The Intergroup Rhabdomyosarcoma Study-II.

Background: Intergroup Rhabdomyosarcoma Study (IRS)-II, (1978-1984) had the general goals of improving the survival and treatment of children with rhabdomyosarcoma (RMS).

Methods: Nine hundred ninety-nine previously untreated eligible patients entered the study after surgery and were randomized or assigned to therapy by IRS Clinical Group (I-IV), tumor site, and histologic type. Outcomes were compared between treatments and with results of IRS-I (1972-1978).

Consistent numerical chromosome aberrations in congenital fibrosarcoma.

Cytogenetic analysis of a congenital fibrosarcoma of the volar forearm from a 2.5-month-old boy revealed a mosaic karyotype 46,XY/49,XY,+11,+17,+20. This pattern of specific trisomies provides additional support to the cytogenetic findings in five cases of congenital fibrosarcoma reported previously.

Second malignancies in very-long-term survivors of childhood cancer.

Purpose: Although most second malignancies are treatment related, their occurrence also may be due to an underlying systemic disease or chromosomal abnormalities shared by multiple organs in which they are tumorigenic. We attempted to identify unusual tumor pairs that might provide a clue to shared genetic etiologies.

Patients And Methods: Medical records and tumor registry correspondence of 1,743 patients (0 to 18 years at diagnosis) were reviewed.

Early structural changes in vascularized periosteal flaps studied in situ.

This study examines the very early stages of osteogenesis in vascularized periosteal flaps and completes a characterization of their behavior after decortication. Pleuroperiosteal flaps based on the intercostal artery were developed in nine dogs and studied in situ by histologic and tetracycline fluorescence methods over periods varying from 1 to 7 days. The earliest changes were noted at 72 hours and were characterized by cellular and capillary proliferation, osteoid deposition, and bright fluorescence.

MR and CT imaging of ethanol-treated liver tumors in an animal model.

To characterize the radiographic appearance of liver lesions over time following ethanol injection, seven New Zealand white rabbits underwent surgical implantation of small fragments of VX-2 carcinoma within the liver. Upon reaching 1 cm in diameter, a tumor nodule was directly injected with absolute ethanol. Another nodule in the same animal was injected with saline as a control.

Ethanol injection of hepatic tumors.

To assess the efficacy of intratumoral injections of absolute ethanol in the treatment of hepatic tumors, 18 New Zealand White rabbits underwent implantation of two 1-mm3 fragments of the VX-2 carcinoma. The animals were reexplored 2 weeks postimplant and the tumors measured. One nodule was treated by intratumoral injections of 2.

Prognostic implications of bronchoalveolar lavage neutrophilia in patients with Pneumocystis carinii pneumonia and AIDS.

The clinical records and bronchoalveolar lavage (BAL) cell differential counts were analyzed in 96 patients at risk for Pneumocystis carinii pneumonia (PCP) from human immunodeficiency virus (HIV) infection to determine if this information may be prognostically useful and to identify possible mechanisms of BAL neutrophilia. In 60 patients with PCP, 15 fatalities or episodes of respiratory failure occurred, and 14 of these patients had greater than 5% BAL neutrophils. Only one of 33 patients with PCP and less than 5% BAL neutrophils died.

Pleuropulmonary actinomycosis associated with a systemic-to-pulmonary artery fistula and contralateral metastatic back mass.

We report a case of systemic-to-pulmonary artery fistula associated with thoracic actinomycosis and with metastatic hematogenous dissemination to the soft tissues of the back. The difficulty in diagnosing thoracic actinomycosis may predispose to the increased incidence of hematogenous spread of this disease. Although resection of pulmonary tissue including the infectious mass was required in previous cases, resection of the pleural mass alone was curative in this patient when combined with penicillin therapy.

Arteriotracheal fistula in patients treated for lymphoma.

Exsanguinating hemorrhage is an unusual cause of death in patients who are being treated for neoplasms. We report two cases of adolescents with mediastinal non-Hodgkin's lymphoma who each developed a fistula between the right brachiocephalic trunk and the trachea following mediastinal irradiation and systemic chemotherapy. During the hospital course, the patients had each suffered a respiratory arrest and undergone difficult endotracheal intubation.

Early onset of childhood pulmonary lymphangiomyomatosis.

The authors present the first case of lymphangiomyomatosis in a pediatric patient. Radiographic findings, biopsy, computerized tomography, and electron microscopy were useful in detailing the presentation of early onset of pulmonary lymphangiomyomatosis. The unusual presentation of this child includes a description of her disease prior to any pulmonary symptomatology and 4 years of follow-up.

Gonadoblastoma: unusual presentation in a patient lacking persistent müllerian ducts.

