Biomedical subjects

E H Soule

Publications and source records attributed to E H Soule.

At least 19 recordsLinked to original sources

PubMed1988

Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I.

The patterns of disease extent, response to treatment, and survival rates were examined in 129 children registered as metastatic (Group IV) soft-tissue sarcoma patients on the Intergroup Rhabdomyosarcoma Study (IRS)-I protocol from November 1972 through October 1978. The patients' ages at diagnosis ranged from 3 months to 21 years (median, 10.5 years); 72 were male patients and 57 were female patients (male-to-female ratio, 1.3:1). The tumor arose in an extremity in 34 patients, the head and neck (exclusive of the orbit) in 27, the genitourinary tract in 25, the retroperitoneum or pelvis in 14, the trunk in 12, the intrathoracic region in nine, the hepatobiliary system in four, the perineum in two, and the orbit in two patients. The histologic types of soft-tissue sarcoma confirmed by the pathology review committee were embryonal rhabdomyosarcoma in 68 patients, alveolar rhabdomyosarcoma in 41, undifferentiated sarcoma in 14, embryonal-botryoid in two, pleomorphic in one patient, and other sarcoma in three patients. Of 105 patients who were confirmed in Group IV and had complete information concerning sites of metastatic deposits, 53 patients presented with metastases restricted to only one anatomic region or tissue; the other 52 had diffuse metastases. The most common sites of distant tumor deposits were the lungs, bone marrow, and other soft tissues. After treatment with vincristine, dactinomycin, and cyclophosphamide with or without Adriamycin (Adria Laboratories, Inc., Columbus, OH) and radiation therapy, 65 (50%) of the 129 eligible patients achieved complete disappearance of all detectable tumor. The likelihood of achieving and maintaining a complete response for 2 years was highest among patients whose tumors originated in the genitourinary tract, retroperitoneum-pelvis, or the hepatobiliary or perineum regions. Thirteen of these 45 children (29%) are alive and disease-free at a minimum of 2 years after initiation of treatment. Only 10 of the other 84 (8%) are surviving disease-free at 2 years (P = 0.02). The presence of restricted rather than diffuse metastases at diagnosis (P = 0.02) was the only other characteristic significantly related to long-term complete remissions. One hundred and two patients (79%) died at a median of 47 weeks after diagnosis, 92 from sarcoma and ten from other causes.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

PubMed1988

Congenital anomalies associated with rhabdomyosarcoma: an autopsy study of 115 cases. A report from the Intergroup Rhabdomyosarcoma Study Committee (representing the Children's Cancer Study Group, the Pediatric Oncology Group, the United Kingdom Children's Cancer Study Group, and the Pediatric Intergroup Statistical Center).

Congenital anomalies were identified in 37 of 115 (32%) children and adolescents autopsied with rhabdomyosarcoma. An analysis of sex, age, site, and histology of cases with or without congenital anomalies showed no significant differences. Of the 45 identified anomalies, 14 were considered major and 31 minor. The distribution of the anomalies by system included central nervous (9), genitourinary (10), gastrointestinal (13), and cardiovascular systems (4). Ten patients had complex or miscellaneous anomalies. There was one child with each of the following: Rubinstein-Taybi syndrome, neurofibromatosis, single horseshoe kidney, hemihypertrophy, and Arnold-Chiari malformation. Aniridia was not noted in any case of rhabdomyosarcoma. Individuals with rhabdomyosarcoma have an increased incidence of genitourinary anomalies similar to that in Wilms' tumor. Recent molecular genetic investigations have suggested that rhabdomyosarcoma, Wilms' tumor, and hepatoblastoma share a common pathogenetic mechanism involving chromosome 11. The uniquely increased association of central nervous system anomalies with rhabdomyosarcoma and absence of aniridia would support a different gene locus operative on chromosome 11 for individuals with rhabdomyosarcoma compared to Wilms' tumor. Extensive epidemiologic studies now in progress in patients with rhabdomyosarcoma should provide the incidence of congenital anomalies and potential linkage with prenatal events.

Cardiovascular Abnormalities↗

PubMed1988

Pathologic features of extraosseous Ewing's sarcoma: a report from the Intergroup Rhabdomyosarcoma Study.

