Female and male differences in the survival of patients undergoing resection for lung cancer.

OBJECTIVE: In recent years much attention has been focused on the rapidly increasing incidence of primary lung cancer in women. The aim of this study was to determine gender differences in patients treated surgically for lung cancer. METHODS: We performed a retrospective review of patients who had lung resection for primary lung cancer from January 1994 to December 1998. RESULTS: There were 530 men and 42 women. Women were younger than men (55.6 +/- 10 versus 57 +/- 9.9). The difference was not statistically significant (p = 0.9). The mean cigarette consumption was greater in men than in women (p < 0.001). We observed a higher operative morbidity and mortality rate in men compared to women (p < 0.05). Men had more squamous cell carcinoma (61.7%). In female patients, squamous cell cancer and adenocarcinoma were found with almost the same incidence (35.7% versus 33.3%). There was a significant difference in the distribution of cell types (p < 0.001). Pathologic stages for women were; I = 38.2%, II = 21.4%, IIIa = 21.4%, IIIb = 9.5%, IV = 9.5% and for men; I = 40.4%, II = 26.6%, IIIa = 24%, IIIb = 6%, IV = 3%. There was no significant difference in the stage distribution between genders (p = 0.2). Median survival for female and male patients were 38 +/- 9.2 and 35 +/- 3.2 months. Female patients' survival was longer than male patients but the difference was not statistically significant (p > 0.05). CONCLUSIONS: This study emphasizes sex differences in cigarette consumption, operative mortality and the distribution of cell types of patients with primary lung cancer. These initial results should strongly encourage additional studies in different countries on the interaction between sex and lung cancer characteristics to improve clarity.

Hydatid cyst of the rib with intramedullary involvement.

Osseous localization is uncommon for a hydatid cyst. We present a case of a hydatid cyst with rib involvement spreading through the bone marrow in a 62-year old woman.

Primary malignant melanoma of the lung in oculocutaneous albino patient.

Oculocutaneous albinism is a rare autosomal recessive disorder characterized by general depigmentation, nystagmus, photophobia, and decreased visual acuity. Malignant melanoma is extremely rare in patients with albinism. We present a 41-year-old albino male patient, who was admitted with a suspected bronchogenic carcinoma. He underwent a pulmonary resection and the diagnosis was primary malign melanoma of the lung. The patient died of tumor recurrence in the postoperative 46th month.

Synovial sarcoma in children and adolescents: thirty three years of experience with multimodal therapy.

BACKGROUND: Synovial sarcoma (SS) is the most common type of non-rhabdomyosarcoma soft tissue sarcoma in childhood, with controversies about its prognosis and treatment. PROCEDURE: We reviewed medical records of 42 children and adolescents with SS treated at our institution between 1966 and 1999 to determine treatment results and assess prognostic factors. RESULTS: With a median follow-up duration of 7.8 years (range 0.2-22.4 years), 5-year progression free survival (PFS) and overall survival (OS) rates were 75.6% (95% Confidence Interval [CI] 62-89.2%) and 87.7% (95% CI 77.3-98.1%) respectively. Eleven patients were dead and four others had progressed but were alive without evidence of disease after further therapy. IRS grouping and tumor invasiveness were found to be significant prognostic indicators (P < 0.01 and = 0.02, respectively). Patients with initial gross total resection (IRS I and II) and non-invasive tumors (T1) were most likely to have prolonged PFS and OS. Patients with small tumors (< 5 cm) (P = 0.09) or with monophasic histology (P = 0.14) had better PFS and OS. CONCLUSIONS: Achieving a complete resection or gross total resection with microscopic residual disease is vital for survival of patients with localized SS. Patients with localized disease who received radiotherapy had improved local control. Chemotherapy did not seem to impact PFS or OS. Future large multi-institutional trials are needed to address whether post-operative chemotherapy is necessary for patients with localized, surgically removed tumors, whether radiotherapy is necessary for patients with completely resected tumors, and to ascertain the order of importance of all the candidate prognostic markers. Med Pediatr Oncol 2001;37:90-96.

Surgical treatment of pulmonary hydatid cysts in children.

