Beverly Raney

The intergroup rhabdomyosarcoma study. A preliminary report

Four hundred and twenty‐three children with newly diagnosed rhabdomyosarcoma have been entered to date in the Intergroup Rhabdomyosarcoma Study (IRS), which began in 1972. Patients were classified into Clinical Disease Groups (Stages) I‐IV, based on disease extent and resectability, and treatment regimens were randomly assigned according to group. Two hundred and seventy‐eight of 423 patients are evaluable for this analysis. Thus far, for Group I disease (localized/completely resected), disease control achieved by vincristine, dactinomycin, and cyclophosphamide (VAC) given in combination for 2 years has not been enhanced by the administration of postoperative radiation to the tumor bed. To date, 92% of patients in both irradiated and nonirradiated groups exhibit no evidence of disease, and 92‐96% are still alive, with the median time of follow‐up being 72 weeks. For Group II disease (microscopic residual/nodal involvement), VAC given for 2 years has not been found to be more effective than vincristine plus dactinomycin given for 1 year, both groups also having received postoperative irradiation. Thus far, over 85% of patients on either treatment have no evidence of disease and 90% are still alive. Survival and complete remission durations range from 1+ to 143+ weeks and the median duration of follow‐up is 45 weeks. Chemotherapy as initial treatment has been studied in Group III (localized sarcoma not treated initially by gross total resection) and Group IV (metastases present at diagnosis) patients. They have received either intensive “pulse” VAC or “pulse” VAC plus Adriamycin, and radiation has been administered after 6 weeks. Eighty‐one percent of patients in Group III and 81‐83% in Group IV have responded favorably, with complete regression of disease having been observed in over one‐fourth of patients even before the start of radiation and in approximately one‐half of all the patients after receiving radiation therapy. There is no indication as yet that one treatment regimen is superior to the other. Seventy‐nine percent of patients in Group III are still alive (0+ to 154+ weeks) and 69% remain in continuous response (0+ to 139+ weeks) with the median duration of follow‐up being 41‐44 weeks. Fifty percent of patients in Group IV are still alive (0+ to 127+ weeks) with a median time of follow‐up of 41‐44 weeks. Tumors arising either from genitourinary sites or the extremities have had a higher incidence of lymphatic spread than tumors in all other primary sites of origin.

Differentiated thyroid cancer in children and adolescents: Clinical outcome and mortality after long‐term follow‐up

Children and adolescents with differentiated thyroid cancer (DTC) have a good prognosis and prolonged survival even when extensive regional disease or lung metastases are present at the diagnosis; very‐long‐term follow‐up is needed to appreciate what, if any, impact the disease may exert on the ultimate outcome.

Fibromyxoid sarcoma in a four-year-old child: case report and review of the literature.

We present a child with a rare and chemotherapy-resistant form of soft-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was 4 years old. He is the youngest patient reported to date. An 11-year-old white male presented to. The University of Texas M.D. Anderson Cancer Centers Department of Pediatrics with a 7-year history of right thigh mass and pulmonary nodules, confirmed on examination. He had undergone extensive prior chemotherapy and surgery. He received chemotherapy with high-dose cyclophosphamide (7 g/m2) and later etoposide (150 mg/m2/day x 5), with only slight shrinkage of the thigh mass and none in the lungs. Subsequently the tumor in his proximal thigh and his lung metastases were resected, and radiation therapy was administered to the thigh. His disease remained stable for 12 months, but he then developed a pleural-based metastasis on the left side and new bilateral lung metastases also. The tumors on the left side were removed; residual disease is stable after treatment for 6 months with subcutaneous alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in children. It grows slowly and metastasizes to distant organs, chiefly to the lungs. It is resistant to conventional chemotherapy, and thus far only surgery seems to have a life-prolonging effect. Newer chemotherapeutic and possibly biologic agents should be tried in future patients, in order to find an effective way to control the disease.

Ascending myelitis after intensive chemotherapy and radiation therapy in children with cranial parameningeal sarcoma

In 1977, a program of early, wide‐field radiation therapy (RT) to the central nervous system and repeated lumbar intrathecal (IT) medications along with systemic chemotherapy was begun by the Intergroup Rhabdomyosarcoma Study (IRS) for patients younger than 21 years of age with cranial parameningeal sarcoma and a high risk of meningeal extension. From 1977 until 1987, 149 eligible patients with high‐risk cranial parameningeal sarcoma were enrolled in IRS trials. None had evidence of lower extremity or sphincter impairment at diagnosis. Five of the 149 (3.4%) had ascending myelitis at 5.5 to 9 months after the initiation of therapy, with loss of sphincter control and inability to walk; this progressed to severe flaccid quadriparesis and necessitated longterm ventilatory support in 4. All five had received vincristine, dactinomycin, cyclophosphamide, and doxorubicin; four also had received cisplatin and three also had received etoposide. All patients received 4770 to 5500 cGy to the primary tumor, and four patients received 3000 cGy of cranial RT. Three patients received cervical RT and two received spinal RT. The patients also received four to seven courses of IT methotrexate, hydrocortisone, and cytosine arabinoside. Three patients died: one after local tumor recurrence with central nervous system extension and two without known recurrence. In one of the latter patients, the results of an autopsy showed necrosis of the cervical spinal cord and caudal medulla. Although the exact cause of this complication is unclear, no additional cases have been reported to the IRS since the protocol was revised in 1987 to reduce the doses of the IT drugs and to limit them to four courses each. Cancer 1992; 69:1498‐1506.

Primary renal sarcomas in the Intergroup Rhabdomyosarcoma Study Group (IRSG) experience, 1972-2005: A report from the Children's Oncology Group.

To describe clinical and pathologic characteristics and outcome of patients with renal sarcomas.

Results in patients with cranial parameningeal sarcoma and metastases (Stage 4) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II–IV, 1978–1997: Report from the Children's Oncology Group†

Determine outcome of patients with cranial parameningeal sarcoma and concurrent metastases treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols II–IV.

Metastatic intracranial chordoma in a child with massive pulmonary tumor emboli

A 27-month-old boy of Hispanic background developed multiple cranial nerve palsies, difficulty swallowing, bloody nasal discharge, and irritability. Radiographic evaluations showed extensive destruction of the clivus by a large tumor that invaded the sphenoid bone, left cavernous sinus, ethmoid sinus, nasal cavity, and left orbit. Multiple pulmonary nodules were also noted. The bone marrow and spinal fluid showed no evident tumor cells. Transnasal biopsy revealed a chordoma. Treatment was initiated with a combination of ifosfamide, mesna, and etoposide along with radiation therapy to the cranial tumor. Shifting pulmonary densities were noted on serial films. Despite some clinical improvement, the child developed rapidly progressive hypoxemia 3 weeks after admission and died. Autopsy showed persistent viable tumor in the primary site and massive pulmonary arteriolar tumor emboli, infarcts, and widespread lung parenchymal metastases. No other sites of tumor involvement were discovered. This is the second child reported with intracranial chordoma, pulmonary metastases at diagnosis, and early death attributed to pulmonary tumor emboli.