Eighty-four cases of extraosseous Ewing's sarcoma (EOE) were found during the pathology review of the Intergroup Rhabdomyosarcoma Study I and II. Patients commonly presented during or after adolescence with the most common primary sites including the trunk, extremities, and retroperitoneum. Males were slightly more affected. Histologic sections of 74 tumors in the pathology repository were re-reviewed with attention to rosette formation (positive in 18 cases) and glycogen deposition (++ in 21, + in 36, +/- in 11, and - in 2 of 70 cases examined). Fourteen tumors (7 with rosettes and 7 without) were selected for immunohistochemical and ultrastructural studies, and 13 showed single or multiple neural markers (neuron-specific enolase in 8, S-100 protein in 6, and neurosecretory-type granules in 9). These possible cases of neural EOE could be divided into three subgroups: tumor with bidirectional neuroblastic and schwannian differentiation (5 cases), tumor with monodirectional neuroblastic differentiation (7 cases), and tumor with monodirectional schwannian differentiation (1 case). EOE with a neural nature may be categorized into a spectrum of peripheral primitive neuroectodermal tumors. Clinical, histopathologic, and biologic differences between this disease and conventional sympathetic neuroblastoma are discussed.

Child↗

PubMed1988

Histopathology of childhood sarcomas, Intergroup Rhabdomyosarcoma Studies I and II: clinicopathologic correlation.

Histopathologic material from 1,782 patients registered in the Intergroup Rhabdomyosarcoma Study Committee (IRS)-I and -II were reviewed by the IRS Pathology Committee in order to provide a uniform approach to classification and correlate patient survival with tumor type. Categories considered eligible were the four types of rhabdomyosarcoma (RMS) (criteria of Horn and Enterline), extraosseous Ewing's tumor (EOE), and a group of somewhat variable undifferentiated sarcomas designated small round cell sarcoma, type indeterminate (STI). Tumors that were clearly sarcomas but were unclassifiable also were included (NOS). The committee diagnoses were embryonal (Emb) RMS in 877 (54%), alveolar (Alv) RMS in 343 (21%), botryoid (Botr) RMS in 88 (5%), pleomorphic (Pleo) RMS in 11 (1%), STI in 135 (8%), and EOE in 84 (5%). One in nine were mixtures of types, eg, Emb and Alv. Five percent of the sarcomas could not be classified because of inadequate material. In general, there was close agreement (94%) between the review committee and institutional pathologists in the diagnosis of RMS, but not in the specific types, particularly Alv RMS (41%) and STI (36%). This observation is important, since patients with Alv RMS and STI tumors had decreased survival compared with the other histologies. The prognosis varied by histology, with Botr having the best, Alv RMS and STI the worst, and Emb RMS and EOE an intermediate prognosis.

Age Factors↗

PubMed1987

Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983.

This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.

Adolescent↗

PubMed1987

Pathology of fatal rhabdomyosarcoma. Report from Intergroup Rhabdomyosarcoma Study (IRS-I and IRS-II).

Patterns of tumor spread were studied by examining clinicopathologic and postmortem data on 274 tumor-related deaths from patients in IRS-I and IRS-II. The activity of the primary lesion and presence or absence of distant metastases at death were related to the histologic subtype and anatomic site of origin, individually and/or in combination. The alveolar subtype had the highest proportion of distant metastases and the lowest occurrence of local progression alone from the primary site. More distant metastases were observed among the patients with extremity primary, and the frequency increased significantly with the alveolar histologic subtype. Parameningeal tumors, irrespective of their histologic subtypes, showed a high incidence of direct extension from the primary lesion especially into the CNS. The lung was the most common site of metastasis at death, followed by the regional lymph node, bone, liver, and brain. Regional node involvement was observed more commonly in patients with lower extremity primary and alveolar subtype.

Autopsy↗

PubMed1987

Pediatric orofacial and laryngopharyngeal rhabdomyosarcoma. An Intergroup Rhabdomyosarcoma Study report.

