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J Fontanesi

Publications and source records attributed to J Fontanesi.

At least 55 records · Page 3Linked to original sources
PubMed1993

Bilateral Wilms tumor. Review of outcome, associated abnormalities, and late effects in 36 pediatric patients treated at a single institution.

BACKGROUND: Patients with bilateral Wilms tumor have an increased incidence of associated abnormalities and nephrogenic rests and require individualized multimodality therapy for cure. The authors reported the associated abnormalities, outcome, complications, and late effects of treatment in a group of children with bilateral Wilms tumor treated at St. Jude Children's Research Hospital, Memphis, Tennessee, over a 28-year period. METHODS: The records of 36 consecutive pediatric patients diagnosed with bilateral Wilms tumor between 1962-1990 were analyzed. Biopsy material was also reviewed, with particular emphasis on characterization of nephrogenic rests and histology. RESULTS: Twenty-nine patients had synchronous tumors and 7 had metachronous lesions. Associated physical abnormalities were present in 12 patients and involved the genitourinary, cardiovascular, integumentary, and musculoskeletal systems. The overall survival for patients with metachronous tumors (71%) was similar to that for those with synchronous tumors (70%). There was no effect of age or the presence of nephroblastomatosis. Two patients with synchronous tumors initially treated with nephrectomy eventually required bilateral nephrectomies for contralateral recurrence after chemotherapy and radiation therapy. Nephrogenic rests were present in 22 of 30 evaluable patients. Two of three patients with metachronous tumors had intralobar nephrogenic rests. Bilateral renal salvage procedures were demonstrated to be technically feasible and effective in controlling disease without compromising renal function or survival. Late effects included scoliosis in three patients treated before 1970, cardiomyopathy in one patient who received 300 mg/m2 doxorubicin and 12 Gy pulmonary irradiation, and benign tumors in two patients, one of whom also had a bowel obstruction. Serum creatinine, urea nitrogen, and blood pressure were normal in 23 of 26 survivors. CONCLUSIONS: The authors' experience supports a favorable outcome with minimal late effects for patients with bilateral Wilms tumor who receive individualized therapy at pediatric oncology centers.

Child↗
PubMed1993

Treatment of choroidal melanoma with I-125 plaque.

PURPOSES: To evaluate efficacy of I-125 episcleral plaque therapy in patients with ocular melanoma and determine survival, eventual visual acuity, and complications. METHODS AND MATERIALS: Between July 1, 1984 and January 1, 1991, 144 patients with diagnosis of ocular melanoma were treated with high activity I-125 episcleral plaques. Tumor volumes ranged from 14 to 3449 mm3. Lesion size included small (n = 15; height < 5 mm, and/or largest basal diameter of 8-16 mm) and large (n = 45; height > 8 mm, and/or largest basal diameter > 16 mm). Apical doses ranged from 74.25 to 83.66 Gy with scleral doses ranging from 41 and 160 Gy. Follow-up has ranged from 25 to 90 months (Med = 46 months). RESULTS: Ocular survival was noted in 130/144. Reasons for enucleation included progressive tumor growth (n = 4), extrascleral extension (n = 4), or blind/painful eye (n = 6), 94 patients developed complications which included cataract (n = 43), optic neuropathy (n = 12), neovascular glaucoma (n = 8) and retinopathies (n = 31). Visual acuity testing pre-episcleral plaque therapy revealed 102 patients with 20/200 vision; at last follow-up 59 patients demonstrated visual acuity testing of 20/200 or better. CONCLUSION: The use of episcleral I-125 plaque therapy allows for safe and effective therapy in patients with ocular melanoma of various size depending on location and probable visual acuity outcome. A total apical dose of 75 Gy given at 60-65 cGy/hour provides durable local control with acceptable complication rates.

Adult↗
PubMed1993

A phase II trial of ifosfamide in previously untreated children and adolescents with unresectable rhabdomyosarcoma.