We report a patient with a disorder of sexual differentiation who presented with a 46,XY karyotype, absent internal Müllerian ducts, a vaginal pouch, hypospadias, and bilateral cryptorchidism with a gonadoblastoma in one testis. A human chorionic gonadotropin stimulation test and tissue 5-alpha-reductase and androgen receptor assays were normal. Except for the absence of internal Müllerian ducts, this patient most closely resembles the disorder of dysgenetic male pseudohermaphroditism (DMP).

Bridging bronchus and posterior left pulmonary artery: a unique association.

A 6-month-old female with a lifelong history of respiratory distress became increasingly difficult to manage and required right upper and middle pulmonary lobectomies for worsening emphysema and mediastinal shift. The postoperative course was stormy and confusing and the patient died despite emergency tracheostomy. The autopsy disclosed an anomalous bronchus to the right lower lobe, originating from the left mainstem bronchus.

Extraosseous Ewing's sarcoma versus primitive rhabdomyosarcoma: diagnostic criteria and clinical correlation.

A light and electron microscopic study of 51 cases of Ewing's sarcoma of bone (ESB) and 33 soft tissue sarcomas (carrying a variety of light microscopic diagnoses, including primitive rhabdomyosarcoma) in children and young adults was performed to clarify the similarities and differences among these tumors. Ultrastructural criteria were developed to evaluate the neoplasms. Remarkable ultrastructural uniformity was found in the cases of ESB.

Extraskeletal Ewing's sarcoma: results of combined modality treatment.

Eleven patients with extraskeletal Ewing's sarcoma (EES) were treated with combined modality therapy at the National Cancer Institute. The diagnosis of EES was reserved for lesions that were identical to Ewing's sarcoma of bone by light and electron microscopy. Diagnostic work-up to rule out a skeletal primary included bone scan, localized views of adjacent bone, and bone tomography.

A sibship with hypokalemic alkalosis and renal proximal tubulopathy.

A new syndrome, characterized by hypokalemic alkalosis, hyperreninemia, aldosterone, high urinary prostaglandin E2 excretion, normal BP, and resistance of BP to angiotensin II is described in three of four siblings. Histologic examination of tissue obtained by biopsy from the kidneys showed an intense staining of the proximal tubular cells, as well as an extreme hypertrophy of the proximal tubular basement membranes, features that previously have not been observed. On electron microscopic examination, the characteristic changes of the tubular cells consisted of very dense cytoplasm, compact mitochondria, and pyknotic nuclei.

Ewing's sarcoma. Characterization in established cultures and evidence of its histogenesis.

We have studied the ultrastructure and collagen biosynthetic profiles of three cultured lines of Ewing's sarcoma (ES) to better understand the histogenesis of this tumor. The histology and ultrastructure of the original tumors were characteristic of ES. Light and electron microscopic appearance of the cell lines and of tumors formed in nude mice from injection of these lines were similar to the native tumors.

Pulmonary histopathologic changes associated with melphalan therapy.

A case of fatal pulmonary fibrosis and atypical epithelial proliferation (AEP) in a patient with multiple myeloma treated with melphalan is presented. Review of 10 other autopsied patients with myeloma treated with melphalan but no thoracic radiation, other cytotoxic agents, or highdose oxygen therapy revealed one other patient who died with extensive pulmonary fibrosis and AEP. Four other patients with AEP not associated with pneumonitis or fibrosis were also found, while no such changes were found in 11 autopsy controls or 11 patients with myeloma who did not receive cytotoxic agents.

Long bone ossifying fibromas.

Ossifying fibromas involving the tibia were seen in two patients. In both patients, the radiographic appearance of the lesions suggested fibrous dysplasia, but histopathologic evaluation demonstrated findings similar to ossifying fibroma of the mandible and facial bones. Radiologic and pathologic recognition of this entity is necessary for proper treatment.

Vasoactive intestinal polypeptide and gastrin-producing islet cell carcinoma.

A 61-year-old woman had watery diarrhea, hypochlorhydria, hypokalemia, and elevated serum gastrin levels. She had islet cell carcinoma of the body of the pancreas with multiple metastases to the liver. Radioimmunoassay and immunofluorescence demonstrated both vasoactive intestinal polypeptide (VIP) and gastrin in the surgically removed carcinoma and in a metastatic focus.

Nonfunctioning argyrophilic tumor (APUDoma) of the hepatic duct: simplified methods of detecting biogenic amines in tissue.

This report documents a foregut endocrine neoplasm in an unusual location, the main hepatic bile duct, and describes simplified methods of detecting similar endocrine tumors. Formaldehyde fume-induced fluorescence (FIF) is a quick, specific method to demonstrate biogenic amines. FIF combined with histochemical procedures showing abundant alpha-glycerol phosphate dehydrogenase and esterases indicates the neural crest origin of these and related neoplasms.