PURPOSE: Ten years' experience is analyzed in pediatric patients with pulmonary hydatid cysts. METHODS: Between 1990 and 2000, 33 pediatric patients (2 to 15 years of age) with pulmonary hydatid cysts were operated on in Department of Thoracic Surgery in Ankara University School of Medicine. There were 17 girls and 16 boys. RESULTS: Twenty-three cases presented as a solitary lung cyst, whereas the remaining 10 were found to have multiple cysts in one or both lungs. Cystotomy and capitonnage were performed in 25 patients, cystotomy was done in 6 patients, and only 2 patients underwent the wedge resection. The authors used no scoliocidal agent in our approach. There was no operative mortality. Recurrence after operation was seen in one patient. CONCLUSION: Pulmonary hydatid cysts in children can be treated successfully by cystotomy and capitonnage or only cystotomy. J Pediatr Surg 36:917-920.

Management of esophageal perforation.

Despite recent advances in thoracic surgery, the management of esophageal perforation remains problematical and controversial. Thirty-one patients were treated for an esophageal perforation between 1986 and 1998. The esophageal perforation was iatrogenic in 25 cases, spontaneous in 2, traumatic in 2, and caused by a tumor and tuberculous lymphadenitis in 2 patients. There were 10 cervical, 19 thoracic, and 2 abdominal perforations. The interval from perforation to operation was less than 24h in 12 patients and more than 24h in 19 patients. The surgical procedures included a primary repair in 12 patients, a resection in 8, and conservative treatment with minor surgical approaches in 11. The mortality rate was 20% (4/20 patients) in the surgical treatment group and 45.5% (5/11 patients) in the conservative treatment with minor surgery group. The overall mortality was 29% (9/31 patients). The prognosis is thus concluded to depend on the cause and location of the perforation, the presence of underlying esophageal diseases, and the surgical procedure chosen.

Atypically located pericardial cysts.

Pericardial cysts are uncommon benign abnormalities and are most often found in either cardiophrenic angle. We present the cases of 3 patients with a cyst in the mediastinum and review the literature. Pathological examination confirmed the diagnosis of pericardial cyst. Clinicians should include pericardial cyst in the differential diagnosis of mediastinal masses.

Non-metastatic Ewing's sarcoma: twenty years of experience suggests that surgery is a prime factor for successful multimodality therapy.

Eighty-five patients (37 female, 48 male; median age 14 years) with non-metastatic Ewing's sarcoma received definitive treatment at the University of Texas M.D. Anderson Cancer Center between 1969 and 1988. Multidisciplinary therapy was administered as follows: combination chemotherapy (CC) and local radiotherapy (XRT): 65 patients; CC, XRT and surgery, 19 patients; and XRT and surgery, 1 patient. This permitted a 10-20 year follow-up for 75% of our patients. The overall survival at 5 and 10-20 years was 46.1%, and 37.2%, respectively. At 5 years, 80.5% of live patients had control of local disease. The influence of sex, age, ethnicity, primary site, size, lactic dehydrogenase (LDH) level, presence or absence of systemic symptoms, and XRT dose (<60 Gy and

Fractionated high-dose cyclophosphamide for advanced pediatric solid tumors.

PURPOSE: The objective of this study was to determine the tolerance and toxicities of high-dose cyclophosphamide (CPA) at 7 g/m2 given in four fractions over 8 h in children with advanced solid tumors. PATIENTS AND METHODS: Twenty children aged 1 1/2-19 years (median, 12 years) received 24 courses of high-dose CPA at 7 g/m2 for the treatment of advanced malignant solid tumor. CPA was given in four 1-h infusions of 1.75 g/m2 each, with 1 h of rest between each dose. MESNA was used as a uroprotective agent and was continued for 24 h after the final dose of CPA. With only one exception, all patients were discharged at the end of MESNA infusion and received granulocyte colony-stimulating factor, prophylactic ciprofloxacin, and co-trimoxazole. RESULTS: Severe but transient myelosuppression was observed. The median time to neutrophil and platelet recovery was 17 and 19 days, respectively. Fever developed after 13 of the 24 courses, and hospitalization was required. Extramedullary toxicities were mild. No patient showed cardiomyopathy or hemorrhagic cystitis. Forty-six percent of the courses were managed entirely on an outpatient basis. Objective tumor response was seen in five patients. CONCLUSIONS: CPA at 7 g/m2 is well tolerated by children with advanced malignancies and should be considered in earlier phases of antineoplastic therapy.

Clinical use of molecular information in the management of multiple endocrine neoplasia type 2A.