BACKGROUND: Children and adolescents with unresectable rhabdomyosarcoma fare poorly when treated with contemporary chemotherapeutic regimens. Evaluation of newly developed agents in these patients is important to improve their outcome. Based on a preclinical rhabdomyosarcoma xenograft model that accurately predicted the activity of new agents, the safety and efficacy of ifosfamide was evaluated as part of a Phase II clinical trial in previously untreated children with unresectable rhabdomyosarcoma. METHODS: Twenty-two children and adolescents (median age, 9 years) with newly diagnosed unresectable rhabdomyosarcoma (Intergroup Rhabdomyosarcoma Study Group III [n = 15] or IV [n = 7]) received two courses of ifosfamide at a dose of 1.6 g/m2 intravenously for 5 days over a 6-week period. Then the patients were evaluated for response, and additional treatment with surgery, radiation therapy, and multiagent chemotherapy (vincristine, cyclophosphamide, dactinomycin, and doxorubicin) was administered. RESULTS: Nineteen of 22 patients (86%) had a partial response to ifosfamide given as a single agent. No complete responses to this agent alone were observed. After administration of additional chemotherapy and local control measures (radiation therapy and surgery), the estimated proportion of patients surviving progression-free at 2 years was 63% (95% confidence interval, 37-80%). Ifosfamide was tolerated well; the most frequent toxicity was nondose-limiting myelosuppression. Transient mild renal toxicity infrequently was observed, and no central nervous system toxicity occurred in this group of patients. CONCLUSIONS: Ifosfamide appears to have significant clinical activity in untreated patients with unresectable rhabdomyosarcomas. These findings provide an accurate estimate of the response rate to single-agent ifosfamide in this group of previously untreated patients and thus provide a foundation for its rational incorporation into multiagent clinical trials. In addition, the potential benefits of this type of new drug development were demonstrated.

Adolescent↗
PubMed1993

Interstitial iodine 125 and concomitant cisplatin followed by hyperfractionated external beam irradiation for malignant supratentorial glioma. Preliminary experience at the University of Tennessee, Memphis.

Between November 1989 and October 1992, 28 consecutive patients with glioblastoma multiforme (n = 18) or anaplastic astrocytoma (n = 10; includes one patient with oligodendroglioma with anaplastic astrocytoma component) were treated with interstitial iodine 125 (60 Gy over 6 days) and with concomitant cisplatin (via infusion on days 2-6 of the implant), then followed by hyperfractionated external beam irradiation (110 cGy delivered twice daily; 66 Gy planned total dose). Of 26 patients (60%) who received both 125I and HEBI, 15 are alive with no evidence of recurrent disease at a median follow-up of 18 months post-125I (range: 11 to 34 months). Four other disease-free patients succumbed to nontumor-related events. Two patients with local control had distant failure outside the HEBI treatment fields. Overall local control is 77%. Local failure occurred in 6 patients (23%) 2 to 11 months post-125I. Time to disease progression ranged from 4 to 18 months (median: 10 months). Survival (measured from the date of diagnosis) has ranged from 6 to 26 months (median: 15 months). All patients have maintained Karnofsky Performance Status within 20 points of their preimplant status, with the exception of a single patient who, following diagnosis of radiation necrosis and surgical intervention for symptomatic relief, had a 30-point drop in KPS. Radiation necrosis or persistent mass effect were noted by neuroimaging in seven patients, four of whom required surgical intervention following failed medical management. Ototoxicity, nephrotoxicity, peripheral nerve dysfunction, or hematologic toxicities have not been observed. This new innovative treatment approach offers a promising alternative to the normally dismal prognosis for patients with malignant gliomas.

Adult↗
PubMed1993

High-dose chemotherapy and autologous bone marrow rescue followed by interstitial and external-beam radiotherapy in newly diagnosed pediatric malignant gliomas.

