesia 98%; cutdown 67%; percutaneous 33%; atrial position 50%, caval position 50%; and perioperative antibiotics 48%. Vein entry was the external jugular 33%, internal jugular 22%, subclavian 35%, cephalic 7%, and saphenous 3%. Insertion was difficult or very difficult in only 10% and operative complications occurred in only 0.7%. Degree of difficulty bore no relationship to device type or patient age. The reasons for removal in 736 devices (67%) were due to complications in 39%, of which infections were the most frequent. There was some variance between centers ranging from 8.5% to 31% for infection; 2.8% to 24% for dislodgment; and 0% to 13% for occlusion. ECs had a higher risk of dislodgment; elective removals were more frequent in TIDs; there was no difference in infection as a cause for removal between ECs and TIDs. Dislodgment was associated with the shortest distance of the cuff to the skin exit (mean, 4 cm): less than or equal to 2 cm, 49%; greater than 2 cm, 28% (P = .009) and occurred most frequently in the younger patient (18.9%, 0 to 1 years; 0.5%, greater than 8 years.

Adolescent↗
PubMed1992

Resection of the primary tumor is appropriate for children with stage IV-S neuroblastoma: an analysis of 37 patients.

Current recommendations for treatment of children with IV-S neuroblastoma (NB) indicate that "supportive care is the cornerstone of therapy" and resection of the primary tumor is not mandated. The presentation and clinical management of 37 IV-S NB patients from three pediatric oncology centers were retrospectively reviewed. The 22 boys and 15 girls presented at an average age of 107 days (range, 4 to 616 days). Primary tumor sites were identified in the adrenal gland in 19 children, the thorax in 5, the retroperitoneum in 4, and 1 each in the pelvis and kidney. At the time of presentation, metastatic disease was documented in the liver (27), bone marrow (19), skin (7), and regional lymph nodes (7). Massive hepatomegaly (below the umbilicus) was demonstrated in 18 patients. Twenty-eight children (76%) have survived on average 90 months following diagnosis. Eight patients (22%) died; six of disease and two of complications of therapy (mean, 9.6 months). One child was lost to follow-up. Only 3 of the 37 patients (8%) were managed solely by supportive care and all are alive. Extirpation of the primary tumor was accomplished in 24 individuals (65%). Twenty patients underwent excision at diagnosis and four had delayed resection with few postoperative complications and no deaths related to resection. One death resulted from progression of disease in a child who had tumor removal. Chemotherapy as a single treatment or in combination with radiation was used in 10 children with three children surviving (30%).(ABSTRACT TRUNCATED AT 250 WORDS)

Female↗