Eighty-nine children with localized rhabdomyosarcoma of orofacial and laryngopharyngeal sites were treated in accordance with the first and second Intergroup Rhabdomyosarcoma Study (IRS) protocols (IRS-I and IRS-II) between 1972 and 1984. Treatment included surgery (or biopsy) and chemotherapy for all patients and radiotherapy in the majority. The actuarial estimate of the three-year survival rate for all patients was 83% and did not differ significantly by primary site, histologic findings, or presence of adenopathy. A trend for a worse survival rate was seen in clinical group III patients and in those less than 5 years of age at diagnosis. Factors associated with an increased risk of local/regional relapse included omission of radiotherapy and a radiation dose of less than 40 Gy (4000 rad). We conclude that treatment of these patients as recommended in the IRS-I and IRS-II protocols results in very good local and regional tumor control and survival rates. Salvage therapy for local/regional recurrence may yield long-term remission and possibly cure.

Adolescent↗

PubMed1986

Lymphadenectomy in the management of stage I malignant melanoma: a prospective randomized study.

A prospective randomized study was initiated at our institution in 1972 to determine the efficacy of routine elective lymphadenectomy in localized (stage I) melanoma. Included in the study were 171 patients, 62 of whom had no lymphadenectomy, 55 of whom had delayed lymphadenectomy, and 54 of whom had immediate lymphadenectomy. No significant difference was found among the three treatment groups with respect to survival or metastasis-free survival. Multifactorial analysis indicated that the level of invasion and the thickness of the lesion were the most important prognostic factors, followed by age (60 years or older), site (legs), and tumor type (nodular). A prognostic index based on these variables was highly predictive of metastasis or death. Even when this score was considered, no significant variation was noted among the three treatment groups. More subsequent complications of melanoma, however, occurred in the group with no lymphadenectomy--36 in this group but only 19 in each of the other treatment groups. This finding was not statistically significant but does indicate that a few additional problems may be associated with leaving regional nodes intact. Further studies are needed, and indeed are being conducted, to determine whether elective lymphadenectomy improves survival sufficiently to offset the costs and the complications associated with this approach.

Adult↗

PubMed1986

Synovial sarcoma.

The contributions of Dr. Arthur Purdy Stout to surgical pathology and his observations pertaining to soft tissue tumors (and, more specifically, to synovial sarcoma) are briefly reviewed. In addition, a report on 185 patients treated at the Mayo Clinic for synovial sarcoma is reviewed. In that study, histologic subclassification stratified the tumors as follows: 33% with a predominant biphasic pattern, 31% with a monophasic pattern, and 36% with a mixed pattern. For all 185 patients, the 5-year survival rate was 38% and the 10-year rate was 23%. For patients treated since 1960, the survival rates were 55% at 5 years and 38% at 10 years. Female patients, young patients, and patients with tumors less than 5 cm in diameter had significantly higher survival rates than did their counterparts. Although histologic subtyping did not reveal significant differences in patient survival, patients with glandular differentiation of the epithelial elements seemed to do better.

Cell Transformation, Neoplastic↗

PubMed1985

Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study.

Ten cases of rhabdomyosarcoma of the biliary tree (RMS/BT) in children are reported in this review of the Intergroup Rhabdomyosarcoma Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable tumors on IRS I and II. Intermittent obstructive jaundice with or without abdominal distention, fever, and loss of appetite is the typical presentation. Attribution of these symptoms to hepatitis commonly delayed definitive treatment. Demonstration of a mass in the porta hepatitis by ultrasound led quickly to surgical consultation and an exploratory laparotomy. Intraoperative cholangiography was a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. Resection of the mass with only microscopic or minimal gross residual disease was possible at the initial surgery in six of the ten patients. Included in this group are all four of the surviving patients. The continuity of bile flow was usually maintained by variations of a Roux-en-Y jejunostomy. Histology was embryonal RMS with some botryoid elements. Consultation with a surgical pathologist during the course of the surgery was of invaluable assistance. Multi-drug chemotherapy and radiotherapy consistent with the IRS protocols were given postoperatively. A second-look surgery was useful in evaluating residual or recurrent disease. Four patients are surviving free of disease at 6 months, 3, 6 1/4, and 6 1/2 years from diagnosis, respectively. This review demonstrates that a multidisciplinary approach in RMS/BT can lead to long-term survival.