PURPOSE: Evaluation of high-dose chemotherapy with autologous bone marrow rescue (ABMR) in pediatric malignant gliomas. PATIENTS AND METHODS: Newly diagnosed (n = 11) and recurrent (n = 2) malignant glioma patients received high-dose chemotherapy within 4 weeks of surgery; three had near total and 10 had subtotal resection/biopsy. High-dose thiotepa (300 mg/m2) and cyclophosphamide (2 g/m2) daily for 3 days were followed by ABMR; response was evaluated at day 30. At day 60, patients with at least stable disease received hyperfractionated (n = 9) or conventional external-beam radiotherapy (n = 2) preceded by local radioactive iodine 125 implantation (n = 2) or radiosurgery (n = 1). RESULTS: Grade III and IV toxicities after ABMR consisted of mucositis (n = 12), cardiomyopathy (n = 1), acute abdomen (n = 1), pneumonitis (n = 2), and infection (n = 2). One complete and three partial responses were observed; the objective response rate was 31% (95% confidence interval, 9% to 61%). Seven had stable disease, one had disease progression, and one died of toxicity before response evaluation. The median overall and progression-free survival durations after combined modality therapy were 14 months (range, 4 to 30+) and 9 months (range, 0 to 30+), respectively. One patient remains progression-free at 30+ months. Radionecrosis and white matter changes occurred in three patients: one after hyperfractionated irradiation, and two after 125I implants. CONCLUSION: For patients with bulky residual disease after surgery, survival with this aggressive chemotherapy and radiation regimen is not better than that reported for conventional treatment regimens.

Adolescent↗
PubMed1992

Treatment of childhood germ cell tumors. Review of the St. Jude experience from 1979 to 1988.

BACKGROUND: The outlook for patients with germ cell tumors was poor before the advent of effective chemotherapy. The authors assessed the outcome of treatment with multiagent chemotherapy (with or without radiation therapy) in children treated for germ cell tumors at St. Jude Children's Research Hospital (SJCRH). METHODS: Sixty children with germ cell tumors were treated between January 1979 and June 1988. Postsurgical treatment was based on tumor site, stage, and histology. Most patients received chemotherapy with vincristine, actinomycin-D, and cyclophosphamide (VAC), or a modified Einhorn regimen (cisplatin, bleomycin, and vinblastine [PVB]); in the absence of response to initial therapy, patients received alternating courses of VAC and PVB (VAC/PVB regimen). Exceptions were patients with Stage I testicular tumors (observation only) and ovarian germinomas (Stage I tumors measuring less than 10 cm, observation only; tumors larger than 10 cm or Stage II-III disease, radiation only; and Stage IV disease, VAC plus radiation). RESULTS: The estimated 5-year survival is 100% for patients with Stage I disease (n = 18), 87% for patients with Stage II (n = 8), 72% for Stage III (n = 25), and 56% for Stage IV (n = 9). Patients with testicular tumors of any stage or with Stage I-II ovarian tumors had 100% 5-year survival. Extragonadal tumors responded poorly to VAC alone with recurrent or progressive disease in eight of nine patients. Treatment for those tumors was changed to alternating courses of VAC and PVB, which failed in only one of seven patients. Nine of 19 patients with advanced ovarian tumors had disease recurrence with VAC; these patients then received PVB, which was effective in four cases. CONCLUSIONS: For patients with advanced germ cell cancers, intensification of therapy or the development of new approaches is necessary. In contrast, future trials in children with limited stage should focus on reducing acute and long-term toxicities.

Adolescent↗
PubMed1992

Hepaticopancreaticogastroduodenectomy with transplantation for metastatic islet cell carcinoma in childhood.

Symptomatic islet cell carcinoma of the pancreas with liver metastasis, a rare tumor cured only by complete resection, was unresponsive to chemotherapy and was treated with hepaticopancreaticogastroduodenectomy en bloc resection with transplantation in an 11-year-old girl. This radical surgery in conjunction with adjuvant chemotherapy and interstitial and external beam irradiation has resulted in symptomatic relief and no evidence of disease at 15 months posttransplant.

Adenoma, Islet Cell↗
PubMed1992

Sarcomas of the flexor fossae in children: is amputation necessary?