One hundred and ninety-seven members of 28 kindreds with multiple endocrine neoplasia type 2A (MEN 2A) were screened for RET proto-oncogene exon 10 and 11 mutations. Seventy-one known affected individuals had mutations of codons 609, 618, 620 or 634, whereas 53 unaffected individuals had no abnormalities. Nineteen out of 54 individuals of unknown status, mostly children, had RET mutations. Four of these children had thyroidectomy based on this analysis and were found to have C-cell abnormalities. We identified one false negative mutation analysis because of a codon 691 polymorphism. We conclude that RET mutational analysis is a cost-effective and accurate method for determination of gene carrier status in MEN 2A.

Malignant solid tumors in neonates: a 40-year review.

To evaluate the outcome of neonatal malignant solid tumors, we reviewed the records of 222 infants under the age of 1 year with malignant disease who were treated at the University of Texas M.D. Anderson Cancer Center over a 40-year period. Forty-five cases of neonatal (< 30 days old at the time of presentation) malignancies were found. Thirty-two infants had solid tumors and form the basis of this report. Diagnoses included soft tissue sarcoma (13), brain tumor (5), neuroblastoma (6), retinoblastoma (3), malignant melanoma (2), hemangiopericytoma (2), and nephroblastoma (1). The mean age at which initial signs and symptoms were noted was 9 days of life. Fifty-nine percent (19) presented within the first week of life, and 47% (15) presented at birth. The mean age at histological diagnosis was 54 days. The head and neck region was the most common site (18), followed by trunk (9), and extremities (5). Thirty-one patients underwent surgical resection of the primary tumor. Thirteen of those neonates received no additional chemotherapy and/or radiation therapy, whereas 18 received some combination of surgery plus perioperative chemotherapy and/or radiation therapy. Overall survival was 78% (25 of 32) with an average follow-up of 8 years (range, 2 months to 29 years). There were no survivors among those patients with distant metastatic disease at the time of diagnosis. Despite delays, prognosis is excellent in the absence of distant metastatic disease, particularly for extracranial tumors.

Iodine 131 thyroid ablation in female children and adolescents: long-term risk of infertility and birth defects.

BACKGROUND: The use of radioactive iodine, or iodine 131 (131I), for remnant thyroid ablation and the treatment of cervical and distant metastatic disease in patients with thyroid cancer is well accepted. 131I concentrates in the bladder, and irradiation to the ovaries has been theorized to increase the risk of infertility and birth defects in subsequent offspring. METHODS: We conducted a retrospective review of the charts of 154 children and adolescents treated at our institution for thyroid cancer between 1951 and 1991. Review of these charts identified 68 females diagnosed with thyroid cancer, < or = 20 years of age, who received 131I as part of their therapy at our institution. Charts were reviewed and patients recontacted, and initial tumor, date of diagnosis, and 131I administration, including doses, were recorded. Complete pregnancy histories including current health status of the children were also recorded. RESULTS: Twenty-two patients who never attempted pregnancy were excluded from analysis. Eleven patients could not be contacted and were considered lost to follow-up and thus excluded from the study. In the remaining 35 patients, mean age at 131I administration was 18.3 years (range 14.1-20.8), mean follow-up, 16.8 years (range 5.6-39.8), and mean 131I dose, 148.53 mCi (range 77.2-250). Three patients were diagnosed infertile after extensive workup (8.6%). The remaining 32 patients had 69 pregnancies resulting in 60 term and four premature deliveries. There were two elective abortions for nonmedical reasons and three spontaneous abortions. Only two children were conceived within 1 year of 131I therapy. Both were born with birth defects that proved fatal within 8 months. No other children were born with birth defects. One other child born with an estimated gestational age of 27 weeks died due to complications related to his prematurity. No anomalies were noted at autopsy. Of the 61 children alive for follow-up, no major health problems were identified other than asthma in two children. CONCLUSIONS: 131I, used in doses up to 250 mCi, is not associated with any long-term risk of infertility. The risks of infertility or birth defects are not different from those of the general population. Because the two children with birth defects were born to mothers treated either during pregnancy or 6 months before conception, it might be wise to suggest avoiding pregnancy for up to 1 year after 131I treatment.

Cardiac diastolic function in pediatric patients receiving doxorubicin.