Bile Duct Neoplasms↗

PubMed1982

Synovial sarcoma.

One hundred and eighty-five patients, whose ages ranged from two to seventy years (mean, thirty years), have been treated at the Mayo Clinic for synovial sarcoma. Sixty-nine per cent of the tumors occurred in the lower extremity: 25 per cent, in the upper extremity; and 6 per cent, in the trunk. Histological characteristics included a predominant bimorphic pattern in 33 per cent of the tumors, a monomorphic pattern in 31 per cent, and a mixed pattern in 36 per cent. For all patients, the five-year survival rate was 38 per cent and the ten-year rate was 23 per cent, with a median survival time of thiry-nine months. However, for those patients treated since 1960, the five-year survival rate was 55 per cent and the ten-year rate was 38 per cent. Female patients had a significantly better survival time than did male patients. Younger patients had a better survival rate, and the prognosis was better for those patients whose tumor were either less than five centimeters in diameter or located in the lower extremity. Survival rates were best for those patients who had wide local excision, but this type of surgery was performed for the lesions that had a more favorable prognosis. A regression study indicated that the size of the tumor was the single most important prognostic variable for survival. Second in importance was the extent of disease on initial presentation, followed by the chronological time of treatment, the age of the patient, and the anatomical site of the tumor.

Adolescent↗

PubMed1980

Malignant fibrous histiocytoma: a retrospective study of 167 cases.

A retrospective study of 167 patients with soft-tissue malignant fibrous histiocytoma of the trunk, extremities, and retroperitoneal region revealed twice as many deeply situated tumors as superficial tumors. Malignant fibrous histiocytoma may be subclassified into fibrous, giant-cell, myxoid, and inflammatory variants. The fibrous variant accounted for two-thirds of the lesions. The prognosis is no different among the histologic subtypes. The depth of the tumor significantly affects survival, and three important groups were identified: superficial tumors, superficial tumors that recur in deep locations, and deeply situated tumors. The group with superficial tumors that subsequently did not recur in deep locations had a significantly better 4-year survival rate than did the other two groups (65% versus 34% and 40%, respectively). Patients with distally located tumors had a better 5-year survival rate than did patients with proximally located tumors (73% versus 28%). Local recurrence was found in 51% of patients who had a "complete" excision. Patients with superficial tumors had a higher local recurrence rate (71%) than did those with deep tumors (41%). Few patients with retroperitoneal tumors were long-term survivors; the 5-year survival rate was 14%.

Adolescent↗

PubMed1979

Atypical lipoma, atypical intramuscular lipoma, and well differentiated retroperitoneal liposarcoma: a reappraisal of 30 cases formerly classified as well differentiated liposarcoma.

Thirty cases formerly diagnosed as Grade I lipogenic liposarcoma (well differentiated liposarcoma) were reviewed. The basic histologic pattern in all was that of adult fat modified by the presence of cells with enlarged, hyperchromatic nuclei; in most cases there was also a component of myxoid and/or fibrous tissue. The length of follow-up ranged from two to 30 years. Nine of the tumors were located in the subcutaneous layer. None of these recurred after excision, not even those which were simply "shelled out," and none metastasized. The term "atypical lipoma" is proposed for this group. Thirteen were located within or between muscles of the limbs, limb girdles, and head and neck. Nine of these recurred at least one, but there were no metastases and no deaths due to tumor. These were designated "atypical intramuscular lipoma." The remaining eight originated in the retroperitoneum. Although none of these patients developed metastases, five suffered inoperable recurrence and three died as a result of the neoplasm. It is suggested that the term "well differentiated retroperitoneal liposarcoma" be retained for cases of this type.

Adult↗

PubMed1979

Cutaneous angiosarcoma of the head and neck.