Successful management of sarcomas of the extremities in children implies not only achievement of local control but also satisfactory function and maintenance of the growth potential. The popliteal and antecubital fossae, because of their complex neurovascular anatomy, all of which is essential, make resection with satisfactory margins difficult. We reviewed our experience with 14 patients (3 to 20 years old; median, 13 years) with soft tissue sarcomas arising in the popliteal (11 patients) or antecubital (3 patients) fossae. There were four rhabdomyosarcomas (3 alveolar, 1 embryonal) and 10 other sarcomas, the most frequent being synovial sarcoma (5 patients). Chemotherapy was given to all patients with rhabdomyosarcomas. The one patient presenting with metastatic disease was treated, after biopsy of the primary, by chemotherapy and radiation and survived 21 months. In three patients, the primary management was an above-the-knee amputation and two of three survived (3 and 43 months). In 10 patients a wide local excision of the primary tumor was performed. Radiation therapy was administered to five, either as external beam (3 patients) or as brachytherapy (2 patients). In this group, there were no local recurrences. Four patients remain free of disease (4 months to 18 years) and one developed pulmonary metastasis. Among the five non-irradiated patients, three developed local recurrences, requiring above-the-knee amputation for disease. The fourth patient relapsed in the lung and only one of the five is free of disease at 36 months. Of the 8 patients not treated with amputation, one acquired a leg length discrepancy, which required correction, and one has a minimal extension deficit of the knee.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Pediatric nonrhabdomyosarcomas of the extremities. Influence of size, invasiveness, and grade on outcome.

Surgery alone or supplemental radiation is effective in local control in a majority of patients with nonrhabdomyosarcoma of the soft tissue. To determine the factors influencing subsequent survival, a retrospective review of 64 children with nonrhabdomyosarcoma of the soft tissue of the extremities was performed. Using the American Joint Committee on Cancer staging system, 32 patients had noninvasive T1 lesions, and 31 of these patients survived disease free. Twenty-two patients had lesions that were smaller than 5 cm; 19 of these lesions were grade G1-2. All 22 patients survived. Ten patients had tumors that were 5 cm or larger, and all were G1-2; nine of these patients survived. Thirty-two patients had invasive (T2) lesions; seven of these lesions were smaller than 5 cm, but four were G3. Only one patient survived. Eighteen (72%) of 25 lesions that were 5 cm or larger were G3. Fifteen (83%) of these 18 patients underwent relapse, with 12 patients dying of the disease. Important prognostic factors included G3 lesions, with a 72% (18 of 25 patients) failure rate, and T2 lesions, which were generally larger than 5 cm, of higher grade, and underwent relapse more frequently than T1 lesions. These patients with histologic G3 and T2 lesions may benefit from chemotherapy.

Adolescent↗
PubMed1991

Concomitant cisplatin and hyperfractionated external beam irradiation for advanced malignancy of the head and neck.

The combination of cisplatin and hyperfractionated external beam irradiation (HEBI), followed by salvage surgery when indicated, was evaluated in patients with advanced stage squamous cell carcinoma of the head and neck. Thirty patients with stage III (n = 5) or IV (n = 25) disease received intravenous cisplatin 100 mg/m2 by 6-hour continuous infusion on days 1, 21, and 42 of HEBI. Radiation fractions of 110 cGy were given twice daily, separated by 4 to 6 hours, beginning within 12 hours after cisplatin delivery. Doses to the primary site ranged from 60 to 76.35 Gy (median: 72.3 Gy), with 60 to 74 Gy to nodal sites. Follow-up ranged from 4 to 28 months (median: 19 months). Clinical complete response of the primary site was seen in 27 of 29 patients (93%), and complete clinical clearance of adenopathy in 20 of 26 (76%). A second biopsy 6 to 8 weeks after completion of treatment showed residual disease in both the primary and nodal sites in three patients, and in only the primary site in one patient. Four patients with persistent adenopathy had pathologic confirmation at surgery. Four patients had recurrence after negative biopsy results 6 to 9 months after treatment biopsy. At present, with median follow-up of 19 months, eight patients (26%) have died secondary to uncontrolled primary or nodal disease. Two patients have died of nonrelated causes. Overall, 10 of 30 patients (66%) remain alive with no evidence of disease. Mucositis and weight loss were the most common side effects of treatment. Seven patients developed significant xerostomia, and four have cisplatin-related hearing loss requiring amplification. The early evidence of excellent response (89% pathologic complete response of primary sites; 78% complete response of nodal sites), coupled with acceptable treatment morbidity, warrants further study of this approach.