The purpose of the study was to compare systolic and diastolic function in pediatric patients treated with doxorubicin. Left ventricular function was evaluated in 61 children prior to and following chemotherapy. None had clinical evidence of cardiac decompensation prior to treatment. All received relatively low cumulative doses of doxorubicin; the majority received the drug by continuous infusion. Systolic function was estimated using fractional shortening; diastolic function was estimated using A wave velocity, E wave velocity, E to A ratio, and deceleration time. There was a small but significant decline in systolic cardiac function as estimated from changes in fractional shortening that could not be appreciated in any of the measured parameters of diastolic function. A variety of reasons that could be responsible for the absence of significant changes in diastolic function are discussed. For the present, estimations of systolic function are preferred over the studied parameters of diastolic function in the evaluation of cardiac status in pediatric patients receiving doxorubicin containing regimens.

A rational approach to the use of fine-needle aspiration biopsy in the evaluation of primary and recurrent neoplasms in children.

Fine-needle aspiration (FNA) has been recommended as a technique for accurate evaluation and diagnosis of childhood malignancies. Very little data exist regarding the limitations and utility of FNA in children. We reviewed our experience over the past 5 years to assess the clinical use of FNA and its limitations. One hundred seventy-three FNAs were performed in 156 patients who ranged in age from 7 months to 18 years. Sixty-three patients were female and 110 were male. Thirty-seven aspirations were obtained to evaluate new masses in children with previously diagnosed malignancies. FNA allowed diagnosis of the malignancy in 90% of patients with solid tumors, whereas only 9 of 19 patients with lymphomas were diagnosed accurately with FNA. The specificity was 99.7%. Inadequate material was obtained in 13 aspirates. Insufficient material was obtained in 14% of patients who had FNA as their initial diagnostic tool. Four false-negatives were obtained while evaluating children for recurrent disease. There were 16 true-negative biopsies. These data suggest that FNA is an excellent tool for diagnosing both recurrent malignancies as well as previously undiagnosed solid tumors in the pediatric population but is inadequate to diagnose children with suspected undiagnosed lymphomas. Negative studies should be considered nondiagnostic and followed by open biopsies when clinical suspicion of malignancy is high.

Pulmonary metastases in children and young adults with differentiated thyroid cancer.

BACKGROUND: The prognostic significance and optimal care of children with differentiated thyroid cancer and pulmonary metastases are not well established. METHODS: Of 209 patients younger than 25 years of age who were treated at University of Texas M. D. Anderson Cancer Center between 1960 and 1990 and for whom there was sufficient information, 19 (9%) had pulmonary metastases at presentation. RESULTS: All of these patients had regional lymphadenopathy at the time of diagnosis. All but two had intense, diffuse radioiodine uptake in the lungs; there were two false-negative scans immediately after surgical procedure caused by competing thyroid residual. The chest radiograph (CXR) was normal in 8 of 17 (42%) patients with abnormal radioiodine scans. After therapy with radioiodine (100-499 mCi), CXR appeared normal in 7 of 9 patients with initial abnormal radiographs (within 6-75 months). Radioiodine uptake by the lungs normalized in 3 of 8 patients with initially normal radiographs, and in 3 of 9 patients with initially abnormal radiographs. There have been no deaths in these 19 patients. CONCLUSION: Pulmonary metastases are not uncommon in children and young adults with differentiated thyroid cancer, especially those who have regional lymphadenopathy. The lung metastases almost always concentrate radioiodine diffusely and may be associated with a normal CXR in almost half of the patients. Pulmonary metastases may be overlooked unless near total thyroidectomy is followed by total body radioiodine scan (TBS) in all children and young adults who have regional lymphadenopathy of the neck.

Pediatric hepatic tumors: clinical trials conducted in the United States.

Hepatoblastomas (HBs) and hepatocellular carcinomas (HCCs) constitute the majority of hepatic tumors in children. Although most children who have HB or HCC present with unresectable disease, chemotherapy may reduce the size of the primary tumor and metastases, thus allowing subsequent successful removal. Clinical trials conducted over the past decade in the United States have demonstrated that chemotherapy regimens consisting of continuous-infusion doxorubicin and cisplatin or high-dose cisplatin alone effectively reduce tumor size. The prognosis of children with HB is better than that of those with HCC. Over half the patients who initially have unresectable HB can be rendered disease free with chemotherapy and subsequent surgery.