Between 1920 and 1970, 13 patients (7 men and 6 women) with cutaneous angiosarcomas of the head or neck were treated at the Mayo Clinic; the mean age of the patients was 66 years. Three clinical patterns were noted: a superficial spreading type, a nodular type, and an ulcerating type. There were no predisposing benign lesions; however, in one patient, angiosarcoma developed in an area of previously irradiated skin. Three modes of therapy were used: surgery alone, radiation alone, and surgery with postoperative irradiation. Of the 13 patients, 2 survived more than 5 years and 2 were alive within 1 year of therapy. Because cervical lymph node spread is common, lymph node clearance is recommended for patients with lateralized lesion or with palpable lymphadenopathy at presentation.

Aged↗

PubMed1978

A prospective randomized study of the efficacy of routine elective lymphadenectomy in management of malignant melanoma. Preliminary results.

To determine whether immediate or delayed lymphadenectomy is more beneficial than none in cases of localized (stage I) melanoma, we undertook in 1972 a prospective randomized study. Patients with midline trunk lesions were excluded as well as patients with lesions situated directly over the node-bearing area. In addition, because of the low risk of metastasis, the protocol was changed to exclude level 2 lesions. Of the 173 patients studied, 63 were randomized to no lymphadenectomy, 56 to delayed (3 months) lymphadenectomy, and 54 to immediate lymphadenectomy. None of these regimens differed significantly from the others in its effect on length of survival or interval to metastasis. And of the 110 patients who underwent elective lymphadenectomy, 103 were without nodal involvement. Our preliminary conclusion is that elective node dissection is not beneficial in management of melanoma. However, disease progression was advanced significantly by age of the patient (greater than 60 years) and by invasiveness (level 4 or 5) and thickness (greater than 1.5 mm) of the melanoma.

Adult↗

PubMed1978

Extraskeletal Ewing's sarcoma: a preliminary review of 26 cases encountered in the Intergroup Rhabdomyosarcoma Study.

Twenty-six of three hundred and fourteen (26/314) patients whose tumors were reviewed by the Pathology Evaluation Committee of the Intergroup Rhabdomyosarcoma Study have histologic subtypes I and II which have morphologic characteristics similar to Ewing's sarcoma of bone. They are somewhat more common in an extremity lesion but have no relation to patient age. Disease-free survival is good; seventeen of twenty-six (17/26) or sixty-five (65%) percent thus far.

Child↗

PubMed1978

Paratesticular rhabdomyosarcoma in childhood.

The paratesticular region was the primary site in 20 of 289 children (7%) entered on the Intergroup Rhabdomyosarcoma Study. The 20 patients were 1.7- to 19-years-old at diagnosis. Fifteen underwent retroperitoneal node dissection (12) or biopsy (3), and 6/15 (40%) had nodal involvement by tumor. Nineteen of the 20 patients had no gross local or metastatic disease after surgery. All 20 received chemotherapy, and 13 also received radiotherapy. Treatment was effective: 16 of 18 evaluable patients (89%) were free of disease at a median of 23 months from diagnosis (range, 8-43 months). Since the incidence of tumor-involved retroperitoneal nodes is high, a dissection should be performed. If the nodes are free of tumor, retroperitoneal radiotherapy may not be necessary. Reduction of morbidity in patients with paratesticular rhabdomyosarcoma is desirable, because the disease has a good prognosis.

Adolescent↗

PubMed1978

Adjuvant radiation therapy for regional nodal metastases from malignant melanoma: a randomized, prospective study.

After nodal metastasis from malignant melanoma, approximately 80% of patients die from disseminated disease. To clarify the role of radiation therapy (XRT) following node dissection. 56 patients with biopsy-proven nodal metastasis participated in a randomized, prospective clinical trial which compares radiation therapy to the regional lymph node area following lymphadenectomy (27 patients) with lymphadenectomy alone (29 patients). Interesting differences in the survival curves (p = 0.09) and in the disease-free interval curves (p = 0.08) for the two treatment groups proved to be attributable to imbalances in the age and nodal distributions in the treatment groups. Covariate analysis identified age and sex as the factors having the most significant (p less than 0.04) effect on survival and identified the number of positive nodes as the covariate having the most significant (p less than 0.02) effect on disease-free interval. Treatment did not have a significant effect upon survival or disease-free interval.

Clinical Trials as Topic↗