Adult↗
PubMed1991

Ewing's sarcoma: local tumor control and patterns of failure following limited-volume radiation therapy.

Sixty children with localized osseous Ewing's sarcoma were treated between 1978 and 1988 with induction chemotherapy (cyclophosphamide, adriamycin), irradiation and/or surgery, and 10 months of maintenance chemotherapy (cyclophosphamide, adriamycin, dactinomycin, vincristine). Following induction chemotherapy, 43 patients received primary radiation therapy to limited radiation volumes defined by post-chemotherapy residual soft tissue tumor extension and initial osseous tumor extent. Irradiation was defined as low dose at 30-36 Gy (median 35 Gy) for 31 cases with objective response to induction chemotherapy and high dose at 50-60 Gy (median 50.4 Gy) for 12 patients with poor response to induction chemotherapy or with tumors greater than or equal to 8 cm. Overall event-free survival at 5 years is 59% and local tumor control is 68%. Initial failures have been local (12), simultaneous local and distant failures (7), and distant (6). In the surgical resection group, 14 patients had complete resection without radiation therapy, and 3 patients had microscopic residual plus 35-41 Gy; 100% local control has been maintained. In 43 patients with primary radiation therapy group, local tumor control is 58% (p = .004). Despite limited radiation volume, 18/19 local failures occurred centrally within the bone, well within the radiation volume. Imaging response to induction chemotherapy predicted local tumor control in the radiation therapy group: 62% with complete response/partial response versus 17% with no response/progressive disease (p less than 0.01). Local tumor control related strongly to primary tumor size in the radiation therapy group; among 31 cases receiving 35 Gy, local tumor control is 90% for lesions less than 8 cm versus 52% for tumors greater than or equal to 8 cm (p = .054). The central pattern of local failure in this experience suggests the effectiveness of limited radiation volume. The overall local tumor control rate following the tested dose level of 35 Gy appears to be inadequate, although results in selected cases with tumors less than 8 cm in greatest tumor dimension indicate potential efficacy in a yet limited experience.

Adolescent↗
PubMed1990

Testicular relapse in children with acute nonlymphoblastic leukemia.

The clinical course and other distinctive features of five children who developed a testicular relapse 4 months to 25 months after the diagnosis of acute nonlymphoblastic leukemia (ANLL) are described. The chief presenting feature at relapse was painless testicular enlargement, as is also seen in children with acute lymphoblastic leukemia who relapse in the testes. By French-American-British convention, the malignant cells were classified as M4 (myelomonoblastic) in four cases and M2 (myeloblastic) in one. All children received a course of multiagent reinduction chemotherapy and all but one received local irradiation to the testes. Only one of these children, whose relapse was a late event after elective cessation of therapy, is a long-term survivor. A comparison with six previously published cases shows similar clinical characteristics and outcome. Given the poor responses of such patients to conventional treatment, it seems worthwhile to consider the use of intensive reinduction chemotherapy with concomitant bilateral testicular irradiation followed by remission intensification and an autologous or allogenic marrow transplant.

Adolescent↗
PubMed1990

Removal of radiation-induced cataracts in patients treated for retinoblastoma.

Experience with removal of radiation-induced cataract in patients treated for retinoblastoma is limited. We retrospectively reviewed the records of 38 patients with retinoblastoma (42 eyes) who underwent removal of radiation-induced cataract from 1973 to 1989. Nineteen eyes (45%) without macular tumors or severe radiation complications had final visual acuities in the range of 20/20 to 20/50. One eye (2.4%) developed a rhegmatogenous retinal detachment and four eyes (9.5%) were noted to have amblyopia after cataract removal. Three eyes (7%) developed retinoblastoma recurrence, one with extension of retinoblastoma into the subconjunctival space through the previous sclerotomy. Exenteration was performed and the patient was alive after 8 years. Cataract removal can be visually beneficial in selected patients with radiation-induced cataracts.

Adolescent↗
PubMed1990

Wilms' tumor: reduced-dose radiotherapy in advanced-stage Wilms' tumor with favorable histology.