Diagnosis of eosinophilic granuloma of bone by fine-needle aspiration with concurrent institution of therapy: a cytologic, histologic, clinical, and radiologic study of 27 cases.

Twenty-seven patients with eosinophilic granuloma (EG) of bone seen at our institution between 1979 and 1991 underwent fine-needle aspiration (FNA) with or without concurrent Tru-Cut biopsy. The 16 males and 11 females ranged in age from 2 1/2 to 61 years (median, 10 yr). Twenty-four patients had monostotic lesions. The clinicoradiologic differential diagnosis included osteomyelitis and Ewing's sarcoma (young patients) and primary and metastatic malignancies (older patients). Twenty-four of 28 FNAs (one patient had two FNAs) were diagnostic of EG, and 10 cases were diagnosed by FNA alone. Smears in these cases showed histiocytes, often with grooved or infolded nuclei, and abundant eosinophils. Multinucleated giant cells, foamy histiocytes, neutrophils, lymphocytes, and plasma cells were present in variable numbers. Four FNAs were misdiagnosed: two as osteomyelitis where smears contained abundant neutrophils, sparse eosinophils, and histiocytes misinterpreted as foamy histiocytes, and two as metastatic carcinoma (in adults) where histiocytes in a scant specimen (one case) and skin appendiceal structures without lesional tissue (one case) were misinterpreted. These cases were correctly diagnosed on repeat FNA (one case), Tru-Cut (two cases), or excisional biopsy (one case); however, three cases diagnosed by FNA had nondiagnostic concurrent Tru-Cut biopsies. Treatment consisted of intralesional injection of 125 mg of methylprednisolone (22 cases). Progressive or complete healing of all lesions occurred. FNA is a rapid and useful technique for the immediate diagnosis of EG that allows concurrent institution of therapy.

A unique case of retinoblastoma masked by retinal detachment diagnosis and management.

A 4-year-old Latin American boy presented with a history of loss of vision of his left eye for 7 months. He was followed by different ophthalmologists and diagnosed with a retinal detachment. Computerized tomograms and magnetic resonance imaging demonstrated a large calcified left eye and markedly enlarged optic nerve. Based on these radiologic findings diagnosis of retinoblastoma was strongly suspected. Initially enucleation was not done because it was thought that cutting through the involved optic nerve would cause dissemination. The patient was treated with chemotherapy which resulted in marked response. Following normalization of optic nerve and after irradiation the eye was enucleated. The diagnosis of retinoblastoma was confirmed. No tumor was found in the optic nerve. The importance of imaging studies in diagnosis of retinoblastoma and the role of chemotherapy in the treatment of primary retinoblastoma are emphasized.

Marked response to preoperative high-dose cis-platinum in children with unresectable hepatoblastoma.

Chemotherapy is the only effective method of treating unresectable hepatoblastoma. Most protocols require the administration of multiple highly toxic agents. We evaluated the ability of single-agent high-dose cis-platinum (HD-CDDP) to shrink unresectable primary hepatoblastoma to allow resection. Seven children aged 11 to 72 months had unresectable hepatoblastoma based on size and location. Initial alpha-fetoprotein (AFP) levels were between 4,900 and 1,840,000 ng/mL (mean, 555,900 ng/mL). Chest computed tomography (CT) scans obtained before beginning HD-CDDP therapy showed multiple or bilateral lung masses in 3 patients. Chemotherapy for each of the 7 children consisted of only HD-CDDP, 150 mg/m2, at 3-week intervals. HD-CDDP was stopped and prompt resection performed when AFP levels ceased to decline significantly (n = 4; mean nadir, 18,600); the corrected creatinine clearance decreased below 60 mL/min (n = 2); or, in one case, significant hemorrhage occurred within the tumor. Therefore, the number of HD-CDDP doses given preoperatively varied between 1 and 7 (mean, 3). No children required dialysis. Tumor cells in the bone marrow of one child disappeared completely after one dose of HD-CDDP. Follow-up CT scans showed complete resolution of the pulmonary metastases in 2 children, a partial response in the third, and a marked reduction of primary tumors to resectable sizes. Six children underwent tumor excision with adequate margins; parents of the seventh child refused permission for surgery. There were 2 operative deaths, 3 deaths due to local or distant disease, and 2 patients survived (postoperative follow-up, 22 and 14 months).(ABSTRACT TRUNCATED AT 250 WORDS)