Fifty-two children with favorable histology Wilms' tumor who had residual abdominal disease (Surgical Stages III and IV) were treated from 1979 to 1988 on a protocol designed to assess the effectiveness of reduced radiation doses. All patients received three-agent chemotherapy, beginning within 1 week after surgery. To permit assessment of disease response to initial chemotherapy, radiation therapy was delayed for a median of 28 days after surgery (range, 14-71 days). Total doses of abdominal radiation were limited to 12 Gy, given as 150 cGy daily fractions; 18 patients with Stage IV disease received 12 Gy bilateral pulmonary irradiation. Two year disease-free survival was 85% and 71% for Stage III and IV, respectively (p = .24). Abdominal relapses occurred in 3 cases (5.7%). The interval between surgery and initiation of irradiation was not related to disease-free survival. Of several patient and disease-related factors analyzed, only patient age was related to outcome. Disease-free survival was 100% at 3 years for children under the age of 3 versus 78% for children greater than age 3 (p = .05). Reduced-dose abdominal radiotherapy in conjunction with multi-agent chemotherapy and surgery provided excellent disease control with minimal toxicity in advanced-stage, favorable histology Wilms' tumor.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1990

Local-regional non-rhabdomyosarcomatous soft tissue sarcomas of the head and neck.

Between 1962 and 1987, 112 consecutive patients were treated at St. Jude Children's Research Hospital for soft tissue sarcomas of the head and neck region; 18 of these children (16%) had histologic subtypes other than rhabdomyosarcoma. We evaluated the impact of surgery, postoperative chemotherapy, and irradiation on local control and survival in these cases. Three patients who had complete resection of tumors and received no further treatment are alive without disease at 36, 42, and 162 months. Local control was achieved in 1 of 2 patients with microscopic residual tumor and 4 of 9 patients with gross residual tumor who were treated with irradiation (2500-5040 cGy). Chemotherapy was the only postoperative treatment in three patients; only one achieved lasting local control. One patient was treated with irradiation only; his primary site showed no tumor cells at autopsy following an automobile accident. Overall, local control was achieved in 50% of patients; the disease-free survival rate at 3 years was 44%. The prognosis for patients with nonresectable tumors remains unsatisfactory because of the difficulty in securing local control. A revised therapeutic approach to these patients is presented.

Adolescent↗
PubMed1990

Treatment of choroidal melanoma: MR imaging in the assessment of radioactive plaque position.

Verification of the position of an episcleral iodine-125 gold plaque in relation to underlying choroidal melanoma is essential during early radiation therapy to ensure accurate plaque placement and thus optimum dose delivery. The authors used magnetic resonance (MR) imaging to examine 15 patients with choroidal melanoma after plaque placement to assess tumor coverage. The relationship of the plaque to the tumor was well defined in all cases, including two tumors anterior to the ora serrata. MR imaging measurements of the plaques were within 1 mm of the actual plaque sizes, while tumor measurements were within 2 mm of the preoperative ultrasound estimations of tumor dimensions. Tumors as small as 3 mm thick were readily visualized with MR imaging. Associated subretinal effusion was demonstrated in seven cases.

Aged↗
PubMed1989

Management and prognosis of head and neck sarcomas.

Between 1962 and 1988, a total of 104 patients with head and neck rhabdomyosarcoma (RMS) and 17 patients with nonrhabdomyosarcoma (NRMS) were evaluated and treated at St. Jude Children's Research Hospital. All parameningeal sites (middle ear, orbit, or nasopharynx) were excluded from further analysis; thus, 50 patients represent the cohort of head and neck sarcomas for this review. Survival was good in this group of patients, 28 of 50 being alive and disease-free at last follow-up. Twenty of the 38 patients with RMS were alive and disease-free. Similarly, 8 of the 12 patients with NRMS were disease-free at a median follow-up of 5 years. However, the site and size of the primary tumor impacted on the extent of the initial resection and further treatment in addition to surgery. Although the treatment policy evolved over time to a stage-specific strategy for treatment modalities, the data suggest that surgery alone may be sufficient initial therapy for a subset of patients. For patients in whom complete resection is not achieved, the addition of radiotherapy and chemotherapy may result in improved survival.

Adolescent↗